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Susan B.

Waltzman
j. Thomas Roland jr.

Third Edition

Thieme
(!Thieme
Cochlear Implants

Third Edition

Susan B. Waltzman, PhD


Marica F. Vilcek Professor of Otolaryngology
Department of Otolaryngology-Head and Neck Surgery
Co-Director, NYU Cochlear Implant Center
New York University School of Medicine
NYU Langone Medical Center
New York, New York

J. Thomas Roland Jr., MD


Professor of Otolaryngology and Neurosurgery
Mendik Foundation Professor and Chairman
Department of Otolaryngology-Head and Neck Surgery
Co-Director, NYU Cochlear Implant Center
New York University School of Medicine
NYU Langone Medical Center
New York, New York

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[DNLM : 1 . Cochlear Implants. 2. Hearing Disorders-surgery. WV 274]
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Contents

Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii

Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . viii

Acknowledgments. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xii

1 History of the Cochlear Implant .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1


Marc D. Eisen

2 Genetics of Hearing Loss and Predictors of Cochlear Implant Outcome .............. 10


Robert W. Eppsteiner, Richard K. Gurgel, and Richard j.H. Smith

3 Consequences of Deafness and Electrical Stimulation on the Peripheral and Central


Auditory System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
james B. Fallon, David K. Ryuga, and Robert K. Shepherd

4 Auditory Neuroplasticity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
Robert C. Froemke, Selena E. Heman-Ackah, and Susan B. Waltzman

5 Mimicking Normal Auditory Functions with Cochlear Implant Sound Processing: Past,
Present, and Future . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Ward R. Drennan, Mario A. Svirsky, Matthew B. Fitzgerald, and jay T. Rubinstein

6 Expanding Criteria for the Evaluation of Cochlear Implant Candidates . . . . . . . . . . . . . . . 61


Susan Arndt, Roland Laszig, Antje Aschendorff. and Rainer Beck

7 Principles of Cochlear Implant Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74


Andrew j. Fishman and Selena E. Heman-Ackah

8 Intraoperative Monitoring During Cochlear Implantation .. . . . . . . . . . . . . . . . . . . . . . . . 100


Mauro K. Cosetti

9 The History of Cochlear Implant Electrode Design.. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108


Maja Svrakic and j. Thomas Roland Jr.

10 Cochlear Implant Surgical Technique .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118


Peter S. Roland and j. Thomas Roland Jr.

11 New Horizons in Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128


Theodore R. McRackan, Robert F. Labadie, j. Thomas Roland Jr., and David S. Haynes

12 A Global View of Device Reliability.. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132


Rolf-Dieter Battmer

13 Revision Cochlear Implantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141


David R. Friedmann, j. Thomas Roland Jr., and Susan B. Waltzman

14 Advancements in Cochlear Implant Programming............................... 148


William H. Shapiro

v
15 Auditory and Linguistic Outcomes in Pediatric Cochlear Implantation . . . . . . . . . . . . . . . 158
Gerard M. O'Donoghue and David B. Pisani

16 Auditory Outcomes in the Adult Population 167


Oliver F. Adunka, Margaret T. Dillon, and Craig A. Buchman

17 Therapeutic Approaches Following Cochlear Implantation . . . . . . . . . . . . . . . . . . . . . . . . 182


Warren Estabrooks, K. Todd Houston, and Karen Maclver-Lux

18 Acoustic and Electric Speech Processing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194


Bruce}. Gantz, Sarah E. Mowry, Rick F. Nelson, Sean 0. McMenomey, Chris}. james, and Bernard Fraysse

19 Music Perception . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203


Alexis Roy and Charles}. Limb

20 Auditory Brainstem Implants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212


Shoun D. Rodgers, john G. Golfinos, and}. Thomas Roland Jr.

21 Applying Cochlear Implant Technology to Tinnitus and Vestibular Interventions .. . . . . . 221


justin S. Golub, james 0. Phillips, and jay T. Rubinstein

22 The Impact of Cochlear Implantation on the Recipient's Health-Related Quality of Life.. . . 235
Selena E. Heman-Ackah

23 Future Technology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 248


Susan B. Waltzman and}. Thomas Roland Jr.

Index ...................................................................... 249

vi
Preface
This fully updated third edition of Cochlear Implants repre technology and its implementation. Patient outcomes, can
sents the superb efforts of many individuals who have didacy criteria, technical design, surgical technique, and
contributed significantly to the field. We have built on the programming and processing concepts are constantly pro
previous edition by adding new chapters relating to genet gressing, and increasing numbers of individuals are gaining
ics, neuroplasticity, expanding criteria for implantation, the significant benefit. Our goal was to create a book that will
application of implant technology to tinnitus and vestibular provide both experienced and new and budding otolaryngol
issues, music perception, intraoperative monitoring, device ogists-head and neck surgeons, neurotologists, audiologists,
reliability and reimplantation, and the use of quality-of-life neuroscientists, neurophysiologists, speech pathologists, tea
and outcome measures-areas that occupy the forefront of chers of the deaf, psychologists, and others interested in
cochlear implantation. Other chapters have been revised to cochlear implants with an extraordinary resource for years
reflect current research and clinical applications and pro to come. Thanks to the contributors and their hard work,
vide the most recent information related to the clinical and dedication, and attention to detail, we feel that this goal has
translational sciences that continue to advance this exciting been exceeded, and we are most grateful to all involved.

vii
Contributors
Oliver F. Adunka, MD, FACS Ward R. Drennan, PhD
Associate Professor Virginia Merrill Bloedel Hearing Research Center
OtologyfNeurotology/Skull Base Surgery Department of Otolaryngology-Head and Neck Surgery
Department of Otolaryngology-Head and Neck Surgery University of Washington
University of North Carolina at Chapel Hill Seattle, Washington
Chapel Hill, North Carolina
Marc D. Eisen, MD, PhD
Susan Arndt, MD Adjunct Clinical Assistant Professor
Department of Otorhinolaryngology Department of Otorhinolaryngology-Head and Neck
Implant Center Freiburg Surgery
University of Freiburg University of Pennsylvania
Freiburg, Germany Philadelphia, Pennsylvania

Antje Aschendorff, MD
Robert W. Eppsteiner, MD
Associate Professor
Otolaryngology Resident T32 Research Fellow
Department of Otorhinolaryngology
Department of Otolaryngology-Head and Neck Surgery
Head of Implant Center Freiburg
University of Iowa Hospitals and Clinics
University of Freiburg
Iowa City, Iowa
Freiburg, Germany

Warren Estabrooks, M.Ed. , Dip.Ed.Deaf, LSLS Cert. AVT


Rolf-Dieter Battmer, PhD
President and CEO
Professor and Director
WE Listen International Inc.
Center for Clinical Technology Research
Toronto, Ontario, Canada
Trauma Hospital
Berlin, Germany
james B. Fallon, PhD
Bionics Institute
Rainer Beck, MD
Melbourne, Australia
Department of Otorhinolaryngology
and
Implant Center Freiburg
Department of Medical Bionics
University of Freiburg
University of Melbourne
Freiburg, Germany
Melbourne, Australia

Craig A. Buchman, MD, FACS


Andrew J. Fishman, MD
Professor and Vice Chairman for Clinical Affairs
Director of Neurotology & Cranial Base Surgery
Chief OtologyfNeurotology/Skull Base Surgery
Director, Cochlear Implant Program
Department of Otolaryngology-Head and Neck Surgery
Cadence Neuroscience Institute
University of North Carolina at Chapel Hill
Winfield, Illinois
Chapel Hill, North Carolina
and

Maura K. Cosetti, MD International Visiting Professor of Otolaryngology-Head &

Assistant Professor and Co-Director of Otology/Neurotology Neck Surgery

Departments of Otolaryngology-Head and Neck Surgery NATO Military Hospital, Bydgoszcz, Poland

and Neurosurgery Professor of Pediatric Otolaryngology

Louisiana State University Health Sciences Center Children's Hospital of Bydgoszcz

Shreveport, Louisiana Bydgoszcz, Poland

Margaret T. Dillon, AuD, CCC-A, F-AAA Matthew B. Fitzgerald, PhD, CCC-A


Assistant Professor Department of Otolaryngology-Head and Neck Surgery
Department of Otolaryngology-Head and Neck Surgery New York University School of Medicine
University of North Carolina at Chapel Hill NYU Langone Medical Center
Chapel Hill, North Carolina New York. New York

viii
Professor Bernard Fraysse Selena E. Heman-Ackah, MD, MBA
Chairman of the ENT Department Otology, Neurotology and Skull Base Surgery
H6pital Purpan Medical Director of Otology, Neurotology, and Audiology
Toulouse, France Division of Otolaryngology-Head and Neck Surgery
Department of Surgery

David R. Friedmann, MD Beth Israel Deaconess Medical Center

Department of Otolaryngology-Head and Neck Surgery Harvard Medical School

New York University School of Medicine Boston, Massachusetts

NYU Langone Medical Center


New York, New York K. Todd Houston, PhD, CCC-SLP, LSLS Cert. AVT
School of Speech-Language Pathology and Audiology
University of Akron
Robert C. Froemke, PhD
Akron, Ohio
Assistant Professor of Neuroscience and Otolaryngology
Department of Otolaryngology-Head and Neck Surgery
Chris j. james, PhD
New York University School of Medicine
CHU Toulouse-Purpan
NYU Langone Medical Center
and
New York, New York
Cochlear France SAS
Toulouse, France
Bruce j. Gantz, MD, FACS
Professor and Head, Department of Otolaryngology-Head
Robert F. Labadie, MD, PhD
and Neck Surgery
Professor and Director of Research
Brian F. McCabe Distinguished Chair in Otolaryngology
Department of Otolaryngology-Head and Neck Surgery
Head and Neck Surgery
Professor, Department of Biomedical Engineering
Professor, Department of Neurosurgery
Vanderbilt University
University of Iowa Carver College of Medicine
Nashville, Tennessee
Iowa City, Iowa

Roland Laszig, MD
john G. Golfinos, MD Head, Department of Otorhinolaryngology
Chairman, Department of Neurosurgery Implant Center Freiburg
Associate Professor of Neurosurgery and Otolaryngology University of Freiburg
New York University School of Medicine Freiburg, Germany
NYU Langone Medical Center
New York, New York Charles j. Limb, MD
Associate Professor
justin S. Golub, MD Department of Otolaryngology-Head and Neck Surgery
OtologyfNeurotologyfLateral Skull Base Surgery Fellow johns Hopkins Hospital
Department of Otolaryngology-Head and Neck Surgery johns Hopkins University School of Medicine
University of Cincinnati Peabody Conservatory of Music
Cincinnati Children's Hospital Medical Center Baltimore, Maryland
Cincinnati, Ohio
Karen Maclver-Lux, MA, Aud(C), LSLS Cert. AVT
Richard K. Gurgel, MD Director
Assistant Professor Maclver-Lux Auditory Learning Services
Division of Otolaryngology-Head and Neck Surgery King City, Ontario, Canada
University of Utah
Salt Lake City, Utah Sean 0. McMenomey, MD
Professor of Otolaryngology and Neurosurgery
David S. Haynes, MD, FACS Department of Otolaryngology-Head and Neck Surgery
Otology Group of Vanderbilt New York University School of Medicine
Vanderbilt University Medical Center NYU Langone Medical Center
Nashville, Tennessee New York, New York

ix
Theodore R. McRackan, MD Peter S. Roland, MD
Department of Otolaryngology-Head and Neck Surgery Professor and Chairman
Vanderbilt University Department of Otolaryngology-Head & Neck Surgery
Nashville, Tennessee Professor of Neurological Surgery
University of Texas Southwestern Medical Center

Sarah E. Mowry, MD Dallas, Texas

Department of Otolaryngology
Georgia Regents University Alexis Roy, MSc

Augusta, Georgia Harvard Medical School


Boston, Massachusetts

Rick F. Nelson, MD, PhD


jay T. Rubinstein, MD, PhD
Department of Otolaryngology-Head and Neck Surgery
Virginia Merrill Bloedel Professor and Director
University of Iowa Hospitals and Clinics
Virginia Merrill Bloedel Hearing Research Center
Iowa City, Iowa
Professor of Otolaryngology and Bioengineering
University of Washington
Gerard M. O'Donoghue, MD
Seattle, Washington
Consultant Neuro-Otologist
Nottingham University Hospitals NHS Trust
David K. Ryugo, PhD
Queens Medical Centre
Curran Foundation Professor
Honorary Professor of Otology and Neurotology
Garvan Institute of Medical Research
University of Nottingham
Darlinghurst, New South Wales
Co-founder, Nottingham Hearing Biomedical
and
Research Unit
Conjoint Professor
Nottingham, United Kingdom
School of Medical Sciences
University of New South Wales
james 0. Phillips, PhD
Kensington, New South Wales
Research Associate Professor
Australia
Department of Otolaryngology-Head and Neck Surgery
University of Washington
William H. Shapiro, AuD, CCC-A
Seattle, Washington
Clinical Associate Professor
Supervising Audiologist
David B. Pisoni, PhD NYU Cochlear Implant Center
Distinguished Professor of Psychological and New York University School of Medicine
Brain Sciences New York, New York
Chancellor's Professor of Cognitive Science
Department of Psychological and Brain Sciences Robert K. Shepherd, PhD
Indiana University Bionics Institute
Bloomington, Indiana Melbourne, Australia
and
Shaun D. Rodgers, MD Department of Medical Bionics
Department of Neurosurgery University of Melbourne
New York University School of Medicine Melbourne, Australia
NYU Langone Medical Center
New York, New York Richard J.H. Smith, MD
Sterba Hearing Research Professor
]. Thomas Roland Jr. , MD Director, Molecular Otolaryngology and Renal Research
Professor of Otolaryngology and Neurosurgery Laboratories
Mendik Foundation Professor and Chairman Vice Chair, Department of Otolaryngology
Department of Otolaryngology-Head and Neck Surgery Professor of Otolaryngology, Molecular Physiology & Bio
Co-Director, NYU Cochlear Implant Center physics, Pediatrics and Internal Medicine (Division of
New York University School of Medicine Nephrology)
NYU Langone Medical Center University of Iowa Hospitals and Clinics
New York, New York Iowa City, Iowa

X
Mario A. Svirsky, PhD Susan B. Waltzman, PhD
Noel L. Cohen Professor of Hearing Science and Vice Marica F. Vilcek Professor of Otolaryngology
Chairman of Research Department of Otolaryngology-Head and Neck Surgery
Department of Otolaryngology-Head and Neck Surgery Co-Director, NYU Cochlear Implant Center
New York University School of Medicine New York University School of Medicine
NYU Langone Medical Center NYU Langone Medical Center
New York, New York New York, New York

Maja Svrakic, MD
Department of Otolaryngology-Head and Neck Surgery
New York University School of Medicine
NYU Langone Medical Center
New York, New York

xi
Acknowledgments
We thank all the contributors to this third edition. In commitment, guidance, and patience during the writing of
addition, we are indebted to J. Owen Zurhellen, Timothy this book; we could not have done it without their profes
Hiscock, and Chris Malone at Thieme Publishers for their sionalism and support.

xii
1 H i sto ry of the Coc h l e a r I m pl a nt
Marc D. Eisen

real-time voice synthesizer that produced intelligible speech


Introduction using circuitry designed to extract the fundamental frequency
The cochlear implant has created a paradigm shift in the treat of speech, the intensity of its spectral components, and its over
ment of sensorineural hearing loss. The impact that the implant all power. The spectral components were extracted with a
has had is far greater than one would expect considering the series of ten band-pass filters covering the frequency range of
brief time over which its development occurred. In less than speech.l He named the synthesizer the "vocoder," a compressed
four decades, the cochlear implant progressed from the first version of "coding the voice." The operating principles of the
attempts to elicit hearing via direct electrical stimulation of the vocoder for condensing speech into its principal components
auditory nerve to a commercially available device that has formed the basis of early speech processing schemes for multi
restored varying degrees of hearing to tens of thousands of deaf channel cochlear implants.
patients. Several themes that can be discerned in the implant's
history are widely applicable to the development of other neu
ral prostheses. For one, the implant's development was truly an
The Cochlear Microphonic
interdisciplinary effort. Significant contributions came from In 1 930, Wever and Bray2 recorded and described the electrical
professionals in fields as diverse as engineering, otology, audiol potentials in the cochlea that faithfully reproduced the sound
ogy, auditory neurophysiology, psychoacoustics, and industry. stimulus. This phenomenon became known as the "Wever-Bray
The interaction among these players was not always harmoni effect." The source of these measured potentials was initially
ous, but the strife yielded synthesis and progress. incorrectly assumed to represent auditory nerve discharges.
Another theme is the courage of a few clinicians to risk their This theory of the origin of these potentials would be equivalent
reputations and eschew scientific dogma in the hope of helping to the "telephone" theory of hearing, referring to the analogue
the patients that sought their care. A third theme is the willing representation of the voice carried along the "cable" of the
ness of patients to take substantial risks in serving as research auditory nerve as it would along the wires of a telephone line.
subjects, sometimes without any promise of individual gain. In truth, what Wever and Bray were recording was not a
This chapter cannot mention each and every contribution that response of the cochlear nerve, but the cochlear microphonic
was made to the implant's early development, but rather cites produced by the outer hair cells in the cochlea. Regardless of
selected events and characters that, with the aid of the "retro the ultimate dismissal of the telephone theory of hearing, it
spectroscope," exemplify these themes and demonstrate the inspired several of the earliest pioneers of the cochlear implant.
progression of events leading toward a device that enables the
patient who has lost all hearing to regain the ability to converse
on the telephone and enables the deaf child to develop near
Electrophonic Hearing
normal speech production and understanding. S.S. Stevens and his colleagues classically described in the 1 930s
The implant's development occurred in several phases. The the mechanism by which the cochlear elements respond to elec
first phase of pioneering and experimentation began in 1 957 trical stimulation to produce hearing.3 This mechanism was
and continued throughout the 1 960s. The second phase, which coined "electrophonic hearing." We now know that electrophonic
entailed feasibility studies, occurred in the 1 970s. These studies hearing results from the mechanical oscillation of the basilar
were conducted to determine whether the implant safely stim membrane in response to voltage changes. The primary tenet of
ulated the auditory pathway and elicited useful hearing. A third their description was the requirement that the cochlea be intact.
phase entailed the subsequent development of a commercially Prior to 1 957, efforts to stimulate hearing electrically were per
viable multielectrode cochlear prosthesis. formed on subjects with at least partially functioning cochleas.
These subjects' responses could be accounted for by electrophonic
hearing rather than by direct nerve stimulation. Furthermore, the
Precursors developers of the earliest cochlear implant efforts had the burden
of proving that the implants were directly stimulating the
Several discoveries made during the first half of the twentieth
cochlear nerve rather than eliciting electrophonic hearing.
century were not directly related to electrical stimulation of the
cochlear nerve, but were influential on the early development
of the cochlear implant and therefore warrant mention. These Pioneers: 1957-1973
include Homer Dudley's work on the synthesis of speech and
his "vocoder," Glenn Wever and his discovery of the cochlear Andre Djourno and Charles Eyries
microphonic, and S.S. Stevens and coworkers' description of Although numerous attempts to treat deafness with electricity
electrophonic hearing. have been reported over the past several centuries,4 the first
reported direct stimulation of the cochlear nerve for the purpose
of generating hearing appeared as recently as 1 957 with the
The Vocoder work of Andre Djourno and Charles Eyries. Despite the revolu
Homer Dudley was a researcher at the Bell Telephone Laborato tionary impact that the cochlear implant has had on all auditory
ries in New York. He described and demonstrated in 1 939 a disciplines, these beginnings in Paris received little attention.
. .

Andre Djourno ( 1 904-1 996) received degrees in both science Both the active coil and the ground electrode were implanted
and medicine, yet he devoted his career to science. His early under the skin of an animal, and stimulation was trans
endeavors were studying the electrophysiology of frog periph cutaneous ( rather than percutaneous). The implantable coils
eral nerve.56 He then ventured into more medical applications were first used to stimulate the sciatic nerve and thus trigger a
of electricity. Several of Djourno's earlier innovations reflected jump behavior in rabbits. Djourno studied numerous aspects of
his inventiveness: a device to measure the pulse continuously,? telestimulation, including electrode biocompatibility (he
high-frequency electrical stimulation to remove metal frag described using one of the first bioresistant resins, araldite, for
ments from bones,8 and the use of electroencephalography example, to coat the electrodes ).1 1 He addressed the effect of
( EEG) to study narcolepsy.9 Perhaps the most prescient devel stimulus frequency on muscle contraction, and he found that
opment from this period was artificial respiration utilizing with higher frequency stimuli, muscles would not contract,
direct phrenic nerve stimulation.10 Although this innovation whereas with lower frequency stimuli muscle contraction was
did not reach widespread clinical implementation, it demon painful. Djourno found the "right" stimulus frequency between
strated Djourno's interest in neural prostheses. 400 and 500 Hz. Since this frequency was within the speech
Djourno focused the next phase of his career on fabricating range, he began to use the analogue signal of his own voice as
and testing implantable induction coils to be used for "telesti the telestimulating stimulus.12 Triggering a nerve with his voice
mulation," or stimulation through inductive coupling without may well have contributed to the idea of stimulating the
wires. Djourno assembled these induction coils himself and cochlear nerve to restore hearing.
called them "microbobinages," as the coils wound with wire Djourno also addressed the safety of repetitive stimulation
resembled small spools of thread ( Fig. 1 . 1 ). on tissue, demonstrating that the sciatic nerve from an
implanted rabbit, when examined histologically and grossly,
showed no changes after 2 years of repetitive stimulation.B
Throughout this time Djourno revealed little interest in hear
ing. He recognized, however, the potential of using the micro
bobinages to stimulate the auditory system, as he noted in a
1 954 publication the possibility of "treating deafness" as a
potential application.12
Charles Eyries ( 1 908-1 996) completed his training in oto
laryngology in Paris in the early 1 940s. Clinically, Eyries earned
early recognition for his description of a procedure to treat
ozena, or atrophic rhinitis, by placing implants underneath the
nasal mucosa to decrease the caliber of the nasal passages.14
This procedure became known in the French literature as the
"Eyries operation." Eyries was named chief of otorhinolaryngo
logy and head and neck surgery at I.:Institut Prophylactique in
1 953, which has since been renamed I.:Institut Arthur Vernes.
Although primarily a clinician, he had research interests in
neuroanatomy and embryology of the facial nerve, and he
wrote about surgical facial nerve repairs.15 Eyries had shown
little interest in hearing at this point in his career and had never
worked with Djourno, although he knew of Djourno because
both he and Djourno had laboratories in the medical school
associated with the hospital.
As the local expert on facial nerve repairs, Eyries was
asked in February 1 957 to provide a consultation for an
unfortunate patient, a 57-year-old man who suffered from
large bilateral cholesteatomas. A right-sided temporal bone
resection was performed 5 days prior to the consultation
and an extensive left temporal bone resection was per
formed several years earlier. Both procedures involved abla
tion of the labyrinth and facial nerve sectioning. As a result,
the patient was left with bilateral deafness and bilateral
facial nerve paralysis. Eyries was consulted to consider a
facial nerve graft for reanimation.16
On examination, Eyries found that the caliber of the
patient's remaining nerve was too small to support a local
Fig. 1 . 1 Example of implantable induction coils ("microbobinages") nerve transfer. Eyries therefore embarked on a search for
assembled by Djourno in his laboratory. I nduction coils like the ones
appropriate graft material. He went to the medical school
shown here were used in various applications, including the stimu lation
of the auditory pathway. The hand holding the coils provides perspective
seeking cadaverous material, where he met Djourno, who
on the implant's size. (Courtesy of the john Q. Adams Center.) offered to help and suggested stimulating hearing at the
same time. Although Eyries was primarily interested in

2
his patient's facial reanimation, he agreed to implant and several words, but could not understand speech.
an electrode into the patient at the time of surgery. The publication that resulted from this work is the seminal
Eyries's justification for agreeing to the implantation was citation for direct cochlear nerve stimulation.1 8
that the cavity was already exposed and the patient had Several months later, during testing, the electrode suddenly
nothing to lose in having the extra procedure.16 From ceased to function. Djourno and Eyries went to the operating
Djourno's standpoint, the patient was deaf and begging room to investigate. They found that a solder joint connecting
to escape from the silence that haunted him, and he was the wires to the ground electrode embedded into the tempora
fascinated by the opportunity to telestimulate the auditory lis muscle had broken, and the implant was replaced. The sec
system.17 ond implant, however, suffered the same fate. Eyries held
The procedure took place on February 25, 1 957. Eyries Djourno responsible for the broken electrodes and refused to
performed the right-sided facial nerve graft using fetal sci perform a third implantation.19 The falling out between the two
atic nerve as the graft material, which purportedly proved men over this problem was the end of Eyries's involvement in
to be successful. At the time of surgery, the proximal the project. After this event, he and Djourno rarely conversed
cochlear nerve stump was found to be significantly shred for the rest of their lives.
ded. Djourno and Eyries chose to seat the active electrode This was not quite the end of the story for Djourno,
into the remaining stump and place the induction coil into however. He went on to address several aspects of hearing
the temporalis muscle. A postoperative lateral skull film con applicable to electrical stimulation. For one, he examined
firmed its placement ( Fig. 1 .2). the oscillographic representation of spoken words in an
Some testing was done intraoperatively. The stimulus effort to give deaf patients a visual representation of speech
waveforms included bursts of a 1 00-Hz impulse signal that they could use for biofeedback when learning to
administered 15 to 20 times per minute, low-frequency speak.2 o After the first implant effort, a colleague
alternating current, and the analogue signal of words approached Djourno to enter into a business venture to
spoken into a microphone. The patient described detecting develop the implant. The colleague proposed that in
auditory sensations. Several qualitative observations were exchange for an exclusive arrangement on the project, he
made: the patient's discrimination of intensity was good ; would provide Djourno with the financial and engineering
frequency discrimination was poor; n o speech recognition support of industry_ 1 7 Djourno was always an academic ide
was evident. The patient underwent an extensive post alist, and he did not believe in profiting from his discoveries.
operative rehabilitation with the implant under the guid Furthermore, he detested industry and would have no part
ance of the speech therapist. Over the ensuing months more in granting exclusivity. As a matter of principle, Djourno
complex stimuli were administered, and the patient was chose to do another implant with a different oto
able to differentiate between higher frequency (described as laryngologist, Roger Maspetiol_l7 This second patient, deaf
"silk ripping" ) and lower frequency ( described as "burlap ened from streptomycin ototoxicity, was implanted with an
tearing") stimulation. He appreciated environmental noises electrode near the promontory, rather than within the tem
poral bone. The patient showed little enthusiasm for her
device, and she was lost to follow-up only a few months
after it was implanted. Djourno subsequently lost funding
for further implant work.21 This signaled the end of Djour
no's participation in developing an auditory prosthesis.
The legacy of the Djourno and Eyries's work was sustained
despite the abrupt departure of the two men. Claude-Henri
Chouard, who was a student in Eyries's laboratory working on
the facial nerve, resumed work on the cochlear implant several
years later. Chouard was instrumental in developing one of the
first functional multichannel implants, and he credits Charles
Eyries as his major source of inspiration.22
Although Djourno and Eyries's implantation on February
25, 1 957 is typically credited as the first cochlear implant, a
closer evaluation of the patient's anatomy raises the ques
tion of whether the cochlear nerve or the auditory brain
stem were stimulated by the implanted electrodes, as
wallerian degeneration may have destroyed the cochlear
ganglion cells.23 Whether Djourno and Eyries stimulated the
cochlear nerve or the cochlear nucleus should not over
shadow the significance of their work. Electrical methods to
treat deafness had been described by numerous practition
ers for almost two centuries prior to 1 957, beginning with
Fig. 1 .2 Lateral sku l l fil m of Djourno and Eyries's first implant following
the classic work of Alessandro Volta in the late 1 8th cen
su rgery. The coil has been em bedded in the tem pora l is m uscle,
tury.4 These previous efforts, however, either were aimed at
whereas the electrodes were placed near the remaining stum p of the
cochlear nerve. (Cou rtesy of the john Q. Adams Center.) treating deafness with therapeutic electrical stimulation or
were examples of electrophonic hearing.

3
. .

A second patient was also implanted in january 1 96 1 . The


Early Developments in the woman had deafness, tinnitus, and vertigo associated with con
Western Hemisphere genital syphilis, and she was brought to the operating room for
a vestibular neurectomy through the middle fossa approach.
Dissemination of the work of Djourno and Eyries was at first During the procedure, a single gold-wire electrode was placed
slow in the Western Hemisphere. This is likely attributable through the middle fossa approach into scala tympani at the
to the fact that their publication appeared only in the basal part of the cochlea. The wire was brought out through a
French-language medical literature. Additionally, of the pair, skin incision. The patient described hearing the square wave
the more likely to present his work among clinicians was stimulation upon waking from anesthesia. Over the ensuing
Eyries, as he was an otolaryngologist. Eyries demonstrated days, the current intensity required to elicit a response
little enthusiasm for the project, however, and his interest increased. For fear of infection or edema, the wire was removed.
was short-lived. Djourno was a physiologist rather than a With the first patient's encouraging responses, and with the
clinician, making interaction between him and American hope of producing discrimination of higher frequencies, House
otolaryngologists less likely despite his continued interest. and Doyle decided to reimplant him with a five-wire electrode
Word of their work reached William F. House in California array inserted through the mastoid facial recess and round win
serendipitously sometime around 1 959, when a patient of dow. The electrode array was attached to a more permanent
his handed him a summary in English of the Djourno and electrode induction system seated in the skull. Over a several
Eyries work.24 The summary was optimistic regarding elec week testing period, the patient's intensity requirement
trical stimulation to replace hearing, and House was
increased and his postauricular skin began to swell. This device
inspired. was also removed for risk of infection. Worries of bio
compatibility of materials ensued.
The theoretical basis for the multiple-electrode design was
los Angeles to spread high-frequency stimuli among spatially separated
William F. House was a dentist-turned-otologist who began electrodes. By stimulating different subpopulations of audi
working with his brother Howard P. House at the Otologic tory nerve fibers at rates slower than their refractory period,
Medical Group in Los Angeles upon completion of his residency they thought, summation among the subpopulations would
in 1 956. Early in his career he had already made significant con purportedly yield an overall high-frequency response along
tributions to otology and neurotology, including the facial the whole nerve. This implant design and its theoretical
recess approach. He was working at the time on the middle basis became the foundation for an early cochlear implant
fossa approach to the internal auditory canal in collaboration patent application submitted by james Doyle and Earle Bal
with john Doyle, a neurosurgeon who also practiced at St. lantyne in 1 961 . The patent was not granted until 1 969.28
Vincent's Hospital in Los Angeles.2s House and Doyle first Despite being founded on what has since been shown to be
sought to record the cochlear nerve response to sound when an erroneous theory of electrical stimulation, the patent was
the nerve was exposed during the middle fossa approach for ironically prescient in its statement that a 1 6-channel unit
vestibular neurectomy as a treatment for Meniere's disease. would be necessary for implant patients to be able to con
Specifically, they sought to record the nerve output associated verse on the telephone.
with tinnitus.26 They relied on Doyle's brother, james Doyle. an Word of the two implanted patients reached the lay press.
electrical engineer, to address the technical challenge of record The brief articles were overly optimistic in their descriptions
ing such signals intraoperatively. The nerve output was of an "artificial ear," going so far as to announce that "surgical
recorded, but no tinnitus was observed. Successful recordings implantation of a transistorized device designed to restore
of sound-induced potentials from the cochlear nerve, however, hearing of deaf persons is scheduled within 30 days."29 These
inspired stimulating the nerve with similar waveforms in order reports prompted readers who were deaf to call House and
to restore hearing. Doyle seeking a cure for their deafness, and aroused the interest
House and Doyle first attempted electrical stimulation to of investors seeking to cash in on emerging medical technology.
elicit hearing during stapes surgery by placing a needle elec House recognized the danger in such publicity, and publicizing
trode on the promontory or into the open oval window. An ear the implant work became an issue of considerable conflict
speculum inserted into the external auditory canal served as a between the Doyles and House. Disagreement over how
ground lead. With square wave stimuli, patients reported hear aggressive to proceed with the implant, given the initial bio
ing the stimulus without discomfort, dizziness. or facial nerve incompatibility problems, opened an irreparable rift between
stimulation. These responses were sufficient to encourage House and the Doyles that brought an end to their collabora
House and Doyle to implant a patient with a hard-wire device. tion. House had a very busy otologic practice, and implant
The first willing subject was a 40-year-old man with severe oto development took a low priority for several years to follow. The
sclerosis and deafness. Promontory stimulation of the right ear Doyles, on the other hand, continued to experiment, implanting
on january 5, 1 961 revealed consistent responses. On january 9, numerous subjects. They collaborated with the Los Angeles
therefore. a gold-wire electrode was inserted under local anes otolaryngologist Frederick Turnbull, whose office was used for
thesia through a postauricular approach into the round win most of the testing. They reported their results in locaJ3 and
dow. The wire was brought out through the postauricular nationaJ31 forums, reporting optimistically that electrical stimu
skin.27 The patient reported hearing the electrical stimuli, but lation could yield speech perception, but not offering system
he had poor loudness tolerance. Several weeks later the wire atic testing or analysis. The Doyles ceased their investigations
was removed. in 1 968 due to a lack of research funding.26

4
History of the Cochlear Implant

Stanford University
F. Blair Simmons had worked as a research associate in the
laboratory of S.S. Stevens at Harvard as a medical student and
then with Robert Galambos at the Walter Reed Institute prior to
his residency in otolaryngology at Stanford University in
Stanford, California. Simmons was an assistant professor in
Stanford's Division of Otolaryngology in 1 962 for less than a
month before he was presented with an unexpected opportu
nity to stimulate the cochlear nerve intraoperatively. The
patient was an 1 8-year-old man who had developed a recur
rence of a cerebellar ependymoma that manifested itself as mild
hearing loss. Exploratory craniotomy under local anesthesia
was planned, and the cochlear nerve would be exposed during
the procedure. Prior to surgery, Simmons discussed stimulating
the patient's cochlear nerve electrically. The patient agreed
to the intraoperative testing and to a preoperative auditory
training session. During the awake craniotomy, the patient Fig. 1 .3 William F. House (left) and Robin Michelson (rig ht)
was asked to describe what he heard when a bipolar electrode collaborating in the early 1 970s. (Courtesy of the john Q. Adams
was used to stimulate the exposed cochlear nerve with 1 00- Center.)
microsecond square wave pulses. The patient described
auditory sensations and was able to discriminate stimulation
frequencies up to 1 kHz.32 implanting their single-channel device in human patients. As
Simmons's first implanted device was then placed 2 years much as any other parameter, the durability and safety of
later, in 1 964. This second subject was a 60-year-old man who the device was on House's mind with this group of patients. Of
had been unilaterally deaf for several years, and whose better several patients implanted in 1 969, one required having his
hearing ear then became deaf. He also suffered from retinitis implant removed due to tissue rejection, and another was lost
pigmentosum and had associated severe sight disability. to follow-up. However, a third patient, Charles Graser, became a
Despite being made fully aware that implantation would likely long-term experimental subject. In Graser, House found stimu
fail and very likely yield no useful hearing, the subject agreed lation levels and results that remained stable over the course of
to undergo the implantation. Local anesthesia was used, and years. This gave credence to the safety of electrical stimulation.
the promontory exposed through a postauricular transmeatal Charles Graser was deaf for 10 years because of ototoxicity.
approach and elevation of a tympanomeatal flap. A 2-mm coch Postimplantation, he worked intensely as a research subject,
leostomy and then subsequent 0.1 -mm drill hole into the and continued to do so enthusiastically for many years.
modiolus were performed. A partial mastoidectomy was also Many of the observations and modifications that House and
performed and the middle ear entered anterior to the facial Urban reported in the 1 960s were based on the testing of only
nerve by removing the incus. A six-electrode array was placed subject-Graser. For instance, one of the surprising findings
through the mastoid opening into the epitympanum and then from the work with Graser was that a 1 6,000-Hz carrier fre
through both the cochleostomy and the modiolar opening to a quency signal helped him appreciate higher frequencies, and
depth of 3 to 4 mm. The electrodes were attached to a plug that amplitude-modulating the carrier with the acoustic signal
was then secured to the mastoid cortex. Psychoacoustic testing generally sounded the best. This signal processor strategy
was carried out both at Stanford and at Bell Laboratories in New became standard on the House/3M cochlear implant. Reporting
jersey.33 Unfortunately, the subject's combination of disabilities of these early results was primarily by testimonial experiences
made psychophysical testing very challenging. Based on these of the individual subjects rather than systematic study. Another
unfavorable experiences, Simmons became pessimistic about important outcome of these early studies was abandoning the
the future of implantation. He estimated the likelihood that multiple electrode systems for the single-wire electrode.Js
electrical stimulation of the auditory nerve could ever provide
a clinically useful means of communication to be "considerably
less than 5%."34 Human implantation at Stanford was postponed San Francisco
until further animal testing could prove its utility. Robin Michelson ( .,. Fig. 1 .3 ) was an otolaryngologist in private
practice in the 1 960s in Redwood City, California. He was
the grandson of the Nobel Prize-winning physicist Albert
House Resumes His Work Michelson. Robin Michelson's inspiration for cochlear implanta
With the advancements in pacemakers and ventriculoperito tion came from seeing a patient, T.l. Moseley, who had severe
neal shunts in the late 1 960s, House's interest in cochlear tinnitus and otosclerosis. Michelson had sought to monitor
implantation was reawakened with more confidence in the the cochlear microphonic during a stapedectomy as a means of
safety and efficacy of indwelling devices ( .,. Fig. 1 .3 ). House was immediate feedback, since he performed the procedure under
working with a talented engineer named jack Urban, a collabo local anesthesia. Moseley, an engineer, agreed to build him a
ration best known for several influential developments in neu high-gain amplifier with an earpiece that Michelson could use
rotologic instruments. House and Urban aggressively pursued in the operating room. Michelson placed an electrode against

5
. .

the round window. Feedback from the amplifier elicited the neurophysiology, was masterminded by Sooy and was an indis
sensation of sound for the patient that Michelson was subse pensable element in the development of the UCSF cochlear
quently able to pitch match.36 implant program.
Michelson, like House, originally subscribed to the telephone One of the first studies Merzenich performed was recording
theory of hearing-that the cochlea presented the auditory the response properties of single units in the cat inferior colli
nerve with the analogue electrical signal of the auditory stimu culus in response to both sound stimulation from one ear and
lus, and that all that was required to restore hearing was to electrical stimulation from the other ear in implanted cats. He
stimulate the auditory nerve with a similar signal. In an attempt showed that the inferior colliculus neurons responded similarly
to demonstrate that electrical stimulation of the auditory nerve to electrical and sound stimuli, but that the tuning curves for
elicited auditory responses, he implanted an electrode into electrical stimulation were very flat and showed little tuning.
the cat cochlea and measured the cochlear microphonic in the Additionally, the responses from animals with ototoxin-induced
opposite ear. He found that electrical stimulation suppressed hair cell destruction showed the same responses to electrical
the contralateral cochlear microphonic similarly to acoustic stimuli as untreated cochlea. This was the first definitive dem
stimuli. He concluded, therefore, that electrical stimulation was onstration that auditory sensation with implants arose from
carried along auditory pathways.37 Although Michelson was direct stimulation of the auditory nerve rather than electro
likely demonstrating electrophonic hearing rather than direct phonic effects. Responses in the cat were then compared to
auditory nerve electrical stimulation, this result inspired him to psychoacoustic measures in the human implanted subjects
implant a human volunteer with hearing loss. using the same electrical stimuli in both groups. The conclu
Michelson's first implant was a single-channel device sions from this work were that with single-electrode devices,
implanted into a congenitally deaf woman. Testing after periodicity pitch up to about 600 Hz is possible, but no place
implantation revealed that she obtained auditory sensations coding of frequency is possible. Thus in order to convey com
from stimulation, and that pitch perception was possible for plex sounds such as speech, multiple-electrode arrays would be
stimulus frequencies less than about 600 Hz. More interesting necessary.40 This work was presented to the American Otologi
to Michelson, however, was that the subject could differentiate cal Society at the 1 973 annual meeting in St. Louis, and it
a square-wave stimulus from a sine-wave stimulus.36 Michelson marked the beginning of the race toward the development of a
interpreted this to indicate that the fine structure of the multichannel cochlear implant.
electrical stimulus could be conveyed along auditory pathways.
The gold-wire electrode hardened several days after the
operation, broke from the rest of the implant, and needed to be
Controversies and Doubts
removed. Several additional patients received fully implantable The year 1 973 represents a crossroads in the cochlear implant's
single-channel devices, and this preliminary work was development. Until this time, cochlear implantation would have
presented to the American Academy of Ophthalmology and been considered, at best, an idea with potential to help some
Otolaryngology in October 1 970; a follow-up study was pre deaf people sometime in the future, and at worst as a danger
sented at the 1 971 meeting of the American Otological Society. ous experimental procedure promising nothing better than
The patients had pitch perception based on stimulus frequency vibrotactile information. Simmons had downplayed the poten
and could recognize speech stimuli but had no word under tial of implants and abandoned human implantation. The only
standing. All implanted patients lost whatever residual hearing clinicians performing human implants, House and Michelson,
they had prior to implantation.38 were surgeons far from the mainstream and whose funding
It was around this time that Francis Sooy visited Michelson in was from private sources. In order for the implant development
Redwood City and saw his work in progress with implantation. to proceed, implants would need to be granted legitimacy as a
Sooy was the chairman of the nascent Department of Oto valid research pursuit with National Institutes of Health (NIH)
laryngology at the University of California at San Francisco, and funding and demonstrate broad-based clinical application. This
this interaction with Michelson confirmed his belief in the section highlights events in the 1 970s that accomplished both.
potential of cochlear implantation. He persuaded Michelson to
join the faculty at UCSF and bring his implant investigations
to the University. Sooy also believed that the successful devel
National Institutes of Health
opment of the cochlear implant required a university-based The NIH itself was partly responsible for giving scientific legiti
scientific foundation. After recruiting Michelson, Sooy then macy to the cochlear implant. In 1 970 the Neural Prosthesis
recruited Michael Merzenich from the University of Wisconsin. Program was established within the National Institute of Neu
Merzenich was a young neurophysiologist whose interest was rological Diseases aimed at promoting extramural research on
mapping the inferior colliculus. He joined the UCSF faculty and neural prostheses primarily by capitalizing on the contract
began working on recordings from the inferior colliculus for mechanism of the NIH.41 Initially the program did not focus on
several months before meeting with Michelson about the an auditory prothesis, but rather on developing a visual pros
cochlear implant. Merzenich was initially quite skeptical about thesis for the blind, and the first contracts focused on this goal.
the merits of the implants and showed little interest in joining In addition to awarding contracts, the Neural Prosthesis Pro
the development effort. After seeing a few patients on a docu gram under the guidance of F. Terry Hambrecht initiated and
mentary film created by the otolaryngology resident C. Robert maintained the annual Neural Prosthesis Workshop. The work
Pettit, however, Merzenich became convinced of its potentiaJ.39 shop brought together a multidisciplinary group of contractors
The collaboration between Michelson, a clinical pioneer, and and consultants to the NIH campus to discuss research findings,
Merzenich, a talented basic scientist with a solid foundation in delineate important problems, and develop strategies for the

6
development of neural prostheses. At the third workshop in cochlear pathology was auditory nerve fiber degeneration.45
january 1 973, auditory prostheses first commanded a signifi Why, then, perform an invasive procedure like the cochlear
cant part of the agenda. Participants included Michelson, implant when an externally worn device such as a
Merzenich, and Simmons. Both Merzenich and Simmons also vibrotactile stimulator could be used to the same end?
obtained extramural NIH funding for their implant-related
research by this time.41 The above concerns did not dissuade the core group of implant
developers from proceeding onward, yet a strong aura of doubt
surrounded the cochlear implant. At the forefront of cochlear
Early Cochlear Implant Meetings implant support, however, was Francis Sooy, who was responsi
Several meetings over the next several years pitted the implant ble for assembling the implant devotees in October 1 974 with
pioneers against the otology/hearing science establishment. the support of the NIH in order to evaluate the progress and
These symposia began to bring cochlear implantation into the define the research goals for the implant, and to establish
limelight, often resulting in considerable controversy. Between guidelines for patient selection and implantation protocols. Two
1 971 and 1 973, significant work removed doubts that the audi important decisions were made at this meeting. First, criteria
tory nerve could be stimulated directly with the implant. Con for implantation were delimited : full informed consent that the
cerns that the prominent oro-scientists expressed toward the procedure is experimental; no useful hearing in either ear; only
implant therefore shifted and coalesced during meetings in those patients able and willing to participate in psychophysical
1 973 and 1 974 of the Otological Society, the First International testing; otherwise healthy patients ; and, finally, adults only.
Conference on Electrical Stimulation in San Francisco, and the Second, a consensus decision was made to stop implanting all
Third Workshop of the Neural Prosthesis Program. These con single-channel devices until an objective evaluation of the
cerns and their rationales are as follows: patients already implanted could be performed.46 The NIH took
the lead in this objective evaluation with a call for applications
Concern: The remaining nerve fiber population in deafness is for a formal objective evaluation of the single-channel recipi
not sufficient to support tonotopic stimulation of the nerve. ents. The future of implant development rested on this objec
This was based on the finding of Hal Schuknecht and tive evaluation, as a finding that the implant had limited utility
coworkers that only a minority of temporal bones examined may have curtailed allocation of further resources from
demonstrated more than two thirds of the normal population the Neural Prosthesis Program. The contract to perform the
of cochlear ganglion cells.42 Additionally, Nelson Y.S. Kiang, a objective assessment was awarded in june 1 975 to a team at
neurophysiologist at the Massachusetts Institute of the University of Pittsburgh led by Robert Bilger.
Technology who had defined how single auditory nerve units
respond to sound in cat, led a vehement opposition to human
cochlear implantation. His point of view was that cochlear
The Bilger Report
implants with the current design could never produce speech Thirteen adult single-channel implant subjects, 1 1 implanted
understanding or "useful hearing" because electrical stimuli by House and two by Michelson, were flown to Pittsburgh for a
could not convey the complex auditory stimuli that the week-long testing session to take part in the study. The subjects
cochlea provided.43 Implanting humans with devices that underwent extensive audiological, psychoacoustic, and vestibu
offered little more than improved lip reading, he thought, lar testing. Several of the results were not surprising: Subjects
could not be considered prudent. could not understand speech with the implant alone, but the
Concern: Electrical stimulation could convey sounds out of the implant helped the patients' lipreading scores. Also not surpris
speech frequency range. Cochlear damage in deafness is ing was the finding that the subjects' quality of life was aided
typically in its basal half, where high-frequency stimuli are by the implant. A surprising finding, however, was that the
transduced. The electrodes described at the time extended subjects' speech production was significantly aided by their
only into the proximal basal part of the cochlea. Therefore, if implants. The investigators concluded from the study that
the place principle were utilized, electrical stimulation would single-channel implants helped deaf patients. While this
yield only sound frequencies higher than the speech range.44 conclusion may not seem profound, it was the first objective,
Concern: The dynamic range of loudness with electrical scientific assessment of implant performance, concluding that
stimulation would be too narrow to convey useful sound the subjects received benefit from the implant with minimal
information. Although loudness grows with sound intensity risk.47 From this, the cochlear prosthesis gained the legitimacy
in the cochlea over a range of nearly 1 00 dB, the intensity needed to justify funded research efforts toward a multichannel
range with electrical stimulation is only about 6 dB, which device. Furthermore, while the world waited for the multi
would severely limit loudness discrimination. The dynamic channel implant, the single-channel device was viable.
range of the firing of cochlear nerve fibers of the cat in
response to both electrical and acoustic stimulation revealed
a similar finding-that the dynamic range in response to Development of a
sound is 20 to 40 dB, and to electrical stimuli 4 dB.43
Multichannel Device
Concern: lntracochlear manipulation that would occur with
cochlear implantation would result in significant damage to the With the Bilger study confirming the utility of a single-channel
cochlea; anything that disturbs scala media would cause device, House moved forward with refinement of his implant.
degeneration of the remaining sensory fibers. This concern He and jack Urban joined forces with the 3M Company. The
arose from studies of Schuknecht showing that one aspect of House/3M single channel device ( .,.. Fig. 1 .4) was implanted into

7
. .

He realized as early as his graduate thesis in 1 969 that the


single-channel device had limited utility,48 and he sought to use
a systematic scientific approach to developing a multielectrode
device. The approach had several fronts : developing speech
processing strategies, optimizing the electrode array, and devel
oping a safe, reliable implantable receiver-stimulator. The
efforts toward what would become the Cochlear Corporation's
Nucleus multichannel implant was primarily an Australian ven
ture, as funding came from Australian national telethons and
government-associated engineering firms, and partly through
government grants.49 Clark and his colleagues reported several
important findings, two of which were that inserting the elec
trode array in an anterograde direction through a single cochle
ostomy at the round window niche into scala tympani was less
traumatic to the cochlear structures than either retrograde
insertion or multiple cochleostomies,s o.sJ and dissolution
of platinum electrodes with biphasic pulsatile stimuli was
minimal, implying safe long-term stimulation.s2 Clark first
implanted a human subject in 1 978, and by 1 981 he showed
that subjects were able to understand some open-set speech
with their implants and without the aid of lipreading.s3 The
FDA approval for the Nucleus multichannel implant (Cochlear,
Melbourne, Australia) was granted for adult patients in 1 985
and children as young as 2 years in 1 990.
Fig. 1 .4 An early version of the House/3 M single-channel device. As several technical challenges were overcome in the 1 980s,
Shown is the implanted receiver/stimulator and wire electrodes. multichannel cochlear implants became a safe option for pro
(Cou rtesy of the john Q. Adams Center. ) foundly deaf adults and children. Patients with the implant
were expected to have a quality-of-life improvement and some
open-set speech recognition. Another development was
several thousand patients by the early 1 980s, and in 1 984 the required, however, to dramatically improve the speech
Food and Drug Administration (FDA) granted approval for the recognition provided by the implant, and this was the develop
device. Other centers, however, concentrated their efforts on ment of high-rate interleaved stimulation. Multiple electrode
researching and developing a multichannel device. At the stimulation relies on the place principle of coding auditory
forefront of this competition were Merzenich, Michelson, stimuli along the cochlea. For separate electrodes to be effective
Robert Schindler, and colleagues at UCSF ( Fig. 1 .5), and in eliciting different frequency responses, the spatial extent of
Graham Clark at the University of Melbourne in Australia. their stimuli must be different. Several studies in the late 1 970s
Clark, a clinically trained otolaryngologist, began investigat and early 1 980s demonstrated that significant interference
ing the cochlear implant as a graduate student in the 1 960s. (known as "interaction") resulted from simultaneous stimula
tion of multiple electrodes.s4.ss It was found that electrode
interaction could be minimized by stimulating the electrodes in
a staggered, nonsimultaneous pattern. 54 Another discovery was
that nonsimultaneous stimulation at pulse rates greater than
1 kHz was especially effective at improving an implant subject's
speech understanding. A collaboration between UCSF and the
Research Triangle Institute ( Research Triangle, NC) resulted in
the implementation and testing of a speech processing scheme
that utilized this concept. The concept was patented and
became known as continuous interleaved sampling (CIS). The
implementation of CIS provided a tremendous improvement in
implant recipients' performance with speech recognition. 56
Since that time there have been numerous noteworthy tech
nological developments in both device design and coding, and
the future promises even greater advances to further enhance
performance in the implanted hearing-impaired population.

Fig. 1 . 5 Early eig ht-channel electrode and epoxy-coated receiver


designed by Robin Michelson and assembled by Mel Ba rtz. The Conclusion
intracochlear portion of the electrode a rray was formed from Silastic to
The development of the cochlear implant began in 1 957 with
fi l l scala tympani. (Courtesy of Stephen Rebscher, Un iversity of
California at San Francisco.) the first attempts to restore hearing with direct electrical
stimulation of the auditory nerve. In the years that followed,

8
the primary proponents of implants were a few otologists try (26] Doyle jB. Interview with Philip Seitz, August 22, 1 993. john Q, Adams Center
Archives
ing to help their patients with single-channel devices, despite
[27] House WF. Cochlear implants: beginnings ( 1 957-1961 ). Ann Otol Rhinal Lar
considerable opposition from leaders in the field. If it were not yngol 1976; 85: 3-6
for these pioneers. cochlear implants may well have been (28] Doyle JB, Ballantyne EW, inventors Artificial sense organ, U.S. Patent
delayed by many years. Following the Bilger study, cochlear 3,449,768, june 1 7 , 1 969
implant research gained mainstream support, and efforts [29] Anonymous . California electronics firm readies "artificial ear" implant. Space
Age News. 1961 ; 3: 1
toward a marketable multichannel device were underway.
(30] Doyle JB, Doyle JH, Turnbull FM, Abbey j, House L Electrical stimulation in
Improvements are ongoing and offer a bright future to the hear eighth nerve deafness. Bull Los Angel Neuro Soc 1 963; 28: 148-150
ing-impaired population. (3 1 ] Doyle JH, Doyle JB, Turnbull FM. Electrical stimulation of the eighth cranial
nerve. Arch Otolaryngol 1 964; 80: 388-391
(32] Simmons FB. Mongeon Cj. Lewis WR. Huntington DA. Electrical stimulation
References of acoustical nerve and inferior colliculus; results in man. Arch Otolaryngol
1 964; 79: 559-568
[ 1 ] Dudley H. Remaking speech. ] Acoust Soc Am 1939; 1 1 ; 1 69-177 (33] Simmons FB, Epley jM, Lummis RC et al. Auditory nerve: electrical stimula
(2] Wever EG, Bray CW. The nature of the acoustic response: the relation tion in man. Science 1 965; 148: 1 04-1 06
between sound frequency of impulses in the auditory nerve. j Exp Psycho! (34] Simmons FB. Electrical stimulation of the auditory nerve in man. Arch Otolar
1 930; 1 3 : 373-387 yngol 1 966; 84: 2-54
(3] Stevens SS. On hearing by electrical stimulation. j Acoust Soc Am 1 937; 8: (35] House WF, Urban j. Long term results of electrode implantation and elec
1 9 1 - 1 95 tronic stimulation of the cochlea in man. Ann Otol Rhinal Laryngol 1 973 ; 82:
(4] Shah SB, Chung JH, jackler RK. Lodestones, quackery, and science: electrical 504-5 1 7
stimulation of the ear before cochlear implants. Am j Otol 1 997; 1 8 : 665-670 (36] Michelson R P . Interview with Phillip Seitz, November 7 , 1 995. john Q. Adams
[5] Djourno A. Strohl A. Modifications du courant de peau de grenouille pendant Center Archives
!'excitation electrique. CR Soc Bioi (Paris) 1 937; 1 2 5 : 625 (37] Michelson RP. The crossed cochlea effect. Trans Am Acad Ophthal Otolaryngol
(6] Djourno A. Variation de l'excitabilite du sciatique de grenouille suivant l'ecart 1 968
des electrodes. CR Soc Bioi (Paris) 1 946; 140: 1 83 (38] Michelson RP. Electrical stimulation of the human cochlea. A preliminary
(7] Djourno A. Sur Ia mesure instantanee de Ia frequence du pauls. Paris report. Arch Otolaryngol 1 971 ; 93: 3 1 7-323
Med (Paris) 1938; 37: 83 (39] Merzenich MM Interview with Marc Eisen, March 26. 2004
(8] Djourno A. Masmonteil, Roucayrol jC. Une application de Ia haute frequence (40] Merzenich MM. Michelson RP, Pettit CR. Schindler RA, Reid M. Neural encod
a !'extraction de protheses metalliques. Soc Electrother Radial. 1 948; 29: ing of sound sensation evoked by electrical stimulation of the acoustic nerve.
637-638 Ann Otol Rhinal Laryngol 1973; 82: 486-503
(9] Djoumo A, Delay j, Verdeaux G Un cas de narcolepsie avec etude eiectroence [41 ] Hannaway C. Contributions of the National Institutes of Health to the Devel
phalographique. Congres d'Electro-encephalographie de Langue franaise, opment of Cochlear Prostheses. Bethesda, MD: National Institutes of Health,
Paris 1 949 1 996
[ 1 0 ] Djourno A. La respiration eiectrophrenique. Presse Med 1 952; 60: 1 532- [42] Kerr A. Schuknecht HF. The spiral ganglion in profound deafness. Acta Otolar
1 533 yngol 1968; 65: 586-598
[ 1 1 ] Djourno A. Excitation eiectrique localisee a distance. C R Acad Sciences. 1 953; (43 ] Kiang NYS, Moxon EC. Physiological considerations in artificial stimulation of
236: 2337-2338 the inner ear. Ann Otol Rhinal Laryngol 1 972; 8 1 : 714-730
( 1 2 ] Djourno A. Kayser D. La methode des excitations induites a distance. ] Radial (44] Lawrence M, johnsson L-G. The role of the organ of Corti in auditory nerve
1 954; 36: 1 1 7- 1 1 8 stimulation. Ann Otol Rhinal Laryngol 1973; 82: 464-472
( 1 3 ] Djourno A , Kayser D , Guyon L Sur I a tolerance par l e nerf d'appareils eiectri (45] Schuknecht HF. Lesions of the organ of Corti. Trans Am Acad Ophthalmol Oto
ques d'excitation indus a demeure. CR Soc Bioi (Paris) 1 955; 149: 1 882-1 883 laryngol 1 953; 57: 366-383
[ 1 4 ] Eyries C. Traitement de l'ozene par un nouveau precede de prothese chirurgi (46] Merzenich MM. Sooy FA Report on a workshop on cochlear implants, Univer
cale. Ann Otolaryng. 1 946; 1 3 : 581 -586 sity of California at San Francisco, October 23-25 1 974
[ 15] Olivier G. Eyries C. Reperes chirurgicaux et aspects du nerf facial extra (47] Bilger RC. Evaluation of subjects presently fitted with implanted auditory
petreux. Med trap. 1 953; 1 3 : 720-723 prostheses. Ann Otol Rhinal Laryngol 1 977; 86 Suppl 38: 1 - 1 76
( 1 6 ] Eyries C. Experience personelle. Cahiers d'Oto-Rhino-Laryngologie. 1 979; 14: (48 ] Clark G. Middle Ear and Neural Mechanisms in Hearing and in the Manage
679-681 ment of Deafness [Doctor of Philosophy Thesis]. Sydney: University of Sydney,
[ 17] Djourno A. Interview with Phillip Seitz. january 12. 1 994. john Q, Adams 1 969
Center Archives (49] Clark G. Sounds from Silence. Adelaide: Allen & Unwin, 2000
[ 1 8 ] Djourno A, Eyries C, Vallancien B. De !'excitation eiectrique du nerf cochleaire [50] Clark GM, Hallworth Rj. Zdanius K. A cochlear implant electrode. J Laryngol
chez l'homme. par induction a distance, a !'aide d'un micro-bobinage indus Otol 1 975; 89: 787-792
a demeure. CR Soc Bioi (Paris) 1957; 1 5 1 : 423-425 (51 ] Clark GM. An evaluation of per-scalar cochlear electrode implantation tech
[ 1 9 ] Eyries C. Interview with Phillip Seitz, january 1 0, 1 994. john Q, Adams Center niques. An histopathological study in cats. j Laryngol Otol 1977; 9 1 : 185-199
Archives (52] Black FO. Wall C, O'Leary DP, Bilger RC. Wolf RV. Galvanic disruption of vesti
(20] Djourno A. Analyse oscillographique instantanee de Ia voix par!ee. CR Soc bulospinal postural control by cochlear implant devices. ] Otolaryngol 1 978;
Bioi (Paris) 1959; 1 5 3 : 1 97-198 7: 5 1 9-527
(21 ] Djourno A. A propos de prothese sensorielle totale. Bull Acad Nat! Med 1 977; [53] Clark GM, Tong YC, Martin LF. A multiple-channel cochlear implant: an evalu
1 6 1 : 282-283 ation using open-set CID sentences. Laryngoscope 1981 ; 91 : 628-634
[22] Chouard CH. Entendre sans Oreilles. Paris: Robert Laffont. 1 973 [54] Eddington OK, Do belle WH, Brackmann DE, Mladejovsky MG. Parkin jL Audi
(23 ] Eisen MD. Djourno, Eyries, and the first implanted electrical neural stimula tory prostheses research with multiple channel intracochlear stimulation in
tor to restore hearing. Otol Neurotol 2003 ; 24: 500-506 man. Ann Otol Rhinal Laryngol 1 978; 87: 1 -39
[24] House WF A personal perspective on cochlear implants. In: Schindler RA, [55] White M. Design Considerations of a Prosthesis for the Profoundly Deaf.
Merzenich MM. eds. Cochlear Implants. New York: Raven Press 1 98513- Berkeley, CA: University of California. Berkeley, 1 978
1 98 5 1 6 (56] Wilson BS, Finley CC, Lawson DT, Wolford RD. Eddington OK, Rabinowitz
(25] House W F. Cochlear Implants: My Perspective. Newport Beach, C A : AllHear, WM. Better speech recognition with cochlear implants. Nature 1 99 1 ; 352:
1 995 236-238

9
I I

2 Genetics of H ea ri n g Loss a n d Predictors of Coch l ea r


I m pl a nt Outcome
Robert W. Eppsteiner, Richard K. Gurgel, and Richard}. H. Smith

loss and later-onset goiter, both appear as NSHL at birth,


Introduction
and thus we refer to these types of SHL as the NSHL mimics.
Cochlear implantation is the standard treatment for hearing Without genetic testing the NSHL mimics cannot be distin
restoration in patients with bilateral, profound sensorineural guished from true NSHL. The scope of the challenge lies in
hearing loss (SNHL). Although cochlear implant candidates all the fact that NSHL is extremely heterogeneous. Over 67
share the diagnosis of SNHL, the divergent causes of hearing NSHL-causing genes have been identified; however, the total
loss create a heterogeneous patient population. This heteroge number is likely double based on data assigning genomic
neity in etiology likely contributes to the spectrum of cochlear positions to 130 NSHL loci ( a locus is a genomic position
implant performance. If we better understood the causation that harbors a NSHL-causing gene).s
of SNHL in deaf patients, we would likely better predict their Most congenital NSHL (80%) is autosomal recessive
hearing outcome after implantation. (ARNSHL), and, quite unexpectedly, mutations in one gene
Deafness results from the interplay of environmental and account for 50% of congenital severe-to-profound ARNSHL in
genetic factors affecting the auditory pathway. With the many different world populations.3 G]B2, encodes a protein
advent of new technologies for genomic enrichment and called Connexin 26, which makes hexameric gap junctions
high-throughput sequencing, the field of genetics has begun a to link cells as a functional syncytium. The relative contribu
renaissance. Sequencing the entire human genome, which took tions made by other genes to severe-to-profound ARNSHL,
over a decade for the Human Genome Project, can now be done and to other degrees of congenital hearing loss, have not
in a matter of days. This new accessibility of genetic information been determined primarily because until recently the exper
is revolutionizing both the molecular study of disease and its imental design was prohibitively complex, expensive, and
management. Among the changes is a paradigm shift in the labor intensive.
evaluation of the deaf/hard-of-hearing person, especially if the Of the 67 genes implicated in NSHL, 38 cause ARNSHL and 25
family history suggests that the hearing loss is inherited. cause autosomal dominant NSHL. There are two X-linked
The cornerstone of the evaluation of hearing loss remains causes of NSHL and, in addition, mitochondrial- and microRNA
the medical history and physical examination, comple associated NSHL occurs.67 These genes encode a wide variety of
mented by a thorough audiological assessment, but the next proteins that are expressed in the cochlea and auditory neural
test that should be ordered is a comprehensive genetic pathway and have a diverse spectrum of functions. Structural
assessment. This algorithm underscores the importance of proteins include actins (ACTG1 ), actin-associated proteins
a person's unique genetic traits in the medical decision (TRIOBP, RDX) and myosins ( MY07A, MY01 5A, MY06, MY01A,
making process.1 This chapter reviews the genetic basis of MYH9, MYH14); cellular junction/adhesion proteins include
hearing loss, novel technologies used for comprehensive otoancorin (OTOA), claudin- 1 4 (CLDN1 4), and the gap junctions
genetic testing, and the impact of genetics on cochlear (GJB2, GJB6); extracellular linkage molecules include cadherin
implant (CI) performance. 23 (CDH23 ) and protocadherin 15 ( PCDH 1 5 ) ; and transporters
and channels include pendrin (SLC26A4) and potassium
channels (KCNQ4) ( "" Table 2.1 ).
Genetic Basis of Hearing Loss
In developed nations, hearing loss is diagnosed in approxi Advanced Sequencing
mately 1 of every 500 newborns, making it the most
common congenital sensory defect.2 It is three times more Technology for Genetic Testing
prevalent than both Down syndrome and cystic fibrosis.3 .4
for Deafness
The causes of congenital SNHL may be environmental (for
example, congenital cytomegalovirus and antibiotic-induced In 1 977, Frederick Sanger, the only two-time Nobel laureate in
ototoxicity) or genetic.3 In 70% of cases, hearing loss is chemistry, developed Sanger sequencing, the gold standard for
nonsyndromic ( NSHL), implying that hearing loss is the only genetic testing.8 Sanger sequencing utilizes chain-terminating
recognized phenotypic abnormality. In the remaining 30% of dideoxynucleotide inhibitors to determine nucleotide sequence,
cases, however. other physical findings, such as heterochro and until recently was the only method used for full-gene
mia iridis or preauricular pits, co-segregate with the auditory sequencing. Over the past few years, however, several advanced
deficit. Collectively, these types of hearing loss are called genomic technologies that parallelize the sequencing process
syndromic ( SHL). (therefore they are often collectively called massively parallel
Most forms of SHL can be recognized from birth, but there sequencing [MPS] technologies) have been developed. These
are two important exceptions, notable because they are both technologies produce millions of sequences at once that are bio
recessive and relatively common. Usher syndrome, of which informatically aligned to the parent human genome and
there are three types, presents with congenital hearing loss queried for nucleotide differences. For an in-depth review of
and later-onset retinitis pigmentosa, and Pendred syndrome, these technologies as applied to hearing loss, the reader is
which can present with congenital or postlingual hearing referred to Shearer et al.9

10
.
. . . .

Table 2.1 List of Genes Included on the OtoSCOPE Platform with Corresponding Protein Name, Function, and Cochlear I mplant Performance
I nformation'
Gene Protein Function Cl Perfonnance Reference
Membranous labY!ith Genes
CDH23 cadherin-related 23 structural (cell ad hesion) + 1 8, 1 9
CLDN 14 Claudin 1 4 structural (tight ju nction) u nknown
COL J JA2 collagen, type XI, alpha 2 structural (extracellular matrix) unknown
ESPN espin structu ral (cytoskeleton of hair bundle) unknown
GJB2 gap junction protein, beta 2 ion homeostasis + 22,28 -33
GJB6 gap junction protein, beta 6 ion homeostasis + 62
ILDR 1 lg-like domain-containing receptor 1 u nknown unknown
LOXHD 1 lipoxygenase homology domain-containing 1 stereociliary protein +

MY03A myosin l i lA motor protein (hair bundle) unknown


MY06 myosin VI motor protein (hair bundle) unknown
MY07A myosin VIlA motor protein (hair bundle) +

MYO JSA myosin XVA motor protein (hair bundle) unknown


OTOA otoancorin structu ral (extracellular matrix) u nknown
OTOF otoferlin synaptic transmission (exocytosis at auditory +

ribbon synapse)
PTPRQ protein-tyrosine phosphatase receptor structural (hair cell shaft connectors) unknown
RDX radixin structural (cytoskeleton of hair bundle) u nknown
SLC26A5 solute carrier family 26, member 5 motor protein (OHC} unknown
STRC stereocilin structu ral (extracellular matrix) u nknown
TECTA tectorin alpha structural (extracellular matrix) u nknown
TMC1 transmem brane channel-like 1 u nknown + 63
TMIE transmem brane inner ear un known unknown
TRIOBP TRIO and F-actin binding protein structural (cytoskeleton of hair bundle) unknown
USH 1 C Usher syndrome 1 C homolog structural (scaffolding protein hair bund le) unknown
WHRN whirlin structural (scaffolding protein hair bund le) unknown
MYH14 myosin, heavy chain 1 4, nonm uscle un known u nknown
POU4F3 pou class 4 homeobox 3 transcription factor unknown
T}P2 tight ju nction protein 2 structural (tight ju nction) unknown
POU3F4 pou-domain class 3 tra nscription factor 4 transcription factor + 64,65,66
SLC26A4 solute carrier family 26, member 4 ion homeostasis + 36 -40
AUG J actin, g a m m a 1 structural (cytoskeleton of hair bundle) unknown
COCH coagu lation factor C homologue, cochlin structu ra l (extracellular matrix) +

CRYM crystallin, m u i o n homeostasis unknown


DFNAS deafness, autosomal dominant 5 un known unknown
SERPINB6 SERPI NB6 protease inhibitor (hair cel ls) u nknown
Spiral Ganglion Genes
ESRRB estrogen-related receptor beta transcri ption factor u nknown
CCDCSO coiled-coil domain containing 50 structural (cytoskeleton of hair bundle) u nknown
G/PC3 GAIP (-terminus interacting protein 3 proposed role in signal acquisition and u nknown
propagation in coch lear hair cel ls
GJB3 gap junction protein, beta 3 ion homeostasis unknown
PCDH 1 5 protocad herin-related 1 5 structural (cell adhesion i n hair bundle) u nknown
PJVK pejvakin auditory pathway signaling (hair cell and u nknown
neuronal)
TMPRSS3 transmem brane protease, serine 3 un known variable
KCNQ4 potassium voltage-gated channel ion homeostasis unknown
MYH9 myosin, heavy chain 9, nonm uscle motor protein (hair cell) +

WFS1 Wolfra m syndrome 1 (wolframin) ion homeostasis u nknown

11
Table 2.1 continued
I Gene Protein Function Cl Perfonnance Reference
I Minimal Data Genes
GPSM2 g-protein sig naling modulator 2 G-protein coupled receptor signaling pathway u nknown -
GRXCR 1 g l utaredoxin cysteine-rich 1 u nknown u nknown -
HGF hepatocyte growth factor m itogen, motogen, and neurotrophic factor u nknown -
LHFPLS lipoma HMGIC fusion partner-like 5 may function in hair bundle morphogenesis unknown -
LRTOMT leucine-rich transmem brane OMT auditory receptor cell development u nknown -
MARVELD2 MARVEL domain containing 2 epithelial barrier formation u nknown -
TPRN ta perin u nknown u nknown -
PRPS1 phosphoribosylpyrophosphate synthetase 1 nervous system development and nucleotide u nknown -
synthesis
GRHL2 grainyhead-like 2 transcription factor u nknown -
MY01A myosin lA movement of organel les along actin u nknown -
filaments
SLC1 7A3 solute carrier family 1 7. member 3 ionic and neurotransmitter transmem brane u nknown -
transport
DIAPH1 diaphanous homologue 1 structural (cytoskeleton of hair bundle) u nknown -
DSPP dentin sia lophosphoprotein biomineral tissue development u nknown -
EYA4 eyes absent homologue 4 transcription factor u nknown -
CLRN1 clarin 1 may modu late neu rotransmission at hair u nknown -
cell - spiral ganglion cell synapse
GPR98 g-protein - coupled receptor 98 may have role in the development of the u nknown -
central nervous system
USH 1 G SANS (aka Usher syndrome type- 1 G protein) structural (hair cell bund les) u nknown -
USH2A usherin 2A structural (cell adhesion) un known -
MSRB3 methionine-R-su lfoxide reductase B3 protein repair un known -
DIABLO diablo homologue, mitochondrial promotes apoptosis by activating un known -
caspases
CEACAM 1 6 carcinoembryonic a ntigen-related cell ad hesion possible structura l protein (between un known -
molecule 1 6 tectorial membrane and stereocilia)
EYA 1 EVA1 protein un known un known II -
FOX/ 1 forkhead box protein 1 1 transcription factor un known II -
11 - I
KCNJ 1 0 adenosine triphosphate (ATP)-sensitive inward ion homeostasis un known
rectifier potassium channel 1 0
Abbreviations: + represents good cochlear implant performance.
0Genes have been divided based on their location of primary expression. Genes listed as expressed in the spiral ganglion in certain cases may also be expressed in
the membranous labyrinth but are listed as spiral ganglion expressed genes.

Multigene Panels Using Microarrays Multigene Panels Using Targeted


One of the first advanced genomic strategies applied to heredi Genomic Enrichment
tary hearing loss was the single nucleotide extension microar An alternative to array-based hybridization is solution-based
ray (HHL APEX).10 These mutation detection arrays incorporate
hybridization, one example of which is targeted genomic
bound primers to which genomic DNA is hybridized. Fluores enrichment (TGE). In TGE, sheared genomic DNA is hybrid
cent nucleotides are added, and their binding to overhanging
ized to biotin-tagged probes (also known as baits) that
primer bases is detected by imaging. HHL APEX does not inter are complementary to specific sequences of interest.
rogate entire genes but rather only 1 98 bases in eight genes Hybridization occurs in solution ( solid-phase hybridization
where pathogenic variants have been described. is also possible), and then the genomic DNA-bait complexes
Another microarray-based platform is OtoCHIP. It offers an are captured using streptavidin beads. Noncapture genomic
important advantage in that it uses resequencing microarrays
DNA is washed away, leaving a library enriched for a
to screen specific genes for any nucleotide changes. Biotinylated specific region of interest. There are currently two
genomic DNA is hybridized to its complementary probe on the
comprehensive panels for hearing loss that employ TGE :
array, which is imaged repeatedly to determine the nucleotide
OtoSCOPE and a panel designed by Otogenetics Corporation
at each genomic position. The current version of OtoCHIP inter
(Atlanta, GA). 1213
rogates 19 genes ( 70,000 bases ).1 1

12
. . . . . ..
Personalized Genomic Medicine like OtoSCOPE, all known genetic causes of NSHL can be
screened simultaneously, moving the role for genetic testing
In addition to the targeted deafness panels described above, it is in patient management to the forefront.
possible to sequence a person's entire exome (coding genetic
sequence) or even a person's entire genome using next-genera
tion sequencing. It is no longer far-fetched to consider clinicians Genetic Testing for Cochlear Implant
having access to a patient's entire genomic sequence to aid in Candidates
disease diagnosis and treatment. For a variety of technical and
bioinformatic reasons, however, it is currently more efficient to Currently, when a child or adult with severe-to-profound
use multigene panels than more comprehensive alternatives. deafness presents for a CI evaluation, genetic testing is not
part of the standard evaluation. We propose an evaluation
paradigm in which genetic testing is part of the preoperative
Role of Genetic Testing in evaluation for all CI candidates with suspected NSHL
( 11> Fig. 2.1 ). Preoperative genetic testing can decrease the
Patient Management
number of screening tests ordered, change surgical manage
The role of genetic testing in the management of the deaf/ ment, and improve patient selection for implantation,

-
hard-of-hearing patient is rapidly evolving. With platforms thereby decreasing healthcare costs.

Genetic Testing Paradigm for


Cochlear Im plant Candidates

..(
Apparent Genetic
Hearl ng Loss


Medical History
Family History
Physical Exam
Audiogram, ABA, ASSR

t t
Apparent Non-Syndromlc
Syndromlc Hearing Loss
Hearing Loss
1.( ) II.( )
(
(

J
Comprehensive Genetic Testing Phenotype-Based
with Multlgene Panel Individual Gene Testing

'

( Genetic Counaellng

J J
Pathogenic Mutation In No Pathogenic Mutation l n Appropriate Testing:
Known Hearing Loss Gene Known Hearing Loss Gene0 Ophthalmology referral, EKG,
Temporal Bone Imaging, etc.

t

(
..( J J ( )
R-ch-Baeed Testing
Non-Syndromlc Non-syndromlc Hearing
Hearing Loss Loss Mimic

' '
(( Genetic CounHI Ing
)) Genetic CounHIIng
Appropriate Testing:
Ophthalmology referra l , EKG,
Temporal Bone Imaging, etc.

-
Fig. 2 . 1 Proposed genetic testing paradigm for Cl
candidates. In cases where inherited deafness does not have synd romic features we recommend
comprehensive genetic testing. This wil l determine if individuals have true nonsyndromic hearing loss or hearing loss caused by a nonsyndromic
hearing loss mimic gene such as in Usher or Pend red syndrome. When a mutation in a nonsyndromic mimic gene is identified, referral for appropriate
further clinical eva luation should be pursued. I ndivid uals for whom a genetic diagnosis is not reached a re excel lent candidates for research-based
testing and novel gene discovery. Abbreviations: ABR, auditory brainstem response; ASSR: auditory steady-state response; EKG , electrocardiogram.

13
I I

Genetic Screening in Cochlear and neither is it logical or cost -effective. As an alternative,


we propose that after an audiogram, every patient with
Implant Candidates with suspected NSHL should have comprehensive genetic testing.
Usher Syndrome If a diagnosis is established, a large number of exploratory
screening tests can be avoided and a directed evaluation can
Usher syndrome, an autosomal recessive type of SNL that falls
be completed. If SHL is suspected, appropriate screening tests
under the rubric of NSHL mimics, is the leading genetic cause
can be ordered as required ( .,. Fig. 2.1 ).
of deafness and blindness and illustrates the utility of genetic
testing prior to cochlear implantation. Second to mutations in
G]B2, mutations in the Usher syndrome genes are the most com Impact of Genetic M utation on
mon cause of hearing loss in the congenitally deaf Cl population
(20% of cases).14 Because the deafness is congenital but the visual Cochlear Implant Performance
impairment is delayed, without genetic testing it can be very
Despite more than 60 reports studying genetic mutation and CI
difficult to differentiate the Usher syndrome neonate from a neo
outcome, there are few firmly established genotype-phenotype
nate with another form of congenital severe-to-profound hearing
CI performance correlations (Table 2.1 ). Intuitively, the site
loss.1s But differentiation is important for many reasons. First,
of pathology along the auditory pathway should be a useful
when the diagnosis of Usher syndrome is made early, precau
framework for predicting the impact of a genetic lesion on Cl
tions such as wearing ultraviolet ray (UV)-canceling sunglasses
performance. For example, mutations in genes expressed in
can be taken to delay the onset of vision loss. Second, in patients
the membranous labyrinth should portend a good CI outcome
with Usher syndrome, simultaneous bilateral implants are advo
because the implant would bypass the lesion and directly stim
cated, because sound localization is improved.16 Because patients
ulate spiral ganglion neurons. In contrast, mutations in spiral
with USH1 eventually develop blindness, early bilateral cochlear
ganglion-expressed genes should portend poorer performance
implantation ensures that hearing outcomes are optimized before
because of dysfunction at the site of electrical stimulation.
vision is lostP Third, preoperative genetic testing provides valu
Prior studies suggest that these suppositions are correct
able prognostic information, as Usher syndrome patients have
( .,. Fig. 2.2 ).25,26
excellent hearing outcomes after cochlear implantation.1819

Improved Patient Selection Membranous Labyrinth


for Cochlear Implantation with Expressed Genes
Genetic Testing The vast majority of established genotype-phenotype correla
tions have been published for genes that encode membranous
It is estimated that 3 to 7% of CJ recipients do not benefit from
labyrinth proteins including G]B2, SLC26A4, OTOF, LOXHD 1,
implantation; however, these patients cannot be identified
KCNQ1 , MYH9, CDH23, MY07A, TMC1, COCH, POU3F4, and the
prior to implantation.2021 Because most CJ recipients with
mitochondrial expressed genes. As a CJ bypasses the membra
NSHL have very similar audioprofiles, audiological testing
nous labyrinth, persons with these genetic types of hearing loss
cannot be used to prognosticate CI outcomes. It is possible,
should be among the group of good CI performers, and indeed
however, that CI performance may be related to the genetic
this is almost always the case.
cause of hearing loss. For example, studies of CJ performance
in persons with G]B2-related deafness have shown that
outcomes are typically excellent, whereas implantation in GJB2
persons with DDP1/TIMM8a-related deafness are not advisa G]B2 is the best studied hearing loss gene and has firmly estab
ble.22,23 The availability of multigene deafness panels like lished genotype-phenotype correlations. The encoded protein,
OtoSCOPE make comprehensive studies of this relationship connexin 26, is expressed in the supporting cells, spiral limbus
possible. Preliminary data suggest that if the hearing loss is and spiral ligament of the membranous labyrinth, and may be
secondary to a gene expressed in the membranous labyrinth important in K+ ion recycling in the supporting cells and stria
( G]B2, CDH23, MY07A), the CI outcome is likely to be a good ; in vascularis.27 There are over 25 reports (including over 420
contrast, if the hearing loss is due to a gene expressed in the individuals ) of G]B2 genotype-phenotype CJ performance corre
spiral ganglion (DDP1/TIMM8a), CJ performance may be poor lations, with the general consensus being that G]B2-related
( see below). deafness is associated with good performance. In fact, some
studies have found that patients with G]B2-related deafness
perform better than patients with other causes of deafness
Decreased Need for Screening Tests when congenitally deaf Cl patients are divided into two groups
The evaluation of a deaf/hard-of-hearing patient with appar based on G]B2 status.222B -33 There is only one report of a patient
ent NSHL is not standardized. After an audiogram, temporal with G]B2-related deafness performing poorly; however,
bone imaging is often reflexively ordered, often with one or the implant was not performed until age 36.34 Consistent with
more screening tests including electrocardiogram, renal this poor outcome, prolonged profound deafness prior to
ultrasound, urinalysis, and ophthalmological evaluation.24 implantation is associated with higher rates of spiral ganglion
Occasionally genetic testing, typically for G]B2 only, is consid cell degeneration, which is an important determinant of CI
ered. This type of evaluation paradigm is not evidence-based performance.2526

14
. . . . . . .

Membranous Expressed Genes


GJB2, SLC26A4, O TO F, LOXHD I, KCNO I,
CDH23, MY07A, POU3F4, MYH9, TMC I,
COCH, Mitochondrial

Fig. 2.2 Genes with establ ished genotype-phenotype correlations and their location of expression. (Adapted from the Heredita ry Hearing Loss
Homepage (hereditaryhearingloss.org) with permission from G. Van Cam p and R.J . H . Smith.)

SLC26A4 Mitochondrial Deafness


SLC26A4 encodes the protein pendrin, a sodium-independent There have been over 14 reports (23 cases) of Cl performance in
chloride/iodide symporter, and is implicated in Pendred mitochondrial deafness. Mitochondrial deafness is usually postlin
syndrome (SNHL, enlarged vestibular aqueduct, thyroid abnor gual, progressive, and in many cases, syndromic. Virtually all cells,
malities). There have been five reports of 49 patients with including those in the inner ear, depend on mitochondria for
SLC26A4-related hearing loss correlating Cl performance with adenosine triphosphate (ATP) production via oxidative phospho
SLC26A4 mutations. The majority of these patients had both rylation. Typically, when a phenotype manifests in mitochondrial
SNHL and enlarged vestibular aqueduct. The pathophysiological associated disease, it does so in cells with high energy require
basis of SLC26A4 hearing loss appears to be secondary to mem ments. Because there are many such cells in many different organ
branous labyrinth dysfunction, and, as such, we would expect systems, the result is often a multisystem syndromic phenotype.
these Cl recipients to perform well.35 All reports confirm that In the cochlea, the cells with the highest energy demands are hair
this is indeed the case.36-4o cells and intermediate cells in the stria vascularis. Consequently,
mitochondrial deafness results in damage to hair cells and the
OTOF stria vascularis, and, as would be expected, patients with mito
chondrial deafness are good Cl performers.47-49
There have been four reports ( 1 2 patients ) of Cl performance
in patients with otoferlin (OTOF) mutations, all of whom
benefited from implantation.41-44 The encoded trans Spiral Ganglion Expressed Genes
membrane protein controls calcium binding and vesicle
Cochlear implants stimulate spiral ganglion cells. Therefore, in
release at the interface of the inner and outer hair cells and
persons with extensive degeneration of these cells, Cl perform
the auditory nerve.45 Hence, otoferlin mutations cause a
ance will be poor. Although there are very few reports of geno
unique phenotype, which mimics auditory neuropathy or
type-phenotype correlations in genes with robust expression in
dyssynchrony. The hearing loss is congenital; however,
the spiral ganglia, in the few cases that are available, it does
patients often pass newborn hearing screens based on otoa
appear that Cl outcomes are more variable.
coustic emission ( OAE) testing but demonstrate profound
deafness on auditory brainstem response testing. These
results reflect the expression pattern of otoferlin in both TMPRSS3
outer and inner hair cells. In the presence of OTOF mutations, TMPRSS3 is robustly expressed in the spiral ganglion, stria
the inner hair cells are affected first ( hence the poor ABR) vascularis, and cochlear supporting cells and encodes a trans
followed by progressive degeneration of the outer hair cells membrane serine protease, which is critical to spiral ganglion
(the OAEs are ultimately lost).46 Because Cis directly stimulate function.s o Although the function of TMPRSS3 has not
the spiral ganglion neurons, the causative lesion is bypassed been completely elucidated, it may be involved in cleavage of
in OTOF-related deafness. neurotrophins in spiral ganglion cells, and, when defective,

15
I I

neurotransmission may be impaired.51 There have been three


published studies on genotype-phenotype correlations in per
Cochlear Implants in Auditory
sons with TMPRSS3-related hearing loss.51 - 53 In the first report, Neuropathy
describing four siblings with postlingual, progressive, hearing
loss, the average age of onset of hearing loss was 6 years, and by Auditory neuropathy (AN) or dyssynchrony is hearing loss
20 years hearing thresholds were in the severe-to-profound caused by aberrant neurotransmission of the cochlear nerve
range. Cl performance was noted to be good; however, the met leading to dyssynchronous temporal processing of auditory
rics to assess performance were not reported. Another report of input.ss Because a CJ does not bypass the auditory nerve, one
nine CJ patients demonstrated improved performance on the might expect AN patients to be poor Cl candidates. However,
AN is a heterogeneous disorder, with mutations in OTOF
consonant-nucleus-consonant (CNC) word test as compared to
controls groups. 51 However, in a third study, two adult Cl recipi accounting for 50% of cases. Because CJ performance in the
ents demonstrated poor performance on both the Hearing-in presence of OTOF mutations is good, reflecting a distal lesion, it
Noise Test ( HINT) as well as the CNC word list.53 Further is likely that within the cohort of AN patients, Cl performance
research is required to clarify the impact of TMPRSS3-related will vary, based on the site of pathology. In general, however,
deafness on Cl performance. AN is associated with good Cl performance.s9-61

CHD7 Limitations of Genotype


CHD7 is diffusely expressed in a large number of tissues
Phenotype Correlations
throughout the body. Expression in the ear is found in spiral
ganglion cells, cochlear hair cells, vestibular sensory epithelia, Genotype-phenotype correlations in CI patients are limited.
and ossicles.s4 Mutations lead to CHARGE syndrome (coloboma, Until recently, it has not been possible to evaluate genotype as a
heart defects, choana( atresia, retarded growth and develop variable in assessing CJ performance. The increased availability of
ment, genital hypoplasia, ear abnormalities), which includes multigene panels, however, now makes it possible for clinicians to
diverse ear phenotypes that can involve any part of the inner, determine the genetic cause of hearing loss in Cl recipients. More
middle, or external ear. There have been two studies examining genetic causes of spiral ganglion degeneration will be found, and,
the impact of CHARGE syndrome on Cl performance. In one, of over time, likely corroborate an association with poor Cl perform
eight persons with CHARGE syndrome, three performed poorly ance. It is possible that cochlear implantation in these patients
with their cochlear implants.55 In the second, five persons will then become an ethical debate. Temporal bone studies of
were studied and again the outcome was variable.56 Because spiral ganglion cell survival may help to resolve this question.
phenotypic variability is common in CHARGE syndrome, it is
not unreasonable to hypothesize that the level of spiral gan
glion pathology may vary, leading to inconsistent results after Conclusion
cochlear implantation. Performance may also be confounded by Advanced genomic and sequencing technology have changed
cognitive disabilities. the evaluation paradigm for the deaf/hard-of-hearing person by
placing genetic testing at the forefront of clinical management.
DDP1 /TIMMBa The incorporation of genetic testing in the evaluation of the Cl
patient will likely enable us to prognosticate CI performance
Deafness-dystonia-optic neuronopathy syndrome ( Mohr more accurately than heretofore possible. Thus far, it appears
Tranebj.:erg syndrome, DFN-1 ) is caused by mutation of the that mutations in genes expressed in the membranous laby
deafness-dystonia peptide (DDPI/TIMMBa) gene. The pheno rinth do not compromise Cl performance; in contrast, muta
type includes postlingual SNHL, dystonia, psychosis, and optic tions in genes expressed in spiral ganglion neurons may lead to
atrophy. Temporal bone histology shows degeneration of spiral poor Cl performance. This observation can be easily confirmed
ganglion cells in the absence of damage to the membranous by applying comprehensive genetic testing to the Cl population
labyrinth, and consistent with this finding, Cl performance is and evaluating genotype as an independent variable against CI
poor_23.57
performance. If a genotypic effect can be substantiated, given
the expense of cochlear implants and the limitation of health
Variability in Outcomes in Spiral Ganglion care dollars, this information may have impact on the decision
Expressed Genes to place cochlear implants and on the aggressiveness of post
operative auditory therapy in predicted poor performers. Ulti
Lack of sensory input to the spiral ganglion cells over a pro mately, applying comprehensive genetic testing to the cochlear
longed time period results in spiral ganglion neuron degenera implant population will allow for more patient-centered care
tion, and a decreased number of spiral ganglion neurons leads based on an individual's unique genetic traits.
to poor Cl outcomes.25.26 The impact of mutations in spiral
ganglion-expressed genes on CJ performance is more difficult
to quantitate because cell degeneration and death follows a References
continuum, which may explain some of the variable outcomes
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affirming this hypothesis. Eng! ] Med 2006; 354: 2 1 5 1 -2 1 64

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( 1 9 ] Pennings Rj, Damen GW, Snik AF, Hoefsloot L, Cremers CW, Mylanus EA. 2003 ; 22: 451 -456
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1 1 3 : 21 35-2140 patient with mitochondrial hearing loss and m.625G > A transition. j Laryngol
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1 980; 89: 5-7 serine protease (TMPRSS3) mutated in deafness DFNBS/10 activates the
(26] Nadol jB, Young YS, Glynn Rj. Survival of spiral ganglion cells in profound sen epithelial sodium channel ( ENaC) in vitro. Hum Mol Genet 2002; I I : 2829-
sorineural hearing loss: implications for cochlear implantation. Ann Otol Rhi 2836
no! Laryngol 1 989; 98: 4 1 1 -416 [51 ] Weegerink NJ, Schraders M, Oostrik j et a!. Genotype-phenotype correlation
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Hear 2003 ; 24: 3 1 4-323 201 1 ; 1 2 : 753-766
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after cochlear implantation in DFNBI patients. Otolaryngol Head Neck Surg hearing loss ( DFNB8): compound heterozygosity for two novel TMPRSS3
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(55( Song MH, Cho Hj, Lee HK et al. CHD7 mutational analysis and clinical consid (63 ] Edvardson S, jalas C, Shaag A et al. A deleterious mutation in the LOXHDl
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Otolaryngol Head Neck Surg 2006; 1 3 2 : 495-500 deafness. Laryngoscope 2006; 1 1 6: 221 1 -2215

18
6 . 6 6 6 .
I I ' I I

3 Conseq uences of Deafn ess a n d El ectrica l Sti m u l ation o n


t h e Peri phera l a n d Centra l Aud ito ry Syste m
james B. Fallon, David K. Ryugo, and Robert K. Shepherd

in the central auditory system have been inconsistent, probably


Introduction
because of the high rates of spontaneous discharges in auditory
Cochlear implants provide important auditory cues necessary for nerve fibers, which are not affected by this manipulation.1 Even
auditory awareness and speech perception in severe to with extreme plugging strategies, the effects can be relatively
profoundly deaf subjects. Over the past three decades more than small and show signs of recovery.2 -4
220,000 adults and children worldwide have received these In contrast, surgical ablation of the cochleas is perhaps the
devices. Clinical experience has shown, however, a large variabil most invasive manipulation and results in a profound SNHL.
ity in outcome among implant users. Factors predicting a The central effects of such a dramatic intervention are depen
successful clinical outcome reflect the importance of auditory dent on the timing (i.e., before or after the closure of the critical
experience-either before an acquired hearing loss or with use periods). with early ablation resulting in cell loss, whereas
of a cochlear implant. Moreover, deaf children, with little or no later ablation results in mostly atrophic changes (see following
prior auditory experience, can obtain significant benefit from sections for more details).
a cochlear implant provided their device is fitted at a young age. At one time, acoustic trauma (or auditory overstimulation)
This clinical experience suggests that such a response can be at caused by an intense sound, was thought to produce localized
least partially attributed to plasticity within the auditory system. cochlear lesions and hearing deficits that were specific to the
This chapter reviews the response of the auditory system to spectral composition of the noise.56 Following acoustic trauma,
both deafness and its reactivation through a cochlear implant, the pattern of hair cell loss correlated with the traumatizing
and includes experimental data from animal models as well stimulus and was used to map cochlear frequency representa
as human material where applicable. Understanding the com tion.7 Other work, however, argued that there was little relation
plexities of this response will help provide a substrate for of hair cell loss to traumatic stimulation; rather, the damage to
understanding the clinical variability evident among cochlear the stereocilia of hair cells was the causal event.89 Although the
implant users. precise details of the damage differed, light and electron micro
scopic analyses suggested that stereocilia damage in the region
of the cuticular plate coincided with the hearing loss. Other
Models of Deafness
details of acoustic trauma raised concerns about result reliabil
In the auditory system of mammals, there is a period during ity because the degree of damage to the inner ear has been
which the normal development of speech and hearing is related to preexposure conditions, strain genetics, and stress
adversely affected by sound deprivation. Following a period of levels.w.n Together, these reports emphasize the complications
such deprivation, the resulting pathologies cannot be remedied surrounding noise trauma as a tool for interpreting mecha
even if sound is fully restored. The nature of this epoch, called nisms of noise-induced deafness in the auditory system.
the "critical period," is conceptually similar across species but An alternative form of deafening is provided by administra
undoubtedly varies in timing and magnitude of effect. Interven tion of ototoxic drugs. These drugs (e.g., amikacin, neomycin,
tion strategies in the case of pediatric patients with a profound kanamycin) are known to cause a loss of hair cell receptors
sensorineural hearing loss (SNHL) have concentrated on and deafness,12-15 thus, as described below, ensuring loss of
cochlear implants, and there is a growing tendency to implant spiral ganglion neurons ( SGNs).16 Ototoxic drugs can also be
deaf children at earlier and earlier ages. It seems necessary, used to produce a partial hearing loss in either neonataP7 or
however, to acquire better knowledge of those factors affecting adult18 animals, with lesions of the cochlea generally proceed
the structure and function of the auditory system when ing in a high-to-low frequency manner with frequencies below
electrical stimulation is used. the lesion typically exhibiting normal thresholds.19
In an effort to understand the influence of acoustic stimula Genetic models of deafness, particularly the mouse20 and the
tion on the developing brain, there have been experimental congenitally deaf white cat21-29 (DWC) represent a fourth alter
manipulations on the end organs to deprive the system of native for these kinds of studies. owes have long been of inter
input. Deafening has been produced by surgical or pharmaco est because an autosomal dominant gene links coat color, eye
logical manipulations of the auditory periphery, by noise pigment, and deafness.JD-34 This well-documented syndrome is
trauma, and by congenital or hereditary defects. Each form a variably penetrant and variably expressive syndrome affecting
of deafness is accompanied by its own potential complication. several characteristics including cochlear pathology3s that was
One must be vigilant for indirect surgical trauma, nonspecific first described in congenitally deaf patients.3637 The pathology
drug effects, inconsistent noise damage, and downstream in the DWC ranges from a slightly outward bulge of Reissner's
genetic influences. membrane to moderate hair cell loss to complete collapse of
The least invasive manipulation on the auditory system is the organ of Corti with corresponding degrees of hearing
produced by the introduction of a conductive hearing loss. loss.22,23,26,38,39 For those animals with a profound SNHL, deaf
This method basically involves the "plugging" of the ear canal ness is manifest by the collapse of Reissner's membrane onto
with a malleable substance or removing one of the ossicles. the undifferentiated organ of Corti, thinning of the stria vascu
Ear plugging results in little change in the cochlea, and changes laris, and malformation of the tectorial membrane.

19
I I ' I I

Fig. 3 . 1 a - c Schematic diagra m of a single cochlear turn illustrating the degenerative changes that occur following loss of the organ of Corti . (a)
Normal cochlea, illustrating the th ree fluid fi lled chambers (scala tympani [ST] ; scala media [SM]; and scala vestibuli [SV] ) . the organ of Corti
containing the sensory hair cells (arrowhead), and Rosenthal's ca nal (RC) containing the spiral gang lion neuron (SGN) soma. (b) The most common
forms of SNHL target the hair cells of the organ of Corti, eventually resu lting in the degeneration of the organ of Corti. Hair cell loss initially induces
loss of SGN peripheral processes (arrow) that wou ld normally innervate the organ of Corti. (c) More gradual degenerative changes following hair cell
loss incl udes extensive loss of peripheral processes, gradual and ongoing loss of SGNs, and significant shrinkage of surviving SGNs. This is typically the
status of the cochleas receiving a cochlear implant. (From Shepherd RK, Wise AK, Fa l lon JB. Cochlear implants. In: Celesia G , ed. Handbook of Clinical
Neu rophysiology, vol . 1 0: Disorders of Peripheral and Central Auditory Processing. New York: Elsevier, in press. Reprinted by permission.)

the sensory epithelium and the support cells of the organ of


The Cochlea
Corti. First. there is a rapid and extensive loss of the
Pathophysiology Following Deafness unmyelinated peripheral processes within the organ of Corti.43
This process is followed by a more gradual degeneration of the
Spiral ganglion neurons, whose cell bodies are located within myelinated portion of the peripheral processes within the osse
Rosenthal's canal (11> Fig. 3.1 ), are the target neurons for cochlear ous spiral lamina and of the cell bodies within Rosenthal's canal
implants. These devices electrically depolarize local populations (11> Fig. 3.1 ).14.44.45 The degenerative process also results in
of SGNs, initiating the generation of action potentials via demyelination of residual SGN soma and possibly part of their
an electrode array typically located in the scala tympani central processes.14-44.46 Finally, the perikaryon of residual SGNs
(a description of the initiation of an action potential via an elec undergoes considerable shrinkage,47 -49 a response that is
trical stimulus is provided elsewhere40 ). It is important to repeated throughout the central auditory pathway following
emphasize that once the action potential has been initiated, an SNHL (see below), although the functional effects of this
the implant plays no further role in its propagation along the atrophy are unknown.
ascending auditory pathway; saltatory conduction along the The secondary degeneration of SGNs following loss of the
central process of the SGN and its passage across synapses organ of Corti is an ongoing process, eventually resulting in
within the cochlear nucleus (CN) or higher auditory centers are very small numbers of surviving neurons ( 11> Fig. 3.2).14.45 As we
carried out via normal physiological processes. will describe below, these pathological changes affect the physi
Survival of these neurons is dependent on the integrity of the ological response of SGNs to an electrical stimulus. The ongoing
organ of Corti ; SGNs are subject to atrophic and degenerative degeneration of SGNs has been widely reported across mamma
changes that occur secondary to an SNHL Although the mini lian species following various etiologies that target the organ of
mum number of SGNs required to achieve acceptable clinical Corti: mouse50; rat51,52; chinchilla53; guinea pig54; and cat.14.23.26,
performance with a cochlear implant is unclear, the greater 45.55-57 It is important to emphasize that the rate of SGN degener
number of viable SGNs available for stimulation is likely to ation exhibits considerable variation across species-a theme we
result in improved clinical performance.41.42 Here. we will dis shall return to when discussing SGN loss in humans.
cuss the pathophysiological changes that occur to the cochlea In the majority of forms of SNHL, SGN degeneration is sec
following SNHL and describe techniques that may lead to SGN ondary to the loss of hair cells and support cells of the organ of
preservation. Corti.5 S -so These changes appear due. at least in part, to the
withdrawal of neurotrophic factors normally expressed by the
hair cells61 -64 and nerve growth factors released by support
Cochlear Pathology cells.s5 This pathway of SGN loss is common to many forms of
Hair cells of the organ of Corti are sensitive to many forms of SNHL described clinically, and would be expected to result in a
pathological damage including acoustic trauma, ototoxic drugs, gradual but ongoing secondary degeneration of SGNs as
congenital abnormalities, and aging. Unlike avian hair cells, reported above. Importantly, etiologies that directly target
mammalian hair cells cannot spontaneously regenerate; loss of SGNs, including viral and bacterial labyrinthitis. mechanical
hair cells results in a permanent SNHL. A number of atrophic trauma, and disruption to cochlear vasculature, are likely to
and pathological changes occur to SGNs following the loss of result in a more rapid and extensive loss of auditory neurons.

20
6 . 6 6 6 .
I I ' I I

1 000
Fig. 3.2 G raph illustrating the rate of loss of SGNs in
the upper basal turn of the cat cochlea as a fu nction
of duration of deafness. Although there is relatively
wide individual variabil ity in the data, there is a clear
800
red uction in SGN survival with increased d u rations of
deafness. Similar degenerative patterns a re observed
"" in other mammals a l beit at different rates. Although
E
E rodents such as gu inea pigs exhibit a more rapid loss
- 600
of SGNs compared with cats, it is im portant to
Qi
emphasize that ganglion cell loss is significantly

slower in humans. These data a re based on cou nts
v;
from 48 cat coch leas including 1 6 normal hearing
c:
Ql
a
400 .a controls. Error bar 1 standard error of the mean
u (SEM).
lJ
Vl


200

:I



0 400 800 1 200


D uratio n of D eafness ( Days)

Reports of degeneration patterns of SGNs in human cochleas throughout the cochleas.7 These results provide encouraging
following a profound SNHL are generally consistent with the findings for pediatric cochlear implantation, and emphasize the
experimental studies already summarized. Degeneration of relatively slow rate of SGN degeneration evident in humans
peripheral processes is more extensive than that of the SGN compared with experimental animals. This situation may
somata or central processes, and the extent of the pathology reflect, at least in part, the lack of a long-term decrease in
typically varies with distance along the cochlear partition in a clinical performance with device use.71
manner that reflects the extent of damage to the organ of Corti
-being most extensive in the basal turn with Jess extensive Physiological Response of the Spiral Ganglion
pathology apicalward.42,ss.s9.GG- G9
Analysis of SGN survival from profoundly deaf adult tem
Neurons to Deafness
poral bones reveals a moderate to severe Joss of auditory Despite the extensive pathological changes that occur to SGNs
neurons. Otte and colleagues68 studied 62 profoundly deaf following an SNHL, these neurons remain capable of initiating
ears and demonstrated that 45% of cochleas had a SGN popu and propagating action potentials elicited via an electrical stim
lation in excess of 1 0,000, that is, approximately one third of ulus, even in cochleas deafened for many years, with surviving
normal (28,4 1 8 3,675). Nadal and colleagues42 studied 66 neural populations of< 5% of normal.45 In general, the basic
profoundly deaf ears and showed that the mean SGN popula response properties of SGNs in deafened cochleas remain simi
tion was approximately half that of normal ears, although the lar to those observed in normal cochleas-that is, the cells show
standard deviation, as in all studies of this sort, was large an increase in the probability of firing and a decrease in both
( 1 4,061 8,063 ). Total SGN Joss tended to be greater in older response latency and the jitter in the timing of the response
compared with younger subjects and, consistent with animal with increasing stimulus current. There are, however, more
studies, greater Joss was observed with longer durations of subtle changes seen in neural response properties in cochleae
deafness. However, the single most significant determinant of subjected to long periods of deafness ; these pathology-induced
SGN loss in humans was etiology,4267 with loss most exten changes have the potential to degrade the perceptual quality of
sive in patients with postnatal viral labyrinthitis, congenital cochlear implants. First, Joss of peripheral process and ongoing
or genetic deafness, or bacterial meningitis-that is, patholo Joss of SGNs results in an increase in threshold.5272-74 This will
gies that directly target SGNs or patients having long dura likely have an adverse effect on power consumption and result
tions of deafness. Patients deafened with aminoglycoside in a reduction in spatial selectivity of the electrode array.75 Sec
antibiotics or sudden idiopathic deafness typically exhibited ond, demyelination results in an increase in membrane capaci
the smallest levels of SGN pathology. tance,7677 reducing the efficiency of a neuron in initiating and
Although many of the findings observed in adult temporal propagating action potentials in response to electrical stimuli
bones have also been reported in temporal bones from pro and a decrease in facilitation.78 In the auditory system, there is
foundly deaf children, two important differences were noted: evidence of reduced temporal resolution in myelin deficient or
( 1) the SGN population in children did not show evidence of deafened cochleas,79 as well as a significant increase in the
ongoing Joss with duration of deafness within the age range of refractory properties of auditory nerve fibers 52 and evidence of
0 to 9 years, and (2) there was a more even distribution of SGNs conduction blockP

21
I I ' I I

01
/lPG it is clear that the combination of electric and acoustic hearing
provides important additional auditory cues for use in speech
comprehension. These clinical findings have led to considerable
interest in the development of strategies to protect hair cells
following cochlear implantation. Provided the cochlea does not
-
produce an adverse inflammatory response due, for example, to
infection, trauma, or the use of nonbiocompatible materials,
experimental studies using small-diameter "free-fit" electrode
arrays typically show good long-term hair cell survival follow
ing cochlear implantation.96 - 1DO This hair cell survival is almost
always restricted to that part of the cochlea apical to the elec
trode array. In approximately 30% of chronically implanted
hearing animals, widespread hair cell loss was observed, which
was almost always associated with an extensive inflammatory
T ime response within the implanted cochlea.96,99,1D1 Therapies
designed to minimize the inflammatory response following
cochlear implantation need to be developed in order to increase
Fig. 3.3 Schematic diagra m ill ustrating the charge-balanced biphasic
cu rrent pu lses typica lly used in cochlear implants. Charge in the the probability of preserving hearing. Finally, chronic electrical
second phase (02) is equal in magnitude but opposite in polarity to stimulation using charge-balanced biphasic current pulses at
the charge in the first phase (01 ). The duration of each phase intensities that are typically used clinically shows no statistical

50 to 200 s/phase for the less efficient bipolar stimu lation.


interphase gap.
lPG,
typically varies from 10 to 50 sf phase for monopolar stimulation and evidence of a functional decrement in hearing ( measured via
click-evoked auditory brainstem responses ) when compared
with the contralateral control ear.99,1D2

Trophic Effects of Electrical Stimulation on


Effects of Electrical Stimulation
Spiral Ganglion Neuron Survival
Safety Issues In deafness, there is a lack of driven neural activity and signifi
Nondamaging electrical stimulation typically consists of short cant reductions in the levels of spontaneous activity,26.57.73.103
duration ( < 200 J.IS/phase) charge-balanced biphasic current such that SGNs rarely undergo depolarization. However, neural
pulses ( Fig. 3.3) delivered using platinum electrodes and activity is considered to play an important role in SGN survival;
operating at charge densities of< 60 J.IC/cm2 geom. per depolarization is sufficient to maintain neuronal survival in
phase.8D-86 These stimulus guidelines ensure that the charge vitro without the addition of neurotrophic factors.104 Mem
injection process is achieved using reversible electrochemical brane depolarization appears to promote SGN survival by ele
reactions localized to the electrode-tissue interface, minimizing vating intracellular calcium levels and cascading a number of
the chance of releasing potentially harmful electrochemical intracellular signaling pathways.1 04 Importantly, the trophic
products into the tissue environment.87-89 Contemporary support of SGNs via depolarization in vitro appears to be addi
cochlear implants operating in a monopolar electrode configu tive with the actions of some neurotrophins.10s
ration would typically produce charge densities of an order of A number of in vivo studies have described significant
magnitude below these levels. increases in SGN survival, to levels of up to 70% greater than
Although theoretically charge-balanced biphasic current nonstimulated control cochleas. in ototoxically deafened ani
pulses should not result in the production of potentially damag mals following chronic electrical stimulation of the auditory
ing direct current, in practice it is not possible to generate nerve.106- 114 However, this is not a universal finding; work from
perfectly balanced stimuli. Neural damage and new bone for other laboratories has reported no such trophic influence. 8082,
mation is observed following chronic stimulation with direct 1 1 5 - 1 1 8 There is general agreement, however, that chronic
current levels greater than 0.4 J.IA.9091 Protection against direct electrical stimulation in deafened ears results in subtle mor
current, and the local pH changes that occur as a result of phological changes to SGNs. For example, a number of studies
charge imbalance, can be achieved by either shorting electrodes have reported a small but significant increase in the soma area
between current pulses or by placing a capacitor in series with of SGNs in deafened and chronically stimulated cochleas com
each electrode.92.93 One or a combination of these techniques is pared with deafened controls.80114 This increase in soma area
used to ensure complete charge recovery in contemporary presumably reflects an increase in biosynthetic activity within
cochlear implant systems. the SGN soma following reactivation via an electrical stimulus.

Effects of Cochlear Implantation and Electrical Therapeutic Support of Spiral Ganglion


Stimulation on Hair Cell Survival Neurons
The clinical performance of cochlear implant patients with
some level of residual hearing is typically significantly greater
Controlling Inflammation
than that of profoundly deaf implant recipients.94.9S Although The implantation of an electrode array will evoke a host
there are a number of factors contributing to this improvement, response designed to externalize any foreign body. The extent

22
6 . 6 6 6 .
I I ' I I

Fig. 3.4 a -f Cochlear histological sections from the


basal turn of guinea pigs. (a) An intact organ of Corti
in a normal cochlea (solid arrow) compared with a
degenerated organ of Corti in a chronically deaf
cochlea (open arrow). (b -f) Representative images of
Rosenthal's canal [e.g . , dashed line in (c) from the left
(treated) cochlea] for (b) normal; (c) deafened
unstimulated (US) cochlea treated with artificial
perilymph (AP); (d) deafened unstimulated (US)
cochlea treated with BDNF; (e) deafened stimulated
(ES) cochlea treated with artificial perilymph (AP); and
(f) deafened stimulated (ES) cochlea treated with
BDNF. SGN somata appear smaller and the packing
density lower in AP cochleas compared with NT
treated cochleas and normal controls. Higher magni
fication images of SGN somata from different
cochleas a re shown in the insets. Morphological
degeneration of the somata was appa rent in AP
animals, unlike NT-treated cochleas. (From Landry TG,
Wise AK, Fallon JB, Shepherd RK. Spiral ganglion
neuron su rvival and function in the deafened cochlea
following chronic neurotrophic treatment. Hear Res
201 1 ;282:303 - 3 1 3.)

of this reaction will depend on many factors including the ( NT-3 ),61 .62.123 with receptors for both of these neurotrophins
extent of any surgical trauma, materials biocompatibility, and expressed on SGNs.6162 Moreover, survival of SGNs in the
the mechanical characteristics of the implant relative to the absence of the organ of Corti has been promoted by exogenous
host environment. The nature of the tissue response will also BDNF or NT-3 in vivo.5 1.54.124-126
vary over time as acute edema and the proliferation of reactive This research has been extended to demonstrate enhanced
inflammatory cells are gradually replaced by a mature tissue SGN survival in deafened cochleas treated with both exoge
capsule around the implant.1 1 9 Minimizing these reactions at nous neurotrophins and electrical stimulation (11> Fig. 3.4).1 1 0
the electrode-tissue interface is particularly important as an 127-130 Importantly, this work has also shown a functional
extensive foreign body reaction and neural loss could signifi advantage, in the form of significantly reduced electrically
cantly affect the performance of a cochlear implant. 120 Gluco evoked auditory brainstem response thresholds in ears
corticoid steroids such as dexamethasone have been used to treated with neurotrophins,127-1 28-130-131 which may be associ
minimize the tissue response following cochlear implantation, ated with a neurotrophin-mediated growth of SGN peripheral
although their efficacy to date has not been clearly demon processes toward the scala tympani.126-129,!30,I32 Reductions
strated.121 Further research in this area is clearly warranted. in electrical thresholds would reduce the power consumption
of a cochlear implant, thereby providing a number of engi
neering advantages including the possibility of smaller, more
Rescusing Spiral Ganglion Neurons numerous electrode contacts.
A number of growth factor families have been shown to play These results are promising, but from a clinical perspective
important roles in the development of, maintenance of, and further research is necessary before neurotrophins can be
protection against injury of SGNs.122 Presynaptic hair cells, combined with cochlear implants. Although neurotrophin
supporting cells, and postsynaptic neurons within the CN delivery has been used clinically to treat a number of neuro
are necessary for SGN survival, reflecting complementary logic disorders, 133 and preclinical studies have shown no signif
neurotrophic support. These neurotrophins include both icant adverse effects of long-term neurotrophin delivery into
brain-derived neurotrophic factor (BDNF) and neurotrophin-3 the inner ear, there remain a number of issues to be addressed.

23
I I ' I I

First, there is evidence of increased tissue response within the


cochlea following exogenous neurotrophin delivery.128 The
extent and implications of this inflammatory response requires
further study. Second, there is evidence of small changes to the
morphology of SGNs exposed to exogenous neurotrophin deliv
ery127130134; the long-term implication of these changes needs
Auditory Cortex
to be explored. Finally, although neurotrophin therapy has been
shown to protect SGNs following an insult to the organ of Corti,
it is not clear whether neurotrophins would be effective in
etiologies that directly target SGNs such as mechanical trauma
or infection.

Drug Delivery Techniques


There is evidence that the removal of exogenous BDNF leads to Medial Genicul ate Nucleu

an accelerated loss of SGNs,m although chronic electrical


stimulation can reduce this loss.136 This finding implies that the
neurotrophin must be supplied over weeks and months rather
than hours and days. The most efficient route is delivery of the
neurotrophin directly into the inner ear. Direct delivery of a
drug into the perilymph will result in its distribution to most
structures within the cochlea including the organ of Corti, the
SGNs, the spiral ligament, and the spiral limbus; only the scala
media and stria vascularis are not accessible via this route due
to the presence of tight junctions.137 The use of large pump
delivery systems should be discouraged because of the risks of
introducing infection into the inner ear. Alternative delivery
methods using viral vectors,n o.138, l39 cell-based therapies,129,l40
slow-release nanotechnology-inspired applications,141 - 143 and
microfluidics-based drug delivery methods144 hold considera
ble promise as clinically viable delivery techniques. A number
of these technologies have the potential to deliver a wide
variety of therapeutic drugs in addition to neurotrophins. These
include antiinflammatory agents to minimize tissue response
following cochlear implantation, antioxidants for hair cell
protection,14S ,l46 neurotransmitters for tinnitus suppression,
monoclonal antibodies for autoimmune inner ear disease, and Fig. 3.5 Schematic diagram of the central auditory system ill ustrating
apoptosis inhibitors for hair cell rescue.147 the major auditory nuclei and the predominant ascending con nections.
M NTB, medical nucleus of the trapezoid body; MSO, medial su perior
olive; LSO, lateral su perior olive.
The Cochlear N ucleus
Auditory deprivation in the developing animal produces severe electrical stimulation of the cochlear on these cells and their
abnormalities in the central pathway ( Fig. 3.5), whereas older synapses differ and are discussed in detail below.
animals are less affected.25.148 - 153 Postmortem studies of those
rare human cases where the profound hearing loss of the
individual is documented have revealed up to 50% somatic
Pathophysiology Following Deafness
shrinkage in the CN, with acknowledgment that many variables As previously mentioned, the effects of the introduction of con
contribute to the nonuniform results.1 54- 157 Clinical observa ductive hearing loss have been inconsistent and relatively small,
tions related to congenital deafness suggest that the best and they show signs of recovery.2-4 However, a week of conduc
candidates for cochlear implants are very young children, and tive hearing loss can result in a change in the expression of exci
that with increasing age, outcomes become less optima[.lS S -160 tatory and inhibitory ionotropic receptors,161 which in turn
The implication is that sensory stimulation, whether natural or could contribute to abnormal processing of auditory signals.
prosthetic, is necessary during early life to ensure the normal In contrast, surgical ablation of the cochleas in neonatal ani
development of the central auditory system. mals results in approximately 50% ipsilateral volume loss,
The CN serves as the gateway to the central auditory system, 30 to 40% cell body shrinkage, and > 50% neural death in the
and the spherical and globular bushy cells represent two major CN.151.162.1 63 Importantly, the loss of central neurons following
cell classes in the ventral CN that have been well studied. cochlear ablation is restricted to a critical period that appears
Spherical bushy cells receive large axosomatic endings called to end at the onset of hearing.1 64165 Although deafferentation
end bbulbs of Held, whereas globular bushy cells received during cochlear development results in widespread neural loss
smaller end bulbs. The effects of long-term deafness and in the CN, similar cochlear damage after the onset of hearing

24
6 . 6 6 6 .
I I ' I I

results in atrophic changes but not neural loss. Given that most Likewise, there was no statistically significant difference in
forms of SNHL occur following the onset of hearing,17.1 66.1 67 nuclear volume between the ipsilateral and contralateral
these results imply that the major transneuronal effects of deaf sides. In a similar study using unilateral electrical stimulation
ness are atrophic with relatively small amounts of neural loss. of ototoxically deafened cats, the size of spherical bushy cells
Ototoxically induced SNHL results in a secondary SGN loss, was reduced 20% by deafening, but stimulation did not pro
which in turn causes a reduction in CN volume and distinct duce any difference between the ipsilateral and contralateral
cell shrinkage in cats, gerbils, and guinea pigs,45.168- 1 70 with the sides.1 78 In related studies, these same comparisons
effect on the ventral CN much greater compared to that on revealed a small (6%) but statistically significant increase in
the dorsal CN. In addition, ototoxically induced SNHL results somatic cross-sectional area between ipsilateral and contra
in a reduction in end-bulb branching, reduction of presynaptic lateral spherical bushy cells.168.179 There were also mixed
vesicles, and flattening and hypertrophy of postsynaptic results regarding differences in volumes of the cochlear nuclei
densities.171 and somatic sizes of various cell types in the stimulated and
The variable degree of SGN degeneration, and its ongoing nonstimulated sides. The most provocative results have been
nature, seen in the DWC,33,39 results in distinct atrophic that chronic electrical stimulation of the auditory nerve
features evident in the CN including a 50% reduction in nuclear restores the morphology of end-bulb synapses on spherical
volume and a differential shrinkage of separate cell types.21 ,2s. bushy cells in the anteroventral CN ( Fig. 3.6). 171,180,181
26.172.173 Furthermore, a hypertrophy of synapses is seen on The conclusion from these observations is that electrical
some but not all CN neurons.82 Similarly to ototoxic deafening, stimulation of the cochlea in deaf animals, regardless of the
congenital deafness results in a reduction in end-bulb branch cause of deafness, restores synaptic structure when the ani
ing, reduction of presynaptic vesicles, flattening and hypertro mal is treated at a young age. In short, the stimulation of
phy of postsynaptic densities, and loss of intermembranous young deaf cats restores the postsynaptic density of auditory
channels. Synapses on spherical bushy cells are severely nerve synapses to their characteristic dome shape and small
affected by an SNHL, whereas those on globular bushy cells are size, and returns synaptic vesicle density to levels consistent
less so. In contrast, synapses on multipolar cells do not appear with those of normal-hearing cats. Importantly, these
to be affected at all ( Fig. 3.6).25.28.1 74 animals show evidence that they can process sounds in a
The structural abnormalities in CN neurons in response to biologically relevant way.
SNHL seem a natural consequence of abnormal inputs from The issue of whether electrical stimulation of a deaf ear
the auditory nerve. Congenital deafness abolishes, and ototoxic has tangible benefits in addition to conveying information to
deafness significantly reduces, the levels of spontaneous activity the brain resides in whether stimulated cells are more
in auditory nerve fibers of the cat.26103 Variations in spontaneous robust than their unstimulated counterparts. Does it matter
activity in auditory nerve fibers have an effect on spherical whether neurons ipsilateral to the cochlear implant are
bushy cells even in normal-hearing cats.175 Deafness-induced larger, show higher survival rates, or are otherwise healthier
changes are expressed as increased thresholds to activation,52 in appearance? On the one hand, it would seem that appear
smaller action potentials, smaller after-hyperpolarizations, and ances do not signify much. Chen et a P 1 8 demonstrated that
shorter membrane time constants.176 the best results of electrical stimulation on synapses
occurred independent of these physical parameters. In con
trast, other researchers claim that stimulation produces a
Effects of Electrical Stimulation small increase in somatic size; however, there are several
The question naturally arises as to whether electrical stimula considerations that temper this interpretation. First, it is not
tion of the auditory nerve can effectively prevent, ameliorate, always clear that the cells sampled were those that were
or reverse the deleterious effects of deafness on the CN neurons. stimulated. Because the stimulating electrodes are placed in
Inherent to the interpretation of implantation and stimulation the basal (high-frequency) end of the cochlea, the sampling
results is a consideration that either or both manipulations must also occur in the high-frequency regions of the nuclei.
can contribute to changes additional to those caused by In several studies the sampling was from spherical busy cells
SNHL, and the nature of these changes is not necessarily in the rostral pole of the anteroventral CN, and this region
predictable. Nevertheless, given the notion of a "critical period" is known to be responsive primarily to low frequencies.182
in auditory system development, deafened animals are being Second, using the contralateral CN as a control may not be
used to develop experimental models. Furthermore, children, appropriate with evidence of strong commissural connec
especially prelingually deaf children, are postulated to receive tions between cochlear nuclei.183 - 187 Stimulation of the ipsi
additional benefit from early electrical stimulation via cochlear lateral CN by auditory nerve fibers undoubtedly affects
implants. The rationale is that the preservation of neurons and activity in the contralateral CN via the commissural projec
synapses represents the substrate for improved development tions as there are clear signs that there is a change toward
of language skills. normal in the structure of synapses.1 81 The bilateral effects
The results of electrical stimulation of the auditory nerve on the central auditory system from stimulation by a single
on CN structure have not been uniform. In deafened guinea unilateral cochlear implant have major relevance to those
pigs, chronic electrical stimulation of one cochlea resulted in considering bilateral cochlear implants (a review of bilateral
an increase in the size of octopus cells in the ipsilateral CN cochlear implants is available elsewhere188). If such change
compared with those on the contralateral side.177 If specific reflects a partial preservation of the contralateral system,
cell types were not considered, analysis of all neurons then the idea of adding a second cochlear implant sometime
suggested that there was no effect of electrical stimulation. after the first becomes more tractable.189

25
I I ' I I

S BC fro m No r m a l Cat SBC fro m Cong enita lly Deaf Cat

20 pm

Cross Sec t i o n
T hroug h Term i n a I

0 .2 5 p m PSD PSD
K itten K itten

20 pm

PSD PSD
0.2 5 pm
-
Fig. 3.6 Summary diagram illustrating end-bulb plasticity under conditions of normal hearing, congenital deafness, and congenital deafness with
coch lear impla ntation. Deafness resu lts in a red uction of the end-bu l b a rborization. The end-bulb synapses of deaf animals exhibit hypertrophic
postsynaptic densities { PSDs), lose their dome shape, and increase synaptic vesicle density. Electrical stim u lation through a coch lear implant in young
but not older cats restores syna ptic morphology. Syna pses regain their small size, dome shape, punctate distribution, and normal synaptic vesicle
density. The end bulb itself does not fully regain its highly branched arborization, nor do the swellings return to the small size typical of those in
hearing cats. SBC, spherical bushy cells. (Modified from Redd EE, Pongsta porn T, Ryugo DK. The effects of congenital deafness on aud itory nerve
syna pses and globular bushy cells in cats. Hear Res 2000; 1 47 : 1 60 - 1 74; O'Neil J N , Limb CJ, Baker CA. Ryugo DK. Bilatera l effects of unilatera l cochlear
implantation in congenita l ly deaf cats. j Comp Neurol 201 0;5 1 8( 1 2):2382 - 2404; and Shepherd RK, Meltzer NE, Fa llon JB, Ryugo DK. Conseq uences of
deafness and electrical stim u lation on the peripheral and central aud itory system. In: Wa ltzman SB, Roland JT, eds. Cochlear Implants, 2nd ed. New
York: Thieme, 2006:25 - 39.)

What emerges from these studies is the conclusion that


congenital deafness, regardless of its cause, results in trans
Higher Auditory Centers
neuronal changes that are expressed at the level of the CN. We The variable synaptic abnormalities within the CN in
shall now examine deafness-induced effects at higher centers response to an SNHL, summarized in Fig. 3.6, may contrib
of the auditory system. Ascending projections out of the CN ute to differential transmission delays along the central
exhibit abnormal pathways163,J90- J92 that impact on the struc pathways ( Fig. 3.5), leading to corrupted temporal process
ture of the target brainstem nuclei, including the superior ing of auditory information initiated by cochlear implants.
olivary nuclei21 24193 and inferior colliculus.194- 197 These wide Changes within the CN could obviously have widespread
ranging abnormalities are implicated as part of the explanation effects because this structure serves as the gateway to
for the variability in clinical outcomes with cochlear implants. the central auditory system. As one ascends the auditory

26
6 . 6 6 6 .
I I ' I I

pathway, it seems that transneuronal changes can occur at cats, though not as large as in the hearing cats, indicating a
all levels. partial recovery of excitatory inputs to MSO dendrite excitation
after stimulation. These results exemplify dynamic plasticity in
the auditory brainstem and reveal that neural activity evoked
The Superior Olivary Complex via electrical stimulation has a restorative effect on synaptic
The deleterious effects of deafness evident within the CN are organization in the MSO.
also seen in more central structures. In the DWC, microscopic
analysis of the lateral and medial superior olive and the medial
nucleus of the trapezoid body demonstrated that neural somata The Inferior Colliculus
and nuclei areas were 33 to 50% smaller in deaf compared to In the midbrain, the central nucleus of the inferior colliculus
hearing cats.21 The sizes of cerebellar Purkinje cells were not ( ICC) represents a key structure in the central auditory path
affected by an SNHL. There was also a 35% decrease in the way. It is tonotopically organized and receives strong excitatory
number of synapses per unit somatic length in these nuclei input from the contralateral ear, although most ICC neurons are
in DWCs.24 Analysis of the medial superior olive from deaf binaurally influenced. Inputs to the ICC arise bilaterally from
humans suggests a 20 to 30% reduction in neural soma area the superior olivary complex via the lateral lemniscus ( decus
for the denervated structure.157 The specificity for this effect of sating fibers represent the major pathway) and contralaterally
deafness was indicated by the size of cerebellar Purkinje cells, from the dorsal and ventral cochlear nuclei.2J2 ,213
which was not affected by SNHL.
The medial superior olive ( MSO) has been considered a "coin
cidence detector," and is implicated in processing interaural Pathophysiology Following Deafness
time differences ( ITDs) used for low-frequency sound localiza Following an SNHL, significant structural abnormalities are
tion. The principal neurons of the MSO have their cell bodies observed in unilaterally deaf young animals when compared
aligned in an elongated column with two bilaterally extending with both normal-hearing and bilaterally deafened animals.
dendrites19B and receive bilateral excitatory inputs from the Such studies reveal an increase in the afferent projection from
spherical bushy cells of the anteroventral cochlear nucleus the normal CN to the ipsilaterai !CC.19D,J9J,2J4 In contrast, bilat
(AVCN).199 -2D3 The inputs from the left AVCN terminate on the erally deaf animals show symmetry of the ascending projection
left-facing dendrites, whereas those from the right AVCN termi that is similar to that in normal hearing animals.27,2JS It would
nate on the right-facing dendrites. Spherical bushy cells are spe appear that these changes are driven by asymmetrical input to
cialized for preserving timing features of acoustic stimuli.2D2.2D4 the central auditory system as a result of unilateral deafness.
As a result, MSO cells are especially suited to compare the timing It is worth noting that the great majority of cochlear implant
of ipsi- and contralateral excitatory inputs. The MSO cell bodies subjects also receive a strongly asymmetrical input via a single
receive inhibitory input from the medial and lateral nuclei of the cochlear implant.
trapezoid body ( MNTB, LNTB), which integrates with the den No difference was found between hearing and unilaterally
dritic excitation to provide lTD sensitivity.2DJ.2DS-2D7 deaf animals when studying ICC ultrastructure after 1 year of
Studies of MSO neurons have revealed spatial refinement of chemically induced deafness, although in bilaterally deaf cats
synaptic inputs during development. In gerbil brainstem slices, there was a reduction in the number of synapses compared
the effect of y-aminobutyric acid B [ GABA(B)] receptors in with partial-hearing or normal-hearing cats.194 A small but sta
young animals is stronger for excitatory compared to inhibitory tistically significant reduction in neuronal soma area may
transmission in the principal cells, but in mature animals GABA be present in bilaterally but not unilaterally deaf cats.196
(B) receptors mainly control inhibition. During this same devel
opmental period there is a shift in location of these receptors
from dendrites to cell body.2 o s This refinement fails to develop
Effects of Electrical Stimulation
in mammals that do not use ITDs for sound localization; deaf In patients who had some exposure to electrical stimulation via
ness disrupts these spatially segregated excitatory and inhibi a cochlear implant, the range of neuronal soma size in the ICC
tory input patterns.2D7 Deafness produces abnormalities was found to be as much as 30% smaller than norma1.157 Small
in structure and function throughout the central auditory study size, sampling bias, and laterality of stimulation may have
system,21 .24-26.s6.2D9.21D so one obvious question is whether the obscured trophic effects if they were present, because all cell
pathology can be reversed by the introduction of stimulation area averages were smaller than in normal-hearing controls.
via a cochlear implant. Cell soma area was smaller in cases of cochlear pathology than
The axosomatic and axodendritic patterns of synaptic organi in cases of auditory nerve sectioning. This finding was postu
zation were determined for principal neurons from the MSO of lated to be due to a dystrophic effect on neurons from a dis
hearing cats, deaf cats, and deaf cats with cochlear implants.2 1 1 eased cochlea, rather than the loss of trophic activity. This
Results show that the proportion of inhibitory axosomatic inference may have implications for models of deafness based
terminals was significantly smaller in deaf animals when com on cochlear abnormalities. Alternatively, neuronal soma size
pared to hearing animals. However, after a period of electrical may not be a relevant (or is at best a crude) parameter in
stimulation via a cochlear implant, the proportion of inhibitory evaluating central nervous system changes due to deafness and
inputs returned to that of hearing animals. Additionally, the electrical stimulation.
excitatory axodendritic boutons of hearing cats were found to Metabolic markers such as 2-deoxyglucose (2-DG) have
be significantly larger than those of deaf cats. Boutons of stimu been used to study the extent of neural activation in the ICC
lated cats were also significantly larger than the boutons in deaf in response to electrical stimulation of the auditory nerve.

27
I I ' I I

Brown et aF16 compared the effects of electrical vs. auditory ied. Data are collected by presenting different electrical stimuli
stimulation on 2-DG uptake in cats. Using acute, unilaterally to deaf implanted animals, and modifying the duration of deaf
deafened animals, Brown et al found that 2-DG uptake evoked ness, the duration of electrical stimulation, or the stimulus
from the deafened, electrically stimulated cochlea resembled characteristics. Evidence suggests that early-onset deafness fol
that seen in the acoustically stimulated contralateral ICC. The lowed by chronic electrical stimulation of the auditory nerve
location and extent of 2-DG uptake was dependent on the elec may enhance the capacity of the ICC to encode high pulse rates,
trode configuration, electrode location, and stimulus intensity, beyond those seen in normal-hearing animals.223 In addition,
although any increase in 2-DG uptake may also reflect an addi there is a maximum electrical pulse rate that may be encoded
tive component arising from the ipsilateral auditory stimula by the ICC. findings that have implications for stimulus parame
tion. In a different set of experiments, bilaterally deafened ters in the application of cochlear implants.195 ,223 Neurons in
guinea pigs did not exhibit significantly reduced 2-DG activity the ICC also appear to have higher temporal resolution when
in the ICC, and remained responsive to acute electrical stimula stimulated using amplitude-modulated electrical pulses com
tion after 15 months of deafness.217 Schwartz et at218 similarly pared with unmodulated stimuJi,224 Most ICC neurons are able
observed normal 2-DG uptake in electrically stimulated deaf to encode frequency-modulated stimuli up to 40 Hz. The carrier
guinea pigs. The 2-DG labeling equaled but did not surpass pulse rate eliciting response of neurons in the ICC was 1 04
levels observed in acoustically stimulated normal-hearing con pulses per second in unmodulated stimuli. By amplitude
trols. This effect was seen at increasing levels of stimulation modulating the stimuli, carrier pulse rate could exceed 600
and with chronic stimulation. Collectively these studies suggest pulses per second. Distortion of the neural response was
that in the unstimulated state. basic pathways for auditory observed as the ratio of modulation to carrier rate approached
information to the ICC in deafened animals remain largely 1 /4 to 1 /6. This observation may be significant, because cochlear
functionally and structurally intact, indicating a powerful role implants can exceed these parameters. Following chronic stimu
for genetic programming of auditory connections. lation at high rates, the ICC seems able to increase its average
Single-cell activity evoked by electrical stimulation of the maximum following-frequency.22s High stimulation rates do not
auditory nerve in neonatally deafened cats has revealed that appear to impact the peak maximum following-frequency.
major physiological features of the ICC are preserved over wide Increased latency and jitter of single unit responses were
durations of deafness.195 The spatial tuning of the ICC studied seen in long-term deaf. acutely stimulated cats.195 One- to two
in these acutely stimulated cats revealed that long-term deaf year-old congenitally deaf white cats exhibited significantly less
ness reduced the distance between basal and apical electrode sensitivity to interaural time differences when bilaterally stim
neural representation in the ICC compared to that seen in cats ulated with electrical pulses. The neurons of the ICC had
with more auditory experience. roughly half as many ITO-sensitive neurons as compared to
Experiments using neonatally deafened, chronically electri deafened cats with previously normal hearing_226 ICC neurons
cally stimulated cats have also been performed. Snyder of congenitally deaf animals exhibited much greater levels of
et aF19 observed an increase in the ICC neural representation spontaneous activity, which might reflect a "release of inhibi
of the electrically stimulated sectors of auditory nerve. Corre tion" due to the diminished proportion of inhibitory inputs to
lating ICC recording depth with neural activity demonstrated MSO neurons.21 1 Chronically stimulating deaf cats at high rates
a significant increase in neural representation of that sector reduced the median latency below that seen in normal-hearing
of auditory nerve subject to chronic stimulation. Moreover, cats225 and it seems reasonable to predict that chronic stimula
chronic electrical stimulation of the auditory nerve can tion could restore more functional parameters in the ICC.
induce a similar degree of plastic expansion in the ICC of Overall, the ICC of deaf animals seems to retain many of the
adult-deafened implanted cats.220 More recently, ICC record basic neural properties exhibited by normal-hearing animals
ings using multielectrode arrays have been implemented.22J but they are degraded by deafness. Chronic electrical stimula
When applied chronically, this technique promises to provide tion may increase the temporal resolving capacity of the ICC.
a longitudinal view of neural activity across the ICC in and the significance of this speculation would be improved ITO
response to cochlear implantation. resolution and spatial hearing for bilateral cochlear implant use
Alternating stimulation of separate areas of the auditory in humans.
nerve may preserve, or perhaps even sharpen, ICC represen
tation of those stimulated sectors of auditory nerve.222 These
The Auditory Cortex
studies indicate that even in prolonged early-onset deafness.
basic cochleotopic and neural representation of electrical In 1 942 Woolsey and WalzF27 reported the first cortical
stimuli may be elicited in the ICC. This developmental fea responses to electrical stimulation of the auditory nerve in
ture, however, is clearly modifiable by sensory experience. normal-hearing cats. Subsequently, the primary auditory cortex
Neural plasticity of the ICC seems to expand the neural rep (AI) in the normal-hearing animal was shown to be cochleo
resentation of chronically stimulated sectors of auditory topically organized, and individual neurons within layer III/IV
nerve, even when those sectors receive stimulation long have well-characterized input-output functions for electrical
after the onset of deafness. stimulation and decreasing response latencies (to a minimum
The ability of neurons in the ICC to synchronize their spike around 8 ms) with increasing stimulus strength.228 - 23J
discharges to an auditory stimulus is referred to as temporal The changes in the auditory cortex after an SNHL are depen
response properties. These properties allow for the encoding of dent on many factors, including the severity of the SNHL,
sound features for speech recognition and word discrimination. whether the SNHL is unilateral or bilateral. and the developmen
Properties such as latency, jitter, and spike rate have been stud- tal stage at the time of the SNHL 232 A further complication in

28
6 . 6 6 6 .
I I ' I I

the interpretation of changes in the auditory cortex in response but basic response properties, including growth functions
to an SNHL is that many "downstream" changes occur as a result and latencies, were similar to those in normal-hearing cats.245
of the SNHL ( see previous sections), which effect the input, and Corroborating results of stable basic response properties have
the organization of that input, into the auditory cortex. At least been reported for ototoxically neonatally deafened cats.249.252
some of the changes that occur in the auditory cortex as a result Long-term SNHL, however, does reduce the temporal processing
of an SNHL are plastic, and the return of input to the auditory within Af,253.254 and is apparent as a delayed P1 latency in
cortex, via electrical stimulation of the auditory nerve, results in humans.255
the reorganization of the auditory cortex. It is the plastic nature Clearly, the AI does not develop entirely normally in the
of the auditory cortex that is likely to be an underlying factor in absence of sensory input; in fact, deafness induces variations in
the continued improvement in performance of cochlear implant the development of different layers within AI. Although the
patients with device use.m owe develops a rudimentary cochleotopic representation in
layers III/IV, there is a decrease in current sinks (and therefore
presumably synaptic currents) at long ( > 30 ms) latencies in lay
Morphological Changes ers II, Ill, and IV.210.2s6 The decrease in currents sinks is even
Following Deafness more evident in the deeper (infragranular) layers IV, V, and VI,
and in these deeper layers the decrease is evident at all laten
In contrast to the lower auditory centers, there is a scarcity
cies. As the infragranular layers are considered to be the output
of data on the morphological changes that occur in
layers of AI, there is a reduction in the output from AI to both
the auditory cortex as a result of a long-term SNHL.55
subcortical and higher order cortical structures.257 These
Nonetheless, it has been reported that rabbits that were
changes result in an overall decrease in the coupling between
unilaterally deafened as neonates showed no change in
AI and other auditory centers, although the pattern of change
neuron soma area, number of dendritic branches, or total
is complex and dependent on other factors associated with
basal dendritic length_234 However, there is an apparent
the end of sensitive periods (reviewed elsewhere258). The devel
reduction in the number of spines along basal dendrites of
opment of the auditory cortex is also heavily influenced by
lamina III/IV pyramidal neurons of the auditory cortex,23s
top-down effects and the multisensory nature of real-world
and a reorganization of "spine-free" but not "spiny" den
events.240
drites, with an increase in "nonspiny" dendrite length
The effects of a restricted unilateral SNHL in adult ani
mostly in a tangential direction.236 In combination, these
mals, resulting from a cochlea lesion, and subsequent expo
results indicate a loss of synapses within the auditory cor
sure to at least some auditory input, rather than a complete
tex after a prolonged deprivation of auditory input during
bilateral SNHL, are apparent in the reorganization of the
development.
contralateral AI ( Fig. 3.7). Under such conditions, the
Although there have been reports that after pure - tone stimu
normal cochleotopic organization of the contralateral AI is
lation the location of cFOS (a protein associated with learn
distorted, and acoustic frequencies that correspond to
ing237) -positive neurons within the auditory cortex matched
regions of cochlea near the lesion-edge become grossly over
the expected tonotopic organization238; there have been no
represented.259-263 Unilateral SNHL not only affects the con
reports of the effects of SNHL on either its localization or
tralateral AI, but also results in the ipsilateral AI becoming
expression. However, an SNHL has been reported to reduce the
more sensitive.261264-266 Such reorganization could be due to
expression of phosphorylated cyclic adenosine monophosphate
"rapid disinhibition or true plastic reorganization with
(cAMP)/Ca2 -responsive element-binding protein (pCREB) and
morphological changes,"257 and a contribution from the
BONF.239 owes also exhibit a prolongation of the normal
reorganization of interhemispheric corticocortical projec
synaptogenesis, with overshoot and elimination thought to be
tions cannot be excluded.
part of the closure of the critical period.240
Many of the studies of SNHL-induced changes in the auditory
cortex utilize congenitally or neonatally deafened animal
Physiological Changes models, which allow access to the early critical period in devel
opment. However, changes in the response of neurons within
Following Deafness
the auditory cortex to an SNHL are not limited to this critical
The motto "use it or lose it" is often apt for cortical centers, and period, but can occur later in development.266 There is a
so reports of the activation of the auditory cortex by other growing body of work supporting the plasticity of the adult
modalities after an early SNHL are not surprising.241 - 244 auditory cortex, including Af.267 - 269
However, as is the case for the ICC, there is evidence for the
development of a "rudimentary cochleotopic representation"
within AI, even when completely deprived of input,sS.24S Response to Chronic
although this finding is not universal.246-249 Other "higher
order" cortical auditory centers, including the secondary audi
Electrical Stimulation
tory cortex, on the other hand appear to be more susceptible to Chronic, behaviorally relevant, intracochlear electrical stimula
"infiltration" from other modalities and exhibit more plasticity tion results in plastic changes in the deaf auditory cortex.
than AI in the cat.244250251 Electrical stimulation increases the expression of pCREB and
Field potentials and single- and multiunit recordings from BONF, as well as activity in the mitogen-activated protein
layers III/IV of the auditory cortex in the owe have shown that kinase signaling pathway,239 which has been implicated in
not only is a rudimentary cochleotopic representation present, long-lasting forms of synaptic plasticity.

29
I I ' I I

Coch l ea Au d ito ry Cortex

N o rm a l

Restri cted
SNHL

P rofo u n d
SNHL

Coch l ea r
I m p l a nt

Fig. 3.7 Summary diagram illustrating the changes in coch leotopic organization of the primary auditory cortex with different hearing histories. A
restricted loss of hig h-frequency hearing results in an overrepresentation of the edge frequency, whereas a complete loss results in a complete
disruption of the organization. The use of a cochlear implant maintains/restores the normal cochleotopic organization.

30
6 . 6 6 6 .
I I ' I I

Compared to unstimulated deaf controls, cats subjected to than in normal-hearing subjects ( magnetoencephalography
chronic electrical stimulation exhibit an increase in long [MEGj2 B4-287 and fMRJ2B8). The ipsilateral-contralateral changes
latency (> 1 50 ms) field potentials, larger current source can also occur in adult subjects as a result of a late-onset
densities (particularly in layers II and III), and more sustained unilateral hearing loss,289 again highlighting the plasticity of
single- and multiunit activity.245270 There is also an expansion the auditory system in the adult brain. Some changes may man
in the area of cortex activated by the electrical stimulation ifest within weeks of the hearing loss,290 but changes continue
of the auditory nerve270 and a maintenance/reestablishment of to occur over a prolonged time course of 2 years or more,2 B9
the normal cochleotopic organization.249 Moreover, with behav suggesting there may be both an unmasking of ordinarily silent
ioral training there is improved frequency-following ability inputs and morphological changes. Interestingly, changes in
of cortical neurons, suggesting some restoration of temporal the response of the ipsilateral auditory cortex may also be
processing in the AI of long-term deaf animals.253254 The result dependent on which side the unilateral deafness occurred,291
ing response properties of AI in chronically stimulated deaf cats indicating that these changes may be at least partially a result of
are similar to those reported in normal-hearing animals. The changes peripheral to the auditory cortex. These results empha
long-latency responses are thought to be mediated by cortico size the difficulties in determining whether functional changes
thalamic loops, and are proposed to be essential for short-term in the response of the auditory cortex are the result of changes
memory and processing in higher order auditory centers.257 within the cortex itself or a result of downstream changes.
The effects of chronic electrical stimulation with a restricted Despite many years of auditory deprivation, during which
SNHL have been less well studied. However, chronic electrical very low levels of activity are evident in AI, its activation can be
stimulation does appear to limit the overrepresentation of seen using a variety of imaging techniques after the return of
lesion - edge frequencies,27l while having little effect on the auditory input via a cochlear implant,292-295 In fact, after long
basic acoustical response properties. The auditory cortex can term cochlear implant use, auditory evoked potentials recorded
integrate "two-tone" stimuli over a broad range of frequencies, from prelingual deaf patients implanted in early childhood
with a complex dependence on the frequency, intensity, and appear nearly normal, suggesting an activation-induced matu
timing of the two stimuli212 ; however, the ability of the audi ration of the auditory cortex.2SS.296 There are also plastic
tory cortex to integrate both acoustic and electric stimulation changes within the "higher order" auditory centers, with the
is less clear. amount of activity in these centers in prelingual deaf patients
Therefore, behaviorally relevant, chronic intracochlear electri with a cochlear implant decreasing with implant use.297 There
cal stimulation appears to allow the auditory cortex to achieve is also less activity seen in the higher order auditory centers in
an experience -dependent maturation, albeit not precisely as it prelingual deaf cochlear implant patients than in postlingual
would have occurred in the absence of a SNHL. deaf cochlear implant patients.298 Finally, long-term cochlear
implant use also appears to enhance "top-down" feedback
modulation of processing within lower auditory centers.273
Clinical Observations A final interesting observation is that there is a positive cor
Many of the reports of changes in the auditory cortex in deaf relation between speech perception and low resting activity in
patients, as in the animal studies of the cortex, have been AI prior to cochlear implantation for the prelingual deaf.297.299
focused on the "use it or lose it" scenario.273 Therefore, it comes This circumstance suggests that although AI and other higher
as no surprise that it has been reported that in prelingual deaf order auditory centers are capable of plastic change, as evident
patients "the brain region usually reserved for hearing may be in the continued improvement in auditory performance with
activated by other sensory modalities."274 However, the audi increasing experience using a cochlear implant,m the best
tory deprivation-induced "takeover" of auditory areas appears clinical outcomes for cochlear implant patients may in fact
to be limited to secondary auditory areas ( supratemporal occur with the most immature auditory cortex, or the
gyrusfperisylvian region), which are normally used for auditory "cleanest sheet."
processing and Ianguage.274-276
Consistent with results reported from the animal models, the
connections to AI appear to be more robustly organized than
Concl usion
those to the secondary auditory areas. There is no evidence of
cross-modal activation of AI using either positron emission Auditory experience plays a key role in laying down the fine
tomography ( PET)277 or functional magnetic resonance imaging organizational structure of the auditory pathway onto a frame
(fMRI),278 though there is evidence of some plasticity of the work generated by genetic cues. A lack of auditory experience
tonotopic organization.279 Auditory evoked potentials recorded during development results in a more rudimentary pathway;
from both cochlear implant subjects and normal-hearing sub however, this reduced level of organization appears sufficient to
jects also suggest near-normal maturation of middle ( IV and provide both the temporal and spatial cues necessary for speech
deep III) cortical layers. However, there appears to be altered perception using a cochlear implant. Importantly, the ability
maturation or input to the superficial (ll and upper Ill) Iayers,2s o of this pathway to undergo plastic reorganization following
particularly in the absence of auditory input during the early cochlear implantation is now recognized as a major factor
critical period.281282 Therefore, as in the animal models, it underlying the clinical success of these devices. This chapter
appears that the auditory cortex, and in particular AI, remains reviewed the degenerative and atrophic changes that occur
in an immature state in prelingual deaf patients.283 within the auditory pathway following an SNHL and the trophic
Ipsilateral-contralateral differences in the responses of and functional reorganization that occurs following reafferenta
the auditory cortex in unilaterally deaf subjects are smaller tion via a cochlear implant.

31
[221 Bosher SK, Hallpike CS. Observations on the histological features, develop
Acknowledgments ment and pathogenesis of the inner ear degeneration of the deaf white cat.
Proc R Soc Land B Bioi Sci 1 965; 1 62 : 147-170
This work was funded by the National Institutes of Health [231 Mair IW. Hereditary deafness in the white cat. Acta Otolaryngol Suppl 1 973;
NIDCD ( HHS-N-263-2007-00053-C), a Life Sciences Research 314: 1 -48
Award from the NSW Office for Science and Medical Research, [24 1 Schwartz IR, Higa JF. Correlated studies of the ear and brainstem in the
NHMRC grants APP1 00507 1 , APP1 009482, and APP1 002430, deaf white cat: changes in the spiral ganglion and the medial superior olivary
nucleus. Acta Otolaryngol 1 982; 93: 9-18
and a grant from the Garnett Passe and Rodney Williams
[25 I Ryugo OK, Pongstaporn T, Huchton OM, Niparko JK. Ultrastructural analysis
Memorial Foundation. The Bionics Institute acknowledges the of primary endings in deaf white cats: morphologic alterations in end bulbs
support it receives from the government of Victoria, Australia, of Held. ] Camp Neurol 1997; 385: 230-244
through its Operational Infrastructure Support Program. Prof. [261 Ryugo DK. Rosenbaum BT, Kim PJ, Niparko JK, Saada AA. Single unit
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Dexter Irvine provided critical comment on an earlier version
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[271 Heid S, jahn-Siebert TK. Klinke R. Hartmann R, Langner G. Afferent projection
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37
. . . . . .

4 Aud itory N e u roplasticity


Robert C. Froemke, Selena E. Heman-Ackah, and Susan B. Waltzman

with internal state variables such as the level of motivation and


Basic Research on
attention to stimuli.6
Neuroplasticity: Relevance Positive changes in speech comprehension and production
with time and training after implantation should be
for Cochlear Implant Design unsurprising, given the large literature on plasticity within the
The cochlear implant is the most widely adopted neuropros human auditory system in relation to language learning and
thetic device, and is used by over 200,000 people worldwide. musical abilities, and after hearing loss_?.s A common theme
The success of this device stems in part from a long history of among these studies is that the auditory cortex in particular
collaboration between basic and clinical research on the func can be rapidly, profoundly, and persistently reorganized by
tion, design, and biological interface of the cochlear implant changes in sensory input and practice with speech and music
with the central nervous system. This chapter briefly reviews production and comprehension. The perisylvian cortex of the
the history of basic research on cochlear implants, focusing on temporal lobe contains Broca's and Wernicke's areas, and has
neuroplasticity-the capacity of the brain to be modified by been found to have an extended maturational period, continu
changes in patterns of sensory experience and motivational ing through at least 14 years of age in relation to speech percep
state. We then discuss more recent research on the plasticity of tion and 30 years of age for gray matter thickness.91 0 Although
the central auditory system, and the impact these studies have there is undoubtedly a critical period during early childhood
on cochlear implant design, training, and long-term outcomes. development for language learning1 1 , recently Schlegel et af12
have used diffusion tensor imaging to examine changes to
white matter in adults learning a second language. Over a 9-
Neuroplasticity in the Human Central month period of an intensive course in modern Chinese, imag
Auditory System ing in Dartmouth students indicated that myelination increased
in cortical language centers, and the degree of change for each
As described in Chapter 1 of this book, the earliest studies of subject correlated with their proficiency with the new lan
cochlear implants were performed in human subjects prior to guage. In a similar manner, practice with musical instruments
substantial work in animal models. After the pioneering work leads to enlarged representations of musical notes in human
on deaf patients performed by House, Michelson, and others in auditory cortex, as measured with magnetoencephalography.13
the late 1 950s to early 1 970s, Michael Merzenich was hired by Thus practice and exposure are linked to positive forms of audi
the Department of Otolaryngology at the University of tory cortical plasticity that might subserve increased profi
California at San Francisco to examine the basic neurobiological ciency with music, speech, and language skills.
mechanisms of cochlear implant function. Merzenich and Importantly, the cortex seems to remain functional and plas
colleagues conducted experiments on the effects of electrical tic after sensory deprivation. Forms of deafness such as conduc
stimulation of the cochlea in cats, recording in both the inferior tive hearing loss and sensorineural hearing loss have been
colliculus and the auditory cortex.1.2 These experiments found to affect the auditory system at multiple levels.14 How
revealed that neurons in the central auditory system of deaf ever, acoustic stimulation to the intact ear evokes detectable
ened animals robustly responded to cochlear stimulation. For responses over both contralateral and ipsilateral auditory cortex
years after implantation, midbrain neurons could be reliably in hearing loss patients.1 s After patients are fitted with cochlear
activated by electrical stimulation in a similar manner as acoustic implants, cortical responses can also be detected over the con
stimulation, although tuning curves were much flatter and tralateral hemisphere after electrical stimulation, including in
responses were artificially more synchronous with shorter patients who are congenitally deaf.16.1 7 Despite the reductions
latency. Thus even in congenitally deaf animals or after prolonged in activity levels and coherence, and potential cross - modal plas
periods of hearing loss, the adult auditory system retains the ticity (e.g., by visual inputs), cochlear implant use seems able to
ability to function and transmit signals to higher brain regions, recover some degree of functionality in central auditory regions
supporting the use of cochlear implants in older subjects.3 in the face of peripheral atrophy and degeneration.9.1 S
In some patients, use of the device is limited or negligible
immediately after implantation but progressively improves over
time. For example, Waltzman et al4 in 1 990 found that speech Neuroplasticity in Animal
production, phoneme recognition, and lipreading could subs Models of Auditory Function
tantially increase after 1 year of use. Similarly, Chatelin et a15
in 2004 observed that word recognition in adult patients
and Perceptual Learning
increased by approximately 50% over the first postoperative Studies in animal models have revealed the basic mechanisms
year, from 30% correct after 3 months to 45% correct after 6 to by which changes in sensory experience modify neural circuits
1 2 months postimplantation. As the device itself is unaltered in an adaptive, behaviorally relevant manner. There is substan
(or if anything, degrades in functionality) after initial implanta tial evidence that changes to neural circuits of the auditory sys
tion, this improved performance is believed to reflect a neuro tem are induced by training. Furthermore, a growing number of
plastic process within the central auditory system, shaped by studies suggest that these modifications are not simply correla
the statistical pattern of electrical stimulation in conjunction tive but causal, supporting behavioral changes and improved

38
. . . .. .

Aduk Young (P14)


6.0 6.0 Fig. 4.1 a - d Development o f excitatory-inhibitory
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balance. (a) In vivo intracellular (whole-cell) recording
from neuron in adult rat primary auditory cortex,


:! l.O / . . .
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displaying balanced excitation and inhibition: (top)
frequency tuning of excitatory (Exc; filled circle) and
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inhibitory tu ning early in development. Recording
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0.0 0.0
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with excitation. (c) Increase of excitatory-inhibitory
frequenc y { k H z ) rreq uenc: y ( kHz)
balance during development. After week 2, excitation
2.0 2.0 and inhibition were uncorrelated. By the end of the
first month, excitation and inhibition were balanced .


(d) Summary of changes to excitatory-inhibitory
! ! . balance over development: (top) correlation between
! 1.0
1.0 excitation and inhibition in young (P1 2 - 2 1 ) and
adults; (bottom) difference in excitatory and inhibi

r. 0. &7 r. ...0 . 0 1 tory best freq uencies in young and adults. (Adapted
from Dorrn AL, Yuan K, Ba rker AJ, et al. Develop

f
0.0 0.0 mental sensory experience bala nces cortical excita
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frequercy ( nS) Frequenc y (nS)
tion and inhi bition. Nature 201 0;465:932 -936.
Reprinted by permission .)

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Age ( PoSI:natil 0) Young

perceptual abilities such as sensory detection and discrimination. the auditory system from brainstem to cortex, perhaps due to
Perhaps most significantly, it is clear that the central auditory failure of the maturational programs that determine the driving
system, and the auditory cortex in particular, remains plastic force for GABAergic neuronal currents and refine interneuronal
throughout life.1920 circuitry to balance excitation and inhibition.3 o-3s
Excitatory-inhibitory balance is a fundamental feature of
neural circuits, and refers to consistent observations (most
Developmental Plasticity frequently in cortical networks of adult animals) that the
Classically, it was believed that the central nervous system was strengths of inhibitory inputs are proportional to co-tuned
largely or exclusively labile during postnatal development.21 For excitatory inputs.3637 For example, in vivo intracellular record
this reason, most studies of auditory plasticity have been per ings from adult rat primary auditory cortex have revealed that
formed in young animals, or in animals that were exposed to or the frequency tuning curves of synaptic excitation and synaptic
deprived of inputs early in life. Much of this work has focused inhibition are co-tuned and highly correlated ( Fig. 4.1 a).
on the effects of developmental conductive or sensorineural This scaling and tuning of inhibitory input to match the pro
hearing Joss, usually in cats and rodents. These manipulations file of excitatory input is important for precise timing of action
greatly affect subcortical stations of the auditory system, lead potential generation, as well as to limit spurious plasticity and
ing to degeneration of peripheral neurons and central neurons, epileptic activity.38 New work from our laboratory has shown
broadening of tuning curves in the midbrain, and disruptions of that excitatory-inhibitory balance emerges in an experience
auditory brainstem responses and threshold elevations.22-26 dependent way over the first postnatal month in rat primary
Hearing Joss also leads to cortical reorganization, including auditory cortex, a period thought to be equivalent to the last
changes to tonotopic map structure, increase in the efficacy of trimester in utero for human children.3940 Hearing onset in
cross-callosal projections in the case of unilateral deprivation, rodents begins at postnatal day (P) 12, as rats and mice are born
and degradation of inhibitory cells and circuits.26- 29 Decreased blind and deaf. At these young ages, inhibitory synapses are
y-aminobutyric acid (GABA)ergic inhibitory strength after functional and can be equally as strong as excitatory synapses.
developmental hearing Joss is a general observation throughout However, the frequency tuning profiles of inhibitory responses,

39
. . . . . .

as measured with pure tones between P1 2 and P21 , were found these is the nucleus basalis of the basal forebrain, the main
to be untuned and mismatched with the profiles of excitatory source of acetylcholine in the cortex. Acetylcholine release is
responses from the same neurons ( Fig. 4.1 b-d). predominantly involved in selective attention and arousal, and
Gradually over the first postnatal month, the correspondence thus is important for learning and memory.49,s o An extensive
between excitation and inhibition increased, as inhibitory number of studies using extracellular recordings have shown
inputs became tuned and calibrated to the local strengths of that pairing pure tones of a specific frequency with electrical
excitatory inputs. This improvement then reflects the native stimulation of nucleus basalis ("nucleus basalis pairing')
plasticity within the auditory cortex possibly similar to other induces large, long-lasting enhancements of spiking.2051 -53
developmental critical periods, for the analysis of more com These changes are robust across species and systems, and are
plex auditory objects and language learning in humans.41 .42 observable at the level of field potentials, single- and multiunit
Successful engagement of this developmental process recordings, and cortical maps as a whole. Acetylcholine has a
required structured and reliable patterns of sensory input. wide range of effects on cortical neurons, but a consistent
Repetitive stimulation with a given tone frequency for several observation is increased excitability and suppression of intra
minutes ("patterned" stimulation, in contrast to the random cortical synaptic transmission.s4- S7 However, it has been
sequence of tones used to initially measure tuning curves) led unclear how these effects could produce long-term response
to a large increase in the strength of both excitation and inhibi enhancement specific for particular stimuli, such as pure tones
tion evoked by that frequency. In addition, synaptic responses of the paired frequency, and how these changes might affect
evoked by frequencies within one octave also increased in size. sensory perception and signal processing of paired inputs.
Conversely, the original best frequencies of excitation and inhi To determine how cholinergic modulation enables adult
bition decreased in amplitude. These forms of long- term synap cortical synaptic plasticity, we combined in vivo whole-cell
tic plasticity had two consequences : first, the best frequency for recording with nucleus basalis stimulation.49 These experi
both excitatory and inhibitory inputs became the frequency ments serve as a model for understanding the basic princi
used during patterned stimulation; and second, the correlation ples of neuromodulation and synaptic plasticity in intact
between excitation and inhibition increased, improving overall animals. Stimulation electrodes were first implanted in
excitatory-inhibitory balance.39 This form of plasticity required nucleus basalis in anesthetized adult rats, and we then made
reliable statistics of sensory stimulation for just a few minutes, whole-cell recordings from neurons of the primary auditory
and was not observed in adult animals in which excitatory and cortex ( Fig. 4.2a). For each cell, we measured synaptic fre
inhibitory inputs are already highly correlated. quency tuning profiles using random tone sequences
( Fig. 4.2b ), and then repetitively paired tones of a specific
nonpreferred frequency with simultaneous high-frequency
Adult Plasticity stimulation of the nucleus basalis, to greatly increase acetyl
Although brief episodes of patterned stimulation were only choline levels throughout the cortical mantle.ss
effective in young, not adult, animals, this does not mean that Cholinergic modulation transiently decreased the strength of
the adult central nervous system is not plastic. As balanced, co inhibitory responses evoked by the paired tone. Consequen
tuned inhibition likely prevents adjustment of neural circuits in tially, this decrease in inhibition enabled excitatory synapses to
adult animals, manipulations that transiently disrupt or reduce become much stronger. These inhibitory and excitatory synap
inhibition have been found to gate or enable synaptic modifica tic modifications were long lasting: we found that nucleus basa
tions in mature neural networks. Prolonged sensory exposure lis pairing led to a long-term increase in the ratio of excitation
for days to weeks can lead to long-term adaptations of auditory to inhibition, due to the cooperative effects of suppression
cortex in adult cats, as cortical neurons become insensitive to of inhibition and enhancement of excitation ( Fig. 4.2c).
background noise.43 Conversely, pairing repetitive presentation However, this change in excitability at the paired tone, and at
of a weak, nonpreferred stimulus with the best frequency of a other tones, continued to evolve for hours after pairing, with
given neuron leads to shifts in tuning curves toward the paired inhibition eventually increasing to a higher level than before, to
weaker stimulus.44 In these cases, sensory stimulation with a rebalance the persistent increase of excitation at the paired
specific pattern of inputs or with background noise presumably frequency ( Fig. 4.2d).
leads to adaptation of inhibitory circuits, transiently rendering These results indicate that the dynamics of inhibitory trans
cortical networks plastic and susceptible to reorganization mission could serve as a synaptic memory trace of the pairing
toward or away from overrepresented stimuli. Other, more event.49 The duration of input-selective disinhibition permits
invasive methods of directly affecting inhibitory synapses are self-reorganization of cortical tuning curves to emphasize the
also effective at opening windows of plasticity in the adult cor new preference for the paired stimulus, in a manner indepen
tex, including application of GABA receptor blockers and trans dent of further evoked neuromodulator release. Under natural
plantation of embryonic progenitor cells into older cortex.45.46 conditions, this memory trace would represent episodes or
However, behavioral training on perceptual learning tasks stimuli that have acquired new behavioral meaning, or might
has been shown to be the most reliable and best-documented be similar to the sorts of cortical changes that occur during
method for modifying central circuits.47.48 Behavioral training is perceptual learning, especially for those tasks requiring focal
believed to drive cortical plasticity via activation of subcortical attention and sensory discrimination. Ongoing work in our lab
neuromodulatory systems that signify behavioral context. Find oratory is investigating the perceptual effects of these changes
ings from several labs have determined that cortical plasticity is to cortical tuning curves, in terms of improving sensory detec
governed by various subcortical structures that project to the tion and recognition of tones paired with reward, arousing
cortex and provide neuromodulatory input. Foremost among stimuli, and directly stimulating neuromodulatory centers. 59

40
. . . .. .

a b

Cel i A:
1. 5 -10 min


g_ 1 . 0
"'
(].J
""

al
.!::!
-;;;
o. 5

0. 0 L......I---'---L----'--1.---'-'""
0. 5 2 4 8 16 32
F req uency ( kH z )

c d

Celi A: Cell B:
1.5 + 30 m in 1. 5 + 1 80 m i n

s:
c:
1.0 g_ 1 . 0
"'
(].J (].J
"" ""

] ]

0. O L......&---'---L----'--1.--...__
0. 5 2 4 8 16 32 2 4 8 16
F req uency ( kH z ) Freq uenc y ( k H z )
Fig. 4.2 a - d Synaptic receptive field plasticity in the adult primary auditory cortex. ( a ) Experimental setup. A stimulation electrode (Sti m) was
acutely implanted (AI) in nucleus basa lis (NB) and whole-cell recordings (Rec) were obtained from auditory cortica l neurons in anesthetized adult rats.
(b) Synaptic frequency tu ning of excitation (Exc) and inhibition (lnh) for the first cell 1 0 minutes prior to pairing 4-kHz tones (arrow) with nucleus
basalis stimulation. Note the initial balance (r: 0.9) and co-tu ning of excitation and inhi bition (original best frequencies of both a re 1 6 kHz, arrowhead).
(c) Frequency tuning of the same cell in b, recorded 30 min utes after pairing. Excitability at 4 kHz was increased due to the enhancement of excitation
and suppression of inhibition after pairing, whereas overall excitatory-inhibitory balance was red uced (r: 0.3). (d) Another cell from the same location
of the aud itory cortex in the same animal as in b and c, recorded 3 hours after pairing. The paired frequency was the best freq uency for both excitation
and inhibition, and the excitatory-inhibitory balance was restored (r: 0.9). (Ada pted from Froemke RC, Merzenich M M . Schreiner CE. A synaptic
memory trace for cortical receptive field plasticity. Nature 2007;450:425 -429. Reprinted by permission.)

Finally, new work in deafened animals fitted with cochlear temporal following abilities of cortical neurons back to control
implants suggests that behavioral training can potently enhance levels, despite the extensive peripheral degeneration.
performance and improve implant use. In 201 1 Vollmer and In summary, the central auditory system remains plastic
Beitel60 examined adult cats with early hearing loss and throughout life, even after long periods of sensory depriva
cochlear implants. Cortical responses to stimulation were tion and hearing loss. Changes in the statistical pattern of
reduced and had poor temporal precision, probably due to sub inputs, including artificial inputs generated by cochlear
stantial loss of spiral ganglion cells (generally < 6% of control cell implants, can successfully modify central synapses, espe
numbers). Passive stimulation had little long-term effect on cially in the auditory cortex, for improved behavioral per
responses, but combining electrical stimulation with behavioral formance. These modifications tend to increase the cortical
training (a mild shock predicted by high-frequency stimulation representation of reliable inputs or inputs that have become
of the cochlea) greatly improved the response rates and the behaviorally meaningful.

41
Auditory Neuroplasticity

reported in prelingually deaf children implanted before the age


Clinical Perspective: of 3.5 years within 6 months of implant use. On evaluation of
Neuroplasticity in Cochlear children implanted over the age of 7 years, abnormal P1 latency
was observed, which never reached normal latencies following
Implant Recipients implant use, and it has been suggested that these differences
Neuroplasticity of the auditory system plays a critical role in may be critical for optimal speech and language development.Gl
the upper level functionality of the cochlear implants, particu Additional studies have investigated the impact of age of
larly in individuals with prelingual deafness implanted after implantation on speech perception testing performance. Harri
early childhood. External stimulation is critical for the normal son et aF5 studied cochlear implant performance as a function
neurologic development of essential functional networks and of age of implantation in 82 children implanted between 2
neural connections during early development.61 62 Auditory years and 1 3 years of age. Children implanted at 5 years of age
deprivation has been found to result in abnormal or delayed and younger were found to outperform their older peers in all
maturation within the auditory cortex.6364 As such, auditory phoneme and word speech perception tasks, with children
perception as well as speech and language development beyond implanted at 2 years of age exceeding all other groups. When
this early period of development is highly dependent on a corrected for duration of deafness, the optimal split was found
degree of neuroplasticity resulting in later synaptogenesis. to be at age 4.4 years and at 5.6 years based on the Glendonald
Auditory Screening Procedure (GASP) word and Test of Audi
tory Comprehension (TAC) scores, respectively. These findings
Clinical Impact of Auditory support the assertion that early cochlear implantation yields
the best cochlear implant performance, which is likely associ
Deprivation ated with a higher degree of synaptic plasticity within the
The impact of auditory deprivation has been long investigated. auditory system. However, these findings also reveal that
Although innate properties of the auditory system exist, devel individuals implanted at a later age do derive benefit from
opment of the auditory system is highly dependent on extrinsic implantation indicative of persistent capacity for neuroplastic
input.GS Various changes have been identified within the audi ity within the auditory cortex and auditory system.
tory system as a function of auditory deprivation including Studies of cortical function within the auditory cortex have
extensive reorganization leading to abnormal development in been performed, yielding findings supportive of the clinical
studies of congenitally and prelingually deaf individuals.GG-69 observations noted in patients with delayed cochlear implanta
Size reduction has been noted in neural cell types within the tion. Truy et aF6 investigated the cortical capacity of auditory
cochlear nucleusJO Deafness has also been associated with a stimulation on patients with longstanding auditory deprivation.
reduction in the volume of neurons within the cochlear In three right-handed prelingually deaf individuals with deaf
nucleus, the development of gliosis, and replacement of much ness duration of 1 6 to 26 years, cerebral regional activity was
of the neuropil by glial processes leading to a subsumption of measured using 150-labeled water and positron emission
the auditory cortex by other processes.71 72 Because of these tomography ( PET) at rest and during electrical cochlear stimu
early findings in auditory deprivation, late cochlear implanta lation (crude constant current squared pulses) of the right ear.
tion in patients with congenital and prelingual deafness had Auditory sensation was perceived in only two of the three
long been disparaged. subjects. However, some degree of activation was noted in the
Changes in the auditory pathway with auditory deprivation auditory cortex in all subjects : the ipsilateral cortex in one sub
have been investigated in cochlear implant patients as a func ject and the contralateral cortex in two subjects. This study
tion of duration of deafness and sensory deprivation. No age-at provides evidence that despite extended periods of sensory
implantation-dependent difference in electrically evoked com deprivation, the auditory pathway may be stimulated even to
pound action potential of the auditory nerve and brainstem the level of the auditory cortex, although the perception of said
following cochlear implantation has been identified.73 However, stimuli as sound may be variable.
differences have been reported in higher order function along Conversely, Lee et al77 investigated neuroplasticity in the
the auditory pathway. Critical variations have been identified auditory cortex of cochlear implant patients by examining the
dependent on age of implantation in studies of cortical auditory correlation between preimplantation function within the audi
evoked potential P1 peak latency time. With age, the latency tory cortex and postimplantation speech perception scores_77
between presentation of auditory stimulus and cortical audi Fifteen prelingually deaf individuals ages 2 to 20 years were
tory evoked peak potential P1 decrease in normal-hearing indi evaluated and compared to 1 7 normal hearing young adults.
viduals, indicating a shorter time period necessary for cortical ! Sf- fluorodeoxyglucose PET and statistical parametric mapping
response to auditory stimulus. was performed preoperatively and correlated with the Korean
Sharma et aF4 investigated cortical P1 latency in children version of the Central Institute for the Deaf (CID) test scores.
following cochlear implantation. Immediately following implan There was a positive correlation ( r = 0.8 1 , p < 0.005 ) between
tation, children implanted at the age of 3 years were found to hypofunction within the auditory cortices and CID scores.
exhibit P1 latencies similar to those of neonates. By 8 months Hypofunction within the regions of the auditory cortex investi
post-implant activation, the P1 latency values were found to be gated decreased as the duration of deafness increased. These
age appropriate. In a subsequent study, Sharma and Campbell61 findings suggest that lack of subsumption of the auditory cortex
compared the cortical P1 latencies of prelingually deaf children by alternative function allows for improved cochlear implant
implanted before 3.5 years of age, between 3.5 and 7 years of function and higher levels of speech perception. Similarly, in
age, and after 7 years of age. Normal cortical P1 latencies were patients with near- normal function within the auditory cortex

42
. . . . . .

despite an extensive history of auditory deprivation, these find in childhood (oral versus total communication or American Sign
ings suggest that the presence of subsumption of the auditory Language), stable versus progressive hearing loss, and time
cortex by alternative functions leads to less successful outcomes without use of a hearing aid on the implanted ear. Patients who
in terms of speech perception. Interestingly, in the investigation used oral communication, had progressive hearing loss, and
of individuals who use sign language as their primary mode of wore a hearing aid on the implanted ear were more likely to
communication, Nishimura et aF2 noted activation of the audi obtain better speech perception outcomes likely secondary to
tory cortex during signing, indicating subsumption of the audi the maintenance of some extrinsic stimulation within the audi
tory cortex. The findings of these two studies provide evidence tory pathway decreasing the degree of subsumption.
to support the finding that individuals who use sign language Similarly, Zeitler et aF9 performed a retrospective review of
as their primary modality of communication tend to perform cochlear implant performance in a cohort of 67 patients with
inferior to their age-matched peers following cochlear implan prelingual deafness. The mean duration of deafness was 1 1 .5
tation.78 - 80 years ; the associated mean age at cochlear implantation was
Additionally, Buckley and Tobey81 provided evidence that 1 2.9 years. Sixty-one percent of patients had a history of con
subsumption of the auditory cortex by alternative processes genital sensorineural hearing loss. Significant improvement in
negatively impacts cochlear implant performance. N1 visual speech perception scores for both the Hearing-in-Noise Test
evoked potential (VEP) was recorded over the right temporal (HINT) sentence list and the consonant-nucleus-consonant
lobe in response to peripheral visual motion stimuli in individ (CNC) monosyllabic word test scores were noted at 12 months
uals with prelingual deafness (n 1 0) and postlingual deafness
= postoperatively with mean score changes of 5 1 . 1 % and 32.2%,
(n 1 2 ). Although the correlation was not statistically signifi
= respectively (p < 0.001 ). The mean changes in HINT and CNC
cant, increased amplitude of the N1 VEP recorded over the scores at the end of study were 60% and 38.7%, respectively
right temporal lobe was associated with decreased speech (p < 0.001 ), indicating that the majority of the improvement in
perception scores in individuals with prelingual severe-to performance occurs within the first year of cochlear implant
profound hearing loss. This association was not identified use. Similar to the findings of Caposecco et aJ,78 adolescents
within the postlingually deaf cohort, again suggesting that with progressive deafness and those using oral communication
cross-modal plasticity of the auditory cortex negatively impacts have significantly better cochlear implant performance than
cochlear implant performance. age-matched peers.
Santarelli et al82 retrospectively studied 18 prelingually deaf
Cochlear Implantation in Late implanted adolescents and young adults (age 13 to 30 years )
assessing cochlear implant performance at 6 months, 1 year,
Childhood and Adulthood 2 years, and 3 years. Phoneme identification, word, and sen
Despite the changes that occur as a function of auditory depri tence identification scores improved significantly following
vation, recent evidence suggests that residual neuroplasticity cochlear implantation in these late-implanted subjects, with
remains within the auditory system, enabling prelingually deaf improvement being demonstrated beyond the 1 -year postim
individuals to derive benefit from cochlear implantation plantation interval. Mean values of approximately 1 0% pre
beyond the first few years of life. There are emerging data sup operatively improved to 38.7%, 5 1 .6%, and 65.6% postoperatively
porting the benefit of late cochlear implantation in individuals for disyllabic, trisyllabic, and sentence scores, respectively.
with prelingual deafness. In a review by Caposecco et aF8 of 38 Significant improvement in auditory perception has similarly
individuals with bilateral sensorineural hearing loss diagnosed been demonstrated following cochlear implantation of prelin
before the age of 3 years who underwent cochlear implantation gually deaf adults. In a review by Yang et aJ,80 prelingually deaf
at 14 years of age or older, cochlear implant outcome perform adults who underwent cochlear implantation exhibited signifi
ance was investigated; 28 patients had a confirmed diagnosis of cant improvement in open-set speech perception ( sentence)
severe to profound prelingual deafness (prior to age 3 years), scores following implantation with a mean score change of 7.0
and 10 patients had a diagnosis of sensorineural hearing loss to 46.7 (p < 0.05 ). Improved performance in this population was
prior to age 3 with progression to severe-to-profound sensori found to positively correlate with higher preimplantation
neural hearing loss thereafter 3 years of age. Despite being thresholds and negatively correlated with the primary use of
implanted at a mean age of 33 years, all patients improved sign language for communication preimplantation.
beyond sound detection. Fifty- three percent of patients According to current definition of success and metrics uti
attained open-set speech recognition with scores of> 30% on lized to assess success, late cochlear implantation in prelin
the City University of New York Sentences (CUNY) and the Cen gually deaf individuals has been demonstrated to be of benefit.
tral Institute for the Deaf Everyday Sentence Lists (CID) testing, However, one may argue that within this population a more
with 2 1 % of subjects attaining scores of greater than 90% on muted definition of benefit and expectations should be utilized.
both CUNY and CID testing. The ability to understand speech In the study by Caposecco et aJ,78 although open-set speech
with visual cues was much improved following cochlear recognition of > 90% was not attained in the vast majority of
implantation in 90% of patients ; 85% of patients felt that the patients, > 90% of patients reported improvement in communi
implant had a positive or somewhat positive effect on their cation ability. The main benefits of cochlear implantation in
employment. Furthermore, 70% of patients reported use of the this population may be to aid in sound awareness, lipreading,
telephone following implantation. Cochlear implants were used and overall communication ability, as well as the possibility of
for greater than 8 hours per day by 8 1 % of patients. Three fac speech understanding as shown in several published articles.
tors were found to have a significant impact of cochlear implant Although the associated performance may not parallel normal
performance within this population: mode of communication hearing age-matched individuals or those implanted at a

43
. . . . . .

younger age, late cochlear implantation in prelingually deaf plasticity, although, as stated in this chapter, recent studies
individuals provides a communication benefit that should not have reported open-set speech perception in patients whose
be negated. Given that these recipients report that implantation demographics might suggest poor performance outcomes.
positively impacts their lives, the ability to communicate, Despite the fact that the literature has supported plasticity
opportunities for employment, and thus quality of life, cochlear within the human auditory cortex by showing significant
implantation in this population should be considered a viable reorganization as a result of changes in input and training, the
option for patients with preoperative counseling, realistic transition to the development of clinical tools to effect these
expectations, and positive motivation. changes has been modest. The possibility of developing training
materials that might create changes in the neural system,
resulting in improved levels of speech understanding with
Auditory Rehabilitation and Training cochlear implants and hearing aids, would truly be exciting.
Despite the numerous advances made in cochlear implant The collaboration among neuroscientists, clinicians, and engi
technology, understanding speech in noise and perceiving the neers involved in improving technology presents a unique
tonal complexity of music still poses a great challenge for many opportunity for increased quality of life for the hearing
cochlear implant users. This is believed to be secondary to impaired population and affords the opportunity to increase
limitations within the current technological spectro-temporal our knowledge of the human brain.
resolution as well as to deficiencies attributable to the
implanted ear.83-86 Interestingly, to surmount this obstacle,
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fields. Neurosci Biobehav Rev 201 1 ; 35: 2 1 05-2 1 1 3 [66[ Zhang Ll, Baa S, Merzenich MM. Disruption of primary auditory cortex by
(36[ Wehr M, Zador AM. Balanced inhibition underlies tuning and sharpens spike synchronous auditory inputs during a critical period. Proc Nat! Acad Sci U S A
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[37[ Tan AY, Zhang Ll, Merzenich MM. Schreiner CE. Tone-evoked excitatory [ 6 7 [ Stanton SG, Harrison RV. Abnormal cochleotopic organization in the auditory
and inhibitory synaptic conductances of primary auditory cortex neurons. cortex of cats reared in a frequency augmented environment. Aud Neurosci
j Neurophysiol 2004; 92: 630-643 1 996; 2 : 97- 1 02
[38[ Hensch TK. Critical period plasticity in local cortical circuits. Nat Rev Neurosci [68[ Kitzes LM, Semple MN. Single-unit responses in the inferior colliculus: effects
2005 ; 6: 877-888 of neonatal unilateral cochlear ablation. j Neurophysiol 1 985; 53: 1483-1 500
(39] Dorrn AL Yuan K, Barker Aj, Schreiner CE, Froemke RC. Developmental [69] Kral A, Sharma A. Developmental neuroplasticity after cochlear implantation.
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465: 932-936 (70] Webster DB, Webster M. Effects of neonatal conductive hearing loss on brain
(40] Romijn Hj, Hofman MA, Gramsbergen A. At what age is the developing cere stem auditory nuclei. Ann Otol Rhinal Laryngol 1 979; 88: 684-688
bral cortex of the rat comparable to that of the full-term newborn human [71 ] Moore JK. Wu BJC. Human cochlear nuclei: shape and volume in normal and
baby? Early Hum Dev 1 991 ; 26: 61 -67 in deaf subjects. Assoc Res Otolaryngol l 992; I S : 1 53
(41 ] Doupe Aj, Kuhl PK. Birdsong and human speech: common themes and mech (72] Nishimura H, Hashikawa K, Doi K et al. Sign language "heard" in the auditory
anisms. Annu Rev Neurosci 1 999; 22: 567-63 1 cortex. Nature 1 999; 397: 1 1 6
[42] Insanally MN, Kover H. Kim H. Baa S. Feature-dependent sensitive periods in [73[ Gordon KA, Papsin BC. Harrison RV. Activity-dependent developmental
the development of complex sound representation. j Neurosci 2009; 29: plasticity of the auditory brain stem in children who use cochlear implants.
5456-5462 Ear Hear 2003 ; 24: 485-500
[43 ] Norena AJ, Gourevitch B. Aizawa N, Eggermont jj. Spectrally enhanced acous [74] Sharma A, Dorman MF, Spahr Aj. Rapid development of cortical auditory
tic environment disrupts frequency representation in cat auditory cortex. Nat evoked potentials after early cochlear implantation. Neuroreport 2002 ; 1 3 :
Neurosci 2006; 9: 932-939 1 365-1 368
(44] Dahmen jC, Hartley DE, King Aj. Stimulus-timing-dependent plasticity of cor [75] Harrison RV, Gordon KA. Mount Rj. Is there a critical period for cochlear
tical frequency representation. j Neurosci 2008; 28: 1 3629-1 3639 implantation in congenitally deaf children? Analyses of hearing and speech per
(45] Harauzov A, Spolidoro M, DiCristo G et al. Reducing intracortical inhibition ception performance after implantation. Dev Psychobiol 2005; 46: 252-261
in the adult visual cortex promotes ocular dominance plasticity. j Neurosci (76] Truy E, Deiber MP, Cinotti L Mauguiere F, Froment jC, Morgan A. Auditory
2010; 30: 361 -371 cortex activity changes in long-term sensorineural deprivation during crude
(46] Southwell DG, Froemke RC, Alvarez-Buylla A, Stryker MP, Gandhi SP. Cortical cochlear electrical stimulation: evaluation by positron emission tomography.
plasticity induced by inhibitory neuron transplantation. Science 2010; 327: Hear Res 1 995; 86: 34-42
1 1 45- 1 1 48 [77] Lee DS, Lee JS, Oh SH et al. Cross-modal plasticity and cochlear implants.
(47] Buonomano DV, Merzenich MM. Cortical plasticity: from synapses to maps. Nature 2001 ; 409 : 149-150
Annu Rev Neurosci 1998; 2 1 : 1 49- 1 86 (78] Caposecco A, Hickson L Pedley K. Cochlear implant outcomes in adults and
[48 [ Karmarkar UR, Dan Y. Experience-dependent plasticity in adult visual cortex. adolescents with early-onset hearing loss. Ear Hear 201 2 ; 33: 209-220
Neuron 2006; 52: 577-585 (79[ Zeitler DM, Anwar A, Green JE et al. Cochlear implantation in prelingually
(49[ Froemke RC, Merzenich MM, Schreiner CE. A synaptic memory trace for deafened adolescents. Arch Pediatr Adolesc Med 201 2 ; 1 66: 35-41
cortical receptive field plasticity. Nature 2007; 450: 425-429 [80] Yang WS, Moon IS, Kim HN, Lee WS, Lee SE, Choi jY. Delayed cochlear
(50[ Parikh V, Kozak R, Martinez V, Sarter M. Prefrontal acetylcholine release implantation in adults with prelingual severe-to-profound hearing loss. Otol
controls cue detection on multiple timescales. Neuron 2007 ; 56: 1 4 1 - 1 54 Neurotol 201 1 ; 32: 223-228

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I I .

[81 [ Buckley KA, Tobey EA. Cross-modal plasticity and speech perception in pre [87] Galvin lJ, Fu QJ, Nogaki G. Melodic contour identification by cochlear implant
and postlingually deaf cochlear implant users. Ear Hear 201 1 ; 32: 2-1 5 listeners. Ear Hear 2007; 28: 302-3 1 9
[82] Santarelli R, De Filippi R, Genovese E, Arslan E. Cochlear implantation out [88] Oba 51, F u QJ , Galvin lJ. Digit training i n noise can improve cochlear implant
come in prelingually deafened young adults. A speech perception study. users' speech understanding in noise. Ear Hear 201 1 ; 32: 573-581
Audio) Neurootol 2008; 1 3 : 257-265 [89] Stacey PC, Raine CH, O'Donoghue GM, Tapper L, Twomey T, Summerfield AQ
[83] Donaldson GS, Dawson PK, Borden LZ. Within-subjects comparison of the Effectiveness of computer-based auditory training for adult users of cochlear
HiRes and Fidelity120 speech processing strategies: speech perception and implants. lnt j Audiol 2010; 49: 347-356
its relation to place-pitch sensitivity. Ear Hear 201 1 ; 32: 238-250 [90] Wu JL, Yang HM, Lin YH, Fu QJ. Effects of computer-assisted speech training
[84] Firszt jB, Holden LK, Reeder RM, Skinner MW. Speech recognition in cochlear on Mandarin-speaking hearing-impaired children. Audiol Neurootol 2007;
implant recipients: comparison of standard HiRes and HiRes 120 sound proc 1 2 : 307-3 1 2
essing. Otol Neurotol 2009; 30: 146- 1 52 [91 ] F u QJ , Galvin lJ . Maximizing cochlear implant patients' performance with
[85] Plant K, Holden L, Skinner M et al. Clinical evaluation of higher stimulation advanced speech training procedures. Hear Res 2008 ; 242 : 1 98-208
rates in the nucleus research platform 8 system. Ear Hear 2007 ; 28: 381 -393 [92] Yucel E, Sennaroglu G, Belgin E. The family oriented musical training
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Ear Hear 2000; 2 1 : 608-624 1 043- 1 052

46
t I I

5 M i m icki n g Normal Aud ito ry Fu nctions with Coc h l e a r


I m p l a nt Sou n d Processi ng : Past, Present, a n d Futu re
Ward R. Drennan, Mario A. Svirsky, Matthew B. Fitzgerald, and jay T. Rubinstein

In the early 1 980s, the Vienna/3M single-channel implant


Introduction
became available. This device incorporated loudness control and
Cochlear implants (Cis) represent the first case where a human compression.3.4 Compression minimized peak clipping, providing
sense has been replaced successfully through electronic means. improved encoding for the temporal envelope. Nevertheless,
Over the years, clinical outcomes of cochlear implantation have minimal frequency information was available, so although out
increased systematically. In the case of postlingually deaf Cl comes improved,s.6 performance was still marginal. See Chapter
users, this happens because the neural activation patterns 1 for more information on the history of the development of Cis.
caused by the implant, although relatively crude in many ways,
are close enough to those that were caused by normal acoustic
stimulation when the patients had acoustic hearing. This mim
Representing Frequency
icking of auditory function occurs within the sound processor, Spatially
the functional core of the cochlear implant.
Sound processors have undergone extensive development The human ear functions, in part, as a frequency analyzer,
over the last 45 years. Development moved from single chan transforming acoustic frequency to place along the basilar
nels to multiple channels, from speech feature extractors to membrane? Thus, hair cells in different parts of the basilar
sound encoders, from simultaneous excitation to interleaved membrane respond preferentially to different frequencies.
excitation, and from slow rates of stimulation to high rates. Although it was originally thought that an implant could not
From initial inception in the 1 950s and 1 960s, the Cl has realistically achieve the fine frequency representation of thou
changed from a device that provides merely a sensation of sands of hair cells, achieving some frequency representation
sound, improving acoustic awareness of the deaf, to a device was certainly possible. An electrode array was created and used
that brings reliable speech understanding to the majority of to deliver frequency-specific information according to place.
users. Current research and development efforts continue to In the late 1 970s and early 1 980s, Australian researchers
show improved hearing with new sound-processing strategies. introduced multichannel processors that extracted speech
Development of the Cl has faced numerous challenges. First, features from the acoustic input. For example, vowels are iden
hardware had to be developed that could safely deliver electri tified by the frequency of their spectral peaks, also known as
cal signals into the inner ear. Second, processors had to be formants ( see The Handbook of Speech Perception 8 for reviews of
speech perception). The FO/F2 processor910 identified a funda
developed that could successfully transmit acoustic information
to the auditory nerve. Third, the conditions of the auditory mental frequency ( FO) and the second formant frequency ( F2).
systems of implant users are widely variable, so devices and FO, extracted with zero-crossings, determined the rate of elec
processors had to be optimized to suit individuals. Outcomes trical pulses delivered to a specific place along the electrode
were widely variable; however, most users, particularly postlin array corresponding to F2, re-creating the frequency encoding
gually deafened adults and early-implanted deaf children, have properties of the inner ear. The stimulation rate was equal to
enjoyed great benefit from their prostheses. This chapter the FO estimate for voiced sounds, and it was random (a few
focuses on the development of sound processors, including hundred Hz on average) for voiceless sounds. The processing
past, present, and future strategies. was implemented using a 22-channel Cl. Such processing pro
vided an improvement in performance over single-channel
devices,1 1 but the scheme provided only a small fraction of the
information present in the speech stimulus.
Single-Channel Implants
In the early 1 980s, the FO/F1/F2 processing strategy was
Early implants used a single channel. The first cochlear introduced.12 This processing scheme added first formant (F1 )
implant prototypes were developed and implanted in France information. Central Institute for the Deaf (CID) sentence
by Djourno et al.l The earliest single-channel implant to be recognition more than doubled (from 16 to 35%) with the addi
mass produced was the House/3M implant, developed by tion of the F1 component.B Northwestern University Auditory
William F. House and his colleagues in Los Angeles. This Test No.6 ( NU-6) word recognition increased more than three
single-channel implant succeeded in transmitting acoustic fold (from 8 to 28%). The processing strategy did not incorpo
information electrically to the auditory nerve, but little could rate higher frequency consonant information that could
be understood as there was minimal frequency-specific improve speech understanding further.
information delivered to the implantee. The device also did not A new strategy called MultiPEAK (MPEAK) was introduced in
provide any compression. The 1 6-kHz carrier had a temporal the late 1 980s. This approach used the FO/F1 /F2 encoder and
envelope code that provided some periodicity information added high-frequency information to help code consonants.
below 300 Hz.2 Duration and some voicing cues in speech were Three bands of acoustic information above 2,000 Hz (2,000 to
discernible. A few "star" listeners could understand speech, but 2,800 Hz, > 2,800 to 4,000 Hz, and > 4,000 Hz) were encoded
for the most part this device provided only acoustic awareness using an envelope detector. This information was passed to the
and served as an aid to lipreading. more basal electrodes using electric pulses. Performance again

47
. . . . . . . . . .

improved markedly with this information. Consonant identifica maximum frequency of the low-pass filter. The Hilbert trans
tion improved from 17 to 28%,14 and sentence recognition also form, used in the modem Med-El ( lnnsbruck, Austria) devices,
improved.1516 If there was competing noise of any kind, however, can also accomplish the same goal. The Hilbert transform con
particularly competing speech, the processors would often make verts the original acoustic wave into two outputs: an envelope
errors in selection of the fundamental and formant frequencies. and temporal fine structure ( Hilbert phase). The extracted
envelope is multiplied by a series of biphasic pulses that are
passed to the appropriate electrodes in the implant after com
Compressed Analogue pression of the acoustic input dynamic range into the electrical
dynamic range of the user. Empirical studies showed that speech
Stimulation
can be understood well using the CIS approach.23 CIS often
Concurrent with development of the Australian speech proces provided superior performance to SAS, and today virtually all
sors, a compressed analogue (CA) strategy was developed in the processing strategies offer some type of CIS-based strategy.
United States.1718 The processor was multichannel, using con The majority of people trying both SAS and CIS strategies
tinuous and simultaneous current flow at each electrode. Origi prefer CIS26 -28; however, Battmer et aF9 have shown that
nally, using the lneraid device, the incoming acoustic wave was some SAS users can achieve excellent speech understanding.
compressed, filtered into four channels, and passed via electri Osberger and Fisher28 showed no significant difference in
cal current to the appropriate electrode. The approach incorpo performance after 6 months of experience but noted a faster
rated compression using an automatic gain control (AGC), learning rate among SAS users. Preferences can depend upon
which compressed the wide acoustic dynamic range into the the specific hardware used. For example, the Clarion "HiFocus"
much more narrow electric dynamic range. Dorman et aP9 electrode array is designed to sit closer to the center of the
reported a broad range of abilities ranging from 0 to 1 00% for cochlea. In a group of 56 Clarion HiFocus users, Zwolan et aPD
CID sentence recognition. Average performance was 45% cor reported that a majority preferred SAS. In another group of
rect, far exceeding performance with single-channel implants. similar size that did not use HiFocus, the majority preferred CIS,
The lneraid device is no longer available; however, CA proc as in the other studies. Zwolan et al speculated that the HiFocus
essing is currently available in the form of the simultaneous array limited channel interactions, allowing better performance
analogue strategy20 (SAS) with the Clarion (Advanced Bionics, with SAS. There has not yet been a demonstration of broad
Valencia, CA) device. SAS provided advancement over CA with a clinical superiority for either strategy, but the vast majority of
post-filter AGC that limited spectral distortions caused by fast implantees currently use some form of interleaved processing.
acting compression implemented prior to filtering. SAS also
used discrete current steps updating every 75 microseconds.
The Clarion II can implement the SAS strategy with up to 1 6 Spectral Peak Processors
channels. The CA and SAS approaches preserved the temporal
waveform electrically including zero-crossings and temporal Building on the CIS concept, an "n-of-m" approach was devel
fine structure; however, users typically could only perceive oped.31 The Cochlear Corporation (Sydney, Australia) and Med
such fine structure up to about 300 Hz.221 Further, the simulta El (lnnsbruck, Austria) devices currently use this approach. An
neous analogue approach caused extensive channel interaction "n-of-m" strategy worked much like CIS except that, given m
due to the summation of electric fields.22 This interaction lim total electrodes, a subset of n electrodes with the highest
ited spectral resolution and the effectiveness of simultaneous filter output levels was selected for presentation during each
analogue approaches. stimulation cycle. The Australian group developed the Spectral
Maxima Sound Processor (SMSP).32 Sound was processed into
1 6 band-pass filters. Pulses were delivered only to the six chan
Continuous Interleaved nels having the maximum output. The current levels in filters
with lower outputs were set to zero. Hence, n (6) of m ( 1 6) elec
Sampling
trodes were activated on each stimulation cycle at a rate of
Wilson et aF3 introduced continuous interleaved sampling about 250 cycles per second. McKay et al33 showed that the
(CIS), which addressed the problem of excessive channel inter processor provided markedly better speech recognition than
action. CIS used rapid, nonsimultaneous sweeps of pulses across the MPEAK speech-feature-extraction approach.
the electrode array to represent the time-varying acoustic spec The SMSP processor was developed into the spectral peak strat
trum. CIS processing used a vocoder process24 to extract fre egy (SPEAK), which is currently available with the Cochlear Cor
quency-specific temporal envelopes from the acoustic signal. poration implants. In SPEAK, as with SMSP, a subset of electrodes
The incoming acoustic wave was filtered into multiple fre is selected to stimulate on each sweep, based on the filters with
quency channels. Then, the envelopes of each channel output the largest outputs. The total numbers of maxima are selected
were extracted using rectification and low-pass filtering. from 20 channels. The number of peaks selected is variable, using
Finally, the amplitude envelopes were multiplied by nonsimul up to 10 channels. Channels below a certain noise threshold
taneous biphasic pulse trains, and each pulse train was deliv would not be selected even if they were among the strongest 1 0.
ered to a different intracochlear stimulation electrode . This approach conserves power and further limits channel inter
.,.. Fig. 5.1 shows a schematic of the process.2s action. SPEAK showed superior performance to MPEAK even after
Extraction of frequency-specific amplitude modulations controlling for the more lenient candidature requirements, which
successfully transmitted spectral information but eliminated allowed Cl users with better preoperative hearing, and presum
periodicity and temporal fine structure at frequencies above the ably healthier auditory systems, to obtain cochlear implants.34

48
t I I

Vo lume Skin
Elec t ro d e
Array
M ic ro p ho ne

Spt=:ech
Processor
Q1------
- - El ec t rod e
C o nt a c t s
Receiver

Fig. 5.1 (Top) A schematic of the cochlear implant. (Middle) A more detailed schematic of the sound processor for continuous interleaved sampling
(CIS}. The input is filtered into four band-pass channels (only fou r are shown, for simplicity). The band-pass outputs are rectified and low-pass filtered,
creating a temporal envelope for each frequency band. The temporal envelopes are mu ltiplied by nonsimu ltaneous biphasic pu lse trains that are
delivered by electrical current through the cochlea via the electrode array. (Bottom) The acoustic wave is tra nsformed to a series of biphasic electrical
pulses. (From Loizou P. Mimicking the human ear: an overview of signal-processing strategies for converting sound into electrical signals in cochlear
implants. IEEE Signal Processing Magazine 1 998;98: 1 0 1 - 1 30. Reprinted by permission)

The SPEAK processor operated at a fixed and relatively slow


pulse rate (250 pulses per second per channel [ ppsfch ] ). The
Development: Addressing
Advanced Combination Encoder35 (ACE) was then implemented Weaknesses in Sound
in the Nucleus (Cochlear Corporation) Cis and used a higher
pulse rate than SPEAK, which was expected to improve tempo Processing by Mimicking
ral encoding. Evaluations of the ACE strategy have usually Normal Auditory Function
shown superior performance to SPEAK.36,37 Such improvements
were shown independent of changing candidature require Ideally, an implant would perfectly re-create the neural excitation
ments.34 Most users preferred ACE processing, suggesting it patterns of a normal-hearing person with acoustic stimulation.
would be appropriate to use that in initial fitting for Nucleus The implant achieves varying degrees of success depending on
users36; however, variability among different users was high. the type of information to be transferred.40 A cochlear implant
Vandali et al,38 for example, noted wide variability in open-set has many limitations. First, the dynamic range in implants is
speech recognition performance among five subjects using ACE, highly limited, leading to complex issues regarding the manner of
with speech presented in multitalker noise. Loizou et aJ39 also compression required. Second, the spectral-resolving power of
reported between-listener variability dependent on pulse rates implants is poor. Third, the ability of implants to deliver temporal
and pulse durations. Thus, for implant users, one pulse rate fine structure is highly limited. Finally, a frequency mismatch
does not fit all. Individual optimization of pulse rate and pulse might occur in which the frequency output of the sound proces
duration parameters might lead to significantly improved sor is mapped to a cochlear location that corresponds to a differ
performance. ent frequency region in a normal-hearing ear.

49
. . . . . . . . . .

The Dynamic Range Problem in speech understanding in noise. One person, with the condi
tioner, had a speech reception threshold (SRT) in babble noise
Small dynamic ranges in CI users require extensive compres of -9 dB, remarkable for an implant user and comparable to the
sion. To decrease the amount of compression, and to minimize SRT of person with a mild hearing loss. Two others showed
background noise, the softest sounds are often eliminated. significant objective improvements for speech understanding in
Processed quiet sounds can also be masked easily by louder noise, with SRTs improving from 5 to 19 dB. Three others
sounds. james et a141 introduced adaptive dynamic range opti preferred the conditioning strategy but did not show objective
mization (ADRO), which is currently available with Cochlear benefit, and three more did not like the conditioning and
Corporation devices. ADRO attempts to make all outputs com switched to their original strategy. The exact reasons for benefit
fortably loud. In doing so, ADRO increases the quieter speech are unclear, but they might be related to the increased dynamic
sounds and improved intelligibility. With no background noise, range with conditioning. It is also speculated that conditioning
ADRO improves open-set sentence recognition at low-to could improve temporal sensitivities, especially if it were
moderate speech levels by 1 6 to 20%. Such increases in low implemented in a processing strategy that encodes temporal
level pulses might reduce suprathreshold refractory effects that fine structure in some way. A number of strategies to encode
can mask quiet sounds.42 temporal fine structure have been implemented without condi
Using another approach, Geurts and Wouters43 introduced tioning, and they are discussed below.
enhanced-envelope CIS ( EECIS ), which was intended to intro
duce rapid cochlear adaptation effects to electrical processing.
EECIS could also increase lower level speech sounds (e.g., Spectral Resolving Power
consonants). Word recognition ability was 7% better with
EECIS than with CIS. The transient -emphasis spectral maxima The spectral resolving power of implant users is also limited.
(TESM)M was also intended to magnify short-duration speech Fishman et a1,50 Dorman et al,51 and Friesen et a1,52 for example,
cues that had low levels. Improvements in consonant and word found that despite having up to 22 processing channels, speech
recognition were observed with TESM. Additional improve understanding does not improve significantly with more than
ments of about 9 to 1 1 % were seen for half of the participants about eight channels. Although eight channels are sufficient for
on sentence recognition in noise at a 1 0-dB signal-to-noise good speech understanding in quiet,53 more channels are
ratio ( SNR). needed for good speech understanding in noise52 and for good
Other issues concerning dynamic range control involve the music perception.5455 Some attempts to improve spectral infor
speed of compression and loudness balancing. Stone and mation include use of modiolar-hugging implants and use
Moore45 noted that fast-action compression could reduce the of electrode configurations that can decrease current spread
ability of implantees to perceive amplitude modulations due to within the cochlea. Neither of the approaches has yet to have a
decreased modulation depth. Fast-action compression also significant impact on clinical outcomes. 1356,57 One reason might
increases comodulation across frequency channels, which could be the compromised nerve survival of implant users.5 S- 6o
increase the likelihood of perceptual grouping of speech and Ambitious work is being conducted to address this problem
noise,46 decreasing the ability of implantees to segregate with investigations of nerve growth factors6l - 63 and hair cell
sounds. regeneration.64-66 Although progress is encouraging, the
Use of a "conditioning" or "desynchronizing" stimulus can amount of time required for development and approval of such
increase the electrical dynamic range in CI users.47 In the future biological treatments could be extensive.
absence of functioning hair cells, the auditory nerve has little One approach with current technology involves improving
stochastic spontaneous activity. The response to electrical the place coding of frequency information using "current steer
stimulation is highly synchronized and deterministic, contribu ing" by altering the current level balance between neighboring
ting to the small dynamic range. A conditioning stimulus is a electrodes.67 Current steering makes use of electrical current
high-rate, low-level pulse train intended to encourage sponta presented simultaneously to neighboring electrodes. The cur
neous activity in the nerve.48 The stimulus is designed to lightly rent on each electrode is balanced to "steer" the current to
activate the auditory nerve at all times, re-creating spontane multiple positions between the electrodes, thus theoretically
ous activity like a normally functioning auditory nerve. Physio increasing the amount of spectral information delivered to a
logical studies have shown that the use of a conditioner creates Cl user ( Fig. 5.2 ).68
a more normal pattern of auditory nerve responses.49 Psycho In its present form, current steering is clinically available with
physical work with a conditioner has shown that the condi the Advanced Bionics implant, which uses a fast Fourier trans
tioner increases the dynamic range.47 Conditioning tends to form ( FFT) to extract spectral information. In this processing,
lower the threshold for electrical stimulation, extending the called "HiRes Fidelity 1 20," there are 1 20 possible frequency
usable dynamic range. "bins" of excitation including multiple places between electro
A conditioning stimulus could theoretically be added to any des. The processing balances the charge between electrodes to
processing strategy. Preliminary, unpublished studies have create the between-electrode bins. Current steering has been
shown that a high-rate ( 5,000 pps), low-level (near threshold) shown to provide more pitch percepts for listeners68 and
conditioning stimulus, implemented with a CIS-like strategy improved spectral resolution.697 Current steering, however,
provides significant and sometimes substantial benefit for has been shown to result in degraded temporal information,
understanding speech in noise in some listeners. Trials con owing to the longer ( - 1 5 ms) sliding time window required
ducted at the University of Iowa showed that about one third of for the FFT, and consequently has not been shown to provide
the participants using this strategy had marked improvements consistent benefit for speech or music perception.7

so
t I I

Basal Contact Apical Contact



(of Pair) . (of P air)

1 00/0 70/30 50/50 30/70 0/ 1 0 0


E 1 4/E1 3 E 1 4/ E 1 3 E 1 4/ E 1 3 E 1 4/ E 1 3 E 1 4/ E 1 3

A B
P itc h / Lo c atio n

Fig. 5.2 A schematic of cu rrent steering between electrodes 13 and 14 of a cochlear implant. Using simultaneous electric stimulation. the current
from these neigh boring electrodes is balanced so that it is "steered" to an intermediate position. Box A shows the target place of excitation at
electrode 14 when 1 00% of the cu rrent goes to electrode 14 and 0% to electrode 1 3 . Box B show the cu rrent balance when the target place of
excitation is 3/ 1 0 of the way from electrode 1 3 to electrode 1 4. thus 3/ 1 0 of the cu rrent goes to electrode 1 4, and 7/ 1 0 to electrode 1 4. (From Firszt
JB, Koch DB, Downing M, Litvak L. Cu rrent steering creates additional pitch percepts in adult cochlear implant recipients. Otol Neu rotol 2007;5:629-
636. Reprinted by permission.)

Temporal Fine Structure structure information could provide cues to speaker segrega
tion based on periodicity. Binaural unmasking resulting from
The lack of ability of Cis to pass the temporal fine structure of interaural time differences (ITDs ) can provide additional benefit
acoustic waves to the auditory nerve is yet another limitation. in a cocktail party situation.79 With bilateral implantation,
The ability to segregate speech from noise,71 to perceive tonal temporal fine-structure information could further improve
speech,n and to hear musical melodiess4-73 all rely heavily on speech understanding in noise that is spatially separated from
temporal fine structure. Further, temporal fine structure is a the signal. Zeng et als o have shown, using Cl simulations, that
critical element of binaural hearing in which interaural time encoding frequency modulations can improve speech under
differences are critical.74-7S For example, a cocktail party, with standing in noise. Frequency modulations could be better
numerous competing speakers, presents a serious problem for encoded with temporal fine structure. Thus, successful delivery
implant users, because they cannot easily distinguish one of temporal fine-structure information to Cl users might pro
speaker from another. The ability to segregate different funda vide clinically meaningful benefits.
mental frequencies ( FO) is one critical element that helps listen There are three sound-processing strategies that could
ers distinguish one speaker from another/6-77 Such information improve electrical delivery of temporal fine structure: ( 1 ) a
is encoded in the periodicity of the acoustic waveform, which is fundamental frequency tracking approach called FOmod; ( 2 ) an
part of the temporal fine structure.78 Delivery of temporal fine- experimental harmonic frequency tracker called a Harmonic

51
. . . . . . . . . .

Single Side-Band Encoder, which uses a unique, distortion-free and cochlear size. Concerning electrode location, current
approach to delivering harmonic information; and (3) Med-El's electrode array designs include two nonstimulating markers,
fine-structure processing ( FSP). separated by a few millimeters, that are meant to guide the
FOmod8182 is based on ACE processing, using the same initial, surgeon as to how far the electrode array is to be inserted into
front-end processing. When unvoiced speech or noise is pres the cochlea. Typically, manufacturer guidelines ( HiRes 90K
ent, it operates like ACE. When harmonics are present with Surgeon's Manual; Nucleus 24 Contour Surgeon's Guide)
voiced speech or with musical tones, FOmod extracts the require the surgeon to insert the electrode array so that the
fundamental frequency and modulates all channels with 1 00% more proximal nonstimulating marker is as close as possible to
modulation at the FO. This processing has been shown to pro the cochleostomy, ensuring a standard electrode array insertion
vide benefit in off-line testing of pitch ranking information, depth past the cochleostomy. Although this approach does
melody contour, and identification,82 as well as for Mandarin much to ensure that the electrode is fully inserted into the
tone detection with male voices.81 cochlea, this "one-size-fits-all" approach does not accommo
The Harmonic Single Side-Band Encoder ( HSSE)83 uses a date different cochlear sizes, raising the possibility that fully
unique, linear temporal coding approach. Unlike traditional inserted electrode arrays may stimulate neural populations
approaches that use the nonlinear processes of half-wave recti of different characteristic frequencies in different patients
fication with low-pass filtering or the Hilbert transform, HSSE depending on the size of their cochleas.
extracts the fundamental frequency from harmonic stimuli and As with the electrode insertion instructions, current software
downshifts in the frequency domain to put the harmonic infor packages for Cl programming use a one-size-fits-all approach
mation in the perceivable range for CI listeners (< 300 Hz). Using because they use a standard (default) frequency-to-electrode
an auditory nerve model, this has been shown to provide table for all patients. Depending on the fitting software version,
temporal pitch information (harmonic fine structure) over and the ability to change the standard frequency table ranges from
above that provided by CIS.B4 Initial results with simulations very limited (SoundWave Professional Suite 2.1 , 201 1 ,
and with CI users with a research interface show HSSE can Advanced Bionics) to relatively flexible (Custom Sound 3.2,
provide benefit for perception of tonal languages84 as well as 201 1 , Cochlear Corp.). However, even when there is flexibility
for timbre perception.8s within the fitting software for adjusting the frequency table,
Fine-structure processing ( FSP) has been implemented com the audiologist's manuals provide no guidance to clinicians as
mercially on the Med-El Opus device. FSP uses the same front to which frequency allocations to select. As a result, the vast
end as traditional CIS and is built on high-definition CIS ( HDCIS) majority of CI users are fit with the standard frequency table
using high pulse rates. FSP extracts zero crossing information recommended by the fitting software. One important problem
from the sound wave and delivers this information to the is that normal cochleas can vary in size by as much as 40%.89 A
lowest one to three apical channels.86 The higher channels recent review of studies of cochlear duct length90 found an
operate with HDCIS. Original testing was done comparing FSP average length of 33.5 mm, a standard deviation of 2.28 mm,
on an Opus processor, which processed stimuli down to 70 Hz, and a difference of 1 3.78 mm between the shortest and the
with CIS processing on an older Tempo + processor that proc longest. Because humans have the same approximate range of
essed stimuli down to 200 Hz.8687 The results indicated frequency sensitivity, a particular basilar membrane location
superior performance for FSP. that is a given distance from the base of the cochlea can be
Riss et al88 later showed that the benefits were likely due to associated with a very different acoustic frequency depending
the extended frequency range. When matched for the frequency on the size of the individual's cochlea. Consequently, the char
range, their study showed no benefit for FSP over CIS for speech acteristic frequency of the spiral ganglion neurons presumed to
in quiet or noise, and no benefit for melody recognition. The ben be stimulated by an electrode in that location will also differ
efit of the extended frequency range in the Opus processor ver significantly between cochleas of different size.
sus the Tempo + might, however, reflects the effects of the com For example, according to Stakhovskaya et al's91 spiral
mon frequency mismatches observed in CI users. The problems ganglion correction to Greenwood's92 equation, an electrode
and potential solutions are discussed in the following sections. location that is 24 mm from the cochlear base would stimulate
spiral ganglion neurons with an average characteristic
frequency of 76 Hz in a cochlea that is 28 mm long, and
Frequency Mismatches 1 ,020 Hz in one that is 42 mm long. Current clinical practice
A potentially significant problem for Cl users is the possible regarding electrode insertion and frequency table assignment
mismatch between the input ( acoustic) frequency and the cannot possibly result in a perfect frequency match between
characteristic frequency of the neurons that are stimulated by input acoustic frequency and characteristic frequency of stimu
the implant. In turn, this would cause a mismatch between the lated neurons for all patients. Furthermore, this mismatch is
listeners' long-term representations of speech, developed when likely to be even more pronounced in cases where the insertion
they had normal acoustic hearing, and the stimulation provided is shallower than normal, such as when an obstruction is
by the Cl. encountered in the cochlea.
Why might this frequency shift present a problem for postlin
Current Clinical Practice and gually deafened Cl users? Unlike their prelingually deaf peers,
who form representations of speech sounds based exclusively on
Individual Differences input provided by the Cl, postlingually hearing impaired (HI) Cl
Frequency mismatch in postlingually deafened Cl users is a patients formed their representations of speech sounds based
function of electrode location, frequency-to-electrode mapping, on the tonotopic frequency-to-place functions inherent in the

52
t I I

cochlea's responses to acoustic input. Thus, a frequency mismatch are both much higher than the frequency range assigned to the
will cause stimulation patterns that differ from what these listen most apical electrode in these subjects. There were also subjects
ers expect, possibly affecting speech perception. whose most apical electrode was as little as 1 90 degrees or as
much as 507 degrees from the base, resulting in characteristic fre
Evidence of Partial or Complete Adaptation quencies ranging between 3,600 and 390 Hz, respectively.
to Frequency Mismatch in Cochlear Implant In summary, most subjects in this study likely experienced
some degree of basalward frequency mismatch-very small or
Users nonexistent in the case of the patient with the 507 degrees, much
It is useful to distinguish between the acute and the chronic more pronounced for the majority of subjects, and extremely
consequences of frequency mismatch on speech perception in large in the case of the patient with the shallow 1 90-degree inser
CI users. Because humans can adapt to some extent to a dis tion. Perhaps more importantly, Skinner et aP00 observed a signif
torted peripheral frequency map,93 - 98 it seems reasonable to icant correlation between insertion depth and speech recognition
hypothesize that the negative effect of frequency mismatch will scores. They speculated that subjects accommodated to the shift
be greatest shortly after initial stimulation (the acute phase), in frequency, but this accommodation was less successful for
and will decrease over time as the listener adapts to the new those with shallower electrode insertion, giving rise to the
input. Indeed, there is converging evidence that postlingually observed correlation. In other words, shallow insertions tend
deafened CI users do adapt, at least to some extent, to shifted or to result in greater frequency mismatch, which is harder to
distorted frequency tables. Evidence for this concept has been overcome completely. In turn, the uncompensated frequency
observed in several experimental paradigms: ( 1 ) longitudinal mismatch resulted in poorer speech recognition scores.
examination and mathematical modeling of vowel identifica Although most frequency mismatches are thought to be
tion and electrode discrimination after initial stimulation98; basalward in postlingually deafened CI users, there are a few
(2) analysis of perceptual vowel space in recently implanted97 documented cases of apicalward mismatch, due to deep
and long-term CI users95 ; (3) comparison of vowel perception electrode insertion. Two subjects described by Kos et aJ11 had
with different frequency-to-electrode tables before and after insertions of 720 degrees and complained of sound being "very
training93 ; (4) long-term studies of speech perception after low pitched," even after years of experience with their device.
imposition of a large frequency shift94; and ( 5 ) pitch matching When they were reprogrammed using the same frequency
between electrical and acoustic percepts in CI users with resid range delivered only to the six most basal electrodes (that is,
ual hearing.96 The latter study showed a remarkable amount of excluding the two most apical ones), speech perception scores
adaptation in users of the Hybrid-S CI, which is inserted only improved for both subjects by an average of 7 percentage point
1 0 mm into the cochlea in order to preserve residual hearing in s. One patient's scores improved further when the electrode
the implanted ear. Eleven of the 20 subjects were followed for was surgically pulled out to a more basal position. Another
at least a year, and for most of them the latest values of pitch study examined five subjects with deep insertions ranging from
matching were quite close (or at least got closer over time) to 605 to 720 degrees.102 All these subjects had higher vowel and
the frequency range assigned by their clinical processors. consonant identification scores when using tables that
On the other hand, there is also evidence that human CI users excluded one or more apical electrodes, which may have
do not have an infinite ability to adapt to frequency shifts. For resulted from minimizing the apicalward frequency mismatch.
example, Fu et al94 tested three subjects using a frequency table These studies support the idea that postlingually deafened Cl
that was about one octave lower than the standard clinical table users do not always adapt completely to frequency mismatch.
these subjects had been using for a long time. All three subjects A new important source of data about frequency mismatch
reached asymptotic levels of speech perception early in the 3- comes from studies examining the increasing numbers of lis
month period, but these levels were lower than those they had teners who have enough acoustic residual hearing to match the
obtained with the standard clinical table. Fu et al suggested that pitch of their acoustic and electrical hearing. The two largest
there might be a limitation in some subjects' ability to adapt to studies to date1D3.104 tested respectively 10 and 14 listeners
a severely shifted frequency map, and this was later confirmed who were implanted with full-size electrode arrays. The most
with mathematical modeling of their data.99 Additional evi apical electrode in the Nucleus device is typically associated
dence that some CI users might not completely adapt to their with a center frequency of 250 Hz. If these listeners had adapted
standard clinical frequency tables is provided by Skinner completely to their clinical frequency table, they should have
et al,100 who studied a group of 26 users of the Nucleus-22 CI matched the most apical electrode to a 250-Hz acoustic tone.
programmed with the SPEAK stimulation strategy. All these This was not the case, though. Francart et al's103 10 subjects
subjects had used their Cl for at least 1 year, likely allowing were all experienced Cl users, but only three of them had pitch
them to reach asymptotic speech perception levels. Following matches within the 240- to 260-Hz range, and only five were
standard fitting practice, the lowest frequency channel for most within the 1 95- to 3 1 0-Hz range. The other five matched their
subjects had a low-frequency edge of 1 20 to 1 50 Hz and a high most apical electrode to frequencies above the range of the cor
frequency edge of 280 to 350 Hz. The typical location of the responding filter, with values ranging from 343 to 483 Hz. In
most apical electrode, associated with the lowest frequency the McDermott et aP04 study, five Cl users were tested before
band, was 3 1 0 to 384 degrees from the beginning of the basal they had any implant experience, whereas the other nine
turn for most subjects. Using Stakhovskaya et al's91 human spi subjects had 7 to 48 months of CI use. The experienced
ral ganglion frequency map, these intracochlear electrode subjects pitch-matched their most apical electrode to different
locations correspond to estimated spiral ganglion neuron frequencies, ranging from about 250 to 500 Hz. Only two of the
characteristic frequencies of 1 ,200 and 725 Hz respectively, which 14 subjects matched their most apical electrodes to a frequency

53
. . . . . . . . . .

within the 1 88- to 3 1 3-Hz range. Interestingly, the five subjects along prespecified dimensions, such as the amount of gain a
who were tested before having any listening experience with hearing aid produces in a given frequency region. A matrix
the CI matched the percepts from the most apical electrode to (often 5 x 5) is created in which each cell corresponds to a
higher frequencies than the experienced users (577 to 884 Hz), signal with given characteristics. As one moves along the x- or
suggesting that postlingually deafened Nucleus users are sub y-axis, the value corresponding to that axis ( such as low
ject to large basalward frequency mismatches upon initial frequency or high-frequency gain) increases or decreases. A
stimulation and that the amount of mismatch presumably paired-comparison forced-choice procedure is then used to
decreases over time. Pitch-matching data from longitudinal compare different cells in the matrix, and an adaptive proce
studies involving subjects implanted with a short electrode array dure is used to determine the pair of stimuli to be compared.
(Nucleus Hybrid device96,IOS) also indicate decreases in the pitch For example, two sentences that varied along a given dimension
mismatch over time. These pitch-matching data suggest that would be presented consecutively, and the listener would
some subjects might not be able to adapt completely to this report which signal was more intelligible. This decision would
frequency mismatch even after months or years of experience. then determine the next set of comparisons, until a "winning"
Current clinical practice with postlingually deafened CI users cell with a given set of stimulus parameters has been identified.
is based on the implicit hypothesis that they will adapt to the This approach has been used primarily to fit hearing-aid
frequency tables that are in standard clinical use. However, the gain, 106,I07.n 4.1 1 5 although it has also been applied to other fea
literature reviewed here clearly shows that auditory plasticity tures such as noise reduction or spectral enhancement.l l 6,1 1 7
in humans has limitations. If listeners adapted completely to Genetic algorithms also make use o f comparisons that can be
standard frequency tables despite differences in insertion used to determine the optimal fitting parameters for a hearing aid
length and cochlear size, there should be no significant correla or a CJ.IIO In this procedure, a "gene" is a combination of different
tion between insertion angle and speech perception scores stimulus parameters that are prespecified by the experimenter.
but there are.IOO If listeners were able to adapt to any frequency For example, a study that used an acoustic simulation of a cochlear
table, the three subjects in the Fu et al94 study should have had implant included three parameters as genes: the number of noise
much higher scores with their experimental table after 3 months vocoder channels, the magnitude of frequency shift, and the total
of experience, but instead they reached low asymptotic scores frequency range.113 Different genes are then compared with one
after a month or so. Likewise, results obtained by Kos et aP01 another and the outcome of that comparison is used to determine
and Gani et aP02 show that CI users who might not have adapted whether a given gene is used in the next set of comparisons
to their standard frequency tables can improve their speech according to the genetic algorithm used. This approach is theoreti
perception scores with the use of alternative frequency tables. cally very flexible, and thus far has been utilized with acoustic
These alternative frequency tables might help reduce frequency simulations of a CJI12113 as well as with CI patients themselves.1 1 1
mismatch in cases where adaptation is incomplete. Finally, if Another approach i s to develop tools that allow quick successive
listeners were able to adapt completely to the frequency tables presentation of stimuli processed with different parameter set
used by the Nucleus speech processor, those who have residual tings (for example, different frequency tables) and have the lis
hearing would pitch-match the most apical electrode to 250 Hz. tener use a method-of-adjustment procedure to select the param
However, this only happens for about half of the subjects who eter or range of parameters that result in maximum perceived
have been studied. These results suggest that the human brain intelligibility. The speed at which different comparisons can be
might not be infinitely plastic when it comes to adapting to dif presented is likely to be important because an extensive body of
ferent peripheral frequency maps. research indicates that comparisons between different sensory
stimuli are most accurate when the time between each stimulus
Development of Tools for Frequency presentation is short.1 181 19 Over the last few years, two speech
processing platforms have been developed to implement this
Table Selection approach.120- I22 One of the tools developed is named RTStream,
Unfortunately, there are few tools, if any, that can be used to reflecting the fact that it streams processed speech in real time.
assess a listener's frequency mismatch between the characteristic RTStream allows the comparison of dozens of frequency tables
frequency of the stimulated neurons and what is assigned by the within a few minutes, which is at least an order of magnitude
frequency table. One possible way to address this issue is to allow faster than what is possible with currently available clinical tools.
the patient to select a preferred frequency table with the intent of The software needs to accomplish four simultaneous tasks: digi
maximizing speech intelligibility. The assumption underlying this tize the incoming signal, perform all necessary signal processing,
approach is that if a patient selects a frequency table that differs deliver the output signal, and monitor keyboard activity in order
from the standard, then the patient might not have fully adapted to change processing parameters in real time. A further complicat
to a potential frequency mismatch imposed by the standard fre ing factor is that the total processing time must be small (ideally,
quency table. This approach, however, requires a tool that can be below 20 ms) so that the electrical stimulation output remains
used to quickly and reliably select a frequency table that maxi synchronized with the speaker's face for audiovisual experiments.
mizes the patient's perceived speech intelligibility. To date, there Moreover, all these tasks must be completed without data dropout
have been few efforts directly addressing this issue, although even when the operating system (Windows) conducts housekeep
there are two procedures which have been previously investigated ing operations. The signal processing is implemented in Microsoft
and could theoretically be used to do so: the simplex proce C + + , and includes all the typical operations in a CI speech proces
dure106- I09 and the genetic algorithm.n o- 113 sor: automatic gain control, dynamic range compression, preem
The simplex procedure is an adaptive procedure that makes phasis, spectral analysis (using Butterworth 6th order filters),
use of paired comparisons between different stimuli differing envelope extraction from each filter output, acoustic-to-electrical

54
t I I

mapping for each stimulation channel, and channel selection ments with Cl users because the exact characteristic frequency of
according to either the CIS or ACE/n-of-m stimulation strategies. the neurons stimulated by a given electrode is not known. How
The output can also be used for an acoustic model of a Cl in which ever, it is possible to address the question indirectly by using
filter outputs modulate bands of band-passed noise that simulate normal-hearing subjects listening to an acoustic model of a Cl. In
electrodes with different intracochlear locations. this case, the noise bands that simulate each electrode can be
A second tool, called Gridstream, has also been developed at precisely specified, and the mismatch between the noise bands
New York University to select frequency tables for users of the and the filter bank selected by the listener can be determined
Nucleus-24 family of Cis. The graphical user interface consists with precision. It is possible that the frequency tables selected by
of a two-dimensional grid. When listeners click on a given listeners will be at least slightly different from a perfectly
square within the grid, they hear a sentence processed with a matched frequency table. Is speech perception with listener
given frequency table. As they click different squares, the selected tables as good as with a perfectly matched table?
parameters of the corresponding frequency table change Preliminary data obtained from normal-hearing listeners sug
systematically. Gridstream requires that the speech stimuli be gests that, when first exposed to an acoustic simulation of a CI
preprocessed for presentation, so the signal processing is not and told to find the frequency table that makes speech sound
actually done in real time with this tool. Instead, stimuli are most intelligible, listeners select a table that closely approximates
processed offline using a given listener's thresholds and most a frequency-matched table that has little frequency shift with
comfortable listening ( MCL) levels. However, Gridstream has respect to the noise bands used in the simulation. However, the
the advantage that it requires no custom hardware interface, average listener -selected table was shifted slightly in the direction
and it also allows for quick successive presentation of a spoken of a "right-information table" that contains most of the informa
sentence using many different frequency tables. tion important for speech perception and approximates the fre
quency range of a standard Cl clinical table (250 to 6,800 Hz). This
result suggests that listeners were attempting to minimize fre
Listener-Selected Tables in an Acoustic
quency shift while obtaining as much low-frequency information
Simulation of a Cochlear Implant as possible about the speech input. Average listener -selected
After developing the platform described in the previous section, tables, as well as representative individual results, are shown in
many feasibility questions arise. Can listeners consistently select Fig. 5.3 together with the frequency-matched table, and the
a preferred table? If so, is that table close to being frequency right-information table. These data are notable, because they sug
matched? Note that this question cannot be answered in experi- gest that self-selection of frequency tables can be a tool by which

1 6, 982 H z

1 0 , 000 6, 800 H z

N'
I
c
......

ClJ
CJl
c
ro

>.
u
c 1 , 000
ClJ 851 H z
::::l
o-
ClJ
'-
l.J.... 67 1 H z

1 00 ------
Rig ht L istener-Selec ted
Right
Information Place
F req u e n c y Ta b l es

Fig. 5.3 Frequency map selection by normal-hearing subjects listening to an eig ht-channel noise vocoder. The following ta bles are shown : rig ht
information (thick black bar on far left), perfectly matched (thick hashed bar on far right), mean listener-selected (thick gray bar), and representative
individual listener-selected maps (thin gray bars). Dashed lines indicate the edges of the perfectly matched map.

55
. . . . . . . . . .

a frequency-matched table can be approximated in actual Cl and intelligibility with different frequency tables, and could use
users; currently there are no available methods for doing so. those changes to guide their ability to select a preferred table
In this acute listening situation, these individuals understood that sounded most intelligible, supporting the feasibility of the
speech even better with the listener-selected table than with approach.
either the "right-place" or "right-information" tables. Phoneme Further, many listener-selected tables obtained in experi
scores for the listener-selected table (47.0%) were slightly but sig enced users of both Advanced Bionics and Cochlear Corporation
nificantly better (p 0.032) than those observed with the "right
= devices had frequency ranges that were reasonably close to the
place" table ( 43.4%), and were clearly superior (p < 0.001 ) to those ranges covered by their clinical table (the one they used every
obtained with the "right-information" table ( 1 8.7%). These data day), but were not always identical to it. However, other listen
suggest that, in acute listening situations (shortly after initial stim ers selected frequency tables that were different from their
ulation), postlingually deafened Cl users might perform better clinical ones. In both data sets, about half of the listeners
with a listener-selected table than with a standard clinical table. selected a low-frequency range that was consistent with their
clinical tables, and about half selected a low-frequency range
that was higher than their clinical table (only one Nucleus Cl
Listener-Selected Tables: Preliminary Results
user selected a frequency range that started lower than the clin
with Cochlear Implant Users ical one). These results suggest that as many as one half of
Listener-selected frequency tables in users of the Advanced postlingually deafened Cl users fail to adapt completely to the
Bionics and the Nucleus-24 Cis have recently been obtained at frequency range that is routinely assigned to the most apical
NYU. Almost all subjects could hear changes in sound quality electrode. Fig. 5.4 shows representative data from seven

6, 339 H z
1 0, 000

N'
I
c

<lJ
en
c
ro
0:::
>.
u
c 1 , 000
<lJ
:J
CT
<lJ
I...
I.J....

2 50 H z
- - - - - - - - - - 1-
426 H z
-

1 00 ------
Sta nd a rd Self-Selec ted Self-Selec ted
Experienced Naive

Freq u e n c y M a p s

Fig. 5.4 Frequency ranges of the standard clinical map (black bar) and the listener-selected maps from seven representative users of the Advanced
Bionics 90K Cl (gray bars). Six were long-term users and the seventh (rightmost bar) was only 1 month post-initial stimu lation .

56
t I I

Advanced Bionics patients, six of whom had at least 1 year of auditory nerve. Initial work included attempts to extract
listening experience with their Cl and one naive listener who speech parameters from the acoustic stimulus. As a general
was tested only 1 month after initial stimulation. Taken rule, delivery of more speech information yielded a better
together, these frequency table selection data suggest that some clinical outcome. Later work showed that interleaved "peak
listeners might have not fully adapted to their clinical frequency picking" strategies were more successful. These are currently
table, or that they may find speech more intelligible when using implemented in the Med-El "n-of-m" strategy and in
a frequency table with a different bandwidth. The newly Cochlear Corporation's SPEAK and ACE strategies. The pri
implanted individual who was tested ( rightmost bar in mary limitation with all sound processors is the inability of
Fig. 5.4), selected a frequency table whose low end was at users to recognize speech in noise, to recognize tonal
426 Hz, almost an octave higher than the low end of her speech, and to hear musical melodies. These limitations
clinical table, and higher than (or equal to ) the low ends of likely result from the limited electric dynamic range, from
the tables selected by most of the experienced Advanced the inability of the sound processors to transmit sufficient
Bionics users. spectral resolution and temporal fine structure, and from
Some of the subjects were able to stay in the laboratory to the frequency mismatches in Cl users.
obtain speech perception scores with two frequency tables : A number of active compression approaches such as ADRO
their listener-selected one and a close approximation of their have been introduced to address the dynamic range problem.
clinical table. Most of these scores were obtained with two Another approach uses a conditioning stimulus. A conditioning
50-word consonant-nucleus-consonant (CNC) word lists for stimulus could be applied to any strategy to increase the
each table. In a few cases, other tests were used, for example dynamic range and possibly improve perceptual abilities.
to avoid floor effects in subjects whose CNC word scores Although still experimental, a conditioning stimulus has pro
were zero. In the case of Advanced Bionics users, speech test vided substantial improvements in approximately one third of
ing with both tables was done using RTStream, to make sure cochlear implantees tested at the University of Iowa and the
that any differences would be due exclusively to the fre University of Washington.
quency table itself and not to factors like the microphone Although physiological limitations slow progress toward
used, input dynamic range, AGC parameters, loudness growth improving spectral resolution, some signal-processing
curves, and many other ones that are potentially different in approaches have addressed the problem. Current steering
RTStream with respect to the clinical speech processor. In the implemented in HiRes Fidelity 1 20 has been shown to improve
case of Nucleus users, they were tested with their own performance on spectral tasks, but current steering has not
speech processors, which were programmed to a close demonstrated consistent clinical benefit for speech or music
approximation of their self-selected frequency table. Most of perception. Delivery of temporal fine structure is absent in
the experienced subjects who were tested did as well with most modern processing strategies, but successful delivery of
their listener-selected table as with the clinical table despite this information to Cl users could provide numerous clinical
the limited exposure they had to the listener-selected table. benefits. Many attempts have been made to transmit temporal
About one third of the subjects showed improvements fine structure. Med-El has a temporal fine structure processing
between 9% and 1 6% when using the self-selected tables. program available, but this has not been shown to provide clini
The most striking difference in performance between the cal benefit when tested with matching frequency maps. Two
listener-selected and clinical tables was observed in the other promising strategies currently under study include
recently implanted Advanced Bionics user, who reported FOmod8l . S2 and HSSE83 - ss developed at Leuven, Belgium, and at
little to no open-set speech understanding at that time. the University of Washington, respectively. Both strategies pro
Consistent with her report. she scored only 2% correct on a vide fundamental frequency cues across all electrodes, thus
Hearing-in-Noise Test ( HINT) sentence list. In contrast, with providing some temporal fine-structure information to users.
her listener-selected table, she scored 1 1 % correct, and These strategies show possible benefits for music perception
reported that she felt she could understand speech much and for tonal language perception. It appears that Cl listeners
better with this table. Globally, these results suggest that cannot completely adapt over time to frequency mismatches
use of a listener-selected table does not hinder speech per and that this mismatch influences speech perception abilities.
ception, even on an acute basis, and may even help a limited A number of approaches are being explored to develop an
percentage of Cl users. It is possible that a greater number efficient method of generating a better frequency map dur
of Cl users might benefit from the use of a self-selected fre ing clinical fitting. All of the efforts described here endeavor
quency table, provided that a sufficient amount of everyday to provide better hearing with a cochlear implant. Some
listening experience with the novel frequency table is or all of these approaches could yield improved clinical
allowed. performance.

Conclusion Acknowledgments
At first, single-channel devices were employed with mar Elizabeth Anderson provided helpful comments on an earlier
ginal success; their users had the benefit of acoustic aware version of this manuscript. This work was supported by the VM
ness, but were, for the most part, poor at understanding Bloedel Hearing Research Center and National Institutes of
speech. Later. multichannel devices were employed using Health grants DC007525, DC0 1 0 1 48, DC00242, DC003937,
simultaneous and successive electrical stimulation of the DC0 1 1 329, and DC009459.

57
. . . .

[291 Battmer RD, Zilberman Y, Haake P, Lenarz T. Simultaneous analog stimulation


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I . I . . . . . . . . . . . .

6 Expa n d i ng Crite ria fo r the Eva l uatio n of Coc h l e a r


I m p l a nt Ca nd idates
Susan Arndt, Roland Laszig, Antje Aschendorff, and Rainer Beck

Introduction Diagnostics of Hearing Loss


Reliable diagnosis of bilateral hearing loss in children younger
Cochlear implantation has developed from an experimental
than 1 year of age is controversial, and there is an inherent
treatment of bilaterally deaf adult patients with permanent
danger of misdiagnosis and a potential risk of overlooking the
sensorineural profound hearing loss in the early 1 980s to a rec
presence and subsequent impact of additional disabilities that
ognized clinical form of therapy for a growing group of hearing
have not yet manifested at such a young age. Moreover, there is
impaired individuals of all ages. The indications for cochlear
the possibility of unpredicted improvement in hearing resulting
implantation have been continuously expanded and are still
from maturation of the hearing pathways. Examinations should
evolving. Initially, bilateral sensorineural profound deafness in
consist of visual reinforcement audiometry (VRA), and objective
adults was considered the sole indication for cochlear implanta
auditory tests, such as transient-evoked and distortion-product
tion. Shortly thereafter, bilaterally deaf children were also
otoacoustic emission (TEOAE and DPOAE), tympanometry and
treated with a unilateral cochlear implant (CI). Nowadays, bilat
acoustic reflex testing, and auditory brainstem response (ABR).
eral cochlear implantation in children is in many areas consid
All objective methods have limitations either in identification
ered routine clinical practice. In recent decades the indications
of mild hearing loss ( OAE, ABR, VRA) or in the differentiation
have been expanded to include patients with low-grade usable
of severe hearing loss from profound hearing loss (ABR).2 More
residual hearing, who could not achieve adequate speech
frequency-specific measurements can be performed with the
understanding even with an optimally fitted digital hearing
auditory steady- state response (ASSR), and thus higher specific
aid. The technological developments in Cis and the results
ity and sensitivity with respect to diagnostics of hearing
achieved have led to discussions and reports of cochlear
thresholds in children are available.3 Using electrocochleogra
implantation even in difficult cases. This chapter presents a
phy ( ECoG), which determines the cochlear microphonic (CM),
current overview of the challenges, conditions. and results of
cochlear summating potential, and compound action potential
expanded indications for cochlear implantation and the critical
(CAP), the diagnosis can be confirmed in unclear cases, or it
considerations for patient selection, evaluation, and counseling
is possible to diagnose or rule out auditory neuropathy/
for these indications.
dyssynchrony and examine children with multiple handicaps.
This examination requires sedation or general anesthesia of
Age at Cochlear Implantation the children, which is often performed in conjunction with
radiological examination. These examination methods should
Careful patient selection is one of the most important prerequi enable determination of hearing threshold in infants, so that a
sites for the success of cochlear implantation, not only in hearing aid trial can be initiated. The majority of authors con
children but also in adolescents and adults. Widespread intro sider a hearing aid trial for a predefined clinically acceptable
duction of neonatal auditory screening ( NHS) has enabled early time frame as obligatory"S to formally document a lack of or
diagnosis of children with uni - or bilateral hearing impairment. only minimal improvement in auditory performance over the
In addition, the number of elderly patients, 70 years of age or time frame.
older, is increasing. The aging population will be accompanied Contrary to this view, Colletti et al6 recommend performing
by an increase in the prevalence and severity of hearing loss, a computed tomography (CT) and magnetic resonance imag
due to the direct correlation between age and hearing loss. ing ( MRI) examination in infants without cochlear micro
Therefore, cochlear implantation is considered a routine inter phonics and without compound action potentials, as well as
vention to compensate for the negative effects of substantial reevaluation by a pediatric neurologist, and performing
hearing loss, regardless of age. cochlear implantation without previous hearing aid trial to
avoid delaying auditory stimulation to the deprived auditory
pathways any longer. At hearing thresholds at or better than
Pediatric Cochlear Implantation 75 dB hearing level ( HL) for objective auditory test measures,
Due to the implementation of NHS programs in many countries, Colletti et al6 recommend a hearing aid trial phase of 3 months
it is now possible to reliably identify children with high-grade prior to making the decision for Cl candidacy. The additional
deafness in the first months of life and to initiate further diag use of questionnaires by the parents, both in suspected hearing
nostics and the decision process for therapeutic treatments.1 impairment and after the hearing aid test, such as the Infant
The resultant possibilities and consequences raise the question Toddler Meaningful Auditory Integration Scale (IT-MAIS), a
of the potential benefit versus the risks of cochlear implantation parental survey of early auditory development, can confirm
in children prior to 1 year of age, balancing the anticipated ben or strengthen the observations made through objective mea
efits, such as improved and accelerated hearing and speech surements?
development, against the potential for age-related issues, such Use of an extensive battery of subjective and objective diag
as the reliability of the available diagnostics, the inherent surgi nostic methods that should be repeated and compared over
cal risks, and the rehabilitation process. time can serve to reliably confirm the presence of a permanent

61
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bilateral hearing loss, especially in the event the hearing aid very thin-less than 1 mm. As consequence, creation of an
trial period has resulted in no measurable additional benefits. adequate bone bed for secured placement of the implant
High-resolution computed tomography (HRCT) and MRI enable receiver-stimulator is often not possible. Many authors have
diagnosis of inner-ear malformations and auditory nerve recommended circular exposition of the dura, formation of a
aplasias. The use of CT alone, MRI alone, or the combined use firm soft tissue pocket, and fixation of the implant with a
of both varies in clinical practice and remains a topic of contro suture.13-16 With sufficient attention to the conditions cited
versy. In the event auditory nerve aplasia is suspected, MRI is above for the young infant and restricting surgery time to under
indicated as it is the most clinically sensitive method to identify 90 minutes, the surgeon can keep the inherent surgical risks at
such physical anatomic anomalies. the same level as that experienced for the older child. Long
term safety following strict surgical procedures has been dem
onstrated by researchers with a follow-up period as long as
Anesthesia Risk 4 and 6.8 years.1015 Postoperative complications reported by
In addition to concern about a surgical procedure in their new researchers are comparable to incidence reported for implant
born child, parents are also afraid of the side effects of the procedures performed in older children.
anesthesia for their child. Earlier studies have, in fact, shown
that children younger than 12 months have a greater anesthesia
Outcomes in Speech Perception and Language
risk with respect to morbidity, mortality, and life-threatening
adverse events than for children 12 months of age or older.s.9 Development
More recent studies and several publications on successful In the past 10 years, many authors have demonstrated that the
anesthesia of infants younger than 12 months of age suggest results of speech production and speech understanding in chil
that healthy children do not have an elevated cardiorespiratory dren implanted at under 24 months of age were significantly
risk.6.7.l O jiihr et aJ1 1 differentiated between patient-related risks better than for children implanted later.71517-19 Based on these
and anesthesia risks. The patient-related risks include the excellent results, following early diagnosis, ideally implantation
young age of the infant, which leads to the ongoing discussion should be performed between the first and second year of life.
of the potential risk of anesthetic agents. Moreover, the This is also based on the knowledge that the physiological
patients' small size and low body weight are significant factors. maturation of the hearing pathways occurs maximally during a
The ratio of a relatively large body surface compared with the critical window of development that is limited to a very short
low body weight coupled with thin, well-perfused skin requires period in the first years of life. The auditory system has maxi
very close control of body temperature to prevent hyperther mum plasticity in the first 2 to 3.5 years of life. Moreover, there
mia during surgery. Another issue is blood volume. Rapid blood appears to be limited plasticity up to the seventh year, but with
loss in the first months of life leads to low hemoglobin concen slower and more limited progress of auditory skill development
tration.1 1 The young pulmonary system with a considerably following implantation.20 Other studies have shown that there
higher oxygen consumption compared with the low body is another sensitive phase from the 6th month of gestation to
weight is also a factor for consideration in assessing age-related the 1 2th month after birth that affects the capacity for phono
risks. With respect to the cardiovascular system, unpredicted logical skills.21 Dettman et al22 made the assumption that
cardiac diseases may be revealed by general anesthesia even in delayed phonological distinction (later than 1 2 months after
apparently healthy infants. The anesthetist's experience in the birth) can lead to longer lasting difficulties in language process
pediatric population is a potential influencing factor upon ing abilities. They compared the communication abilities of 1 9
the rate of complications arising from the anesthesia.1 1 For this children implanted under 1 2 months o f age with those of
reason, infants at a very young age should undergo surgery only children who were older than 12 months at the time of implan
in dedicated hospitals with appropriate knowledge and exper tation. The children implanted earlier showed significantly
tise in anesthesia and the ready availability of special intensive better receptive and language skills than the children implanted
care wards for neonates.9-12 Longer operation times, such as later, as measured with the Rosetti Infant-Toddler Language
may be needed for bilateral implantation in the young infant, Scale ( RI-TLS). The results for the younger implantees were
without complications are possible under good control of the comparable to those for normal-hearing peers. Holt and
anesthesia and monitoring of the child during and after surgery. Svirsky20 were also able to demonstrate better receptive
Nonetheless, the shorter the operation time and the anesthesia language skills in children implanted under the age of 1 year,
time, and the smaller the physiological impact, the lower the but found no difference with respect to expressive skills.
risk of complications under the supervision of an experienced In an uncontrolled observational study, Colletti et al6 com
anesthetist. pared four groups of infants implanted at successive chronolog
ical ages-between 2 and 6 months (n = 1 2), between 7 and
12 months (n = 9), between 13 and 18 months (n = 1 1 ), and
Surgical Risk between 19 and 24 months (n = 1 3 )-after 48 months' follow
The most significant potential problem during surgery is exces up time. The infants implanted before the age of 6 months
sive blood loss. Bone marrow and emissary veins have the showed significantly better results in auditory performance
greatest danger of trauma leading to significant blood loss. In (Categories of Auditory Perception Scales II), in receptive
the young infant the mastoid bone is often still only slightly vocabulary ( Peabody Picture Vocabulary Test-revised [ PPVT
pneumatized, and the facial nerve is positioned close to the R]), and in a speech production test ( Bortolino Test) than did
surface and laterally. The middle ear and inner ear have already the older age groups tested postimplantation. No significant
reached adult proportions. In addition, the skull bone is often differences were found in word and sentence comprehension

62
O , I . . . . . . . . . . . .

scores among the groups. The authors attribute the significantly with the Oldenburg Inventar questionnaire. Also, postoperative
better results observed in the youngest group to early auditory tinnitus annoyance and perceived stress was reduced to a simi
stimulation during the early critical and sensitive phase lar level in both groups. The pre- to postoperative improvement
responsible for phonological development and later for spoken in disease-specific quality-of-life scores measured with the Nij
language. Of course, longer term results must be obtained, and megen Cochlear Implant Questionnaire was more pronounced
a larger number of infants is required to substantiate this in the elderly than in the younger group of study participants
advantage of very early implantation further. (p < 0.0001 ). Elderly CI recipients were found to seek less post
operative support in coping with their hearing loss (p < 0.05 ).
Carlson et aP2 reported similar postoperative complication
Cochlear Implantation in the Elderly rates in a group of 50 Cl users with age at implantation of
Globally, hearing loss is the leading cause of disability, and the 80 + years compared with a group of 208 younger recipients.
prevalence of hearing loss is particularly high in the elderly. In a Because on average the elderly had worse preoperative physical
study sample representative for the United States population, Lin status classifications, there were more anesthesia-related com
et at23 found mild or worse hearing loss in 63% of individuals plications in that group leading to a reduced percentage of out
over the age of 70. Moderate and severe or profound hearing loss patient treatments. Nevertheless, the authors conclude that,
was prevalent in 26% and 0.6%, respectively. Old age is not a con given thorough preoperative planning, the favorable safety pro
traindication to cochlear implantation. In general, elderly hear file warrants routine cochlear implantation in octogenarians
ing-impaired individuals following implantation can expect to and nonagenarians. Coelho et aP3 reviewed the medical charts
achieve similar postoperative hearing abilities as their younger of 70 CI recipients age 70 + , which they divided into a group
counterparts. General anesthesia is well tolerated by elderly with lower (American Society of Anesthesiologists [ASA] I/11,
patients undergoing cochlear implantation, and the intraopera n 44) and higher (ASA lll/lV, n 26) anesthesia risk. There were
= =

tive complication rate is more related to preoperative risk than to no anesthesia-related complications in the lower risk group and
age per se. The need to undergo full anesthesia has been identi 1 2% of the individuals in the higher risk group experienced
fied as the major obstacle for CI candidates in accepting treat complications with temporary consequences.
ment. The reasons for this potential under-referral might be
rooted in a lack of knowledge concerning the safety of cochlear Summary
implantation and in unclear expectations among referring profes
sionals and candidates about the hearing benefits for the elderly. The positive auditory-based results following implantation in
Though age, hearing loss, and cognitive loss are positively inter bilaterally deaf infants under 1 2 months of age indicates that
related,2425 and auditory processing abilities have been shown to early intervention is both appropriate and necessary for many
decline with age,2627 cochlear implantation in the elderly is aspects of the child's development, such as early auditory and
highly effective, and hearing performance postimplantation is verbal communication with their parents, and from a socio
considered close to or the same as the level achieved by younger economic point of view. The risks of surgery and anesthesia can
recipients. In a sample of 78 postlingually deafened individuals be reduced to a minimum if undertaken in specialized centers.
aged 65 + years, Friedland et a[28 did not find a significant correla Prerequisites to undertake early intervention include reliable
tion between age at implantation and 1 -year postoperative hear diagnosis of profound hearing loss, professional counseling of
ing performance in quiet conditions, though a moderate trend the parents, an experienced and excellently trained pediatric
toward a decrease in speech understanding tested on sentences anesthesia team with provision for pediatric intensive care
in noise was reported. Similarly, Budenz et at29 compared 60 facilities, surgeons experienced in neonatal surgery, and experi
individuals with age at implant of 70 + years to a group of 48 enced pediatric audiologists, speech therapists, and teachers.
younger CI users with matched preoperative performance. They Cochlear implantation is considered a routine intervention to
found similar intergroup performance for sentence scores in compensate for the negative effects of substantial hearing loss,
quiet and noise and slightly higher consonant-nucleus-consonant regardless of age. Further clinical experience is needed to better
(CNC) scores in the younger group. This difference correlated predict the additional benefit of bilateral cochlear implantation
more with duration of deafness than with age at implant. for selected elderly individuals. Special consideration should
Noble et aP0 concluded from the literature that "adult chron be given to preoperative counseling regarding the overall
ological age does not seem to have a bearing on the effective medical assessment of the patient and any possible existing
ness of cochlear implantation." In their own data, they did not comorbidities that may have additional impact on surgery, as
find a significant difference in word recognition or localization well as postimplant care after surgery.
ability between CJ users above and below 60 years of age at
implantation. Using the Hearing Handicap Inventory for the
Elderly and the Hearing Handicap Questionnaire, Noble et al
Bilateral Cochlear Implantation
found similar levels of postoperative hearing-related handicap and Cochlear Implant in Single
in both age groups, with a trend toward a larger pre- to post
operative handicap reduction in the group of unilaterally Sided Sensorineural Deafness and
implanted elderly; the trend was the opposite in the bilateral CI
Asymmetric Hearing loss
condition. Olze et aP1 compared a group of 20 unilateral CI
users with age at implantation of 70 + years to a group of 35 Whereas the difference between hearing in one ear and not
younger recipients. Both groups achieved similar postoperative hearing at all is quite evident, the functional differences to daily
auditory performance in quiet and in noise and as measured life between hearing in one ear only ( monaural) versus hearing

63
. . . . .. .

in both ears (binaural) is much more difficult to discern. interaural time difference ( ITO) and interaural level difference
Multiple effects, among them the differing spatial position of ( ILD) in comparison to the ear more distant from the sound
both ears and a very distinct central processing relying on source. In addition, the form and mass of the head dampens the
input from each ear, come into play and result in the emer wavefront on its way to the second ear. This effect is more
gence of binaural hearing. Some components are well under prominent for the higher frequencies, as the lower frequencies
stood, but others are still too complex to be easily applied to diffract around the skull due to their longer wavelengths
everyday situations. Therefore, the problems associated with instead of being reflected or absorbed. The head therefore
monaural hearing shall be described first, followed by a sum creates a "sound shadow" for the sound components with
mary of the most relevant psychoacoustic effects contribu higher frequencies ( > 2,000 Hz). Whereas theoretically the ITO
ting to a binaural gain, leading to the discussion of bilateral is not frequency specific, it is only measurable if the peaks
cochlear implantation and the treatment of unilateral hearing arriving at both ears can be correlated clearly. This is only true
loss (UHL). for frequencies below 800 Hz, as shorter wavelengths do not
allow for clear mapping of the corresponding peaks. ITO and
ILD are therefore basic cues used in binaural hearing, ITO being
The Crux of Monaural Hearing
relevant for low-frequency sounds, ILD for high-frequency
In everyday life, the landscape of sound surrounding us is com sounds, resulting in the head shadow effect, the summation
plex: relevant information is interwoven with distracting sound effect, and the squelch effect.40
sources like moving cars or the ringing of cutlery. To separate
relevant from irrelevant acoustic events, the auditory system
constantly processes and evaluates the stream of sound and Head Shadow Effect
raises the bits we try to follow to our consciousness, being ever When noise and target signal are positioned on opposite sides
ready to change the focus at a moment's notice. This phenome of the head, the ear ipsilateral to the target source yields a
non is referred to as auditory scene analysis 1 and is essential for better SNR due to the ILD and thus does not require any special
verbal communication in an environment where noise is ever central processing. In people with good bilateral hearing, one
present. In this respect, school classes are prototypical for a dif ear is always closer to the target source, whereas in people
ficult listening situation. Everything a teacher or a classmate with unilateral hearing, the "good side" may well point away
utters has to be understood, so the student cannot be from the target source, making listening all the more difficult
disturbed by the noises produced by the rest of the class. This because of the head shadow effect.
difficulty leads to misunderstanding and missing clarification of
facts in monaural-hearing children (e.g., UHL, dysplasia of the
ear canal). Therefore, they tend to perform worse than their Summation Effect
normal-hearing peers and have a higher risk of needing to The threshold for a sound that is presented to both ears simul
repeat a school year.34-36 Special seating, optimizing acoustics, taneously is 3 dB softer than the hearing threshold of any ear
or technical support like wireless transmitters designed to alone. This may contribute to increased speech understanding
increase the signal-to-noise ratio (SNR) for monaural-hearing in difficult listening situations but is helpful only if the compet
children may attenuate some of this effect.3738 Adults with UHL ing sound source is positioned differently and does not benefit
will suffer the same limitations. Their environment usually is from the summation effect as well.
not as restrictive as a school setting, making it possible to adapt
to their hearing impairment in different ways. Monaural-hear
ing adults may adapt seating arrangements for their benefit or Squelch Effect
limit their professional choices to avoid difficult listening Using ITO, ILD, and spectral differences, the brainstem is able to
situations. But they still experience stress related to verbal com extract a clearer representation of speech and noise signals
munication, resulting in a loss of quality of life.36 Although less stemming from spatially different acoustic sources. This results
impairing than limited speech understanding in noise, the in effective suppression of competing sounds and heightens
inability to localize sound sources is a hindrance as well. Some speech understanding.
monaural-hearing persons implement strategies like head
turning to achieve good results at localization tasks,36,39 but
most are severely limited. This results in potentially dangerous Localization
situations in traffic, as well as nuisances like elevator doors Differences in ITO and ILD are the main sources for the localiza
closing before the available elevator could be identified. tion of sound sources. As both components require the wave
front to reach both ears, listeners are able to localize sound
sources positioned in front of them most precisely and poorest
Building Blocks of
when they are positioned to either side.
Binaural Perception
As a sound reaches one ear, the percept of the second ear differs
Bilateral Cochlear Implantation
mostly in two aspects : amplitude and latency. Both are a direct
result of the spatial arrangement of the sensory organs and the Normally, humans perceive sound with two working ears ; that
resulting path of the respective wavefront. In most cases, one is, binaural hearing is the natural listening condition for the
ear will be closer to the sound source and the wavefront will auditory system. Keeping the implications of monaural hearing
arrive earlier and with higher amplitude, resulting in a specific in mind, bilateral cochlear implantation can be seen as a tool

64
Expanding Criteria for the Evaluation of Cochlear Implant Candidates

for restoring "natural" binaural hearing. The potential benefits because neural pathways form in the first years of life. In
of bilateral cochlear implantation are based on several different addition, other factors such as the amount of hearing aid use,
effects : physical benefits, benefits due to central processing, etiology of hearing loss, or the time elapsing from detection of
and medical considerations. sound to electrical stimulation of the auditory nerve may be
influential. It is very difficult to control all covariates, making it
hard to observe the effects of central signal processing; never
Physical Benefits theless, some evidence of true, working, binaural hearing pro
Two autonomous implants on opposite sides of the head pro cesses exists in some patients. In addition to the summation
vide two distinct signals with the obvious feature of making an effect, some users of bilateral Cis demonstrate an additional
improved SNR possible, as well as providing redundancy as a gain of 1 to 2 dB SNR if the sound and noise source are located
backup system in the case of a device failure. In the future, as frontally. This may be due to binaural redundancy, as both ears
more children are implanted earlier and the general Cl popula receive identical (and partially complementary) parts of the
tion grows older, the per-lifetime likelihood of device failure auditory information, allowing for increased sensitivity to
may increase. This is especially true in young children because intensity and frequency. This effect cannot be seen in all
head trauma is the major cause of defective implants in early patients with bilateral Cis, as it may depend on the history of
childhood. Loss of hearing due to device failure can be amelio hearing loss.44.45 Similarly, the binaural squelch effect can only
rated only by diagnostics, surgery, and postoperative rehabilita be demonstrated in some patients.46 This is because the mecha
tion, taking 4 to 6 months until usable hearing is restored. Being nism requires developed structures in the brainstem, and it
mainstreamed in the education system and, later, in profes may be even more susceptible to the history of hearing loss.
sional life means that falling back into deafness is unacceptable Although binaural squelch offers advantages, the head shadow
for the patients suffering a device failure. A second Cl provides effect already contributes more gain in SNR.
security and stability as it serves as a fail-safe option and as a The last substantial quality of binaural sound processing is
link to the environment that is supported by oral-verbal com localization. Where monaurally hearing people may show some
munication. It provides a level of security to the parents and ability to locate sound sources, this is predominately a function
eases decisions regarding recreational activities or, eventually, of bilateral hearing. The essential auditory clues are provided
choices in personal career for the patient. Notwithstanding by lTD and ILD, as described above. The ILD can be perceived
other benefits, redundancy alone may be considered an argu when using bilateral Cis under the condition that both implants
ment sufficient for bilateral cochlear implantation. are fitted to elicit similar loudness percepts ; that is, loudness
The physical separation of two microphone arrays attached balancing has been performed between the two implants. To
to two different implants results in another benefit of bilateral discriminate small differences in arrival time of wave fronts,
implantation: there will always be one ear/microphone array signal processing of both sides should be "in synch." The delay
that is located closer to the sound source. This simple circum due to converting signals from acoustic to electric stimuli must
stance provides a better SNR and potentially increases speech be very small and comparable on both sides. This, however,
understanding. Patients with Cis are very sensitive to input may not always be the case for bilateral Cis as signal processing
SNR because the signal transmission is limited by the dynamic strategies may be different due to independent fitting strategies
ranges of the electrode/neural interface, thus providing a or the usage of different speech processor models. It can be con
reduced output -SNR in the first place. In addition, Cl users are cluded that localization abilities are best in normal-hearing per
open to being addressed from both sides because they are able sons, followed by hearing aid users (preservation of temporal
to discern sound sources bilaterally and do not depend on a cues), and lastly by those with bilateral Cls.47 Nevertheless, indi
single locus of sound perception-a reliable, valuable, and stable viduals with bilateral Cis perform better than those implanted
effect, observable in everyday life. The benefit described is monaurally due to their ability to estimate ILD.41 Whether some
related to the head shadow effect that provides increased SNR patients are able to use ITO and the specific circumstances
due to the dampening of unwanted sound sources opposite to are the subject of intensive research.
the receiving microphone array simply because of the head
being between the two microphones. Therefore, this effect can
be seen in all patients with bilateral Cis, especially for those with
Medical Considerations
reasonable speech understanding in both ears.41-43 Equally Recommending bilateral cochlear implantation should not be
important is the summation effect also seen in almost all motivated only by the prospect for true binaural processing
patients implanted bilaterally. It is manifested as reduced current strategies, but should also be motivated by the intention to pro
levels when C-levels (comfortable G listening levels) are adjusted vide the best achievable speech perception in quiet conditions
for the bilateral versus the unilateral hearing condition. as well. This is most clearly observed in patients having limited
speech understanding who are using their first Cl because they
benefit mostly from the increased acoustic information availa
Benefits Due to Central Processing ble to them.48 Because neural pathways break down as a result
Compared with the head shadow and the summation effects, of longer periods of nonuse or are not formed to begin with,
benefits arising purely from binaural signal processing strate patients receiving simultaneous bilateral Cis or one implant
gies of the brain are much harder to verify. It is evident that after the other with a short delay ( < 2 years) show better out
neural networks in charge of binaural processing develop dif comes and have a better potential to benefit from binaural
ferently in individuals suffering from congenital deafness when signal processing. This is especially true for children born with
compared with those with late-onset, progressive hearing loss severe hearing loss, as prolonged monaural stimulation may, in

65
. . . . . . .

fact, inhibit the development of contralateral pathways; there together on one side), the question arises as to whether cen
fore, bilateral implantation is most effective when performed tral integration of electrical stimulation on the one side and
early in life.49 Lastly, implantation of both ears ensures that the acoustic stimulation on the other side may be possible to ena
side with better preconditions will always be an ear of choice ble binaural hearing in patients with SSD. Preliminary reports
for implantation. This is a major consideration because it is the of successful cochlear implantations in patients with UHL and
usual practice that the ear with less residual hearing or longer normal hearing or moderate hearing impairment on the con
term deafness is implanted first, providing limited benefits tralateral side were published in 2008.54 The intention of
compared with the results of the better-hearing ear. cochlear implantation in these 2 1 patients was not, however,
binaural hearing rehabilitation and improvement of direc
tional hearing, but rather the treatment of very bothersome
Summary tinnitus. Researchers have reported a reduction or total sup
Most benefits from bilateral cochlear implantation can be pression of tinnitus while using the implant reported on
traced back to relatively simple conditions: redundancy with visual assessment scales and subjective questionnaires ; upon
respect to device failure, separate positions of microphone switching the Cl off, the tinnitus returns to the original levels.
arrays allowing for the head shadow or summation effect, In addition to tinnitus reduction, these patients reported con
increased localization skills, and guaranteed implantation of siderably improved speech understanding in competitive
the ear showing the best preconditions. Whether true binaural noise and in directional hearing. 55 With these positive results,
processing strategies play a role in the benefits achieved for the authors have thus refuted the initial reservation that the
individual Cl users is still unclear and mostly an academic dominance of the better or normal-hearing ear over the Cl
consideration. The most significant consideration is that advan implanted ear or interference with sound perception in the
tages gained from bilateral implantation are experienced by contralateral ear may lead to possible discontinued use or
individual Cl users in real-life situations. even nonuse of the Cl. Since then, the published studies of
AHL patients have shown benefits for speech understanding
in spatially separated and coincidentally presented competing
Cochlear Implant in Sing le noise,ss - ss localization ability, 55 - 57-59 and subjectively through
standard assessment scales over the better ear alone condi
Sided Sensorineural Deafness tion.55 - 59 Furthermore, the evidence suggests that central
and Asymmetrical Hearing Loss processing, known as binaural integration of the signal arriv
ing through the Cl to the poorer ear with the auditory signal
Asymmetric hearing loss (AHL) is defined as normal or close to received by the normal-hearing ear, is possible. 55-57-59,60
normal hearing in one ear and severe to profound unilateral Arndt et at56,57 published reports on successively examined
sensorineural hearing loss in the other. Profound AHL, also SSD adults enrolled in a single-subject prospective comparative
referred to as single-sided sensorineural deafness (SSD), is an study design involving repeated longitudinal measures of hear
example of the most extreme asymmetrical hearing loss config ing performance to assess outcomes for conventional hearing
uration, that is, where the poorer ear presents with profound impairment treatments, and also weighed the outcomes against
sensorineural deafness and the contralateral ear displays nor the benefits obtained from Cl in this population. They con
mal hearing or close to normal hearing thresholds (i.e., up to a cluded from a controlled clinical comparison of treatments over
mild hearing loss with pure-tone average ( PTA] thresholds of 1 1 subjects undergoing Cl treatment that a significant improve
40 dB HL or less ).50 Asymmetrical hearing loss (AHL) has not yet ment was noted even as early as 6 months post-CI surgery over
been defined, but the difference between the PTA of the better performance with CROS and bone-anchored hearing aids ( Baha)
ear and that of the poorer ear should be at least 30 dB to comply devices in a variety of daily listening situations ( Fig. 6. 1 ;
with the asymmetry criterion. Fig. 6.2). Furthermore, they report consistent daily use of the
So far, two types of amplification systems are available for Cl in conjunction with the normal-hearing ear. In addition,
patients with unilateral hearing loss that transfer signals arriv within 6 to 12 months post-CI surgery, binaural hearing benefit
ing at the side of the head with the poorer ear to the better for localization, speech understanding in noise, and absence of
hearing ear via contralateral routing of sound. This is achieved any detrimental effects upon hearing with the contralateral ear
either externally using a contralateral routing of offside signals were demonstrated. Moreover, they reported a significant
(CROS ) hearing aid, or transcranially via application of a bone reduction in tinnitus in subjects using the Cl and overall
conduction auditory implant (bone-anchored hearing systems improvement in the quality of life.5657 The subjects scored
( Baha ] ). But these two systems are not able to offer the patient better in all sections of the Speech, Spatial, and Qualities of
the advantages of binaural hearing, which requires sound to Hearing (SSQ) scale with the Cl ( Fig. 6.3 ).
arrive at each ear independently for the processing of minute To date, no report has been published investigating the bene
differences between the sounds from each ear by the brain as fits of a Cl in children with SSD and AHL. Our own experience of
described before. two children with acquired SSD and AHL using the same evalu
After reports of successful bimodal cochlear implantation ations as for our adult patients confirms significant benefits for
in patients with adequate residual hearing on the contra both children after Cl treatment in speech understanding in
lateral side and the possibility of integrating acoustic input by quiet and noise and in localization ( Fig. 6.4; Fig. 6.5). Fur
means of a hearing aid on one side and electrical input on the thermore, compared with the adult SSD and AHL group, the
side with the CI,sl - 53 as well as reports of successful hybrid children show greater binaural squelch effects, leading to more
treatment (i.e., cochlear implantation and hearing aid significant improvements for speech understanding in noise

66
I . I . . . . . . . . . .. .

* *
1 00

T
0
0
t:;1 t;1 )I(
90 '-'
...1.. 0
*
80
1- -
- - )I(
70 1-- - -
)I(

60 ...
1
l

0
so
**

u
ill!

40

u
)I(

30 1-- -

20
**
T
-
T (")

r
)I(

10 ,...... '"'

0 ......
Una ided CROS Ba ha Cl Unaided CROS Baha Cl Unaided CROS Ba ha Cl

I S ssd N nh SONO S nh N ssd

Fig. 6.1 Box-whisker plots of the Hochmair-Schulz-Moser (HSM} sentence in noise test (SNR 0 dB} percent correct scores of 1 1 subjects for the
presentation configurations SONO (speech and noise from the front), Snh Nssd (speech from the normal-hearing side/ noise from the unilatera l deaf
side), and Sssd N n h (speech from the unilateral deaf side/ noise from the normal-hearing side) in the unaided as wel l as the aided conditions with a
CROS hearing aid, a Baha, and a Cl after 6 months. Significant improvement using the Cl compared with each of the other conditions is shown on top
of their box plots: p < 0.05; p < 0.01 (Wilcoxon signed-ra n k test). (From Arndt S, Aschendorff A, Laszig R, et a l . Comparison of pseudobinaural
hearing to rea l binaura l hearing rehabilitation after coch lear implantation in patients with unilateral deafness and tin nitus. Otol Neurotol 2 0 1 1 ;32:39 -
47. Reprinted by permission.)

than observed for the adult patients following implantation the earliest possible time to enable adequate maturation of the
(unpublished data). This suggests good potential to tap the auditory path and thus create the best possible conditions for
younger brain's ability to readapt to the presence of a signal optimal academic performance. It must be remembered that
from the hearing-impaired ear and integrate the signal from maturation of the auditory path is essentially completed by the
this ear readily with that received from the normal-hearing ear. sixth year of life.
It remains to be investigated if this trend is maintained as the
numbers of children and adults with UHL increase. We assume
that UHL children may obtain at least as much benefit from a Cl
Summary
as shown by SSD and AHL adults. These positive results indicate the benefits for SSD and AHL
This research raised the following question: Up to what patients of Cl treatment over the preimplant conditions of
duration of deafness would the Cl be successful in SSD and AHL listening with one normal ear, as well as over the conventional
patients? Until now, most SSD and AHL patients in whom good CROS and Baha devices. Nevertheless. careful patient selection
results were to be expected in the research had a relatively is necessary. Instruction concerning advantages and disadvan
short acquired duration of deafness from 1 month to 1 5 years. tages of all therapy options and their testing for at least 2 weeks
jacob et al59 reported on the case of a 27-year-old woman is necessary. The handicaps resulting from unilateral deafness
suffering possibly congenital deafness. It was also possible to and the patient's expectations regarding the Cl must also be
achieve localization in this patient, although the speech under checked. The necessary rehabilitation and the patient's own ini
standing developed more slowly than in other patients. We tiative for training the implanted ear must be made clear. Under
must, however, take into account that there may be a higher this premise, unilateral Cl treatment of individuals with SSD
number of nonusers among patients with congenital unilateral and AHL may help to restore hearing abilities experienced with
deafness and long duration of deafness than among patients sound input to two ears, such as understanding speech in noisy
with shorter duration of deafness. situations and localization of sounds and speech. It may also
Children with congenital deafness, and also children with help to reduce fatigue when listening and bothersome tinnitus
bilateral high-grade deafness should undergo implantation at when using the device.

67
. . I I ,

70

60

50


c::n
40
<II
e.
..2!
g> 30
<

20

10

0
Una id ed CROS Bah a Cl

Fig. 6 . 2Box-wh isker plots o f local ization deviations o f 1 1 subjects i n t h e u n a ided as wel l as t h e aided cond itions with a CROS hearing a i d . a Baha
l ntenso, a n d Cl after 6 months. Significant i mp rovement using the Cl compared with each of the other conditions is shown on top of their box
plots: * * p < 0.01 (Wi lcoxon's signed ra n k test) . ( From Arndt 5, Aschendorff A, Laszig R, et a l . Co mparison of pseudobi n a u ra l hearing to real
binaura l hearing rehabil itation after cochlear impla ntation in patients with u n i l atera l deafness and tinnitus. Otol Neurotol 201 1 ;32:39 -47.
Reprinted by permission.)

particular challenges for the implantation team, both in diagno


Additional Handicaps
sis and in treatment.
The primary goal of any cochlear implantation is to achieve or
restore communication through audition. In the Cl treatment of
children, the focus is also on expressive and receptive language Definition of Additional Handicaps
development, educational needs and achievement, and, ulti
and Outcome of Rehabilitation
mately, long-term professional or occupational development for
the future. Ideally, early intervention would be done with Cl In a review of the published literature, there are already
treatment, often performed bilaterally, which enables develop several reports including observation and evaluation of out
ment of normal or near-normal language abilities with normal, comes following Cl treatment in children with multiple
age-appropriate development and intelligible spoken language handicaps.62 - 65 Additional handicaps reported include
production. Clinical reports indicate that additional handicaps delayed or reduced mental capacity, with additional physical
are found in more than 30% of hearing-impaired children.61 handicaps. The complexity and nature of additional handi
Concurrently, the introduction of national neonatal hearing caps make comparison of outcome over time of intrasubject
screening programs has led to earlier diagnosis of hearing or intersubject groups challenging. At the same time, there
impairment and fitting of hearing aids and the subsequent need are different syndromes or also acquired diseases, such as
to face the decision to implant in cases of severe or profound those resulting from bacterial meningitis or cytomegalovirus
bilateral deafness as young as 8 to 10 months of age. At such a ( CMV) infection, which may affect mental development.
young age, it is difficult if not impossible to identify or assess Lesinski et al62 suggested a classification of additional handi
the type and degree of additional handicaps. Furthermore, caps differentiated according to the influence on outcomes
many types of functional deficits may not yet be apparent. One following cochlear implantation. In this classification, addi
example is autism in early childhood, which becomes sympto tional handicaps having no influence upon auditory percep
matic in the 1 0th to 1 2th month of life; in the case of Asperger tion skills, such as medical diseases, endocrinologic diseases,
syndrome, a form of autism, the deficits are manifested only or anatomic anomalies, were differentiated from central
after the third year of life. As a consequence, implantation is processing limitations that were considered to have an
undertaken in an increasing number of children often without influence, such as motor deficits, visual limitations, or
the knowledge of existing additional handicaps. This raises epilepsy. However, as exceptions do exist, the boundaries to

68
I . I . . . . . . . . . . . .

1 0 ,-------, 1
0 0 0

9 +-4r----------,_--+---r-

0 +-----__J I
Una ided CROS Ba ha Cl Una id ed C R O S Ba ha Cl Unaided CR O S Ba ha (I
Speec h Underst a nd ing Spat ia l H ea r i ng H ea r i ng Q u a l ity

Fig. 6.3 Box-whisker plots of the scores of 1 1 subjects for each section of the SSQ in the unaided as wel l as the aided conditions with a CROS hearing
aid, a Baha l ntenso, and a Cl after 6 months. Significant improvement using the Cl compared with each of the other conditions is shown on top of their
box plots: * p < 0.05; * * p < 0.01 (Wilcoxon's signed ran k test). (From Arndt S, Aschendorff A, Laszig R, et al. Comparison of pseudobinaura l hearing to
real binaura l hearing rehabilitation after cochlear impla ntation in patients with unilateral deafness and tinnitus. Otol Neu rotol 201 1 ;32:39-47.
Reprinted by permission.)

D Unaided Aid ed : Child 1 Aid ed : Chi l d 2 Aided : Ad u lts' M ean ( n - 20)


1 00

90

80

70
60

0
50
u
40

30

20

10
0
C hild 1 C h i ld 2 Ad ults Child 1 Child 2 Ad ults
(n - 20) ( n - 20)
Sssd/ Nnh SONO

Fig. 6.4 Results of HSM sentence in noise test (SNR 0 dB) percent correct scores of two children with acq uired UHL ( 1 0 and 1 1 years old), and mean
resu lts of 20 adult UHL subjects for the presentation configurations SONO (speech and noise from the front), SnhNssd (speech from the normal
hearing side/noise from the unilateral deaf side). and Sssd N n h (speech from the unilateral deaf side/noise from the normal-hearing side) in the
unaided condition and with a Cl after 12 months.

69
. . . . . . .

D Unaided Aid ed : C hi ld 1 Aid ed : Child 2 A i d ed : Ad ults' M ea n (n - 20)


1 00

90

80

70

60
....
u

50
0
u

40

30

20

10

0
C hi ld 1 Child 2 Ad ults (n - 20)

subjects in the unaided condition and with a Cl


Fig. 6.5 Resu lts of localization percent correct scores of two children with acquired U H L ( 1 0 and 1 1 years old). and mean resu lts of 20 adult UHL
after 12 months.

differentiate between handicaps that have an impact upon Holt and Kirkfi7 evaluated speech development in children
auditory performance and those that do not are not fixed. with cognitive retardation and found that in the presence of
Consideration of additional handicaps for determining the mildly limited cognitive development, very good results in
( re)habilitation path including Cl treatment represents a very hearing and speech development can be expected. It was nota
complex issue. Each individual case must be examined to ble that children with mild cognitive retardation required more
address the potential prognosis and, even more importantly, time and experience with their Cis to achieve sentence compre
the specific needs and capacity of the child to undergo and hension comparable to that of children with normal develop
derive benefit from the rehabilitation process. In liberalizing ment. Contrary to speech perception, however, the capability
the CI indications, multidisciplinary CI teams that decide for speech production and receptive vocabulary skills differed
whether or not to recommend Cl treatment should be able to significantly. Expressive language also showed less favorable
estimate the influence of additional handicaps, especially to development than in children without cognitive limitations.
support counseling of the patients and their parents to help Overall, however, there was considerable variability between
them develop appropriate expectations following treatment as the individual children. In 201 0, Lee et al68 showed that positive
well as understand the special requirements for involvement in results could be recorded for children with mild mental retar
the rehabilitation process. The most frequent handicaps are dation 2 years after cochlear implantation. However, speech
retardation in motor and mental development. Amirsalari perception and production were negatively correlated with the
et aJ66 examined the rehabilitation outcomes for a group of 262 degree of mental retardation. It was also shown that children
children with prelingual profound deafness. Ten percent of the with moderate mental retardation had received their Cl later
children had mild and moderate motor deficits, whereas chil than the children with only mild mental retardation. Thus, the
dren presenting with severely retarded motor development and older age at implantation may have had an influence on the less
or additional cognitive handicaps had been excluded from this favorable postoperative development. Basically, it can be con
study. For assessment of their auditory perception skills, the sidered more favorable when children receive the Cl as early as
Categories of Auditory Perception Scales and the Speech Intelli possible, coupled with appropriate support, relatively indepen
gibility Rating (SIR) were used both prior to surgery and again dent of the extent of mental retardation present. It must be
at 2 years after activation. They report no significant difference emphasized that children who are born deaf with additional
in performance between children with mild or moderate retar handicaps cannot cooperate with several evaluations due to
dation of motor development and children without. Cognitive their short attention spans or insufficient cognitive skills.
limitations and retarded development are found in a significant Therefore, for the future, there is a need for new objective test
number of cochlear implantation patients.61.62 For patients procedures to be developed that are capable of rating both
undergoing early Cl intervention, it may be difficult to estimate pre- and postoperative performance independent of the child's
the extent of the cognitive limitations. ability to actively respond.ss

70
I . I . . . . . . . . . . . .

To date, relatively little information is available on the devel addresses the needs of these complex children. 6373 In a study
opment and examination of children with cerebral palsy. Steven of children with CHARGE syndrome ( coloboma, heart defects,
et al69 reported on 36 implanted children with cerebral palsy. It choana! atresia, retarded growth and development, genital
was observed that children demonstrated positive development hypoplasia, and ear abnormalities). Arndt et aJ73 showed
following Cl implant in the case of mild cognitive limitations. implantation at an older age may also be a negative influence
Patients with a congenital CMV infection, which is the most factor on the development of speech perception and speech
common infectious cause of congenital hearing loss, comprise production. In our opinion, care of these special children at
another important group of Cl candidates with potential appropriate expert centers is mandatory. Only a multidiscipli
additional handicaps. Yamazaki et aJ7 showed that 9 out of 1 1 nary team of experienced physicians, technicians, and thera
Cl patients with congenital CMV infection presented with psy pists, in cooperation with neuropediatricians, psychologists,
choneurologic anomalies, such as attention deficit hyperactivity and others. can contribute to define and tailor the individual
disorder (ADHD) syndrome, retarded development, or mental rehabilitation program to the specific needs of the multihan
retardation. In general, patients with congenital CMV infection dicapped child to achieve the best possible outcome for the
showed significant delays in speech development skills com patient and their parents.
pared with patients with hearing loss due to genetic causes.
Two patients for whom speech development was considerably
retarded also presented with autistic-spectrum disorders. Malik
Future Clinical Perspectives
et aJ71 also showed less favorable outcomes for a group of 1 4 As this chapter has demonstrated, the indications for cochlear
children with congenital CMV infection compared with other implantation are under continuous development and have
children. However, there was also considerable variability in evolved from extremely strict guidelines to include hearing
performance outcomes, which correlated negatively with the impaired individuals who were previously excluded from CI
severity of additional motor and cognitive handicaps present. treatment but now demonstrate clear benefits. As a conse
Patients with autistic-spectrum disorders also comprise a very quence of the ongoing development and advancements in a
heterogeneous group in terms of the outcomes displayed post variety of clinical aspects-speech-coding strategies for Cis,
implantation. The difficulty exists especially in treating very diagnostic radiological possibilities and resolutions. pre- and
young children for whom such a disorder is not detected or not intraoperative technical aids, and the cumulative clinical expe
yet evidenced symptomatically. It must be at least assumed that riences gained with the variety of and large numbers of patients
this disease pattern may have an unfavorable influence on the treated to date-CI indications will continue to grow and change
development of speech and hearing skills.64 Basically, in treat in coming years. The widespread neonatal auditory screening
ing patients with multiple handicaps, it is important to consider and conscientious follow-up of neonates diagnosed as hearing
not only the individual constellation of the separate symptoms impaired lead to very early diagnosis of congenital uni- or
and comorbidities, but also to define expectations from bilateral hearing impairment. This enables very early diagnos
cochlear implantation treatment. Establishing contact between tics of hearing impairment as well as initiation of immediate
the Cl child and family members, even in the event that spoken rehabilitation measures, such as fitting with hearing aids and
language is intelligible only for the parents, can be a considera logopedic therapy of the children as well as determination for
ble advantage in the communication capacity of the afflicted Cl candidacy in a stepwise manner. These measures set the
patient and subsequent development.64 For this reason, course for sufficiently early cochlear implantation if speech
patients with additional handicaps or limitations should not be development is not apparent by the end of the first year of life,
denied treatment with Cis. or even by the end of the 6th month after birth. If bilateral,
Overall. it appears difficult to generally make valid predic profound inner-ear hearing impairment is confirmed, positive
tions; nonetheless. the study by Nikolopoulos et al64 showed experience indicates that these children can undergo simulta
that long-term results for patients with additional handicaps neous bilateral implantation without anticipating additional
depend essentially on the number of additional handicaps. complications when appropriate care is taken. This requires
The study showed that 70% of the Cl children with additional highly experienced pediatric anesthesiologists and special
anomalies could develop intelligible speech, whereby the postoperative care, as well as a surgical team with sufficient
quality of speech was clearly lower than in the control group experience to reduce the operation time for bilateral implan
of Cl patients without additional handicaps. To estimate long tation to a minimum. Moreover, a specialized rehabilitation
term results. Wiley et aJ72 performed a prospective study team is needed that has experience in the rehabilitation of
applying the Pediatric Evaluation of Disability Inventory. It infants and very young children and in the adjustment
was found that receptive speech development plays a decisive of bilateral Cis.
role in the social functional capability of these patients. For Moreover. cochlear implantation in unilaterally deaf patients.
children considered to be complex cases. the goals of Cl treat taking selection criteria into account, will become established
ment are not necessarily the achievement of open-set speech for hearing rehabilitation coupled with the reduction of tinni
comprehension and corresponding oral/aural communication tus. Neonate auditory screening will also lead to considerably
capabilities. The goal may be recognition of the parents' voices earlier diagnostics in children with UHL or profound hearing
or enabling awareness. contact, and a sense of security impairment, making it necessary to decide in the near future
with their environment, which concurrently has a positive how these children are to be treated. These children should not
influence on the functional capabilities in areas of social or be denied binaural hearing so that they have the same chance
emotional behavior. It is, however, enormously important that for education and occupational training as children with normal
the rehabilitation process after Cl treatment appropriately binaural hearing.

71
. . . . . . .

Furthermore, there is a need for screening of hearing [ 1 9 1 Waltzman SB, Cohen NL Cochlear implantation in children younger than 2
years old. Am j Otol 1 998; 1 9 : 1 58-162
impairment in the elderly population because it might have
[201 Holt RF, Svirsky MA. An exploratory look at pediatric cochlear implantation:
an important social impact: Cl can offer improvement in is earliest always best? Ear Hear 2008 ; 29: 492-51 1
hearing and independence and quality of life even in our [ 2 1 1 Ruben Rj. A time frame o f critical/sensitive periods o f language development.
aging population. Age and comorbidities in the elderly are Acta Otolaryngol 1 997; 1 1 7 : 202-205
not contraindications, and relative benefits are comparable to [221 Dettman Sj, Pinder D. Briggs Rj. Dowell RC. Leigh JR. Communication develop
ment in children who receive the cochlear implant younger than 12 months:
younger patients.
risks versus benefits. Ear Hear 2007 ; 28 Suppl: 11 S-18S
In addition, an increasing number of children will be diag [231 Lin FR. Thorpe R, Gordon-Salant S, Ferrucci L Hearing loss prevalence and risk
nosed through screenings who were born deaf or hearing factors among older adults in the United States. j Gerontal A Bioi Sci Med Sci
impaired with additional syndromes and handicaps. Many 201 1 : 66: 582-590
studies have shown that multiply handicapped children may [241 Lin FR. Metter Ej, O'Brien Rj, Resnick SM, Zonderman AB, Ferrucci L. Hearing
loss and incident dementia. Arch Neurol 201 1 : 68: 2 1 4-220
also profit demonstrating benefits of varying degrees from [251 Lin FR, Ferrucci L. Metter Ej, An Y, Zonderman AB, Resnick SM. Hearing loss
cochlear implantation and that "the earlier-the better" applies and cognition in the Baltimore Longitudinal Study of Aging. Neuropsychology
to these children as well. 201 1 : 25: 763-770
[261 Schvartz KC, Chatterjee M, Gordon-Salant S. Recognition of spectrally
degraded phonemes by younger, middle-aged, and older normal-hearing lis
teners . ] Acoust Soc Am 2008 ; 1 24: 3972-3988
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[ 8 1 Morray JP, Geiduschek J M , Ramamoorthy C e t a l . Anesthesia-related cardiac [341 Bovo R, Martini A, Agnoletto M et al. Auditory and academic performance of
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[ 1 0 1 Colletti L . Mandala M, Zoccante L . Shannon RV, Colletti V . Infants versus older 772-781
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Pediatr Otorhinolaryngol 201 1 : 75: 504-509 children with unilateral and mild bilateral degrees of hearing loss. Trends
[ 1 1 1 johr M , H o A, Wagner CS, Linder T . Ear surgery i n infants under one year of Amplif 2008; 1 2 : 35-41
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2008 ; 29: 3 1 0-3 1 3 loss in children. Arch Otolaryngol Head Neck Surg 2004; 130: 524-530
[ 1 2 1 Keenan RL, Shapiro J H , Kane FR, Simpson PM. Bradycardia during anesthesia [391 Gray L. Kesser B, Cole E. Understanding speech in noise after correction of
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[ 1 3 1 Balkany Tj, Whitley M, Shapira Y e t a l . The temporalis pocket technique for squelch but not in use of head-shadow. lnt j Pediatr Otorhinolaryngol 2009;
cochlear implantation: an anatomic and clinical study. Otol Neurotol 2009; 73: 1 28 1 - 1 287
30: 903-907 [401 Colburn HS, Latimer JS. Theory of binaural interaction based on audi
[ 1 4 1 Davids T , Ramsden j D . Gordon KA, james AL, Papsin B C . S o ft tissue com- tory-nerve data. Ill. joint dependence on interaural time and amplitude
plications after small incision pediatric cochlear implantation. 2009 ; 1 1 9: differences in discrimination and detection. J Acoust Soc Am 1 978; 64:
980-983 95- 1 06
[ 1 5 1 Roland JT, Cosetti M, Wang KH, Immerman S, Waltz man SB. Cochlear implan- [41 1 van Haese! Rj, Tyler RS. Speech perception, localization, and lateralization
ration in the very young child: long-term safety and efficacy. Laryngoscope with bilateral cochlear implants . ] Acoust Soc Am 2003 ; 1 1 3 : 1 6 1 7-1 630
2009; 1 1 9: 2205-221 0 [421 Laszig R, Aschendorff A, Stecker M et al. Benefits of bilateral electrical stimu
[ 1 6 1 Hassepass F, Arndt S , Beck R , Schild C , Maier W , Aschendorff A . [Modified lation with the nucleus cochlear implant in adults: 6-month postoperative
cochlear implant fixation using a single suture I Laryngorhinootologie 201 1 : results. Otol Neurotol 2004; 25: 958-968
90: 262-263 [431 Litovsky RY, johns tone PM, Godar SP. Benefits of bilateral cochlear implants
[ 1 7 1 May-Mederake B. Early intervention and assessment of speech and language and/or hearing aids in children. lnt j Audiol 2006; 45 Suppl 1 : S78-S91
development in young children with cochlear implants. lnt j Pediatr Otorhi- [441 Ramsden R. Greenham P, O'Driscoll M et al. Evaluation of bilaterally
nolaryngol 201 2 ; 76: 939-946 implanted adult subjects with the nucleus 24 cochlear implant system. Otol
[ 1 8 1 Miyamoto RT, Hay-McCutcheon Mj, Kirk Kl, Houston D M , Bergeson-Dana T. Neurotol 2005 ; 26: 988-998
Language skills of profoundly deaf children who received cochlear implants [451 Tyler RS, Dunn CC, Witt SA, Noble WG. Speech perception and localization
under 12 months of age: a preliminary study. Acta Otolaryngol 2008; 1 2 8 : with adults with bilateral sequential cochlear implants. Ear Hear 2007 ; 28
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(46( Litovsky RY. Parkinson A, Arcaroli j et al. Bilateral cochlear implants in adults (61 [ Holden-Pitt L. Albertorio j. Thirty years of the Annual Survey of Deaf and
and children. Arch Otolaryngol Head Neck Surg 2004; 1 30: 648-655 Hard-of-Hearing Children & Youth: a glance over the decades. Am Ann Deaf
(47( Verschuur CA. Lutman ME, Ramsden R, Greenham P, O'Driscoll M. Auditory 1998; 1 43 : 72-76
localization abilities in bilateral cochlear implant recipients. Otol Neurotol [62[ Lesinski A. Hartrampf R, Dahm MC. Bertram B, Lenarz T. Cochlear implanta
2005 ; 26: 965-971 tion in a population of multihandicapped children. Ann Otol Rhinal Laryngol
(48 ( Steffens T, Lesinski-Schiedat A, Strutz j et al. The benefits of sequential bilat Suppl 1 995; 1 66: 332-334
eral cochlear implantation for hearing-impaired children. Acta Otolaryngol [63( Meinzen-Derr j, Wiley S, Grether S, Chao Dl. Language performance in
2008 ; 1 2 8 : 1 64-176 children with cochlear implants and additional disabilities. Laryngoscope 2009
(49( Gordon KA, Papsin BC. Benefits of short interimplant delays in children [64] Nikolopoulos TP, Archbold SM, Wever CC, Lloyd H. Speech production in deaf
receiving bilateral cochlear implants. Otol Neurotol 2009; 30: 3 1 9-331 implanted children with additional disabilities and comparison with age
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(51 [ Ching TYC, van Wanrooy E. Dillon H. Binaural-bimodal fitting or bilateral [65( Cosetti MK. Waltzman SB. Outcomes in cochlear implantation: variables
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(52( Iwaki T, Blarney P, Kubo T. Bimodal studies using adaptive dynamic range [66( Amirsalari S, Yousefi j, Radfar S et al. Cochlear implant outcomes in children
optimization (ADRO) technology. lnt j Audiol 2008 ; 47: 3 1 1 -3 1 8 with motor developmental delay. lnt J Pediatr Otorhinolaryngol 201 2 ; 76:
( 5 3 ( Sucher CM, McDermott Hj. Bimodal stimulation: benefits for music percep 1 00-1 03
tion and sound quality. Cochlear Implants lnt 2009; 1 O(Suppl 1 ): 96-99 [67( Holt RF, Kirk Kl. Speech and language development in cognitively delayed
(54( Van de Heyning P, Vermeire K, Diebl M. Nopp P. Anderson I. De Ridder D Van children with cochlear implants. Ear Hear 2005 ; 26: 1 32-148
de HP. Incapacitating unilateral tinnitus in single-sided deafness treated by [68] Lee YM, Kim LS, Jeong SW, Kim JS, Chung SH. Performance of children with
cochlear implantation. Ann Otol Rhinal Laryngol 2008; 1 1 7 : 645-652 mental retardation after cochlear implantation: speech perception, speech
(55( Vermeire K. Van de Heyning P. Binaural hearing after cochlear implantation intelligibility. and language development. Acta Otolaryngol 2010; 130: 924-934
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Neurootol 2009 ; 14: 1 63-171 Cochlear implantation in children with cerebral palsy. lnt j Pediatr Otorhino
(56( Arndt S, Aschendorff A, Laszig R et al. Comparison of pseudobinaural hearing laryngol 201 1 ; 75: 1 427-1430
to real binaural hearing rehabilitation after cochlear implantation in patients (70] Yamazaki H, Yamamoto R, Moroto S et al. Cochlear implantation in children
with unilateral deafness and tinnitus. Otol Neurotol 201 1 ; 32: 39-47 with congenital cytomegalovirus infection accompanied by psycho-neurolog
(57( Arndt S, Laszig R, Aschendorff A et al. (Unilateral deafness and cochlear ical disorders. Acta Otolaryngol 201 2 ; 1 3 2 : 420-427
implantation: audiological diagnostic evaluation and outcomes] HNO 201 1 ; [ 7 1 [ Malik V, Bruce lA, Broomfield Sj, Henderson !., Green KM, Ramsden RT. Out
59: 437-446 come of cochlear implantation in asymptomatic congenital cytomegalovirus
[58[ Buechner A, Brendel M, Lesinski-Schiedat A et al. Cochlear implantation in deafened children. Laryngoscope 201 1 ; 1 2 1 : 1 780-1 784
unilateral deaf subjects associated with ipsilateral tinnitus. Otol Neurotol [72( Wiley S, Meinzen-Derr j, Grether S, Chao Dl, Hughes ML. Longitudinal func
2010; 3 1 : 1 3 8 1 -1 385 tional performance among children with cochlear implants and disabilities: a
[59[ jacob R, Stelzig Y, Nopp P, Schleich P. [Audiological results with cochlear prospective study using the Pediatric Evaluation of Disability Inventory. lnt j
implants for single-sided deafness I HNO 201 1 ; 59: 453-460 Pediatr Otorhinolaryngol 201 2 ; 76: 693-697
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cochlear implantation in patients with unilateral hearing loss: a case series. j management in children with CHARGE syndrome. Otol Neurotol 2010; 3 1 :
Med Case Reports 201 1 ; 5: 343 67-73

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7 Pri nci p l es of Coc h l ea r I m p l a nt I ma g i n g


Andrew}. Fishman and Selena E. Heman-Ackah

requirement for cochlear implantation is the presence of an


Introduction implantable cavity in proximity to stimulable neural elements
Radiographic imaging plays a major role in cochlear implanta whose projections connect to the auditory cortex. Accordingly,
tion with regards to preoperative candidacy evaluation, intrao the first question that must be answered is: are there any coch
perative monitoring, post-operative evaluation, as well as leovestibular anomalies that preclude implantation?
research and experimental techniques. At a minimum, success
ful cochlear implantation requires that electrical impulses be Embryology
delivered to a surviving spiral ganglion cell population, and that
these impulses be transmitted to a functioning auditory cortex To fully appreciate the wide variety of possible cochleovestibu
by an existent neural connection. Accordingly, imaging the lar malformations it is helpful to first review the embryogenesis
auditory pathway of the implant candidate is necessary to of the inner ear. We will consider separately the formation
screen for morphologic conditions that would preclude or of the membranous labyrinth, the bony otic capsule, and the
complicate the implantation process. Increasing resolution of cochleovestibular nerves and ganglia.
computed tomography (CT) and magnetic resonance imaging The development of the combined cochlear and vestibular
( MRI) technology has provided the clinician with more detailed membranous labyrinthine system begins with the formation of
information about the integrity of the auditory pathway. As the otic placode as an ectodermal thickening that forms on the
technologies evolve, a clear understanding of what information surface of the neural tube in the 3rd gestational week. The otic
can be obtained as well as the limitations of various imaging placode invaginates from the surface and forms the otocyst in
modalities is essential to proper candidacy evaluation, and the 4th gestational week. The otocyst develops three infolds in
selection of the ear to be implanted in complex cases. the 5th week. The resultant pouches represent: the primordial
Also important is the effect that the presence of a cochlear endolymphatic sac and duct; the utricle and semicircular
implant has on future imaging the head and neck region of an canals ; and the saccule and cochlea. Beginning in the 6th week,
implantee. In the past, the presence of a cochlear implant was the cochlear duct grows from its primordial bud beginning from
considered to be a major contraindication to MRJ.I Because it is the basal region spiraling apically to reach its full 2.5 to 2.75
now possible to obtain useful images with advances in CT and turns by the 8th to 1 0th week. The neuroepithelial end organs
MRI technology, the issue of device MRI compatibility has continue to develop beyond this period with the organ of Corti
opened up a new area of investigation. completing its formation in the 25th week.
The semicircular canals begin their formation as three small
folded evaginations on the primordial vestibular appendage.
Preoperative Imaging They develop as disk-like outpouchings whose centers eventu
ally compress and fuse to ultimately form the semicircular duct
Preoperative imaging is instrumental in determining the
structure. By the 6th week of gestational life, this compression
feasibility and facility of cochlear implantation. Analysis is per
and fusion has taken place in first the superior and then the
formed in a stepwise approach, answering of the following
posterior canals. The three canals continue to enlarge and com
three questions: Are there cochleovestibular anomalies that
plete their formation to full adult size in sequence beginning
preclude implantation? Is there evidence of luminal obstruc
with the superior around the 20th week, and followed by the
tion? Are there additional findings that may complicate the
posterior and finally the lateral semicircular canals. Interest
surgery or subsequent patient management? This section is not
ingly, the endolymphatic sac and duct are the first to appear
intended to review principles or techniques of image acquisi
and the last to complete their development.
tion, but rather to provide a platform for discussion between
The osseous otic capsule eventually forms from a morphologi
the implant team and the radiologist.
cally fully developed cartilage precursor model via 14 centers of
ossification, beginning around the 1 5th gestational week and is
Are There Cochleovestibular Anomalies completed during the 23rd gestational week. The cartilage model
and underlying membranous labyrinth continue to grow in the
That Preclude Implantation? region of the posterior and lateral semicircular canals, while
Up to 35% of patients with congenital sensorineural hearing loss other structures, which have previously attained there final
(SNHL) have radiographically identifiable morphologic abnor shape and size, have begun ossifYing. The cochleovestibular
malities of the inner ear.2.3 In general, inner ear malformations nerves and ganglia develop in concert with the membranous lab
can be associated with a wide range of hearing sensitivity.4 yrinth and cochleovestibular end organs. They are of neural crest
These patients can manifest progression of hearing loss though origin and migrate between the epithelial layer and basement
many may retain useful hearing into adult life. As a general rule, membrane of the otic vesicle during the 4th gestational week.
however, the severity of deformity is positively correlated with
the severity of hearing loss.4 Due to the variability and progres
sive nature of hearing loss in these disorders, most large implant
Cochlear Malformations
centers are likely to evaluate a number of patients with a variety There is much confusion in the literature regarding the nomen
of malformations. Given the current technology, the minimum clature of cochlear morphologic anomalies especially regarding

74
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the term Mondini malformation. In 1 79 1 , Carlo Mondini pre authors acknowledge that their classification and could not
sented his findings on an anatomical dissection of a young deaf describe all observable abnormalities but was meant to
boy.5 According to his writings, prior reports of human deafness serve as a framework upon which other describable anoma
were attributed to abnormalities of the external auditory canal lies could be added, which by their supposition would have
and eustachian tube, tympanic membrane, middle ear and resulted from aberrant, rather than arrested development.
ossicles, or compression of the auditory nerve. During his dissec This body of work deserves mention as it is often cited, and it
tion on the posterior face of the petrous bone he discovered serves well as an initial systematic basis for the interpretation
significant vestibular aqueduct enlargement and commented of images. jackler et al. formulated their classification system
that the usual bony lip that "protects the vestibular aqueduct" upon review of polytomes and CT scans of 63 patients with 98
was missing and was replaced by a membranous plate of dura. congenitally malformed ears, and provided the following cate
He noted that the vestibule was not deformed but was of greater gorization4:
than usual size. He also noted an increase in the size of the
elliptical recess though it was normal in shape. He commented Absent or malformed cochlea
that the semicircular canals appeared normal and that the posi 1. Complete labyrinthine aplasia
tions of their openings into the vestibule were unremarkable. In 2. Cochlear aplasia
observing the medial opening of the vestibular aqueduct, he 3. Cochlear hypoplasia
commented that it was quite enlarged and was larger than the 4. Incomplete partition
size of the common crus. With regards to the cochlea, it was 5. Common Cavity
described to possess only 1 .5 turns. He described the cochlea Normal cochlea
as ending in a cavity corresponding to the last spiral turn, 1. Vestibule: lateral semicircular canal dysplasia
and he described an incompletely formed interscalar septum. 2. Enlarged vestibular aqueduct
Mondini malformations have been identified as isolated
findings or in association with other named syndromes. The disorders identified as having normal cochleas were subdi
namely: Klippel-Feil, Pendred and DiGeorge.6 The more con vided out solely for the purposes of jackler's classification
temporary term incomplete partition is commonly used to scheme. It is important to realize that disorders of the vestibule,
describe this classic anomaly and denotes this specific aspect semicircular canals and vestibular aqueduct are also often
of the deformity? found in conjunction with cochlear malformations. Inner ear
Phelps et al. reserve the term "Mondini deformity" for deformities tend to occur bilaterally in 65%, 4 and when they
cochlea whose basal turn are normal and possess a defi are bilateral, there is a 93% chance that they will be similar
ciency of the interscalar septum of the distal 1 .5 coils. 8 They though various combinations of morphologic classes have been
differentiate these cochleas from those termed "dysplastic" documented.4
owing to their widened basal turn being in wide communi More recently, in 2002 Sennaroglu and Saatci proposed a
cation with a dilated vestibule. According to Phelps, the sig modified classification system for cochlear malformations
nificance is in the clinical absence of spontaneous CSF leak based upon radiographic findings that has gained popularity
and meningitis in patients with his strict definition of Mon ( Table 7.1 ).7 Michel deformity is the most severe and rarest
dini deformity as opposed to those patients with dysplasia malformation ( Fig. 7.1 ).7 Michel aplasia represents labyrin
who did manifest these complications in a series of 20 thine aplasia ( complete absence of all cochlear and vestibular
patients studied. structures). This results from embryologic arrest prior to for
Since the writings of Mondini, a number of investigators mation of the otocyst resulting in complete absence of inner
have documented a variety of inner ear malformations. ear development.4 Cochlear aplasia is defined as complete
Though not the first to describe or name these malforma absence of the cochlea. This is the second rarest cochlear
tions, jackler et al. proposed a classification system for the malformation, representing approximately 3 to 7% of identi
congenitally malformed inner ear in 1 987 based on the the fied cochlear malformations.47 The term common cavity ( CC)
ory that a variety of deformities result from arrested deformity is used to denote confluence of the cochlea and
development at different stages of embryogenesis.4 The vestibule into a common rudimentary cavity that usually lacks

Table 7.1 Sennaroglu and Saatci classification system for cochlear malformations.
dassification Description
Michel deformity Complete absence o f a l l cochlear and vestibular structu res
Coch lear aplasia Complete absence of the coch lea
Common cavity deformity A cystic cavity representing the cochlea and vestibule without showing any differentiation into cochlea
and vestibule
Coch lear hypoplasia The cochlea and vestibule are separate from eachother but their dimensions are smaller than normal
Incomplete partition type I ( I P-1) The cochlea is lacking the entire modiolus and cribiform area, resulting in a cystic a ppearance.
Incomplete partition type I I ( I P-11} Also known as a Mondini malformation. The cochlea consists of 1 .5 turns, in which the middle and apical
turns coalesce to form a cystic apex, accompanied by a dilated vestibule and enlarged VA.
Incomplete partition type I l l (IP-1 1 1 ) Bulbous fu ndus and basal turn of the cochlea, absence of the lamina cribosa.
Data from Sennaroglu L. Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002; 1 12: 2230-4 1 .

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. . . . .

Fig. 7 . 1 Computed tomography (CT) sca n of a patient with a common cavity deformity on the right and complete cochleovestibular a plasia on the
left. (a-c)Axial sections a re depicted from su perior to inferior. Image b demonstrates the internal auditory canal (lAC) on the right com municating with
the common cavity. Image (a) demonstrates a narrow lAC on the left containing only a facial nerve. (d-f) Coronal sections through the left tem pora l
bone demonstrate the absence of the otic capsu le with only the carotid and facial nerve canals visible in the region. The tensor tym pani muscle is seen
in d. This patient was successfully implanted in the right ear.

an internal architecture and is often associated with abnor tion type Ill ( IP-111) has been described ( .,. Fig. 7.5).9-1 3 IP-111
mally formed semicircular canals.4,7 This is the third most is associated with X-linked deafness that has been both
common abnormality according to this modified classification radiographically described and genetically identified.910 It is
system ( .,. Fig. 7.2).7 seen in some severely deaf males that possess a deficiency of
Cochlear hypoplasia, according to Sennaroglu & Saatci (2002), bone between the lateral end of bulbous lAC and the basal
describes a malformation in which the cochlea and vestibule turn of the cochlea.9 Additionally absence of the lamina
are separate from each other, but the dimensions are smaller cribosa has been described in association.1 1 13 The clinical
than normaL7 This represents 7 to 1 5% of all cochlear malfor implications of these findings is for abnormal communica
mations ( .,. Fig. 7.3 ).47 Incomplete partition type I (IP-1) refers tion between the CSF containing lAC and the cochlea. This
to malformations in which the cochlea is lacking the entire has been associated with an increased risk of internal audi
modiolus and cribiform area resulting in a cystic appearanceJ tory canal insertion of multichannel electrodes during
This was the second most common malformation noted by cochlear implantation. 1 2 ,14,1 S
Sennaroglu and Saatci? The final malformation described by Finally, abnormalities o f the internal auditory canal have
Sennaroglu and Saatci is incomplete partition type II ( IP-11), been described. A narrow lAC of a diameter 2.0 to 2.5 mm or
which represents the classic Mondini malformation previously less on either conventional imaging or CT has been reported in
describedJ This comprised the majority of malformations association with a normal inner ear as well as a variety of inner
encountered in their series_7 It is felt to represent arrest in ear malformations.9.l 6 It has been reported unilaterally and
development during the 7th gestational week, a time at which bilaterally, in association with a variety of other congenital
the cochlea would have completed 1 .0 to 1 .5 turns.4 Radio anomalies and as an isolated disorder. The clinical significance
graphically, these cochleas possess only 1 .5 turns comprised of this finding with regards to preimplant evaluation is that
of a basal turn leading to the appearance of a confluent middle there is a high likelihood that this represents the presence of
and apical turn which may also be viewed and described as only a facial nerve and the absence of the cochleovestibular
incomplete partitioning by a deficient interscalar septum nerve or cochlear nerve deficiency. Traditionally, an lAC of less
( .,. Fig. 7.4).47 than 2.0 to 2.5 mm as demonstrated by CT of the temporal
In addition to the malformations described in the modified bones was considered by many authors to be an absolute con
classification system described above, an incomplete parti - traindication to cochlear implantation because it was associated

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Fig. 7.2 a-d Axial a scans of the right tem poral bone from the patient in 1> Fig. 7 . 1 , depicted from su perior to inferior. Note the labyrinthine facial
nerve passing anteriorly and su periorly to the common cochleovestibular chamber. I n the patient shown in a , the semicircular canals are absent. The
bony coch lear aq ued uct is visible in images c and d.

with cochlear nerve deficiency or absence.91617 This concept is Patient Evaluation


evolving.18 Recent studies have suggested that lAC diameter
alone is an unreliable marker for cochlear nerve integrity.19- 21 Initial radiologic evaluation of the cochlear implant candi
Cochlear nerve canal diameter on high-resolution computed date is typically performed with either an HRCT scan of the
tomography (HRCT) of the temporal bones and MRl of the lAC temporal bones or high-resolution MRI of the otic capsule
has demonstrated to have a higher correlation with cochlear and internal auditory canal. Contrast is rarely indicated. The
nerve deficiency or absence.19- 21 A recent study purports that a protocol by which HRCT and MRI are employed in patient
decrease in the diameter of the cochlear nerve canal on HRCT of evaluation remains somewhat variable. The choice of initial
the temporal bone may be more highly indicative of cochlear imaging modality is largely dependent upon the age of the
nerve deficiency, that is less than 1 .5 mm.19 Currently, MRI of patient, the presumed etiology of hearing loss, and the
the lAC has become the preferential imaging modality in evalu imaging evaluation protocol developed by the respective
ating patients with a suspicion for cochlear nerve deficiency or cochlear implant center.
absence. Within the adult population with acquired hearing loss,
Another recently identified anomaly of the internal auditory HRCT of the temporal bones is commonly performed. HRCT is
canal is a narrow duplicated IAC.22 The presence of a narrow beneficial in identifying position of the facial nerve, labyrin
duplicated lAC is typically associated with a lack of or deficient thine ossification, aeration of the temporal bone, position of
cochlear nerve.22-25 However, facial nerve function and the sigmoid sinus, high-riding jugular bulb, carotid artery
anatomic integrity remains intact_22-25 As such, this finding on dehiscence, j ugular diverticuli, and size of the vestibular
preoperative imaging may represent a contraindication for aqueduct. Confirmation of the presence or absence of a
cochlear implantation. cochlear nerve is not of concern in this population. Therefore,

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Fig. 7.3 a-d CT scans of a patient with bilatera l cochlear hypoplasia. I mages are shown from the right temporal bone which was successfully
implanted . (a) A coronal section through the vestibule demonstrates the relatively normal formation of the vestibular apparatus as wel l as the
presence of an ova l window. The oval window and ossicles a re also seen in axial image .. (b) An intraoperative transorbital plain radiogra ph of the
multichan nel electrode a rray implanted in this patient. This is the expected appearance of the array placed into this small cavity. Note that the
morphology is quite similar to the coronal section in image (a). The vestibule (v) is marked for reference. (c) These a re useful surgical landmarks
because they al low for the formation of a topographic road map when im planting abnormal cochleas. Only the proximal basal turn of the cochlea is
present. (d) The middle or apical turns a re absent.

MRI of the otic capsule and lAC typically is reserved for adult eral semicircular canal dysplasia, evaluation of the internal
patients with etiologic risk factors (e.g. history of meningitis, auditory canal neural contents, and the caliber of the
temporal bone fracture, labyrinthitis, etc.) for hearing loss to cochlear nerve ( .,.. Fig. 7.6).
assess for cochlear patency in there is a concern for the pres Each of these modalities has its own strengths (i.e. MRI in
ence of labyrinthitis ossificans. This will be discussed in identifying ossification of the labyrinth and cochlear nerve
greater detail later in the chapter. hypoplasia or agenesis; HRCT in identifying enlarged vestibu
The use of HRCT and MRI are more variably used in the lar aqueduct and caliber of the cochlear nerve canai).26-2B No
pediatric population for initial imaging evaluation. Within benefit has been demonstrated in utilizing dual modality
the pediatric population, various anomalies of the temporal (HRCT and MRI) screening in the pediatric population prior
bone may be identified by HRCT: cochlear dysplasia, labyrin to cochlear implantation.28 Because the specificity and nega
thine ossification, position of the facial nerve, aeration of tive predictive value for both MRI alone and HRCT alone are
the temporal bone, position of the sigmoid sinus, high-riding both high and without significant difference in predicting
jugular bulb, carotid artery dehiscence, jugular diverticuli, abnormalities of the inner ear,28 the choice of primary
size of the vestibular aqueduct, narrowing of the cochlear screening modality in imaging prior to pediatric cochlear
nerve canal, modiolar deficiency, and lateral semicircular implantation may vary by cochlear implant team and center.
canal dysplasia. Similarly, MRI is beneficial in evaluating the It is important to highlight however that CT and MR studies
anatomy of the otic capsule and internal auditory canal: are only macroscopic evaluations of the cochleovestibular
cochlear dysplasia, labyrinthine ossification, the position of apparatus ; form does not necessarily imply function. Evi
the sigmoid sinus, the size of the vestibular aqueduct, nar dence of the existence of a stimulable auditory neural path
rowing of the cochlear nerve canal, modiolar deficiency, lat- way, either by documentation of prior or residual hearing or

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. . .

Fig. 7.4 ad I mages from a patient with bilatera l incomplete pa rtition type I I . (a) Axia l CT scan clea rly depicts an i ntact basal turn a n d confl uent
middle a n d apical turns. (b) Note that a m u ltichannel electrode a rray was implanted nearly a fu l l turn with a few stiffening rings remaining outside
the cochleostomy. This patient a lso has a wide vestibular aq ueduct as seen in the axia l CT image (c), as wel l as i n the T2 weig hted M R image
(d) ma rked by a n . I ntraoperatively, eg ress of cerebrospin a l fluid (CSF) was easily controlled with packing of fascia a round the a rray at the
cochleostomy.

Fig. 7.5 Axial CT scan of the right tem pora l bone


from a patient with incomplete partition type I l l .
Absence o f t h e lamina cribosa is highlig hted b y the
black arrow. The characteristic bulbous fundus is
highlig hted by the black a rrowhead.

by utilization of promontory stimulation testing, predict a differentiation from a common cavity deformity by a combi
more favorable outcome ( Fig. 7.7; Fig. 7.8). nation of MRI and promontory stimulation in selected
A few cochleovestibular anomalies do preclude implanta patients in order to evaluate the possible presence of an
tion. Complete labyrinthine aplasia would be an absolute adjacent stimulable cochlear nerve ganglion cell population.
contraindication for implantation on the affected side. The Traditionally, the failure to identify a cochlear nerve by
determination of cochlear aplasia should involve the careful high-resolution MRl would also contraindicate implantation

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. . . .

Fig. 7.6 T2-weig hted MRI demonstrating non-osseous partitioning of a common cavity. These images a re from the inner ear shown in 11> Fig. 7 .4. (a)
Note the bright signal from fluid seen in the lAC and the cochleovestibular chamber i n the axial section There are low signal intensity septations visible
within the common cavity on both the axial (a) and coronal (b) images that are not seen on CT scanning (white arrows).

Fig. 7.7 Axial CT scan images in another patient with a common cavity deformity. Michel aplasia was present on the contra lateral side. Panels a to c are
depicted from su perior to inferior. Note the formation of rudimenta ry semicircular canals (b). The lAC becomes apparent i n section c. This patient
demonstrated some preoperative subjective auditory sensations and language development. Promontory stim u lation, as well, indicated the presence
of auditory perception. She was successfu lly implanted with a m u ltichannel device, and cu rrently derives significant benefit from implant use.

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. . .

Fig. 7.8 T2-weighted coronal MRI of the inner ear depicted in Fig. 7.6. Panels a to c a re depicted from a nterior to posterior. Note the narrow lAC leading
to the fluid containing common cochleovestibular cavity (a). I mages (b,c) The formation of rudimentary semicircu lar canals that also contain fluid.

regardless of the presence of an implantable cavity. How ogen in association with post meningitis hearing loss.4849 Pneu
ever, recent studies have challenged this concept reporting mococcal meningitis which presents a gram positive exotoxin,
auditory perception following implantation in patients with is additionally associated with severe ossification, while the
radiographic findings of cochlear nerve aplasia or deficiency ossification associated with Hemophilus is generally less severe
although outcomes have typically been reported to not meet owing to the effects of endotoxins which may be diminished by
that of peers without cochlear nerve anomalies.29 - 32 corticosteroids. 5 Some degree of cochlear nee-ossification may
With careful patient selection and preoperative planning, be encountered intraoperatively in approximately 70% of
utilizing the various imaging and electrophysiologic testing patients deafened by meningitis.41
modalities available, and experienced device programmers,
many patients with a variety of cochlear malformations have
been successfully implanted. 3,33-4D Pathophysiology of Labyrinthine Ossification
The cochlear aqueduct is a bony channel that connects the sub
Is There Evidence of arachnoid space of the posterior cranial fossa to the scala tym
pani. It opens adjacent to the round window and is lined with a
luminal Obstruction?
loose network of fibrous tissue termed the "periotic duct,"
In the absence of morphological contraindications to implanta which is an extension of the arachnoid. 51 This is felt to be the
tion, here is the next question that must be answered: Is there site of origin of the inflammatory process into the inner ear in
any evidence of luminal obstruction? Inner ear inflammation, cases of meningitis. Other possible routes include the lAC and
abnormalities of bone metabolism or trauma, may ultimately modiolus. the middle ear windows secondary to otitis media,
result in luminal obstruction either by ingrowth of fibrous lateral canal fistulization secondary to chronic inflammatory
scar tissue or pathological neo-ossification. The etiology most processes, trauma, and hematogenous spread.5253 When
commonly encountered, especially in pediatric cochlear implant encountered, ossification is nearly always severest in the region
candidates, is postmeningitic labyrinthitis ossificans.41 Other post of the round window and proximal scala tympani in the basal
inflammatory causes include suppurative labyrinthitis secondary turn, adjacent to the opening of the cochlear aqueduct.46 The
to otitis media or cholesteatoma, and hematogenous infections middle and apical turns are less commonly affected and the
(e.g. septicemia, mumps, Rubella, or other viral infections). Meta scala vestibuli is often spared. 54 Because most cases of labyrin
bolic bone disorders include otosclerosis and Paget's disease.42 thitis ossificans are partial and the extent of obstruction com
Common posttraumatic causes include labyrinthectomy and monly manifests asymmetrically within an individual patient,
temporal bone fractures. Wegener's granulomatosis, sickle cell preoperative imaging plays an essential role in selection of side
anemia, autoimmune inner diseases, and Cogan's syndrome have to implant. 55 Total cochlear ossification may occur. and is more
also been reported to result in labyrinthine ossification.43-46 commonly seen in children than in adults. 54- 56
Bacterial meningitis is the most common cause of acquired Changes in temporal bone architecture have long been
severe sensorineural hearing loss in children.47 Some degree of described with cochlear ossification. Cochlear ossification
hearing loss has been reported in 5 to 35% of survivors of men following meningitis is associated with a severe loss of cochlear
ingitis.48.49 Deafness may follow bacterial meningitis in children hair cells, as well as with a decreased spiral ganglion cell popu
in 2 to 1 1 %.47 -49 The organisms commonly responsible for post lation. 57 There is no clearly predictable relationship between
meningitic deafness are Haemophilus influenza and Streptococ the extent of ossification and the number of injured spiral gan
cus pneumonia.4B.49 Neisseria meningitidis is also a causative glion cells. Hinojosa et al. studied the temporal bones of deaf
organism, though is felt to result in a lower incidence of post patients with labyrinthitis ossificans and found that the
infectious deafness.48.49 Although the rates of bacterial meningi remaining neuronal cell population ranged from 6,3 1 0 to
tis have declined significantly with current immunization 28,1 96, with a mean of 1 7,1 52.58 This is in comparison to the
protocols, H. influenza is the leading causative organism in total cochlear neuronal population of approximately 35,500 in
meningitis ; however, a higher proportion of individuals with the human infant.59 Linthicum et al. studied the post-mortem
meningitis in association with pneumococcuus develop hearing effects of implants on neuronal population and found that
loss. Pneumococcus remains the most common microbial path- benefit may occur with as few as 3,300 neurons.Go Cochlear

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. . . . .

ossification does not contraindicate cochlear implantation per foci of otosclerosis within the areas of neo-ossification.46
se: it does ; however, complicate electrode insertion.61 All their specimensdemonstrated the pathology to be limited to
There are several theories regarding the pathogenesis of lab the first 6 mm of the basal turn in the scala tympani.
yrinthine neo-ossification. In 1 936, Druss described two types
of new bone: metaplastic bone originates from ingrown fibrous
scar or connective tissue, whereas osteoplastic bone originates Evaluation of Cochlear Patency
from the adjacent otic capsule after disruption of the endo Several authors have reported discrepancies between the CT
steum.62 Postlabyrinthitis ossification is felt to occur via the scan interpretation of cochlear patency and the findings at
metaplastic process. During the initial acute stage of infection, implant surgery. This is likely due in part to the thicker image
bacteria within the perilymphatic spaces induce an acute slices available at the time these studies were performed, as
inflammatory reaction characterized by leukocyte infiltration well as the early stage of experience of the image interpreters.
as well as fibroblast proliferation.63 Labyrinthine fibrosis is Early fibro-ossific changes frequently encountered during
considered to be the early stage of ossification and may occur surgery in post-meningitic patients are frequently not identi
within weeks of initial infection.46.63,64 Ossification eventually fied on CT scanning, particularly early in the time course for
ensues, which is termed the osseous or late stage of labyrinthitis fibro-ossific changes. This would be especially likely when there
ossificans. According to Suigura and Paparella, undifferentiated is little ossification within the fibrous matrix. The time course
mesenchymal cells originating in the endosteum, modiolar for metaplastic ossification is quite variable but is felt to begin
spaces and basilar membrane, likely differentiate into fibroblasts with fibrosis as early as 8 days to a few weeks after the initial
and either subsequently or directly into osteoblasts, and form insult.6S-67 The ultimate time frame and extent of eventual
local or diffuse osseous deposits.63,6S osseous deposition is quite variable. It has been reported to be
Several authors have postulated that the pathogenesis of detected as early as two months post- meningitis in humans by
metaplastic bone formation may be related to disruptions of CT scanning.64 Evidence of ongoing ossification has also been
cochlear blood supply, which has been demonstrated both detected to be present histologically in human temporal bones
experimentally and observed histologically in the temporal as late as 30 years past the initial insult.46
bones of patients having undergone a variety of surgical pro The reported accuracy of high resolution CT scan identifi
cedures.66-71 This theory has been claimed to be supported by cation of cochlear ossification and fibrosis is limited ranging
cell cultures experiments performed by Gorham and West in from 53% to greater than 90%.4.73,74 Because of the variability
which low oxygen tension favors bone formation whereas in reliability of CT findings with regard to cochlear ossifica
high oxygen tension favors osteoclastic resorption.72 Other tion and fibrosis, high-resolution MRI of the otic capsule
investigators have commented on the similar findings and lAC has become the imaging modality of choice in eval
between the ossification of vascular occlusion and that of sup uating cochlear patency. T2-weighted images high-resolution
purative labyrinthitis.63,6S MRI of the lAC allow for visualization of endolymph and
The two types of neo-ossification were further character perilymph within the cochlea indicative of cochlear patency.
ized in histological studies performed by Kotzias and In 2003, Gleeson et al. found MRI findings to correlate with
Linthicum on human temporal bones with a variety of path intraoperative findings in 88% of patients during cochlear
ological processes including patients who had undergone a implantation.28 In 2009, Isaacson et al. sought to determine
variety of neurotologic procedures. 66 The metaplastic form sensitivity and specificity of MRI in the evaluation of
is characterized by high cellularity and the relative cochlear patencyJS The sensitivity and specificity of MRI in
absence of eosinophilia. There are no osteoblasts on the evaluating cochlear patency has been estimated to be 94. 1 %
surface. Though its margins are indistinct, it is confined to and 87.5%, respectively.75 Similarly, the positive and negative
the lumen of the cochlea. The osteoplastic form occurs only predictive value has been demonstrated to be approximately
when there has been disruption of the endosteum such as 95% and 88%, respectively ( Fig. 7.9; Fig. 7.1 0; Fig. 7.1 1 ;
occurs during trauma or with a surgical defect. It is charac Fig. 7 . 1 2 ).75
terized by less cellularity and increased eosinophilia and is
characteristically lamellar in form, with clear margins and
osteoblasts on the surface and not clearly distinct from the Are There Additional Findings That May
endosteal layer. Complicate the Surgery or Subsequent
The postmeningitic neo-ossification is thought to occur via
Patient Management?
the metaplastic process with the ectopic bone being typically
chalky white whereas the native otic capsule bone is generally The initial objectives of preoperative sectional imaging are the
ivory in hue.73 The difference in color and its being confined determination of cochlear morphology and luminal patency.
to the lumen of the cochlea aids in differentiation of the neo Additional useful information may be derived that can opti
ossified bone and the native otic capsule during drilling of the mize the safety and facility of surgery, as well as influence
ossified cochlea during the implantation procedure. subsequent patient management. Proper surgical planning
Advanced otosclerosis may in rare cases cause luminal must involve a careful review of sectional images so that
obstruction that is usually limited to the round window or first potential complications may be anticipated and properly
few millimeters of the scala tympani.46 It has been suggested by managed. Preoperative imaging often provides valuable infor
Kotzias and Linthicum, that the otosclerotic process may dam mation that would not preclude implantation, but rather helps
age the endosteal layer resulting in the osteoplastic form of determine which ear would be the technically easier ear to
neo-ossification.66 In 1 991 , Green et al. histologically identified implant.

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Fig. 7.1 1 CT scan of a patient bilaterally deafened by meningitis


Fig. 7.9 CT scan of a patient bilatera lly deafened by meningitis
demonstrates extensive osseous obliteration i nvolvi ng all turns of the
demonstrates osseous obstruction limited to the proximal basal turn.
cochlea. The opposite side appeared patent. Note the unusual bulbous
Though the middle and apical turns appear patent on CT, fu rther
appearance of the lAC.
evaluation with MRI is warra nted to further assess the possibil ity of
luminal fibrosis. Note that the relationship between the round window
and coch lear aqued uct a re nicely demonstrated in this section .

wall has been reported in 1 .6% and a high riding jugular bulb
may be present in 6% of the general population.76 It is rare,
Vascular Anatomy though possible, that a jugular bulb or diverticulum may
Aberrant middle ear vascular anatomy that might complicate overlie the round window niche or promontory ( .,.. Fig. 7.1 3 ).
mastoidectomy and a facial recess approach to the cochleos The distance between the round window and carotid artery
tomy may be anticipated by the routine acquisition of pre may be determined in cases where a drill-out procedure is
operative CT scanning. An extreme anterior displacement of planned. Abnormal course or dehiscence of the carotid canal
the sigmoid sinus with approximation against posterior canal may also be detected.

Fig. 7.1 0 a,b This patient bilaterally deafened by meningitis demonstrated osseus obliteration of the cochlea extending into the middle and apical
turns on the right side (a). The left cochlea appea red patent by CT scan; however, the coronal M R image (b) demonstrates the presence of
i ntermediate signal within the basal turn (white arrow) which is suggestive of luminal fibrosis.

83
. . . . . . .

MRI of the same patient depicted in Fig. 7 . 1 0 . Note the bilateral ly abnormal bulbous morphology of the lACs as demonstrated by the
Fig. 7 . 1 2
bright signal from CSF fluid on this T2-weighted image. A bright fluid signal is also present in the lumen of the right cochlea but absent on the left side,
which demonstrated extensive osseous obliteration on CT sca n .

The Facial Nerve between the apical turn of the cochlea and the pregeniculate
facial nerve canal permits unwanted stimulation of the facial
Preoperative cr scanning is especially useful in identifying the
nerve during implant use.7879 Careful analysis of the cr study
position of the aberrant facial nerve that may be associated
helps to anticipate that certain electrodes will require depro
with cochlear malformations. It has been well documented in
gramming ( Fig. 7.1 5).
such cases that the course of the facial nerve may be quite
unusual and at increased risk of injury during implantation sur
gery.s4 By careful preoperative mapping of the course of the Mastoid and Tympanic Cavity
facial nerve canal, such patients may be safely and successfully The mastoid air cell system and tympanic cavity should also be
implanted ( Fig. 7.14). Careful review of the position of the included in the analysis of preoperative Cf studies. The degree
facial nerve is also warranted as well in patients without of mastoid pneumatization is especially useful information
cochlear malformations, as there may be dehiscences of the when operating on very young children. Though considered
intratympanic portion that may be encountered during fully developed at birth, the depth of the facial recess as well as
approach to the cochleostomy site. its degree of pneumatization may be anticipated.
In some patients with otosclerosis, findings of otosponeosis on Radiographic findings in conjunction with clinical severity
HRCf has been noted to correlate highly with histopathologic may be considered in side selection as well as determination
findings of otosponeosisF The presence of spongiotic bone of the most appropriate course of therapy for patients with
associated chronic ear disease. Chronic ear disease need not
be an absolute contraindication for cochlear implantation if
carefully selected patients are managed with staged proce
dures. Traditional canal -wall-up surgery or a more extensive
exenteration with a blind-sac and oversew type operation is
appropriate in more severe cases. Subsequent implantation
may be performed in a stable, well-protected, and wellOhealed
fat-obliterated mastoid cavity.
The usual landmarks for performing mastoidectomy and
facial recess may be distorted or absent in patients with
cochlear abnormalities. Careful review of CT images is essential
to safely performing surgery on these patients. There are often
associated morphological abnormalities of the vestibular system
and ossicular chain.4.7 Overall, the lateral semicircular canal is
considered the most frequently malformed inner ear structure,
which is speculated to be due to its late embryonic formation.4

The Vestibular Aqueduct


The association of enlargement of the vestibular aqueduct and
congenital sensorineural hearing loss is well recognized.so-s3
Radiographically, it may occur in conjunction with other identi
Note how a dehiscent jugular bulb may extend onto the
Fig. 7 . 1 3
promontory, potentia lly interfering with the drilling of a cochleostomy. fiable inner ear anomalies as previously discussed, or as an iso
lated finding on Cf or MRI ( Fig. 7.4; Fig. 7.1 6). Enlargement

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. . .

Fig. 7.1 4 a,b Coronal CT scan of a patient with a common cavity deformity. (a) The facial nerve passing su periorly over the common
cochleovestibular chamber (black arrow). (b) A more posterior section, demonstrating that the facial nerve travels along the teg men. I ntraoperatively,
the nerve was identified in its descending portion and followed su periorly to the tegmen in the antral region. Here, it formed its second genu with the
tym panic portion that cou rsed along the teg men tym pani as seen in image b. Preoperative knowledge of this anomalous cou rse was felt to enha nce
the surgical safety of cochlear implantation in this patient.

of the vestibular aqueduct exists when the aqueduct's diameter association with various syndromes (e.g. Pendred syndrome,
is greater than 1 .5 mm at its midpoint, measured between the distal renal tubular acidosis, Waardenburg syndrome, X-linked
common crus and the external aperture into the posterior fossa congenital mixed deafness, branchio-oto-renal syndrome,
based upon the Valvassori criteria.s4 otofaciocervical syndrome, and Noonan syndrome).s2.87-94
The large vestibular aqueduct syndrome is traditionally con The major traditional hypothesis regarding the pathogenesis
sidered to be a distinct clinical entity in patients with radio of this anomaly involves aberrant or arrested development of
graphic evidence of enlargement of the vestibular aqueduct.ss.s6 the endolymphatic duct and sac system, which is based on the
Hearing loss is typically bilateral and progressive, with stepwise observation that in early embryogenesis the duct is shorter,
decrements often associated with episodes of relatively minor straighter. and proportionally much broader than in later
head trauma. Enlargement of the vestibular aqueduct is consid maturity.ss There are a variety of speculative etiologies of the
ered as well to be a relatively common finding in children with
congenital sensorineural hearing loss.s2 Enlarged vestibular
aqueduct may occur as a solitary malformation or may occur in

Fig. 7.1 5 CT scan of a patient with otosclerosis. Note the otospongiotic


changes present adjacent to the labyrinthine portion of the facial nerve
in this coronal section (white arrow). Such pathology could pred ispose Fig. 7 . 1 6 Axial CT image in a deaf child with an enlarged vestibular
the patient to facial nerve stimulation by the electrodes in this region. aqued uct.

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. . . . .

hearing loss associated with this disorder based mostly on clini


cal, radiographic, and surgical observations, as well as some
analyses of endolymphatic chemical composition. Among them
are damage secondary to transduction of intracranial CSF pres
sures; reflux of protein rich hyperosmolar endolymph from
endolymphatic sac into the cochlea through the widely patent
duct; and inherent functional abnormalities of endolymphatic
sac system leading to abnormal intracochlear fluid composition
and dynamics.s2
The clinical significance of radiographic enlargements of ves
tibular aqueduct and endolymphatic duct and sac system with
regards to the cochlear implant candidate is twofold. It may
serve both as a diagnosis as well as an indicator of the potential
for the need to manage an intraoperative CSF leak at the time
of cochleostomy. Despite this risk, many such patients have
been safely and successfully implanted with the appropriate
management.9S-97
Fig. 7. 1 7 Plain radiograph of a cadaver head with an intracochlear
Summary electrode a rray in place. Barium markers were placed at the round
window (a) and coch leostomy (b). The position of the cochleostomy
Preoperative sectional imaging is a vital tool for confirming the can be approximated relative to the point at which the electrode array
presence and nature of an implantable cochlear lumen. Imaging crosses the dense otic capsule bone, that is, the inferior cochlear
analysis should include the detection of malformations, luminal margin (arrowheads). The cou nted rings that lay posterior to this
i ntersection a re considered to be clearly outside the cochlea.
obstruction, and anatomic variants or middle ear pathology
that could complicate the implantation process. Preimplant
evaluation of these patients is often quite complex, taking into
account a variety of clinical and electrophysiological data. surgery, prior to reversal of anesthesia. This image will
confirm foremost that the implant has been placed intraco
chlearly in normal cochleas and in cases with severe mal
Postoperative Imaging formation. These films also serve as a baseline for comparison
Postoperative imaging goals for cochlear implant patients with any future films. Further, it makes the implant team
include both imaging of the implant hardware itself as well as aware of malpositioning or damage to the electrode array
diagnostic imaging of either adjacent or distant structures. Plain which would be extremely useful if there was undue difficulty
film radiography can confirm proper initial surgical placement during insertion or suspected device malfunction on intrao
of the cochlear implant electrode array. Interval plain films may perative electrophysiological monitoring. Additional informa
be obtained when there is suspicion of movement or mal tion that can be gained from analysis of intraopertive and
function that is often heralded by changes in program maps. postoperative plain films include detection of device extrusion
Patients with cochlear implants may also require diagnostic as an etiology of malfunction, and electrode positioning rela
sectional imaging for reasons unrelated to the implant itself. tive to the labyrinthine portion of the facial nerve in cases of
Because cochlear implants contain metallic components with undesired postoperative facial nerve stimulation. It is our pol
varying ferromagnetic properties, its presence affects both the icy to compare a recently obtained postoperative plain film
safety and ability to obtain useful images of adjacent structures with an earlier film in the evaluation of suspected malfunction
depending upon which sectional imaging technique is utilized. or significant changes in psychophysical measurements.
With the increased popularity of MR as a diagnostic imaging Intraoperative portable films are usually taken in an oblique
modality, it is important to understand the issues surrounding anti-Stenver's view or a transorbital orientation. A three-view
its compatibility with the currently manufactured as well mastoid series (Stenver's, transorbital, base) may be obtained
as previously implanted devices that may be encountered in on postoperative day one. Follow-up films are obtained in mul
patients today. tiple views so that an appropriate film can be found to best
match the intraoperative film orientation.
It is essential to be familiar with the characteristic appearan
Determination and Monitoring of ces of electrode arrays in both normal and malformed cochleas
and to utilize a consistent method of determination of electrode
Electrode Position insertion depth. Trigonometric techniques of determining
Currently at most cochlear implant centers, intraoperative round window location on plain radiographs have been
plain radiographs of the temporal bones are available to assist described.99- 101 Intracochlear electrode position is then dis
in the determination of cochlear implant electrode position. cerned from this reference point. These techniques are depen
Plain radiographs remains a simple, inexpensive, reliable dent on good-quality radiographs with appropriate views so
method of determining electrode position.9s Many centers, that the important landmarks are readily visible. Intraoperative
routinely obtain an intraoperative portable plain radiograph films are often of poorer quality than postoperative films due to
to confirm final electrode position at the conclusion of the nature of portable x-ray equipment and the inability to

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. . .

place the implanted side closer to the x-ray film. They are fre
quently of low contrast and do not allow the easy visualization
of the fine structures of the labyrinth. In these situations, we
have found it useful to utilize a computer enhancement method
for determination of cochleostomy position. Our method of
interpretation was developed through the analysis of cadaver
temporal bones and confirmed using video analysis of several
clinical cases. A description of the process is detailed in
Fig. 7.1 7 and Fig. 7.18.

Fig. 7.1 9 a-c Normal postoperative plain radiograph of a Clarion


multichan nel cochlear implant electrode array implanted into
the right cochlea. (a) Stenver's view, (b) Transorbital view, and
(c) Base view.

Plain Radiographs
Fig. 7.1 9, Fig. 7.20, Fig. 7.2 1 , Fig. 7.22, Fig. 7.23,
Fig. 7.24, Fig. 7.25, Fig. 7.26, Fig. 7.27, Fig. 7.28
illustrate the expected appearance of both properly and
improperly placed electrode arrays as determined by plain
radiographs. Several interesting clinical case examples are
Fig. 7.1 8 a c Confirmation o f t h e va lidity o f t h e landmark for
also provided.
electrode cou nts was evaluated with severa l clinical cases. The
i ntraoperative video ana lysis is compared to the im mediate post
operative radiog raph. The films are digitally scan ned into a g raphics
workstation . I mage contrast filters enhance the visibility of the
Magnetic Resonance Imaging
electrode a rray and surrou nding osseous structu res. The infero Compatibility of Cochlear Implants
posterior cochlear margin is readily visible and determination of the
point at which the electrode array enters the dense bony otic capsule is Magnetic resonance imaging is the diagnostic study of choice
made. (a) Video-ca ptured post-insertion image from a clinical case for many disease entities. Three electromagnetic fields are gen
reveals that band 10 is intracochlear whereas band 9 is at the erated during acquisition of data for MR images. A constant,
cochleostomy. (b) Image of digitally sca nned X-ray with reference strong, static magnetic field aligns protons; a rapidly changing,
markers placed to facilitate counting. (c) Threshold adjustments
small magnetic field gradient is developed for spatial localiza
demonstrate the infero-posterior coch lear margin (arrowheads) and the
relative positions of the electrode bands. Note the positions of bands 9
tion; radio frequency ( RF) pulses produce proton energy state
and 1 0. changes.102 The strengths of the fields are proportional to the
rating in teslas (T) of the MRI magnet. Imaging time, pulse

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Fig. 7.21 Intraoperative porta ble plain radiograph (transorbital pro


jection) of the patient depicted in Fig. 7 . 1 9. Note that despite the
diminished image resol ution, the electrode a rray can clea rly be
identified within the density of the otic capsule and conforms
appropriately to the shape of the cochlear lumen.

the generation of heat or electrical currents in or around elec


tronic devices that might damage surrounding tissues, damage
the device, or cause unintentional device output. Internal mag
nets can become demagnetized making them dysfunctional
and distortion of needed images may render the studies unin
terpretable or inadequate.
When it is impossible to adequately study an anatomical
region in proximity to an MRI compatible device, the ade
quacy of alternative imaging modalities ( CT scan) must be
weighed against the risks and benefits of explantation. How
ever, it is a reasonable assumption that every child born in
the United States will need to have an MRI at some point
during his or her lifetime.1 03 Issues of compatibility and
potential human harm arise when the recipient of an
implanted medical device incurs the need for an MRI study.
The patient with a cochlear implant and the potential
implant candidate requiring an MRI or serial MRis, present
the physician with a unique clinical decision-making process.
Cochlear implants often contain ferromagnetic electronic
parts and casings and an internal magnet that aligns an
external antenna on the overlying scalp.
Traditionally, the presence of a cochlear implant was
deemed a contraindication for MRI. During the consent pro
cess, patients should be informed of the concern regarding
Fig. 7.20 a-c Normal postoperative plain radiogra ph of a Nucleus 24
MRI exposure.103 Various studies evaluated the impact of
multichannel coch lear implant electrode array implanted into the left
cochlea. (a) Stenver's view, (b) Transorbita l view, and (c) Base view. I n
MRI exposure in individuals with cochlear implant devices.
a, t h e lAC ( * ) , vestibule (v), and su perior semicircu lar c a n a l (white At higher magnetic field strength, Dubrulle et al. tested
arrow) are seen. implant devices in vitro and in vivo attached to the scalps
of volunteers in 2013 to assess demagnetization, tempera
ture change, and electronic malfunction using a 3 T MRI.1 04
sequences, and RF emissions also contribute to field strength. The degree of demagnetization was noted to be dependent
Although harmless to normal human tissue, these fields have on the angle between the implant magnetic field (bi) and
the potential to generate torque and force on the ferromagnetic the MRI magnetic field. With an angle of 90 degrees, the
components of implanted devices. Additional concerns include risk of demagnetization was found to be low (6.6%); with

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. . .

Fig. 7.22 a.b (a) I ntraoperative portable plain radiograph of a MedEl multichannel cochlear implant electrode array. (b) Note the cloverleaf
configuration of the free g round electrode.

angles less than 90 degrees, no demagnetization was 1. Does the device contain an internal magnet? All major
observed. However, in angles greater than 90 degrees, cochlear implant companies currently manufacture
demagnetization occurs in almost 60% of patients. These standard devices with internal magnets. The companies
findings correlate with the 2008 report by Majdani et ai.1 05 also manufacture "special-order" devices without
On cadaveric analysis using a 1 .5 T MRI, moderate to internal magnets for implantation in patients with
severe displacement of the magnet from the internal device known MRI needs. These special devices require the
was observed.106 However, application of a compression use of an adhesive-backed external scalp magnet to
dressing can prevent this displacement when exposed to position the external antenna over the receiver. If
a 1 .5 T MRI.106 the status of a particular implant is unknown, the
In the event that an implantee requires an MRI, device clinician should contact the company, providing them
compatibility must first be determined. The answers to the with the patient's identification number and device
following three questions guide the clinical decision-making serial number.
process. 2. Is the magnet removable? For most Advanced Bionics and
Nucleus devices, the magnet is removable. Magnets within
the Med-El devices are not removable.
3. Are the internal components and casing ferromagnetic
and resistant to damage by the MRI machine's generated
magnetic fields? Device construction can be determined
by plain film analysis of radio-opaque lettering and/or by
contacting the company with the device serial number
and patient identification. Because of the diversity of
devices currently circulating within the cochlear implant
population, patients and providers are encouraged to
contact the manufacturer with any question regarding
internal device ferromagnetics.

If it is determined by the above-cited three questions that a


patient's device is MRI compatible, scanning can be under
taken. All external hardware must first be removed from the
patient, as these components are not MRI compatible. Addi
tionally, there will be a zone of susceptibility artifact with
Fig. 7.23 Normal plain radiograph of a n lneraid m u ltich a n nel distortion of the local magnetic fields in the region of
cochlear i m p l a nt electrode a rray impla nted i nto the right the implant regardless of its resistance to internal damage
cochlea (Stenver"s projection). Note the screws that fix the
or local tissue injury. .,.. Fig. 7.29 depicts a sequence of
tra nscuta neous pedestal to the sku l l . Note that there a re six
ball electrodes as wel l as two g round electrodes (promontory
MR images obtained on a patient implanted with a modified
a n d free). Nucleus Mini-22 device who had an acoustic neuroma on
the opposite side. Satisfactory serial images were obtained

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. . . . .

Fig. 7.24 a,b (a) Image after insertion of a Clarion m u ltichannel cochlear implant electrode array. Note that the tip has kinked and doubled over onto
itself (arrow). (b) Image after reinsertion of the same device. Note the proper position of the dista l electrode pair (arrow).

on a 1 .5 T machine, providing necessary information for further away the anatomical area of concern for study is
patient management. from the device, the less of an issue the magnetic field
Susceptibility artifact is increased with the strength of the strength of the magnet becomes.
magnet in teslas and is related to the pulse sequence uti In cases where MRI was considered a medical necessity,
lized. Consideration should be given to utilizing a lower protocols for removal of the magnet associated with the
testa MRI system in order to obtain adequate imaging. Imag cochlear implant device have been developed.107 In the
ing time and RF settings should also be optimized. The United States, devices produced by three manufacturers have

Fig. 7.25 a,b Postoperative plain radiographs taken of a m u ltichan nel electrode array inserted into a hypotympanic air cell tract (a). Note that in a,
the electrode has an u n usual configuration and is located below the dense otic capsule bone. (b) Base view of the same implant.

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. - .

Fig. 7.27 I ntraoperative porta ble plain radiograph (lateral projection)


of a m u ltichannel cochlear implant electrode array impla nted into the
common cavity of the patient depicted in Fig. 7 .4. Note how the
electrode assumes a cu rved configuration and is located within the
density of the otic capsule bone. I n this image, there are superimposed
Fig. 7.26 Transorbita l plain radiograph taken after implantation of a electrode cables that are used for intraoperative electrophysiological
m u ltichannel electrode a rray i nto the d ri ll-out of a straight tunnel into monitoring.
the basal turn of a patient with extensive bilateral labyrinthitis
obliterans.

including the determination of the adequacy of alternative


imaging modalities or temporary or permanent explantation
been approved by the Food and Drug Administration ( FDA) : with the possibility of reimplantation with an MRI compati
Advanced Bionics, Cochlear Americas, and Med-El. Devices ble device.
manufactured by Advanced Bionics and Cochlear Americas In the past, the presence of a cochlear implant was consid
are currently designed with removable magnets. This allows ered to be a major contraindication to MRI.1 With advances
for removal if necessary for MRI compatibility. The current in implant technology and an understanding of the RF, pulse
devices manufactured by Med-El do not have a removable sequences, and image time factors, it is now possible to
magnet. obtain useful images. In practice, the cochlear implant candi
In june 2013, the FDA approved the first cochlear implant date with known future MRI needs should be implanted
devices as MRI compatible for 1 .5 T MRI scans. Three model with a device considered MRI compatible by the above-men
devices produced by Med-El were approved. Further study is tioned criteria. Implantation of a device with a removable
underway to increase the MRI compatibility of cochlear implant magnet would potentially provide the patient with the
devices. option of a magnet retrofit should future MRI no longer be
If it is determined that a patient's device is not MRI required. Alternatively, an FDA-approved device for 1 .5 T MRI
compatible, then a number of options may be entertained, may be considered for implantation, thereby negating the

Fig. 7.28 a,b (a) Axia l CT image of a patient with a severely hypoplastic coch lea. The cochlea is a small spherical cavity, which is seen in
this image a nterior to the vesti bule a n d ova l window. (b) A m u ltichannel electrode a rray assumes a pigta i l configu ration as it coils inside the
cochlear l u m e n .

91
. . . . .

need for magnet removal and retrofit. Additionally "special


order" magnetless device with nonferromagnetic internal
components can be obtained from all of the major implant
manufacturers.

Fluoroscopically Assisted
Cochlear Implantation
Fluoroscopically assisted cochlear implantation allows visual
ization of electrode insertion in real time. This technique
was initially developed for laboratory study of electrode
prototype and to evaluate surgical technique. 108 Insertion
angle, depth, and cochleostomy position were studied and
optimized with fluoroscopic evaluation. Complication simula
tion provided useful information regarding the avoidance of
cochlear and electrode damage. Cadaver temporal bones
were implanted under fluoroscopic guidance in order to eval
uate insertion dynamics and mechanisms of intracochlear
trauma using conventionally implanted electrode arrays as
well as a variety of peri-modiolar prototypes. The informa
tion gleaned from these studies provided necessary feedback
that eventually affected electrode design by the engineers.
Real-time fluoroscopy provides a visual image that correlates
with tactile sensations that a surgeon may experience during
electrode insertion. 1 09 Intraoperative fluoroscopy have been
found to be beneficial in assisting with visualization of
cochlear implant trajectory during implantation of patients
with cochlear malformations.ID9.1 J o

Technique
Intraoperative fluoroscopy (IF) is performed using the C-arm
unit utilized for conventional neurosurgical, orthopaedic and
angiographic surgeries. The patient is placed on the table in
a standard position for cochlear implantation: supine with
the head turned away from the operating surgeon. The
C-arm is placed with the beam generator beneath the table
directed in an anti-Stenver's view (in contrast to traditional
Stenver's, where the ear of concern is placed against the
image plate. ) Narrowing and centering the beam on the
cochlea magnifies the image. Fluoroscopic assistance may
be employed in the case of inner ear malformation at the
time of cochleostomy for determination of proper placement,
as well as during electrode insertion. Images are recorded in
real time on video for future analysis. Care is taken to avoid
placement of radiodense monitoring leads in the imaging
path. Preset settings used for orthopaedic procedures typi
cally yield the best image quality. Adjustments of both posi
Fig. 7.29 a-c (a) Tl -weig hted contrast e n h a n ced MR showing
tion and beam characteristics are made so that the dense
large left-sided acoustic neuroma in a patient with contra lateral
(AD) long-term deafness. (b) MRI taken post-implantation of a otic capsule is well visualized with minimal interference
mod ified Nucleus M i ni-22 magnetless cochlear implant in the from adjacent skull structures and instrumentation. Surgery
right ear. Note that susceptibil ity a rtifact generated by the is performed as per routine ( .,.. Fig. 7.30).
magnetless device stil l a l l ows for monitoring of a contra latera l
disease process. (c) Baseline post-operative MRI fol lowing tra ns
labyrinth ine total tumor remova l delineates anatomy i n tumor Safety
reg ion despite susceptibility a rtifact from a contra lateral
magnetless cochlea r i m plant. Fluoroscopy is utilized as briefly as possible in order to
keep exposure levels well below exposure tolerance of the
human lens, the structure most vulnerable to exposure.1 1 1
Narrowing the beam will minimize the radiation received

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. . .

Fig. 7.30 a.b (a) Axia l and (b) coronal views of a patient with a common cavity malformation who was impla nted under fluoroscopic guidance. Note
the common cavity (cc) and internal auditory canal (iac). { From Fishman AJ, Roland jr JT, Alexiades G, Mierzwinski j, Cohen NL. Fluoroscopically
Assisted Cochlear Impla ntation. Otology and Neu rotology 2003;24{6):882886. Reprinted by permission.)

by the patient. Direct penetration of the orbit should be utilized during cochleostomy localization and electrode
avoided. Consultation with the radiation safety officer is insertion. The cochleostomy was performed on the promon
recommended, at which time, information regarding the tory bulge inferior to the oval window. The patient had no
fluoroscopy unit's calibrated skin entrance exposure level may round window. The position was determined by correlation
be obtained. Most modern units produce a dose well below of CT images with intraoperative surface landmarks as well
1 0 rads (0.1 gray) per minute of use. The dose to the lens may as confirmed by intraoperative fluoroscopy ( Fig. 7.32a).
be estimated by calculating 20% of this value. The total exposure There was no egress of CSF. All 22 active electrodes were
should be kept below 200 rads (2 gray). The typical exposure inserted in a C shaped fashion ( Fig. 7.32b). Intraoperative
time should be no more than a total of 1 to 3 minutes of short electrophysiologic monitoring revealed present stapedial
multiple exposures for cochlear implantation yielding a maxi reflexes and Neural Response Telemetry in multiple electro
mally calculated dose of 10 rad/min x 3 minutes x 0.2 6 rads for
= des tested throughout the array. Her postoperative course
a 3-minute exposure. Exposure can usually be limited to well has been unremarkable.
under 1 minute. Lead aprons and thyroid shields are worn by
the operating-room staff. The patient is similarly protected Case 2
( Fig. 7.30).
This patient is a congenitally deaf female with a common
cavity malformation who required reimplantation of a right
common cavity malformation 3 years after the original sur
Case Studies of Inner
gery. She was originally implanted with a Nucleus Cl24 M.
Ear Malformations Her right cochleovestibular apparatus is the larger of her
bilateral defects and is comprised of a small common cavity
Case 1
measuring 6.5 mm in maximum diameter with hypoplastic
The patient is a 4-year-old congenitally deaf female. She was semicircular canals and an absent endolymphatic duct
recently implanted with a Nucleus CI24 M device in her right ( Fig. 7.33a). This is associated with an lAC measuring
ear. Preoperative radiographic evaluation revealed that her 2.5 mm in mid-diameter ( Fig. 7.33b).
right cochleovestibular apparatus is comprised of a small At the time of initial implantation, the cochleostomy was per
common cavity measuring 7 mm in maximum diameter with formed anterior to a common window depression. There was no
hypoplastic semicircular canals and an absent endolym egress of CSF during cochleostomy. Seventeen electrode bands
phatic duct ( Fig. 7.3 1 ). Her left cochleovestibular was con passed easily. With the introduction of three additional electrode
sistent with cochlear aplasia and was therefore not suitable bands, a copious egress of CSF was encountered. This could not
for implantation. be controlled with packing the cochleostomy alone and the
Implantation surgery was performed via a trans-mastoid patient was managed with a course of continuous lumbar spinal
with facial recess approach. Intraoperative fluoroscopy was drainage. The CSF leak resolved without consequence.

93
. . . . .

Fig. 7 . 3 1 a,b
I ntraoperative real time fluoroscopic images taken from same patient in Fig. 7.2. (a) The cochleostomy position is verified by
placement of a fine pick (a rrow). Cc, common cavity; iac, interna l aud itory canal. All 22 active electrodes are inserted in a (-shape configuration withi n
t h e common cavity. (b) Note that t h e morphology on fluoroscopic i m a g e approximates t h e coronal C T image. { From Fishman Aj, Roland jr JT,
Alexiades G, Mierzwinski j, Cohen NL. Fluoroscopica lly Assisted Cochlear Implantation. Otology and Neu rotology 2003;24{6):882-886. Reprinted by
permission.)

Intraoperative plain radiography revealed the electrode tion, at which time she had increased channel interaction
array had traversed the common cavity and passed into the and decreased performance. A decision was made to reposi
lAC ( Fig. 7.34). Because most of the electrodes were tion the array under fluoroscopic guidance so that intramea
located within the common cavity, the decision was made tal insertion could be avoided and the maximum number of
not the reposition the array for fear of cochlear or facial available electrodes could be positioned into the common
nerve damage. Four electrode bands required deactivation cavity. The same array was first removed and then rein
secondary to facial nerve stimulation. Although she had not serted with 1 7 electrode bands forming a c shape configura
achieved open-set speech recognition, improvements have tion inside the common cavity ( .,.. Fig. 7.3 5 ). There was
been noted. no cerebrospinal fluid leak. The patient uses 1 7 electrodes
She was performing well, mainstreamed in a normal class postoperatively with performance currently improved from
room with an oral interpreter until 3 years post-implanta- the pre-operative condition.

Fig. 7.32 a,b Axia l CT (a) and coronal MRI (b) in patient with common cavity ma lformation. Note the small common cavity ( * ) with the narrow lAC
(arrow). The tem pora l lobe is labeled for orientation on coronal image (t). Used with permission from Fishman AJ, Roland j r JT, Alexiades G , Mierzwinski
j , Cohen NL. Fluoroscopically Assisted Coch lear Impla ntation. Otology and Neurotology 24{6):882-6. November 2003.
Sou rce: From Fishman Aj, Roland j r JT, Alexiades G, Mierzwinski j , Cohen NL. Fluoroscopically Assisted Cochlear Implantation. Otology and Neurotology
2003;24{6):882-886. Reprinted by permission.

94
. . .

Fig. 7.33 A transorbital intraoperative view taken during surgery of the patient in 1> Fig . 7.4. Note that the a rray has passed i nto the lAC. The arrow
denotes the j unction between the common cavity and the lAC as seen in this orientation. The inset outlines the l u men of the common cavity (cc) as
wel l as the interna l aud itory canal (iac). Used with permission from Fishman AJ, Roland jr JT, Alexiades G, Mierzwi nski j, Cohen NL. Fluoroscopically
Assisted Coch lear Impla ntation. Otology and Neurotology 24(6):882-6. November 2003.
Source: From Fish man Aj, Roland j r JT, Alexiades G , M ierzwinski j , Cohen NL. Fluoroscopically Assisted Cochlear Implantation. Otology and Neurotology
2003;24(6):882-886. Reprinted by perm ission.

Case Studies of Cochlear Luminal as visualized fluoroscopically. Seventeen electrodes are active
in the program map.
Obstruction with Split Array
Case 1 Case 2
A 44-year-old female with labyrinthine ossification secondary An 1 1 -year-old female with deafness secondary to meningitis
to meningitis received an incomplete insertion of a Cl24 M was originally implanted at another center with a Nucleus
electrode array AS at another institution. 1> Fig. 7.35 ademon Cl22 at age 4 with four intracochlear electrodes. The patient
strates a kinked array on plain film. Due to a device failure, was receiving some benefit from the device until 5 years
she underwent revision at our center and received a Nucleus postoperatively when the device failed electronically. At revi
CI24 Double Array under fluoroscopic guidance. A basal turn sion surgery a Cl24 Double Array was placed under fluoro
drill-out allowed full insertion of all 1 1 electrodes of the lower scopic guidance. An 8-mm basal turn drill - out was performed
array. Seven of the 1 1 available upper electrodes were with insertion of 10 lower electrodes and 5 upper electrodes
inserted through the apical cochleostomy. .,.. Fig. 7.35b demon through an apical cochleostomy. She currently uses 13 elec
strates the split array in good position, without kinks or bends trodes in the program map.

95
. . . .

s
Fig. 7.34 ad (a) I ntraoperative fluoroscopy reveals the implant has been repositioned entirely within the common cavity. (b) The outline of the
common cavity lumen (cc). Insertion was stopped at this point due to the visualization of cessation of adva ncement and kinking. (c) The postoperative
plain fi l m confirms proper placement. Arrows denote the cc-iac junction. (d) Detail of same A detail view revea ls the common cavity (cc) lumen outline
and the internal auditory canal (iac) outline. Used with permission from Fishman AJ, Roland j r JT, Alexiades G, Mierzwinski j , Cohen NL. Fluoroscopically
Assisted Coch lear I m plantation. Otology and Neurotology 24(6):882-6. November 2003.
Sou rce: From Fishman Aj, Roland j r JT, Alexiades G, Mierzwinski j , Cohen NL. Fluoroscopically Assisted Cochlear Implantation. Otology and Neurotology
2003;24(6):882-886. Reprinted by permission.

Fig. 7.35 a,b (a) Plain x-ray reveals kinked array and partial insertion in a patient with a history of meningitis. Patient was referred for reimpla ntation
for a device failure. (b) I ntraoperative fluoroscopy was utilized to confirm proper placement during revision surgery of same patient. A Nucleus 24
Double a rray was utilized . Arrows denote the upper and lower electrode arrays in proper position without kinking or bending. Used with permission
from Fishman Aj , Roland j r JT, Alexiades G , Mierzwinski j , Cohen N L.Fiuoroscopical ly Assisted Cochlear Impla ntation. Otology and Neu rotology 24
(6):882-6. November 2003.
Source: From Fishman Aj, Roland j r JT, Alexiades G , Mierzwinski j , Cohen N L. Fiuoroscopically Assisted Cochlear Implantation. Otology and Neurotology
2003;24(6):882-886. Reprinted by permission.

96
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(23] Demir 01, Cakmakci H. Erdag TK, Men S. Narrow duplicated internal auditory
Summary canal : radiological findings and review of the literature. Pediatr Radio! 2005;
35: 1 220- 1 223
Intraoperative fluoroscopy is a useful adjunct to cochlear
[24] Lee SY. Cha SH, jeon MH et al. Narrow duplicated or triplicated internal audi
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an investigative strategy. Otol Neurotol 2007 ; 28: 3 1 7-324

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8 I ntraoperative M o n itori ng D u ri n g Coc h l e a r I m pl a ntation


Mauro K. Cosetti

the electrode, incomplete insertion or extrusion of the elec


Introduction trode array, and, more rarely, a faulty or damaged electrode
Optimal, atraumatic electrode placement within the scala contact.2 -4
tympani of the cochlea is a well-recognized prerequisite for Programming software from each CI manufacturer allows
maximizing cochlear implant (CI) success. In an ideal setting, measurement of El intraoperatively. Published reports on
detection of incorrect electrode placement or a malfunctioning intraoperative monitoring suggest abnormalities in imped
or failed receiver-stimulator would occur at the time of surgery, ance telemetry occur in 6 to 8% of cases.s.6 Up to 50% of ini
thus affording the opportunity for immediate action while the tially abnormal intraoperative values normalize when
patient remains under anesthesia. Toward this end, a variety remeasured after 4 to 6 minutes, suggesting that transient
of electrophysiological and radiological techniques have been irregularities in the electrode-tissue interface ( such as an air
utilized for intraoperative monitoring during cochlear implan bubble generated by electrode insertion) may be responsi
tation. Currently, practice standards for monitoring vary among ble.6 Compared with intraoperative testing, abnormal
institutions and individual surgeons and range from minimal or impedance telemetry is less common in the postoperative
no testing to sophisticated, research-based protocols. Tests used period and may range from 0.1 to 5.0%.57 Because Cis are
for intraoperative monitoring may also be influenced by the programmed to maintain a constant current, changes in
patient's anatomy, such as the use of fluoroscopy during electrode impendence are offset by voltage changes so as
implantation of patients with inner ear malformations, or not to directly impact the threshold (T- ) and comfort (C-)
access to a particular technology, such as intraoperative levels associated with programming. Changes in impedance
computed tomography (CT) scanning. telemetry may reflect stimulation parameters, the amount of
This chapter discusses various electrophysiological and radio device use, or intracochlear fibrosis, and are expected in the
logical monitoring techniques utilized during cochlear implan postoperative period.2 However, progressively increasing
tation in traditional adult and pediatric candidates with normal impedance values, or fluctuating or alternating patterns,
anatomy. Techniques specific to patients with cochlear malfor may result in individual electrode circuit failures requiring
mations, as well as emerging data on intraoperative monitoring deactivation. Although not directly correlated with speech
in patients with residual acoustic hearing, are also discussed. performance, postoperative deactivation of more than three
Lastly, the intraoperative monitoring protocol followed at the or five electrodes has been shown to be worrisome for
editor's institution is briefly described. eventual device failure requiring reimplantation.57 To date,
eventual electrode deactivation has not been correlated with
intraoperative testing.56 In Carlson et al's5 study, three of
Electrophysiological Testing 636 patients with three or more individual circuit failures
At present, all commercially available Cis have telemetric underwent reimplantation for poor performance ; none of
capabilities, meaning that information about the status of these had abnormal impedance telemetry intraoperatively.
the implanted electronics can be conveyed to the speech Similarly, in their review of revision cases, Cosetti et al6
processor for evaluation by the clinician.1 This so-called found no unique pattern of intraoperative El. Further, among
reverse telemetry enables monitoring of device functionality patients with abnormal intraoperative El, none developed
and neural responsiveness to electrical stimulation. Teleme device failure within the study period.
try has allowed a variety of electrophysiological tests to be Emerging data suggest that impedance values can also be
applied to the operative setting. These include electrode affected by a variety of physiological and otologic disease states,
impedance (EI), evoked compound action potential ( ECAP), including diabetes, inflammatory disorders, and Meniere's
spread of excitation (SOE), and electrically elicited stapedial disease, or medications, such as steroids or human growth
reflex thresholds (ESRT). The following sections discuss each hormone.8 Research into the direct effect of these conditions on
test in detail, highlighting its application to the intraopera the intracochlear microenvironment, the electrode-tissue
tive setting. interface, and specifically on their implications for intraopera
tive impedance testing is not currently available.
As described above, El testing does not convey information
Electrode Impedance regarding the location or position of the electrode array, and
Electrode impedance is a function of the array itself and normal values may be obtained when the electrode is
the resistive characteristics of the fluid or tissue surround extracochlear as long as the electrode-tissue interface is
ing each electrode. El measurements provide information maintained. Normal impedance telemetry has been obtained
about individual electrode integrity, such as short or open when the Cl electrode array was located in the carotid,
circuits, as well as the electrode-tissue interface.2 Short superior semicircular, and internal auditory canals.6910 Thus,
circuits are characterized by low impedance values and although El may be included as part of a battery of intrao
indicate a common conduction pathway between two or perative tests, abnormalities obtained during surgery do not
more electrodes. In contrast, open circuits generate high have clear implications for postoperative device function or
impedance measurements and relay information about the performance and, as such, should not influence intraopera
adjacent tissue environment, such as an air bubble around tive decision making.

1 00
. . . . . I t

Eclectically Evoked Compound response to electrical stimulation or a dysfunctional device.


Inability to obtain NRT thresholds for implanted electrodes is a
Action Potential rare event that should not guide use of the backup device.6
Peripheral stimulation of the auditory nerve results in a series With respect to electrode position, measurable ECAP
of measurable potentials, including the cochlear microphonic responses have been demonstrated in cases in which the CI
(discussed in a later section), the summating potential, and the electrode was extracochlear or had an intracochlear tip rollover.
compound action potential (CAP) of the auditory nerve. In There are five reports of electrode placement in the superior
severely to profoundly deafened individuals, only the CAP curricular canal.6924-26 In four of these reports, intraoperative
remains.1 Once inserted into the cochlea, it is possible to electri monitoring demonstrated measurable ECAP responses on
cally stimulate the auditory nerve using the Cl electrode, multiple electrodes despite the extracochlear electrode
thereby generating an electrically evoked compound action location.624-26 In addition, normal ECAP responses have been
potential or ECAP.11 This peripheral, objective measure of the documented in multiple cases of tip rollover.627 In all cases of
neural response of the auditory system to electrical stimulation electrode malposition, electrode location was detected by
was first measured in CI recipients in 1 990.12-14 Presently, each radiographic imaging (either x-ray or CT). All cases required
manufacturer has software allowing telemetric intraoperative revision surgery.
ECAP measurement: neural response imaging (NRI) introduced Although not indicative of performance, device functionality,
for Advanced Bionics ( Los Angeles, CA) systems, auditory nerve or electrode position, intraoperative ECAP measurements have
response telemetry (ART) for Med-El (lnnsbruck, Austria), and demonstrated significant value in other areas of cochlear
neural response telemetry (NRT) from Cochlear Corporation implantation, such as postoperative programming. ECAP
(Sydney, Australia).1415 ECAP measurements are generated by threshold values obtained during surgery can serve as a basis
applying a biphasic stimulus pulse or series of pulses to an for initial program creation in very young children and other
electrode in the intracochlear array and then measuring the populations in which behavioral programming can be challeng
voltage response on an adjacent or nearby electrode.1216 Fol ing.17.2 S-3 o The relationship and correlation between ECAP val
lowing transcutaneous transfer to an external receiver through ues and behaviorally obtained threshold (T-) and comfort (C-)
the reverse telemetry system, these electrophysiological levels have been examined in a multitude of studies in both
thresholds may be evaluated visually by a clinician or, if availa adults and children.B1718.30-37 Although the bulk of the
ble, automatically calculated using an amplitude growth algo research suggests only modest correlations between ECAP pre
rithm in the manufacturer's software.141 7 In general, ECAP dicted and psychophysically assessed T- and C- levels, studies
recording is rapid, unaffected by motion artifact or the type on speech perception following ECAP-based speech processor
or depth of anesthesia, thus making it feasible in both the oper fitting suggest that, although not ideal, these objective mea
ating room and the clinic.12 sures can provide a valid basis for initial programming.38 -42
Intraoperatively, ECAP can be used to assess the response of a
patient's auditory system to electrical stimulation immediately
following intracochlear insertion of the Cl electrode.14-1 S Post
Spread of Excitation
operatively, these intraoperative threshold ECAP (threshold Intraoperatively, spread of excitation (SOE) has been used to
NRT [ tNRT]) values have not been shown to correlate with detect a tip rollover of the electrode array. When correctly
speech performance at one year. In a recent study, intraopera placed within the cochlea, each electrode or channel should
tive tNRT values of adults (n 73 ) and children (n 24) with
= = theoretically stimulate a unique population of neurons. Although
normal inner ear anatomy implanted with the Nucleus Freedom there is undoubtedly some overlap in the neurons stimulated by
device between 2005 and 2008 were examined for a relation each electrode, neuronal selectivity and maximization of per
ship with age-appropriate tests of speech perception, including formance with the CI necessitates correct, linear placement of
the monosyllabic consonant-nucleus-consonant (CNC) test in the electrode array with the cochlea. When a fold-over or tip
adults and the Multisyllable Lexical Neighborhood Test ( MLNT), rollover of the array occurs, two portions of the cochlea are
Phonetically Balanced Kindergarten ( PBK) test, Lexical Neigh affected : the area distal to the fold-over is deprived of stimula
borhood Test (LNT), and the Glendonald Auditory Screening tion, whereas a proximal region receives competing stimulation
Procedure (GASP) in pediatric patients.20- 23 No relationship was from multiple electrodes. It is this channel interaction in the area
found between intraoperative tNRT values and speech perform of the electrode fold-over that is detected by SOE.
ance at 1 year postimplantation. In this technique, current applied to a selected electrode
Additionally, tNRT values showed no significant correlation (probe) generates the greatest neuronal response from the
with other variables such as age at implantation, etiology and spiral ganglion cells in the immediate vicinity of the probe elec
duration of deafness, progressive nature of hearing loss, years trode. Amplitude recordings from other electrodes in the array
of hearing aid usage, preoperative pure-tone average (PTA), or should vary in relation to their physical distance from the probe
preoperative CNC word score. Notably, in this study, abnormal electrode: numerically adjacent electrodes should have higher
NRT (defined either as a tNRT of 0 or no response) was amplitude than electrodes located farther from the probe_27.43
uncommon, occurring in 23.7% (23 of 97) patients, and a com Using this inverse relationship between amplitude response
plete lack of NRT response on any electrode was rare, occurring and distance, measurements from consecutive electrodes create
in only 4% (three adults and one pediatric patient). In all cases, an SOE curve that reflects the spread of excitation across a
absence of NRT response did not correlate with postimplant given neuronal population. When the amplitude of response is
open-set word discrimination, suggesting that a lack of meas plotted as a function of recording position, one local maxima or
ureable ECAP does not necessarily indicate a lack of auditory peak is seen for each stimulating (probe) electrode. Two or

1 01
. . . . . I I I I

more local maxima of the SOE curve suggest electrode mal muscle. In normal-hearing individuals, a unilateral acoustic sig
position. SOE measurements provide information regarding the nal travels along the vestibulocochlear nerve ( cranial nerve
selectivity of neural excitation fields around each electrode; [CN] VIII) to the cochlear nucleus in the brainstem where it
when these fields overlap, they can suggest a tip rollover. A activates the superior olivary complexes and facial nerve
detailed characterization and in-depth discussion of abnormal (CN VII) nuclei bilaterally. Resulting motor activation of the
patterns in SOE measurements can be found in Cohen et al.43 stapedius muscle (innervated by CN VII) leads to movement of
As with other instances of electrode malposition, tip rollover the stapes bone away from the oval window and subsequent
requires revision surgery. If discovered intraoperatively, tip roll dampening of acoustic transmission. During CI surgery, CN VIII
over could be rectified at the time of implantation by removing is activated electrically through the implanted CI electrode
the malpositioned array and reimplanting the backup device. array, and the thresholds for stapedius muscle contraction are
Thus far, SOE has had limited but promising application to the evaluated visually by direct observation. This electrically eli
intraoperative setting. Grolman et aF7 compared intraoperative cited stapedial reflex ( ESRT) can be elicited with any CI device
SOE measurements with intraoperative imaging, and found SOE and has been used as a guide for postoperative comfort (C-)
to be a reliable, rapid, and effective method of detecting elec level settings during initial CI program creation in children and
trode tip rollover. In their prospective, double-blinded trial of other difficult to program populations.2B.3D.42.44-47
72 patients implanted with the Nucleus Freedom device, they In addition to visual assessment of stapedius contraction,
detected four instances of electrode fold-over using SOE. In each various studies have demonstrated the feasibility of evaluat
instance, patients with more than two local maxima on the SOE ing intraoperative ESRT with tympanometry or electromyo
curve (as described above) had confirmed malposition of the graphic recordings from a bipolar hook electrode placed into
electrode array on intraoperative imaging ( specifically 30 rota the stapedius muscle.44.45.48 Although visual observation is
tional X-ray, discussed in more detail in section 8.3, below.) inherently subjective, direct comparison between ESRTs
Additionally, an experienced audiologist was further able to obtained using intraoperative tympanometry and those
identify the specific electrode at which the fold-over occurred. assessed visually showed no significant difference.44 In many
All four patients underwent immediate reimplantation while circumstances, however, direct observation may be challeng
under anesthesia. Intraoperative NRT was also performed in all ing as the muscle or stapedius tendon may be obscured or
cases, and, consistent with the published literature, did not compromised by middle ear conditions, such as chronic ear
correlate with malposition of the electrode array. Notably, disease, anatomic variation, blood, or fibrous tissue. Addi
detection of tip rollover using SOE required an audiologist tionally, there is dose-dependent suppression of the ESRT
experienced in interpretation of the SOE curve. Visual interpre with general anesthesia and specifically with various volatile
tation of SOE maxima by a trainee audiologist who was anesthetics commonly used in implant surgery.49 Even under
unfamiliar with this testing suggested a tip rollover in one ideal circumstances, the acoustic reflex may be absent in
patient that was not confirmed by radiological imaging, and certain individuals.50 These challenges may lead to overesti
revision electrode insertion was not performed. Other authors mation of intraoperatively obtained ESRTs. It is possible that
have similarly demonstrated the ability of SOE measurements creation of initial programs based on these values may lead
to detect tip rollover intraoperatively.6 to erroneously high maximal comfort levels with subsequent
Currently, a SOE measurement tool is available in the Nucleus impact on speech performance.49
Custom Sound-EP software (Cochlear Corp.). However, lack of Although generation of ESRTs requires both an intact
automated SOE algorithms with the program necessitates mon acoustic reflex pathway and a functional device, there are
itoring by an experienced CI audiologist specifically familiar no specific studies examining the use of ESRT as an intrao
with generation and visual interpretation of the SOE curve. perative test of device functionality and electrode position.
SOE testing capability is not presently incorporated into the Based on available data reviewed above, the inability to
software of other CI manufacturers. Increased availability and elicit an ESRT may be explained by a variety of factors and
optimization of the SOE software to allow for widespread use does not necessarily indicate device dysfunction. Further,
during implantation has been advocated.627 although as yet undocumented, it is theoretically possible
In summary, there is reliable, although limited, evidence to that ESRTs (like ECAP) may be obtained despite the extraco
support SOE as a feasible and effective intraoperative test of tip chlear position of the electrode array. More data are neces
rollover. Additional data as well as modification of the currently sary to evaluate the immediate and long- term implications
available software may encourage more widespread implemen of intraoperative ESRT testing.
tation of this electrophysiological test during surgery. Because
SOE has not demonstrated an ability to detect extracochlear
electrode malposition, it should be used in combination with Radiographic Imaging
radiological imaging for a comprehensive intraoperative test
Intraoperative radiographic imaging has been reported to
battery.
provide confirmation of correct, intracochlear placement of
the electrode array. Plain radiographs using modified Stenver's
Electrically Elicited Stapedial view are a safe, low-cost, and reliable tool for evaluation of
electrode location, position, and presence of tip rollover
Reflex Threshold ( Fig. 8.1 ).6.19.51
The stapedius (or acoustic) reflex is an autonomic response to A variety of more sophisticated imaging modalities have been
loud sound that results in reflexive contraction of the stapedius introduced into the operative setting for use during cochlear

1 02
. . . . . I t

nal auditory canal. Revision surgery with intracochlear


placement of the array was undertaken while the patient
remained under anesthesia.55
For patients with cochleovestibular abnormalities, use of
intraoperative fluoroscopy is a safe, effective, and more
readily available imaging modality for confirmation of elec
trode position.5657 A dynamic, "real-time" assessment of CI
electrode placement, fluoroscopy is unique in its ability to
provide two-dimensional planar visualization of electrode
position during insertion. Effective use of fluoroscopy
requires specific considerations with respect to intraopera
tive setup. As previously described, the C-arm should be
placed with the bean generator below the head of the
table and directed in an anti-Stenver's view. The path of flu
oroscopy should be checked to ensure that radiodense
equipment, including facial nerve monitoring electrodes,
electrocardiogram leads, and bispectral index (BIS) monitor
cable, is avoided and does not compromise visualization of
the acquired images ( Fig. 8.2). To minimize radiation expo
sure to the patient and staff, fluoroscopy should only be
used as necessary during the procedure, and appropriate
safety precautions should be followed closely. These include
lead aprons and thyroid shields placed under the patient
preoperatively and worn by all staff in the room during
image acquisition.
In all cases, intraoperative radiographic imaging is the
gold standard for confirmation of electrode position. For
routine Cl surgery in patients with normal anatomy, data
suggest that the use of plain film x-ray is the most effective,
Fig. 8.1 I ntraoperative transorbital or modified Stenver's view plan widely available intraoperative test of electrode position.6 As
radiograph of a right coch lear implant demonstrating mal position (tip anatomic malformations of the inner ear complicate the
rollover) of the electrode array. ( From Cosetti MK, Troob SH, Latzman cochlear implantation procedure and are more commonly
J M , Shapiro WH, Roland JT j r, Waltzman SB. An evidence-based associated with electrode malposition, available data support
algorithm for intraoperative monitoring during cochlear implantation.
the use of additional imaging modalities, such as intraopera
Otol Neurotol 201 2;33:1 69- 1 76. Reprinted by permission.)
tive fluoroscopy, if available, for intraoperative confirmation
of electrode position.

implantation, including three-dimensional (30) rotational


tomography X-ray and cr. although intraoperative use of this Emerging Techniques for
technology is uncommon.27.s2 - ss As described by Carelsen
et al,52 intraoperative 30 rotational imaging (BV Pulsera with
Intraoperative Monitoring
30-RX; Philips Healthcare, Best, Netherlands) utilizes a motor Thus far, the use of intraoperative monitoring has primarily
ized C-arm that acquires 450 images in a 30-second rotational focused on assessment of device functionality, electrode position
sequence. Obtained with a comparatively low dose of radiation and acquisition of data for later use in postoperative program
(0.07 mSv versus 1 .0 mSv for high-resolution temporal bone ming. Emerging data have introduced various techniques for
Cf), these images are processed over 6 minutes at an intraoper monitoring of intracochlear physiology during implantation. Early
ative workstation and can be reconfigured in a variety of plane data suggest these tests may be useful for assessment of cochlear
s.2752 Intraoperatively, these high-resolution images can iden trauma during electrode insertion as well as preservation of
tify tip rollover as well as intrascalar electrode position, residual hearing.
allowing immediate revision surgery when electrode mal As expanded criteria for cochlear implantation have evolved
position is detected_27.53 to include patients with some residual hearing, preservation of
Use of intraoperative CT has been reported as a useful these acoustic thresholds has been the focus of considerable
method of electrode position confirmation in patients with research. Individuals with significant low-frequency hearing
malformed cochlea. Bloom et al55 describe use of the 0-Arm may be candidates for electroacoustic stimulation ( EAS), a tech
Imaging System ( Breakaway Imaging, Medtronic, Louisville, nique that involves atraumatic insertion of a shorter CI elec
CO) for cochlear implantation in a single patient with a trode array into the scala tympani of the cochlea in an attempt
common cavity malformation. Utilizing a telescoping gantry to preserve the native acoustic function of the apical region, the
sterilely placed around the patient, high-resolution temporal site of low-frequency signal transduction.ss Postoperatively,
bone CT images were obtained in approximately 30 seconds these patients use both a hearing aid and a Cl in the same ear,
showing initial placement of the electrode within the inter- allowing low-frequency acoustic stimulation with traditional

1 03
. . . . . I I I I

n amplification and high-frequency electric stimulation using the


NO R M A L A BNO R M A L
Cochleolbular Cochleolbular
Cl electrode array. Results suggest combined acoustic and
Anatomy An.1tomy electric stimulation may afford distinctive benefits including
improved hearing in noise, enhanced ability to appreciate
music, improved spectral discrimination and pitch perception,
a constant awareness of sound (including times when the Cl is
not worn), and a less "mechanical" quality of sound.s9 - 6s More
recently, studies have demonstrated the ability to preserve
residual hearing in Cl patients implanted with standard-length
electrodes. Using "soft" atraumatic surgical techniques, they
present evidence for preservation of pure-tone acoustic thresh
olds after implantation in traditional or non-EAS candidates.66-70
Thus far, the functional benefits of residual acoustic hearing in
patients utilizing only a Cl for amplification remain unclear.
Independent of effects on Cl performance, there are many
l or More
U1able to compelling considerations to support hearing preservation and
Open o r Short Normal rfrom
atraumatic electrode insertion techniques. Preservation of
Tetemetry, loss
E trodes
of lock residual hearing may be a marker of overall cochlear "well
being," leading to a larger electrical dynamic range and ulti
Repeat C hec k/Exchange mately allowing a more complex stimulation of the auditory
Elec trode Monitoring Equipment
Imped a nc e (Cable, Coil, External nerve.69 Atraumatic, "soft" surgery techniques are superior at
after 4-6 Processo r and Monito r i ng
M lr.Jtes Software) and Reattempt El
limiting intracochlear damage such as that resulting from mod
iolar fracture, fibrosis, inflammation, and molecular proapop
totic pathwaysJl -74 Minimization of intracochlear damage may
Sti l l Unable
to Perform
also have implications for revision implantation, an important
Te4emetryf consideration in very young children who may require replace
ment of their devices during their lifetime as well as for instan
ces of device failure.
Research specifically examining the use of intraoperative
monitoring during hearing preservation surgery is minimal, but
initial data suggest that real-time measurements of electrode
impedance, cochlear microphonics (CMs), and electrocochleog
raphy ( ECoG) may be feasible and valuable.
As described in the ECAP section above, peripheral stimula
tion of the auditory nerve results in a series of measurable
potentials, the first of which is the CM, a very early potential
generated by the inner and outer hair cells. In an animal model
of normal-hearing gerbils, deterioration of CM correlated with
intracochlear trauma as observed with microendoscopes
during electrode insertion. lntracochlear damage was con
firmed on postprocedure histological analysis.7576 Adunka
et aF7 described the feasibility of intraoperative monitoring
using CM in a single case of cochlear implantation in a patient
with auditory neuropathy. Despite profound hearing loss,
patients with auditory neuropathy maintain normal hair cell
function, allowing monitoring of CM during implantation
despite lack of residual hearing.78 In this case, CMs were elicited
_j with click stimuli that are inherently not frequency specific.
Fig. 8.2 Flowchart of the New York University intraoperative monitor Recently, Radeloff et aF9 reported the use of intraoperative
ing protocol. El, electrode impedance; tNRT. neural response tele monitoring with frequency-specific CM in six patients with
metry. f i n these rare circu msta nces, it is also possible to seek support/ residual hearing. Four patients received full insertion of the
guidance from the device manufacturer. *When intraoperative Flexsoft Med-El electrode array through an anterior-inferior
fluoroscopy is performed, the desired electrode position is confirmed
cochleostomy, whereas two patients were implanted with the
using fluoroscopy and a plain film x-ray is not necessary. + Abnormal
electrode configuration can include tip rollover, kinking, or other
limited length FlexEAS20 electrode (designed for hearing pres
anomalous positioning. Use of the backup device is based on X-ray of ervation) via a round window insertion. Intraoperatively, CMs
the electrode position and configuration. If the correct intracochlear were assessed before and after cochleostomy creation, after
position is verified, the device is typically not removed regardless of the initial electrode insertion, and following complete insertion of
electrophysiological results (including the inability to perform elec the respective electrode array. CMs were also performed 1 week
trophysiological testing). postoperatively. In all patients, CMs were unaffected by opening
the cochlea (either via cochleostomy or round window).

1 04
. . . . . I t

Four patients (two with standard-length electrodes and two surgeon. Results are recorded for potential use in the initial pro
with the shorter EAS electrode) had measurable CMs at the gram creation. At present, SOE capability is included in the soft
conclusion of surgery. In the latter two patients, CMs correlated ware from only one manufacturer (Cochlear Corp.). In Nucleus
with preservation of residual hearing in the initial post device recipients, SOE are performed only when requested by
operative period. All four patients who received full insertion of the surgeon, in part due to lack of optimization within the Cus
a standard-length electrode had no measurable CMs 1 week tom Sound Software. As previously described, identification of
after surgery. Of note, intraoperative imaging was not per more than one local maximum in the SOE curve for two or
formed in this study, and one patient was found to have kinking more probe electrode positions are considered suggestive of tip
and tip rollover of the electrode array on postoperative X-ray. rollover. Any abnormalities on electrophysiological testing are
This patient underwent revision with correct placement of the noted, but do not lead to device removal.
Cl electrode surgery 4 days later.79 Next, an intraoperative modified Stenver's view plain radio
Mandala et a180 described intraoperative monitoring of ECoG graph is obtained in all cases and interpreted by the operating
using a custom-made cotton wick electrode placed near the surgeon while the patient remains under general anesthesia.
round window. As in Radeloff et a1,79 opening of the cochlea via When an abnormality of electrode location or position is
cochleostomy or round window had no discernible effect on detected on intraoperative radiograph, the indwelling device is
ECoG. To assess the effect of ECoG monitoring on surgical tech removed and a new device is placed during the same surgery.
nique, 27 patients were prospectively divided into two groups, Identical intraoperative monitoring is performed on the new
and postoperative hearing preservation outcomes were com device following reimplantation.
pared. In the first group, the operating surgeons received imme Immediate "out of the box" or "hard" failures are rare and can
diate feedback regarding ECoG measurements and "modified" be characterized intraoperatively by a loss of telemetric "lock"
their surgical technique by performing a slow and stepwise elec or an inability of the external processor to communicate with
trode insertion when ECoG abnormalities were detected. In the the receiver-stimulator, thus preventing telemetric functional
second group, surgeons were blinded to ECoG measurements. assessment.6 If encountered, difficulty with intraoperative tele
Rates of complete hearing preservation (loss of< 1 0 dB) were metry is first addressed by checking and, if necessary, exchang
found to be significantly higher in the feedback group (85% vs. ing all monitoring equipment such as the cable, coil, external
33%), leading the authors to assert that ECoG monitoring during processor, and monitoring software. If still unresolved, the next
electrode insertion may impact hearing preservation.80 step of the algorithm, an X-ray, is performed. Upon confirma
"Real-time" measurement of intraoperative El has is another tion of correct, intracochlear electrode placement, no additional
method of delivering immediate feedback to the CI surgeon action is taken. Although lack of telemetric lock can be related
regarding intracochlear insertional trauma. Tan et al81 intro to skin flap thickness, current surgical incisions have evolved
duce prototypical software that allows continuous impedance such that thinning of the flap over the device is no longer possi
telemetry during electrode insertion. In two cadaveric temporal ble without extending or enlarging the postauricular incision.
bones as well as two patients, impedance measurements Additionally, even if technically feasible, flap thickness may be
reflected changes in electrode position as seen on fluoroscopy. exacerbated by edema during surgery and may be unresponsive
Although none of these investigational techniques specifically to thinning. For this reason, in combination with the rarity of
assessed device function or electrode position, initial data out of the box failures, we do not remove the device even if loss
support these methods as feasible and potentially valuable of telemetric lock is suspected.
methods of monitoring intracochlear trauma and hearing For patients with cochlear malformations, electrophysiologi
preservation during Cl surgery. cal testing is performed as described above. Although exact
rates are unknown, abnormalities of EI and ECAP may be more
common in patients with abnormal cochleovestibular anatomy
The New York University and do not influence device removal. Intraoperative fluoros
Protocol copy is performed during electrode insertion as described
above for confirmation of intended electrode position. In these
Currently, there is no clinical practice guideline regarding intra challenging cases, decisions regarding device removal and
operative monitoring during cochlear implantation. Analysis of immediate revision surgery may be influenced by individual
the currently available data has led to a feasible, efficient, patient factors, such as type of device used and cochlear anatomy.
evidence-based algorithm of intraoperative monitoring used
during primary and revision cochlear implantation of adults
and children with normal cochlear anatomy ( Fig. 8.2).
Concl usion
Following electrode insertion, intraoperative electrophysio
logical monitoring is performed remotely by an experienced CI Immediate intraoperative determination of device functionality
audiologist using the device - specific software. Remote intrao and optimal electrode placement is advantageous. Electrode
perative monitoring has previously been found to be feasible, malposition has been cited as the cause of CI revision in up
efficient, and cost-effective.29 EI is first obtained on all electro to 13 to 1 6% of cases.82 -85 Each of these patients required
des. If one or more open or short electrodes are discovered, EI is additional revision surgery to correct electrode misplacement,
repeated at the discretion of the surgeon after approximately 4 thereby unnecessarily incurring the risks associated with
to 6 minutes. ECAP values are then obtained from four electro additional surgery and general anesthesia. Additionally, as CI
des (typically ES, E 1 0, E 1 5 , E20), with additional electrodes performance is maximized with optimal electrode placement,
tested when deemed prudent by the monitoring audiologist or each of these patients experienced a variable time period in

1 05
. . . . . I I . .

which their Cl experience was suboptimal. Evidence suggests [181 Botros A, Psarros C. Neural response telemetry reconsidered: I. The relevance
of ECAP threshold profiles and scaled profiles to cochlear implant fitting. Ear
that revision surgery and the lost time associated with elec
Hear 2010; 3 1 : 367-379
trode misplacement can be avoided with careful and judicious [ 1 9 1 Cosetti MK. Shapiro WH, Green JE et al. Intraoperative neural response tele
use of intraoperative monitoring. Multiple electrophysiological metry as a predictor of performance. Otol Neurotol 2010; 3 1 : 1 095-1 099
tests are feasible in routine CI surgery, including El, ECAP, SOE, [201 Peterson GE, Lehiste I. Revised CNC lists for auditory tests. J Speech Hear Dis
and ESRT, although none should be used as an independent ord 1 962; 27: 62-70
[ 2 1 1 Kirk Kl, Pisani DB, Osberger MJ. Lexical effects on spoken word recognition by
barometer of device function or electrode position. Intraopera
pediatric cochlear implant users. Ear Hear 1995; 16: 470-481
tive radiographic imaging, specifically the widely available and [221 Haskins HL A Phonectically Balanced Test of Speech Discrimination for Chil
cost-effective plain X-ray, allows immediate detection of elec dren. Evanston, IL: Northwestern University, 1 949
trode malposition and affords the opportunity for revision [231 Auditory Training NE Washington, DC: Alexander Graham Bell Association,
1 982
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1385-1391 between NRT measurements and behavioral levels in children with the
[121 Abbas PJ, Brown CJ, Shallop JK et al. Summary of results using the nucleus Nucleus 24 cochlear implant may change over time: preliminary report. Int J
Cl24 M implant to record the electrically evoked compound action potential. Pediatr Otorhinolaryngol 2001 ; 58: 1 53-1 62
Ear Hear 1 999; 20: 45-59 [371 Thai-Van H, Truy E, Charasse B et al. Modeling the relationship between psy
[ 1 3 1 Dillier N, Lai WK, Almqvist B et al. Measurement of the electrically evoked chophysical perception and electrically evoked compound action potential
compound action potential via a neural response telemetry system. Ann Otol threshold in young cochlear implant recipients: clinical implications for
Rhinal Laryngol 2002 ; 1 1 1 : 407-41 4 implant fitting. Clin Neurophysiol 2004; 1 1 5 : 281 1 -2824
[ 1 4 1 van Dijk B, Botros A M , Battmer RD et a l . Clinical results of AutoNRT, a com [381 Seyle K, Brown Cj. Speech perception using maps based on neural response
pletely automatic ECAP recording system for cochlear implants. Ear Hear telemetry measures. Ear Hear 2002 ; 23 Suppl: 72S-79S
2007; 28: 558-570 [391 Smoorenburg GF, Willeboer C, van Dijk JE. Speech perception in nucleus
[ 1 5 1 Alvarez I, de Ia Torre A, Sainz M, Roldan C, Schoesser H. Spitzer P. Using Cl24 M cochlear implant users with processor settings based on electrically
evoked compound action potentials to assess activation of electrodes and evoked compound action potential thresholds. Audio! Neurootol 2002; 7:
predict (-levels in the Tempo + cochlear implant speech processor. Ear Hear 335-347
2010; 3 1 : 1 34-145 [401 Basta D, Dahme A, Todt I, Ernst A. Relationship between intraoperative eCAP
[ 1 6 1 Abbas P, Brown CJ, Etler CP. Electrophysiology and device telemetry. In: thresholds and postoperative psychoacoustic levels as a prognostic tool in
Waltzman S, Roland JT, jr, eds. Cochlear Implants. New York: Thieme, 2006: evaluating the rehabilitation of cochlear implantees. Audio! Neurootol 2007;
96-109 1 2 : 1 13-1 1 8
[ 1 7 1 Holstad BA, Sonneveldt VG, Fears BT et al. Relation of electrically evoked com [41 1 Kaplan-Neeman R , Henkin Y , Yakir Z e t al. NRT-based versus behavioral
pound action potential thresholds to behavioral T- and C-levels in children based MAP: a comparison of parameters and speech perception in young
with cochlear implants. Ear Hear 2009 ; 30: 1 1 5-127 children. J Basic Clin Physiol Pharmacol 2004; 1 5 : 57-69

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(42 [ Van Den Abbeele T, Noel-Petroff N, Akin I et al. Multicentre investigation on (64[ Lorens A, Polak M, Piotrowska A, Skarzynski H. Outcomes of treatment of par
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these objective measures relate to implant programming parameters? 1 1 8 : 288-294
Cochlear Implants lnt 201 2 ; 1 3 : 26-34 [65) Turner CW, Gantz Bj, Vidal C, Behrens A, Henry BA. Speech recognition in
(43 ( Cohen LT, Saunders E, Richardson LM. Spatial spread of neural excitation: noise for cochlear implant listeners: benefits of residual acoustic hearing.
comparison of compound action potential and forward-masking data in j Acoust Soc Am 2004; 1 1 5 : 1 729-1735
cochlear implant recipients. lnt j Audiol 2004; 43 : 346-355 [66) Carlson ML, Driscoll CL, Gifford RH et al. Implications of minimizing trauma
(44[ Pau HW, Ehrt K, just T, Sievert U, Dahl R. How reliable is visual assessment of during conventional cochlear implantation. Otology and Neurotology 201 1 ;
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surgery, compared with tympanometry? j Laryngol Otol 201 1 ; 1 2 5 : 271 -273 [67) Garcia-lbanez L Macias AR, Morera C et al. An evaluation of the preservation
(45 ( Pau HW, Zehlicke T, Sievert U, Schaudel D, Behrend D, Dahl R. Electromyo of residual hearing with the Nucleus Contour Advance electrode. Acta Otolar
graphical recording of the electrically elicited stapedius reflex via a bipolar yngol 2009; 1 29: 651 -664
hook electrode. Otol Neurotol 2009; 30: 1 -6 [68) Balkany Tj, Connell 55, Hodges AV et al. Conservation of residual acoustic
(46( Allum JH, Greisiger R, Probst R. Relationship of intraoperative electrically hearing after cochlear implantation. Otologz and Neurotology 2006; 27:
evoked stapedius reflex thresholds to maximum comfortable loudness levels 1 083-1 088
of children with cochlear implants. lntj Audiol 2002; 41 : 93-99 [69) D'Eiia A, Bartoli R, Giagnotti F, Quaranta N. The role of hearing preservation
(47( Lorens A, Walkowiak A, Piotrowska A, Skarzynski H. Anderson I. ESRT and on electrical thresholds and speech performances in cochlear implantation.
MCL correlations in experienced paediatric cochlear implant users. Cochlear Otol Neurotol 201 2 ; 33: 343-347
Implants lnt 2004; 5 : 28-37 [70) Brown RF, Hullar TE, Cadieux JH, Chole RA. Residual hearing preservation
(48 ( Almqvist B. Harris 5, Shallop JK. Objective intraoperative method to record after pediatric cochlear implantation. Otol Neurotol 2010; 3 1 : 1 2 2 1 - 1 226
averaged electromyographic stapedius muscle reflexes in cochlear implant [71 [ Eshraghi AA, Van de Water TR. Cochlear implantation trauma and noise
patients. Audiology 2000; 39: 1 46-152 induced hearing loss: apoptosis and therapeutic strategies. Anat Rec A Discov
[49( Crawford MW, White MC, Propst EJ et al. Dose-dependent suppression of Mol Cell Eva! Biol 2006; 288: 473-481
the electrically elicited stapedius reflex by general anesthetics in children (72( Somdas MA, Li PM, Whiten DM, Eddington DK, Nadal jB. Quantitative evalua
undergoing cochlear implant surgery. Anesth Analg 2009; 108: 1 480-1487 tion of new bone and fibrous tissue in the cochlea following cochlear implan
(50( Hall JW. Audiologists Desk Reference, val. 1: Diagnostic Principles and Proce tation in the human. Audio! Neurootol 2007; 1 2 : 277-284
dures. London: Singular Publishing Group, 1 997 (73 [ Briggs Rj, Tykocinski M, Saunders E et al. Surgical implications of perimodio
(51 ( Xu j, Xu SA, Cohen LT, Clark GM. Cochlear view: postoperative radiography lar cochlear implant electrode design: avoiding intracochlear damage and
for cochlear implantation. Am j Otol 2000; 2 1 : 49-56 scala vestibuli insertion. Cochlear Implants lnt 2001 ; 2 : 1 3 5- 1 49
(52( Carelsen B, Grolman W, Tange R et al. Cochlear implant electrode array inser [74) Roland jT. A model for cochlear implant electrode insertion and force evalua
tion monitoring with intra-operative 30 rotational X-ray. Clin Otolaryngol tion: results with a new electrode design and insertion technique. Laryngo
2007 ; 32: 46-50 scope 2005 ; 1 1 5 : 1 325-1339
(53( Aschendorff A, Kromeier j, Klenzner T, Laszig R. Quality control after insertion [75) Ahmad Fl. Choudhury B, De Mason CE, Adunka OF, Finley CC, Fitzpatrick DC.
of the nucleus contour and contour advance electrode in adults. Ear Hear Detection of intracochlear damage during cochlear implant electrode inser
2007 ; 28 Suppl: 755-795 tion using extracochlear measurements in the gerbil. Laryngoscope 2012;
[54( Labadie RF, Balachandran R, Mitchell JE et al. Clinical validation study of 122: 636-644
percutaneous cochlear access using patient-customized microstereotactic [76( Campbell AP, Suberman TA, Buchman CA, Fitzpatrick DC, Adunka OF. Correla
frames. Otol Neurotol 2010; 3 1 : 94-99 tion of early auditory potentials and intracochlear electrode insertion proper
(55( Bloom jD, Rizzi MD, Germiller jA. Real-time intraoperative computed tomog ties: an animal model featuring near real-time monitoring. Otol Neurotol
raphy to assist cochlear implant placement in the malformed inner ear. Otol 201 0 ; 3 1 : 1 3 9 1 - 1 398
Neurotol 2009 ; 30: 23-26 (77( Adunka 0, Roush P, Grose j, Macpherson C, Buchman CA. Monitoring of
(56) Coelho DH, Waltzman SB, Roland jT. Implanting common cavity malforma cochlear function during cochlear implantation. Laryngoscope 2006; 1 1 6 :
tions using intraoperative fluoroscopy. Otol Neurotol 2008; 29: 914-91 9 1 0 1 7 - 1 020
(57( Fishman Aj, Roland JT, Alexiades G, Mierzwinski j, Cohen NL Fluoroscopically [78( Gibson WP, Sanli H. Auditory neuropathy: an update. Ear Hear 2007; 28
assisted cochlear implantation. Otol Neurotol 2003 ; 24: 882-886 Suppl: 1 025-1 065
(58( Lehnhardt E. lntracochlear placement of cochlear implant electrodes in soft [79) Radeloff A, Shehata-Dieler W, Scherzed A et al. Intraoperative monitoring
surgery technique. HNO 1 993 ; 41 : 356-359 using cochlear microphonics in cochlear implant patients with residual hear
[59[ Skarzynski H, Lorens A. Electric acoustic stimulation in children. Adv Otorhi ing. Otol Neurotol 201 2 ; 33: 348-354
nolaryngol 2010; 67: 135-143 [SO) Mandala M, Colletti L Tonoli G, Colletti V. Electrocochleography during
(60( Turner CW, Gantz Bj, Karsten 5, Fowler j, Reiss LA. Impact of hair cell preserva cochlear implantation for hearing preservation. Otolaryngol Head Neck Surg
tion in cochlear implantation: combined electric and acoustic hearing. Otology 201 2 ; 146: 774-781
and Neurotology: official publication of the American Otological Society, Ameri [ 8 1 [ Tan C-T, Svirsky M, Anwar A et al. Real-time measurement of electrode
can Neurotology Society[ and[ Eur Acad Otol Neurotol 201 0; 3 1 : 1 227-1 232 impedance during intracochlear electrode insertion. Laryngoscope 2012
(61 ( Golub jS, Won jH, Drennan WR, Worman TD, RubinsteinjT. Spectral and tem (82( Lassig AA, Zwolan TA, Telian SA. Cochlear implant failures and revision. Otol
poral measures in hybrid cochlear implant users: on the mechanism of elec Neurotol 2005 ; 26: 624-634
troacoustic hearing benefits. Otology and Neurotology: official publication [83( Marlowe AL, Chinnici JE, Rivas A, Niparko JK, Francis HW. Revision cochlear
of the American Otological Society, American Neurotology Society[ and[ Eur implant surgery in children: the johns Hopkins experience. Otol Neurotol
Acad Otol Neurotol 201 2 ; 33: 147-1 53 2010; 3 1 : 74-82
(62( Gantz Bj, Hansen MR, Turner CW, Oleson Jj, Reiss LA, Parkinson Aj. Hybrid [84( Rotteveel Lj, Proops DW, Ramsden RT, Saeed SR, van Olphen AF, Mylanus EA.
10 clinical trial: preliminary results. Audio! Neurootol 2009; 14 Suppl l : 32-38 Cochlear implantation in 53 patients with otosclerosis: demographics, com
[63( Lenarz T, Stover T, Buechner A, Lesinski-Schiedat A, Patrick j, Pesch j. Hearing puted tomographic scanning, surgery, and complications. Otol Neurotol
conservation surgery using the Hybrid-L electrode. Results from the first clin 2004; 25: 943-952
ical trial at the Medical University of Hannover. Audio! Neurootol 2009; 1 4 (85( Sorrentino T, Cote M, Eter E et al. Cochlear reimplantations: technical and
Suppl l : 22-31 surgical failures. Acta Otolaryngol 2009; 1 29: 380-384

1 07
. .

9 The H i story of Coc h l e a r I m p l a nt Electrode Desig n


Maja Svrakic and}. Thomas Roland Jr.

of electrical impulse to the adjacent neurons (the spread of


Introduction
excitation).
Evolution of the cochlear implant electrode over the past 50 A single-channel electrode is limited in its exploitation of the
years has been driven by three key principles : tonotopic organization of the cochlea. Even with varying input
signals through that one electrode, the performance of patients
1 . Multichannel stimulation allows superior discrimination to with a multichannel implant has consistently been better than
that of a single-channel device. for those with a single-channel device. This was demonstrated
2. Placing the stimulating signal of the electrode closer to the in the late 1 980s and early 1 990s, and has led to the National
target or directing the current toward the cochlear modiolus Institutes of Health (NIH) consensus statement in 1 988 regard
is probably optimal for high spatial specificity. ing better efficacy of multichannel implants. The addition of
3. Atraumatic and precise placement in the scala tympani the spectral information provided by multichannel electrodes
with attempts at preservation of fine cochlear anatomy enables implant recipients to recognize speech without visual
and residual hearing allows integration of electrical and cues, to better identify environmental sounds, to perform
acoustic stimulus ( EAS) and in many cases leads to better in noise, and to achieve significantly more speech
improved performance. reading enhancement.12
Djourno and Eyries pioneered the insertion of the first
In addition to these principles, universal surgical dogmas, such cochlear implant, a single-channel device in 1 957. A year later
as diminishing the rate of iatrogenic infections and shortening Djourno built a "sound analyzer" capable of performing real
the operative time, have led to further advancements in both time analysis of human speech. His idea consisted of directing
the configuration and design of the implant and modifications different speech frequencies into separate rather than single
of the operative procedure and electrode insertion methods. stimulating channels (electrodes). This is the earliest recorded
Round window and cochleostomy techniques have been design of a multichannel-type electrode, although it was never
modified to limit trauma. Further, technological advances implanted into a live patient.3 - s Another early version of a
in microphonics, speech processing, microprocessors, signal multichannel device with five electrodes was implanted by
transduction, and biologically inert materials have finessed the William House in 1 961 into a single live patient who poorly
original designs to their current state. These designs have tolerated the device; the implant was subsequently removed.6
included smaller and more cosmetically appealing external In 1 962, Doyle from Lorna Linda University implanted a stimu
devices, thinner and more easily implanted receiver/stimula lating array of four electrodes into a patient's cochlea; the
tors, longer battery life, improved speech discrimination, mag patient was able to repeat certain words with this multichannel
netic resonance imaging ( MRI) compatibility, and avoidance of stimulation?
implant rejection or infection. Enhanced outcomes in patient Approximately 15 years after these early multichannel device
performance, quality, and safety have encouraged the regula designs, Chouard and MacLeod8 in 1 976 and Simmons et al9 in
tory bodies to consider binaural implantation, younger age at 1 977 reported their preliminary results with eight- and four
implantation, and implanting individuals with residual hearing electrode implants, respectively. In the 6 years that followed,
or single-sided deafness. Clark (predecessor of the Cochlear Corporation device),10-13
All of these factors have played a crucial role in the push for Eddington,141s Hochmair, 1 617 and Michelson1s followed suit
a design of a better cochlear implant and higher performance with their versions of multichannel implants, with Cochlear's
electrode. This chapter discusses the factors that have impacted 22-channel implant, the Nucleus-22, having the highest num
the structural design of the electrode and their historical ber of available electrodes. Fig. 9.1 shows the various elec
context. trode designs as they varied in the number of available chan
nels. As can be easily seen, the growth in the number of electro
des rapidly increased in the late 1 970s but has plateaued over
the last 30 years.
The Key Principles Affecting The maximum number of electrodes is limited by overlap
ping populations of neurons that are stimulated with one
Electrode Design channel. The spread of excitation in the highly conductive
perilymph of scala tympani is difficult to control, although it
Multichannel Stimulation can be constrained to a degree by using a bipolar instead
Multichannel stimulation allows superior discrimination of monopolar electrode configuration19.20 or with closer
compared to that of a single-channel device. Theoretically, apposition of the electrodes to the inner wall of the scala
the larger the number of electrodes and discrete points of tympani.21 All four of the current major implant manufac
stimulation, the finer is the resolution for coding frequen turers ( Cochlear, Advanced Bionics, Med-El, and Neurelec)
cies. However, the upper limit of resolution is constrained have explored both bipolar and monopolar strategies in
by the number of neurons that are available for stimulation electrode design to overcome the overlap in stimulated
at a particular site in the cochlea and also by the spread neurons. Going even further with the attempts to focus the

1 08
. . . . . . .. .

Cl a rk/Coc hlea r

--+- M ic helso n {Sc h i nd lerf Ad v a nc ed


Bio nic s

--+- H o c hma i r /V i e n n a/ M ed - EI

D journo{Choua rd I Neurelec

H ouse/3 M

--+- Ed d i ngto n { l nera id

- + Doyle

- S i m m o ns

- .., Peeters{Antwerp{ Phili p s

- +- Banfai/Dorfman

Douek

0 1-----.----.---.--,--
1 956 1 962 1 968 1 974 1 980 1 986 1 992 1 998 2004 201 0

Fig. 9.1 Graph of the increase in n u m ber of intracochlear electrode contacts over time and across research g roups/man ufacturers.

electrical stimulus, Advanced Bionics has explored tripolar


and even quadripolar excitation of electrodes 22 after
preliminary tripolar models from Miyoshi et al's group in
japan.23 Interestingly, patients' word recognitions scores did
not improve with tripolar and quadripolar stimulation
schemes.2224
Despite implants with as many as 22 electrodes, current
research suggests that no more than four to eight independent
sites are available in a speech-processor context at any one
moment using present electrode designs.25 -31 It is likely that a
future large gain in the number of independent sites may well
require a fundamentally new type of electrode, or a fundamen
tally different placement of electrodes, or a fundamentally
different type or mode of stimulation.26

High Spatial Specificity Fig. 9.2 Electrode impla ntations in the auditory nerve. A small hole is
drilled through the promontory and into the sca la vestibuli about
Placing the stimulating signal of the electrode closer to
2.5 m m anterior to the oval window. (From Simmons FB. Mathews RG,
the target or directing the current toward the cochlear Walker MG, White RL. A functioning m u ltichannel auditory nerve
modiolus is probably optimal for high spatial specificity. stimulator. A preliminary report on two human volu nteers. Acta
lntramodiolar placement was described in Simmons's32 Otolaryngol 1 979;87: 1 7 1 . Reprinted by permission.)
initial report in 1 966 ( Fig. 9.2). During the 1 970s and early

1 09
. .

Gg

Ill

Fig. 9.4 Banfai's 1 984 extracoch lear electrode desig n . (From Banfai P,
Kubik S, Hartma n n G . Our extra-sca lar operating method of coch lear
implantation. Experience with 46 cases. Acta Otolaryngol Suppl
1 984;41 1 :9-1 2 . Reprinted by permission. )
Fig. 9 . 3 Chouard's 1 976 design o f 1 2 separate cochlear fenestra
tions for a 1 2-electrode i m p l a nt. Cl, internal ca rotid ; FR. rou n d
window; Gg, geniculate gangion. ( Fro m Lacombe H , Meyer B,
Chabolle F. Chouard CH. S u rgical proced u re a n d impla nted material limit this imposes is that the electrodes are relatively far
description . Acta Otolaryngol Suppl 1 984;41 1 :20-24. Repri nted by from the target tissue ( spiral ganglion), even for perimodio
permission.) lar electrodes that are adjacent to the most inner wall of
the scala tympani. Close apposition of the target and
the electrode is necessary for a high spatial specificity of
1 980s, various insertion techniques were explored. ln 1 976 stimulation.2637 We are likely limited by the distance and
and 1 977, Chouard (predecessor of today's Neurelec Chori interposing bone, soft tissue, and fluid.
mac device ) suggested that eight and later 12 separate coch In the 1 990s and at the turn of the 2 1 st century, all of the
leostomy sites be drilled for the multichannel electrodes major companies were attempting to position the electrode
( Fig. 9.3 ) ; these were later modified to one cochleostomy array closer to the modiolus in an effort to decrease power
site.33 In 1 984 Banfai et af34 in Vienna described an extraco consumption, prolong battery life, as well as provide more
chlear design of placing the electrodes on the promontory specific neuronal subpopulation stimulation ( Table 9.1 ).
( Fig. 9.4). Cochlear's Contour electrode was a precurved electrode
Most cochlear implant pioneers preferred inserting an whose perimodiolar placement was facilitated with an inser
intracochlear electrode via an opening at or near the round tion stylet ( Fig. 9.5).
window, much like it is performed today via a cochleostomy Advanced Bionics's initial HiFocus electrodes had a Silastic
or round window technique. These included the early positioner that was inserted into the cochlea after electrode
designs by House and Urban in 1 973 ( House-3M device),35 placement. Unfortunately, the positioner was associated with
Clark in 1 979 (later Cochlear Corp.'s Nucleus device ),12 and an increased risk of meningitis and was taken off the market.
Michelson and Schindler in 1 98 1 (University of California Advanced Bionics went on to develop precurved HiFocus
San Francisco multichannel implant, later Advanced Bionics's electrodes as well: the 1j electrode with a j shape and the Helix
Clarion ).18.36 precurved electrode ( Fig. 9.6).
All of the current implants are intracochlear, that is, the The latest Advanced Bionics electrode, the Hi Focus Mid-Scala,
electrodes are within the scala tympani of the cochlea. The is intended to be placed with atraumatic techniques in the

- - -.

Fig. 9.5 a,b Cochlear precu rved Contour electrode with stylet in place (a) and stylet removed (b). (Cou rtesy of Coch lear Corp., Australia.)

110
. . . . . . .. I

Table 9.1 M u ltichannel Electrodes


Manufacturer Device Year I nsertion Electrode Position Insertion strategy
Depth
Cochlear N ucleus-22 { Freedom), 1 982, 1 988 24 m m Straight " K " Lateral wal l o f ST Stiffening ri ngs
Nucleus-24
Double Array Cl551 1 999 1 6 mm Straight Stiffening ri ngs
Contour' 2000 Precurved Perimodiolar Stylet
Contour Adva nce 2002 1 9 mm Precurved Perimodiolar Stylet AOS
Cl 5 1 2
Hybrid L24 HFHL 2008 1 6 mm Straight Lateral wal l of ST Microforcep
Slim Straight 0422 201 1 25 mm Straight Lateral wal l of ST Microforcep
Advanced Bionics Clarion , Clarion 1 987, 1 99 1 25 mm Precurved I ntermediate Retractable Teflon ca rrier
Hi Focus I ' . Hi Focus I I ' 2000 25 m m Precurved Perimodiolar Silastic positioner
HiFocus 1j 2000 25 mm Precurved Lateral I nsertion tube
Hi Focus Helix 2004 24.5 m m Precurved Perimodiolar I nsertion tool
Hi Focus Helix Slim 2005 26 m m Precurved Perimodiolar Stylet AOS
Med-El Standard 1 997 31 mm Straight Outer wa l l Microforcep a n d claw
Medium 1 997 24 m m Straight Outer wa l l Microforcep a n d claw
Compressed 1 997 1 5 mm Straight Outer wa l l Microforcep and claw
Split 1 997 1 0 mm Straight Outer wa l l Microforcep and claw
FLEX { EAS) 2005 3 1 m m 28 m m Straight Outer wa l l Microforcep and claw
24 m m
Neurelec Chorimac' 1 983 1 8 mm Straight I ntermediate/lateral Electrode carrier
Digisonic 1 992 20 m m Straight I ntermediate/lateral Electrode carrier
Digisonic SP 2004 25 m m Straight with I ntermediate/lateral Electrode carrier
shape memory
l neraid Symbion ' 1 988 24 m m Straight I ntermediate/lateral I nsertion tool
Abbreviation: AOS, Advance Off-Stylet; ST. scala tympani.
*Devices no longer commercially available.

perimodiolar or midscalar position. Med-El's original electrodes proximity of electrode contacts to the modiolus,39.43 most
occupied a more lateral position (toward the outer wall of have demonstrated improved performance with both peri
the cochlea) but are now manufactured with softer and more modiolar scala tympani insertion as well as with an
pliable and flexible materials intended to minimize outer wall increased number of total electrodes placed in the scala tym
trauma ( Fig. 9.7 ).3839 The smaller French company Neurelec pani.44.45 Aschendorff et al46 have shown improved speech
introduced a softer, longer electrode, Digisonic SP, in 2004. performance in patients implanted with Cochlear's Contour
Another advantage of perimodiolar electrode placement is a Advance electrode over the Contour electrode due to
slightly lower electrode extrusion rate as compared to straight increased number of contacts in the scala tympani as
electrode; this is likely related to the vector of force imposed opposed to scala vestibule. Finley's group47 reported that
by the outer wall of the cochlea.40.41 across all patient groups, significant positive relationships
Further attempts at positioning electrodes closer to the target from low- to-high performance in consonant-nucleus-conso
spiral ganglion cells involved manufacturing electrode contacts nant ( CNC) scores were identified for tighter perimodiolar
in such a way that they span only the half of the cross-sectional wrapping of the electrode array independent of electrode
circumference of the electrode that was closest to the modiolus ; manufacturer. Research into new intramodiolar devices and
that the 1 998 design is full banded or circumferential, whereas in promoting growth of neurites from ganglion cells toward
the newer electrode contacts are half banded and focused the electrodes with controlled delivery of neurotrophic drugs
toward the area of contact. The early Clarion (Advanced Bionics) has continued in the last decade.26
arrays consisted of eight pairs of contacts ; each pair spanned a Another challenge of bringing the target neurons to the
quarter of the cross-sectional circumference of the electrode, electrical stimulus is imposed by patients with malformed or
oriented at a 90-degree angle to each other and directed medi ossified cochleas. The original designs of electrodes were con
ally toward the target cells of the modiolus.42 Early Med-El elec structed with normal human cochleas in mind. Initially, some
trodes contained paired electrode contacts at 1 80 degrees to older designs were looked at as possibilities: Chouard et al48
each other, whereas the new FLEX tip design arrays contain five suggested using the eight or 1 2 separate cochlear fenestra tech
medially oriented contacts at the apical end (Med-El Electrode nique in patients with abnormal cochlear anatomies. Ineraid
Arrays Catalog for Professionals). evaluated the possibility of reviving its design in these special
Although some studies did not find significant correlation anatomic considerations in the market dominated by Cochlear,
between patients' postoperative speech performance and the Advanced Bionics, and Med-El in the 1 990s.49 Of the three

111
. .

Fig. 9.6 a-c Adva nced Bionics's precu rved Hi Focus electrodes. (a) Hi Focus l j (lateral). (b) HiFocus Helix (perimodiolar). (c) Hi Focus Mid-Scala
(median). (Cou rtesy of Advanced Bionics AG, Stafa, Switzerland.)

Fig. 9.7 The Med-El Standard straight electrode. (Cou rtesy of Med-El Corp., Durham, North Carolina.)

112
. . . . . . .. .

---- !

Fig. 9.8 a,b Electrodes for severely ossified or obstructed cochleas. (a) The Cochlear Double Array. (b) The Med-El Spilt Array electrode. (a is courtesy
of Cochlear Corp., Austra lia; b is cou rtesy of Med-El Corp., Durham, North Carolina.)

major companies, the straight Cochlear Corporation K electrode such as a round window instead of a separate cochleostomy.
and the Med-El Standard and Compressed electrodes are best Trauma of insertion forces and the amount of drilling can be
suited for the common cavity malformation and the hypoplastic limited by utilizing proper inserting tools and paying more
cochlea. Cochlear's Double Array and Med-El's Split Array are attention to surgical technique and insertion trajectory.
best suited for the dual cochleostomy technique in the Some of these factors that led to changes in electrode design
obstructed or ossified cochleas ( Fig. 9.8 ).50 are discussed below.

Integrating Electrical and Shorter Electrodes


Acoustic Stimulus Short electrodes were designed so that they do not traumatize
or provide a stimulating signal to the most apical spiral gan
Atraumatic and precise placement in the scala tympani with
glion cells ( SGCs). The mean length of cochlea spanned by SGCs
attempts at preservation of fine cochlear anatomy and residual
is 13.7 to 1 6.0 mm (about 1'% cochlear turns, as compared with
hearing allows integration of electrical and acoustic stimulus
33.1 to 35.6 mm spanned by the organ of Corti. Short hearing
( EAS ) and in many cases leads to improved performance. The
preservation electrodes ( Cochlear's Hybrid and Med-El's FLEX)
first decade of the 2 1 st century focused on the discovery of
do not reach the apical most SGCs and thus allow integration of
significant benefit of integration of residual hearing stimulated
the electrical and acoustic stimulus.5859 Investigations with
by acoustic energy and the electrical energy provided by
short, 1 0-, 1 6-, and 20-mm electrodes have shown good hear
the implanted device.51 -56 Therefore, the ideal device would
ing preservation of the lower frequencies that are based in the
contain components of a hearing aid (usually for residual
apex of the cochlea.54.56,6o Interestingly, some of the earliest
low-frequency hearing) and that of a typical cochlear implant
implant designs were shortest ( such as the electrodes designed
(high-frequency Joss). Benefits of the combined electroacoustic
in the 1 960s and 1 970s), followed by a period of rapid growth
stimulus include improved listening to speech in quiet, in
in the length of the electrodes. With the more recent push for
noise, and in competition, and improvement in identification of
hearing preservation, especially toward the apex of the cochlea,
melodies and reception of musical soundsY
the design pendulum swung back toward the shorter electrodes
In light of these findings, the dogma over the past 1 0
( Fig. 9.9).
years i n cochlear implants has been to minimize trauma
caused by implant insertion. Less traumatic insertion can be
achieved in several ways. An advance off-stylet technique Minimizing Drilling Trauma
that does not touch or engage the outer cochlear wall
inserts the electrode into space. A softer and more flexible
Smaller Cochleostomy: Thi n ner Electrode Design
electrode is gentler on the cochlear infrastructure and limits Drilling on the cochlear promontory with exposure o f
the force of insertion on the outer cochlear wall. A shorter endosteum may cause acoustic trauma of up t o 1 3 0 dB.61
electrode is Jess traumatic to the apex of the cochlea. Drill Therefore, drilling should be minimized or avoided in the
trauma can be minimized by using a different port of entry hearing-preservation goals. This can be achieved by using a

113
. .

Fig. 9.9 a-c Shorter electrodes. (a) Cochlear original


ful l banded straight array. (b) Cochlear Hybrid L24
(half banded). (c) Med-El FLEX electrode. (a and b a re
cou rtesy of Cochlear Corp., Australia; c is cou rtesy of
Med-El Corp., Durham, North Carolina.)

Fig. 9. 1 0 Thinner Electrode: Cochlear Cl422 Slim (0.3 m m tip to 0.6 mm base). (Cou rtesy of Cochlear Corp., Australia.)

thinner electrode, which requires a smaller cochleostomy. Reducing Outer Cochlear Wall Forces
Larger, up to 1 .5-mm cochleostomies required for stylet
based models can cause up to 25 dB SN drop in some Most electrode designs produce outer wall contact and forces
cases.62 The thinner electrode designs from Cochlear, during insertion. For the precurved designs of Cochlear's Con
Advanced Bionics, and Med-El have taken into consideration tour and Advanced Bionics's Helix Slim electrodes, an Advance
this design concept ( Fig. 9. 1 0 ). Off-Stylet (AOS) insertion technique has been utilized to
minimize force exertion on the outer cochlear wall and trauma
less Dri l l i n g : Rou n d Window a n d Modified to the cochlear structures.67 Perimodiolar placement in the
scala tympani with AOS technique also results in better clinical
Round Wi ndow Insertion
outcomes.46.68 Similarly, deep insertion of a straight electrode
As was the case with reverting to shorter electrode designs, has significant impact on the lateral cochlear wall and basilar
recent electrodes have also been designed to accommodate membrane, which can rupture the membrane and result in
round window insertions, a technique employed in the early scala vestibuli displacement.45.69 A modiolar research array
implants of the 1 960s and 1 970s. Drilling a separate cochle ( MRA) has recently been proposed by Briggs et aJ70 that
ostomy site initially provided a surgical advantage in negoti employs a very thin, precurved, and no-stylet design electrode.
ating the "hook region" and allows a more appropriate angle Advanced Bionics has developed a slim midscalar electrode for
of insertion. If drilling is to be avoided, an alternative entry this purpose (Advanced Bionics Research Bulletin 2007). Med-El
into the scala tympani is through the round window. Round holds the only currently approved FLEX electrode design with
window insertions may cause less damage to intracochlear a tapered, softer tip to minimize insertion trauma to the
structures in some cases and may reduce the postoperative cochlea ( Fig. 9.1 1 ).
vertigo caused by drilling.s5.63 - 66 Further, minimal drilling in Another strategy proposed by Zhang et aF1 in 201 0 is to
the round window niche minimizes trauma and also allows insert steerable intracochlear electrodes through the round
for a better insertion angle (the "modified" or "inferiorly window or a cochleostomy with robotic devices. The feasibility
extended" round window technique ) and is compatible with study showed a significant decrease in insertion forces and
new slim design electrodes.s7.6s prevention of buckling; a second-generation robotic device

Fig. 9.1 1 Flexible and soft electrode: Med-El FLEX 24. (Courtesy of Med-El Corp., Durham, North Carolina.)

114
. . . . . . .. .

may involve haptic control, preprogrammed path planning As mentioned earlier, the FDA removed the dual-component
based on imaging, real-time impedance measures guidance, Advanced Bionics electrode from the market in 2002 due to an
and force-sensing capability. increased rates of meningitis. This is an example of an attempt
to optimize performance with one design concept (perimodio
lar placement) leading to a newly created, significantly morbid
Other Considerations in problem of meningitis.77-79

Electrode Design
Optimizing Stimulus Intensity
Preventing Ascending Infection
Continuous electrical stimulation to the ganglion cell may
The current strategy for preventing ascending infection itself be toxic. Indeed, there is a limit to the stimulus
from the middle ear to the cochlea involves sealing the frequency (2,000 pulses per second) and charge density
cochleostomy after electrode insertion and pneumococcal (directly related to decreasing electrode surface area) above
vaccinations. Sealing techniques with muscle, fascia, Teflon, which undesired fibrotic reactions could occur.8081 However,
and Dacron materials were first investigated in the early stimulus intensity seems to be directly related to promoting
1 980s primarily as means to prevent Staphylococcus neurotransmitter production and maintaining electrical path
pyogenes and Staphylococcus aureus from the middle ear to ways, and thus a balance between toxicity and improved
enter the cochlea through the cochleostomy site. The fascial neurotrophic potential in cochlear implant electrode design
graft was found to be most successful in providing a barrier would be idea1.8283
for infection and also to be the least inflammatory. In
addition, the fascial graft also prevented Streptococcus
pneumoniae, common in the middle ear in the younger Prolonging Battery Power
than 2 age group, from ascending into the cochlear environ Most current devices utilize monopolar stimulation because
ment.72 it requires less current and therefore less battery power.
A cutaneous infection can track into the middle ear if there Monopolar stimulation also lowers the differences in threshold
is no barrier ( such as intact skin) separating the external for individual electrodes, which simplifies fitting of speech
from the internal components of an implant device. The link processors.s4.ss As mentioned earlier, bi-, tri-, and quadripolar
between external components ( such as a microphone) and stimulation theoretically focuses the electrical signal ( "current
internal components (electrodes) can be achieved via a trans steering" ) and allows for better signal discrimination. However,
cutaneous or a percutaneous method. The simpler, per there seems to be no significant performance advantage22-24.86
cutaneous design has the advantage of having no implanted and thus multipolar stimulation does not, at present, justify
electronics other than the electrodes. The signal, produced by increased battery consumption.
the stimulator, is not constrained by another device such as a Further, closer opposition to target neurons at the modiolus
receiver, and can be directly channeled toward the electrodes. reduces energy requirements. This optimal positioning leads
This type of implant does not employ magnets, and is thus not only to reduced threshold levels but also to more localized
compatible with MRI. neural excitation patterns, an increase in the number of bipolar
The major disadvantage, however, is the possibility of cutane electrodes available, and an increase in electrode dynamic
ous infection tracking toward the middle and possibly the inner range.87
ear. In their early work, Chouard et aF3 predicted the problem
of infection and in 1 976 switched the percutaneous device to
the transcutaneous, radiofrequency-based receiver. Early Concl usion
designs by Michelson in San Francisco and Banfai in Cologne
Evolution of the cochlear implant electrode design is as com
also involved a percutaneous connection, which was modified
plex now as it was 50 years ago. Although the initial challenges
to a transcutaneous for similar concerns.3436 Interestingly, the
of simply stimulating the cochlear nerve with electrical current
first cochlear implant developed by Djourno and Eyries in 1 957
have been overcome, the fine tuning of electrode design, its
utilized a transcutaneous connection similar to what is in use
ideal intracochlear placement, and optimized stimulus delivery,
in today's modern implants.74
which all ultimately affect patient performance, will continue
The spread of infection that is possible with a percutaneous
to drive innovation in this field.
connection largely contributed to the Food and Drug Adminis
tration ( FDA)-Ied discontinuation of the Ineraid Symbion
device originally developed by Eddington and later Parkins in References
Utah despite its comparable performance to the other major
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N Engl j Med 1 993; 328: 233-237
nicity, and reduce ingress of antibodies and antibiotics, allowing
131 Djourno A. Instantaneous oscillographic analysis of the spoken voice. C R
for development of cutaneous and subcutaneous infections, Seances Soc Bioi Fil 1959; 1 5 3 : 1 97-198
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1 0 Coch l ea r I m p l a nt S u rg ica l Tech n i q u e


Peter S. Roland and}. Thomas Roland jr.

the absence of a fluid signal within the labyrinthine, and


Introduction anything that eliminates the fluid signal from the cochlear
Cochlear implantation has been utilized for hearing restoration canals ( ossification, fibrous tissue, etc.) will appear as an
with increasing frequency for over three decades. Although, for abnormality on MRI.
the vast majority of surgeons, the basic surgical approach used Because MRI visualizes soft tissue well, MRI can visualize
for cochlear implantation has not changed, it has been signifi the cochlear nerve, which CT cannot visualize. An absent or
cantly refined, especially with respect to the skin incision, significantly hypoplastic nerve on MRI indicates a poor
management of the receiver/stimulator, and how the cochlea is prognosis for the acquisition of open-set speech discrimina
approached and opened. Most surgeons continue to place tion and may be a contraindication to cochlear implantation.
cochlear implants (Cis) through a posterior tympanostomy Current evidence suggests that at least some individuals
(facial recess), but alternative techniques have been proposed with radiographically absent cochlear nerves on MRI may
and are used by a minority of surgeons. This chapter focuses receive greater benefit from brainstem implantation rather
on the standard technique. than cochlear implantation. If MRI demonstrates a signifi
cantly healthier cochlear nerve on one side than on the
other, the side with the more robust cochlear nerve should
Preoperative Considerations be selected for implantation. The MR sequences most useful
for evaluating Cl candidates are heavily T2-weighted images
Imaging ( constructive interference steady state [ CISS ) . fast imaging
Radiographic imaging of the temporal bone is used to iden employing steady-state acquisition ( FIESTA] ) that enhance
tify any potential anatomic variance that might contra the fluid signal in the cochlea, semicircular canals, and cere
indicate the operation or require alterations to the usual bellopontine angles. Sagittal reconstructions at right angles
procedure by alerting the surgeon to anatomic and patho to the auditory canal should be performed to evaluate the
logical anomalies.1 The findings on preoperative imaging can presence or absence of a separate cochlear nerve. MRI can
also help determine which of the many available electrodes identify defects of the modiolus that can warn surgeons
to implant. Traditionally, radiological evaluation of Cl candi about potential intraoperative cerebrospinal fluid ( CSF)
dates has utilized high-resolution computed tomography "gushers." High-resolution CT. on the other hand, allows for
( CT), but it is now recognized that magnetic resonance better characterization of hypoplasia, aplasia, and partition
imaging ( MRI) also has a role in the evaluation of Cl candi ing defects ( Mondini deformity). CT scan allows for fairly
dates.2 Some centers rely predominantly on CT scanning and precise identification of the course of the facial nerve within
use MRI only in special circumstances. Other centers rely the middle ear and mastoid.
principally on MRI and use CT in special circumstances. An anomaly of the facial nerve is uncommon in CI recipients.
There are advantages and disadvantages to both techniques When present, a facial nerve anomaly usually occurs in
( .,. Table 1 0. 1 ). The techniques are complementary; they conjunction with abnormalities of the vestibule, semicircular
each can provide important information not provided by canals, or cochlea that are easily identified on MRI. See
the other. MRI is generally more sensitive in identifying Chapter 7 for more detailed information.
early labyrinthine obstruction. Even high-resolution CT
scanning may miss cochlear obstruction in up to 50% of Meningitis
cases. CT cannot detect labyrinthine obstruction until frank
ossification has developed. On the other hand, MRI relies on Cochlear implant recipients are more likely to develop men
ingitis than individuals who have not received a CI. It is
unclear if this vulnerability is a consequence of the presence
Table 1 0. 1 Magnetic Resonance Imaging vs. Computed Tomography of the Cl or whether it merely reflects the heightened vul
nerability of individuals with malformations of the labyrinth
MRI a
to meningitis.
Labyrinthine + +
It does appear that the use of a plastic "positioner" to
architecture
push the electrode close to the modiolus was associated
Cochlear luminal + + I-
with a significantly heightened incidence of meningitis. This
obstruction
technique is no longer in use, and the plastic positioner has
Facial nerve location +
been withdrawn from the market. The overall risk of menin
Cochlear nerve +
gitis associated with cochlear implantation appears to be
characteristics
about 1 /1 ,000.3 Fatal episodes of meningitis are infrequent
Modiolar defects {CSF + +
but when they occur they are usually caused by the bacteria
leak risk)
Streptococcus pneumoniae. Immunization against this
CNS abnormalities + + I-
organism is highly recommended. Immunization recommen
Abbreviations: CSF, cerebrospinal fl uid; CNS, centra l nervous dations for Cl recipients against pneumococcus can be found
system; + f-, some ability to detect.
on the Centers for Disease Control and Prevention ( CDC)

118
website (www.cdc.govfvaccinesfvpd-vacfmeningfcochlear/ Equipment
dis-cochlear-gen.htm) and include both Prevnar 13 and
Pneumovax 23. An otology drill and an operating microscope are necessary for
every CI operation regardless of the technique employed. A
variety of drills and microscopes are available, any of which are
Counseling appropriate provided only that they are designed for otologic
As is true of any surgical procedure, patients and families use. Use of a facial nerve monitor is highly recommended and is
need to be fully informed of the potential benefits and risks utilized by the vast majority of experienced surgeons.
associated with the procedure ( Table 1 0.2). A devas