ASSESSMENT AND

MANAGEMENT
OF TINNITUS AND
HEARING LOSS
Douglas E. Mattox

KEY POINT:

Hearing loss
and tinnitus
should be
considered
separate
symptoms
because
neither is
predictive nor
prognostic for
the presence
or progression
of the other.

ABSTRACT
Hearing loss and tinnitus are common symptoms among the general population, and
both increase in frequency and severity with age. The initial evaluation determines the
site of the lesion: conductive hearing loss from the middle ear, or sensorineural
hearing loss from the inner ear. Unilateral hearing loss is of most concern because of
the possibility of a mass lesion. Most forms of hearing loss can be helped with
amplification (hearing aid), surgical correction, or cochlear implantation in the case of
profound hearing loss.

Hearing loss and its commonly associated symptoms tinnitus and hyperacusis
are the most common sensory deficits,
especially in older persons. Congenital
profound hearing loss or deafness occurs in 2 per 1000 live births, and an
additional significant proportion of
children have mild to moderate hearing loss. Hearing impairment increases
with age and has been estimated to
occur in 25% to 40% of the population
older than 65 years (Yueh et al, 2003).
The prevalence accelerates in very elderly people; more than 80% of patients aged older than 85 years have
hearing impairment (Ciurlia-Guy et al,
1993). Untreated hearing impairment
is likely to be associated with depression, anxiety, decreased social activity,
and isolation (Dalton et al, 2003).
Tinnitus is the perception of a sound
that is not present in the environment.
It also affects a large percentage of the
population, estimated to be 17% of the
general population of the United States,
and increases to over one third of patients aged older than 65 years. Al-

though tinnitus commonly occurs in the

hearing-impaired population, either tinnitus or hearing loss can exist without
the other symptom. Hearing loss and
tinnitus should be considered separate
symptoms because neither is predictive
nor prognostic for the presence or
progression of the other.
ANATOMY AND PHYSIOLOGY
OF HEARING
The ear is divided into the outer ear,
the middle ear, and the inner ear
(Figure 7-1). The outer ear consists of
the pinna and external auditory canal,
which together function as sound collectors. The resonance frequencies of
the pinna and external canal match the
dominant speech frequencies; thus, the
ear canal provides mild benefit to verbal
communication. The middle ear consists of the tympanic membrane, an aircontaining middle ear space, and the
ossicular chain (malleus, incus, and
stapes). The middle ear serves as a
transformer that bridges the impedance

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135

" TINNITUS AND HEARING LOSS

KEY POINTS:

The middle
ear serves as
a transformer
that bridges
the impedance
mismatch
between sound
vibrations in
air, which
has very low
density,
with the
high-density
fluid-filled
spaces of the
inner ear.
The inner ear
is the
transducer
that converts
mechanical
vibratory
energy (sound)
into auditory
nerve impulses.
Hearing loss
is divided
into two main
categories:
conductive and
sensorineural.

mismatch between sound vibrations

in air, which has very low density, with
the high-density fluid-filled spaces of
the inner ear. Without this impedancematching mechanism, most of the sound
impinging on the inner ear would be reflected because of the difference in mass
between air and water. Most of this impedance matching is provided by the
area difference between the tympanic
membrane and the footplate of the
stapes. The severity of the hearing loss
caused by abnormalities of the middle
ear is dependent on the specific abnormality. Small perforations of the tympanic membrane or middle ear effusions
cause moderate loss, while complete loss
of the middle ear and its transformer
function can cause up to 60 dB of
hearing loss.
The inner ear is the transducer that
converts mechanical vibratory energy
(sound) into auditory nerve impulses.
Movement of the stapes produces movement of the fluids in the inner ear, which,
in turn, causes movement of the basilar
membrane. The basilar membrane is frequency tuned, not unlike the strings on
a piano, based upon its width and tension at any given point. Low-frequency
sound preferentially vibrates the apex of
the cochlea and high-frequency sound,
the basilar end near the stapes.

136

FIGURE 7-1

Anatomy of the ear. A schematic of external,

middle, and inner ears.

The hair cells of the organ of Corti are

the sensory receptors of the inner ear.
They rest on the basilar membrane and
are stimulated by a specific sound frequency based on their individual location on the basilar membrane. Two sets
of hair cells are within the organ of
Corti. The inner hair cells receive the
majority of the auditory nerve fibers and
are the major generators of the auditory
nerve action potentials. The outer hair
cells are highly specialized and have
the capacity for high-frequency motion, both contraction and lengthening.
This movement occurs in response to
sound and serves as a positive feedback
amplifier, which increases the sensitivity
of the organ of Corti by up to 60 dB.
The auditory nerve carries signals generated in the cochlea to the cochlear
nucleus and thereafter to higher brain
stem and cortical centers for cognitive
interpretation.
PATTERNS OF HEARING LOSS
Hearing loss is divided into two main
categories: conductive and sensorineural. Conductive hearing loss implies that
sound is prevented from reaching the
inner ear. Thus, simple occlusion of the
external auditory canal, malfunction of
the ossicular chain, or replacement of
the air within the middle ear with fluid
all produce conductive hearing loss.
Sensorineural hearing loss implies malfunction of the transducer mechanism
of the cochlea or, rarely, auditory nerve
dysfunction. The outer hair cells at the
basilar end of the cochlea are more
sensitive to most forms of auditory insult (noise, ototoxic drugs, and aging);
therefore, high-frequency sensorineural
hearing loss is the most common audiometric pattern seen. Fortunately, in most
cases, even if a substantial number of
hair cells are lost, a large percentage of
the auditory nerve fibers are preserved.
Rehabilitative measuresincluding hearing aids and cochlear implantsare
generally successful at supplementing

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or replacing the inner ear function.

Correcting auditory neuropathy is difficult because both hearing aids and
cochlear implants rely on a functioning
auditory nerve.

TABLE 7-1

"

External Ear
Congenital atresia
Acquired stenosis

DIFFERENTIAL DIAGNOSIS OF
HEARING LOSS
Conductive Hearing Loss
The differential diagnosis of conductive
hearing loss is shown in Table 7-1 and
includes disorders of the ear canal,
tympanic membrane, and middle ear.
Disorders of the external canal should
be apparent to the conscientious examiner before audiometric testing, but
cerumen impaction or foreign body in
the ear canal, congenital or acquired
stenosis, and canal skin edema from
acute or chronic infection all cause
conductive hearing loss.
Middle ear effusion is the most common cause of conductive hearing loss.
Air within the middle ear cleft is continually reabsorbed by the lining mucosa,
and the eustachian tube ventilates the
middle ear with each swallow or yawn.
Effusions result from acute or chronic
dysfunction of the eustachian tube.
Acute effusions are associated with acute
upper respiratory infections causing eustachian tube dysfunction or may linger
after the resolution of an acute bacterial
otitis media. Marginally functioning eustachian tubes may produce no symptoms until the system is stressed, eg, the
barometric pressure changes with commercial airline flight leading to acute
effusion or middle ear hemorrhage.
Adult-onset unilateral middle ear effusion can be the first symptom of nasopharyngeal carcinoma or other nasopharyngeal mass and should prompt a
fiberoptic examination to exclude this
possibility.
The severity of conductive hearing
loss caused by tympanic membrane perforation is dependent upon its size and
location. Small perforations may have

Differential Diagnosis of Conductive

Hearing Loss

Trauma
Chronic external otitis
Osteoma or exostosis
Otitis externa
External canal neoplasm
(basal cell, squamous cell)

"

Tympanic Membrane
Tympanosclerosis
Perforation
Thickened tympanic membrane from infection,
trauma, or surgery

"

Middle Ear
Congenital ossicular abnormality
Acute or chronic otitis media
Middle ear effusion
Cholesteatoma
Ossicular discontinuity (trauma, infection, cholesteatoma)
Otosclerosis
Tympanosclerosis
Malleus head fixation
Temporal bone fracture
Surgical complication
Trauma
Middle ear neoplasm (glomus tumor, adenoma)
Superior semicircular canal dehiscence

"

Syndromes Causing External and/or Middle

Ear Abnormalities
Treacher Collins syndrome
Pierre Robin syndrome
Crouzon disease
Apert syndrome
Goldenhar syndrome
Turner syndrome

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137

" TINNITUS AND HEARING LOSS

KEY POINTS:

Adult-onset
unilateral
middle ear
effusion can
be the first
symptom of
nasopharyngeal
carcinoma
or other
nasopharyngeal
mass and
should prompt
a fiberoptic
examination
to exclude
this possibility.
Mutations
of the gap
junction protein
connexin
26 account
for half of
nonsyndromic
congenital
hearing loss.
Noise exposure
is the most
common
preventable
cause of
hearing loss
in adults.

very little impact on hearing, but perforations greater than 20% usually cause a
30-dB conductive loss. In addition to the
perforation, the hearing loss may be
compounded by thickening or scarring
of the drum, tympanosclerosis (white
hyaline deposits in the tympanic membrane), or associated ossicular damage.
Chronic infection, cholesteatoma (keratocyst in the middle ear), and trauma can
damage the ossicular chain, most commonly the incus, which further contributes to the hearing loss. The worst
hearing loss is caused by a discontinuity
of the ossicular chain behind an intact
tympanic membrane because the drum
then functions as a sound defender,
and there is no mechanism to conduct
sound vibrations from the drum to the
inner ear.
An adult-onset progressive conductive hearing loss without a history of infection or trauma is most likely caused
by otosclerosis (Case 7-1). Otosclerosis
is a process of reactive bone growth
limited to the otic capsule and the oval
window around the stapes footplate
in particular. When the otosclerosis is
sufficient to inhibit movement of the

stapes footplate, a conductive hearing

loss occurs.
Sensorineural Hearing Loss
The differential diagnosis of sensorineural hearing loss is shown in Table 7-2.
Congenital hearing loss is categorized as
syndromic or nonsyndromic, depending upon the presence of other congenital developmental stigmata. Although
notable exceptions exist, syndromic
hearing loss is relatively uncommon
and tends to exhibit autosomal dominant inheritance, while nonsyndromic
hearing loss is much more common
and is usually recessively inherited.
Mutations of the gap junction protein
connexin 26 account for half of nonsyndromic congenital hearing loss.
Noise exposure is the most common
preventable cause of hearing loss in
adults. Although most major industries
now monitor noise levels and require
hearing protection in noisy areas, many
unregulated sources of recreational
noise remain, including firearms, unmuffled gasoline motors, and highintensity audio speakers. The typical
hearing loss from noise exposure is

Case 7-1
138

A 35-year-old woman complains of a progressive bilateral hearing loss

over the past 4 to 5 years. The left ear is worse than the right. She has
no vestibular complaints. She denies a history of childhood hearing loss,
otitis media, trauma, or previous surgery. She has a sibling with similar
symptoms. Physical examination shows normal tympanic membranes
with good mobility and no evidence of effusion. Tuning forks show air
better than bone on the right, but bone greater than air on the left.
The audiogram shows a bilateral conductive hearing loss of 15 dB to 20 dB
on the right and 35 dB to 40 dB on the left. The diagnosis is otosclerosis.
Comment. Adult-onset conductive hearing loss without infection,
trauma, or other obvious external cause is almost certainly otosclerosis.
A positive family history can be elicited in 20% of cases. Note that the
conductive hearing loss must be 25 dB or more before the tuning forks
reverse (bone greater than air). Therefore, she still has a normal response
to the tuning forks on the right but reverses her response on the left.
Therapeutic options include hearing aid and surgical correction, both
of which offer her excellent results.

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TABLE 7-2

"

Differential
Diagnosis of
Sensorineural
Hearing Loss

Congenital and Early Acquired

Syndromic
Waardenburg syndrome
(dystopia canthorum,
confluence of eyebrows,
heterochromia iridis)
Alport syndrome
(interstitial nephritis)
Usher syndrome
(retinitis pigmentosa)
Nonsyndromic
Connexin 26 mutation (50%
nonsyndromic hearing loss)
Malformation of otic
capsule (30%)
Large vestibular aqueduct
syndrome

"

Acquired
Infectious
Measles
Mumps
Cytomegalovirus
Syphilis
Lyme disease
Noise-induced hearing loss
Ototoxic
Aminoglycosides
Quinine
Salicylates
Cisplatinum
Autoimmune
Menie`res disease
Acoustic neuroma
Sudden hearing loss
Presbycusis

worst at high frequencies, with a

notch at 4000 Hz. A useful rule of
thumb is that if the ambient noise level
is sufficient to require two people 6 feet
apart to shout to communicate, then
the ambient noise level is potentially
damaging. Noise trauma is dependent
upon both intensity and duration. Current Occupational Safety and Health
Act guidelines permit 85-dB exposure
for 8 hours; each increase of 5 dB
reduces the permissible time of exposure by half.
Ototoxicity from aminoglycosides and
platinum derivatives are still important
causes of hearing and vestibular dysfunction. The most commonly used aminoglycoside, gentamicin, is more likely to
cause vestibular loss than hearing deficit.
A mitochondrial DNA mutation seen
most commonly in Asian populations
makes them particularly susceptible
to aminoglycosides (Usami et al, 1997).
Unfortunately, aminoglycoside ototoxicity is not precisely dose- or peak-level
dependent, and profound audiovestibular loss has been reported after relatively
small doses of these drugs. Screening
measures for both blood levels and
audiometric function have not entirely
eliminated this problem.
The medical workup of slowly progressive bilateral hearing loss is limited.
Diseases usually associated with microvascular disease, eg, diabetes and sickle
cell anemia, are infrequently associated
with hearing loss even in the face of
severe retinopathy or other severe manifestations. Hypothyroidism is associated
with hearing loss but not without clinically obvious cretinism or myxedema.
Lyme disease and tertiary syphilis are
treatable causes of hearing loss, and appropriate serology tests will identify the
occasional patient requiring treatment.
Some hearing loss results from autoimmune processes either alone or in
combination with known autoimmune
disorders. Hearing loss is a defining
symptom in Cogan syndrome (hearing

KEY POINT:

A useful rule
of thumb is that
if the ambient
noise level
is sufficient
to require
two people
6 feet apart
to shout to
communicate,
then the
ambient noise
level is
potentially
damaging.

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139

" TINNITUS AND HEARING LOSS

KEY POINTS:

140

In Menie`res
disease, the
patient feels
perfectly
fine between
vertigo attacks;
patients
with chronic
disequilibrium
without
spinning
attacks
probably do
not have
Menie`res
disease.
Special attention
must be paid
to patients
with unilateral
or significantly
asymmetrical
hearing loss.

loss and nonsyphilitic interstitial keratitis of the eye). Hearing loss may be
seen with polyarteritis nodosa, temporal arteritis, Sjo
gren syndrome, lupus,
scleroderma, and other autoimmune
processes. Hearing loss as a sole manifestation of autoimmune process is
more difficult to establish. The clinical
setting of a rapidly progressive (weeks
to months), bilateral but not necessarily
symmetrical, sensorineural hearing loss
has been attributed to autoimmune
processes and is frequently steroid responsive. Some of these patients go
on to develop other manifestations of
autoimmune disease. There are no pathognomonic laboratory tests, but a
serum assay for antibodies to a 68-kD
inner ear antigen is positive in 60% to
70% of patients. Treatment is usually
with intermittent high doses of corticosteroids and occasionally other immunosuppressive agents like cyclophosphamide or methotrexate, but these
patients frequently become cochlear
implant candidates.
The combination of hearing loss and
episodic vertigo suggests Menie
`res disease. The current diagnostic criteria established by the American Academy of
OtolaryngologyHead and Neck Surgery
(Committee on Hearing and Equilibrium,
1995) require the patient to have hearing
loss (typically low frequency and fluctuating), episodic vertigo, and either
tinnitus or fullness in the affected ear.
The vertigo attacks are the most dramatic part of the syndrome and include
episodes of violent-spinning vertigo
with associated nausea and vomiting
typically lasting 1 to 3 hours. The patient
often has fluctuation of hearing and
tinnitus coincident with the attack. The
hearing loss in Menie`res disease is
characteristically in the low frequencies, whereas almost all other forms of
sensorineural hearing loss start in the
high frequencies and progress downward. The attacks are unpredictable and
may be rare, frequent, or clustered. An

important distinction is that between

the attacks the patient feels perfectly
fine; patients with chronic disequilibrium
without spinning attacks probably do not
have Me
nie`res disease. The etiology of
Menie`res disease is unknown, but the
pathological correlate is hydrops or
dilation of the endolymphatic spaces
of the inner ear at the expense of the
perilymph spaces. The mechanism of
an individual Menie
`res attack is probably from continued dilation of the
endolymph until one of the limiting
membranes of the inner ear ruptures.
Escape of the potassium-rich endolymph into the surrounding perilymph is thought to trigger the vertigo attack and fluctuation of hearing.
Special attention must be paid to
patients with unilateral or significantly
asymmetrical hearing loss. Symmetrical
hearing loss usually can safely be attributed to systemic, metabolic, genetic,
or age-related phenomena, but unilateral hearing loss raises the possibility
of pathology unique to that ear. Sudden hearing loss is the symptom of
the abrupt loss of hearing in one ear
(occasionally both) instantaneously or
over 24 to 48 hours. It is usually accompanied by tinnitus and may or may
not have associated vertigo. Etiological
hypotheses for sudden hearing loss
include vascular and embolic events,
viral infection, intracochlear membrane
ruptures, and autoimmune phenomena. The prognosis is suggested by the
severity of the initial loss, with mild and
moderate losses having a good prognosis for recovery and severe losses,
especially accompanied by vertigo, having a poor prognosis for recovery. Treatment is unsatisfactory, and studies are
confounded by a significant spontaneous recovery rate. Some studies have
suggested that the early institution of
corticosteroids is useful; antibiotics and
decongestants are of no benefit. Fortunately, sudden hearing loss is rare in the
contralateral ear (Case 7-2).

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Case 7-2
A 55-year-old man awakens in the morning complaining of a blocked
left ear. He is unsuccessful in cleaning the ear at home. He seeks the
attention of his primary care physician the next day, who notes a clear
ear canal, questions if there is a middle ear effusion, and prescribes oral
antibiotics and decongestants. By the following day the hearing has
deteriorated, and the patient develops spinning vertigo and tinnitus in
the ear. He has a past history of mild cardiovascular disease, has had an
angioplasty in the past, and is on daily aspirin.
Physical examination reveals a brisk nystagmus with the fast phases
to the right. Microscopic examination of the ear shows a normal tympanic
membrane without effusion. Audiogram shows a profound sensorineural
hearing loss on the left. Magnetic resonance imaging (MRI) with contrast
is normal. The diagnosis is sudden hearing loss.
Comment. Sudden hearing loss is a relatively common syndrome of
acute sensorineural hearing loss with or without vestibular symptoms.
Although the cause usually cannot be determined in a given patient,
candidate hypotheses include viral inflammation, intracochlear membrane
breaks and embolic phenomena. As with any unilateral hearing loss,
it is important to exclude a cerebellopontine mass with imaging studies.
It is common for the first health care provider who sees such a patient to
diagnose middle ear infection or effusion; however, pneumatoscopy with
magnification demonstrates a normal middle ear. Overall, two thirds of
patients recover some hearing; severe initial hearing loss and accompanying
vertigo are poor prognostic signs. Since the exact cause is unknown, it is
difficult to design a rational therapeutic plan. Steroids may, however, be
beneficial if there are no medical contraindications to their use. Patients
are always concerned about the risk to the contralateral ear, and fortunately
this risk is small.

A second important cause of unilateral

hearing loss is acoustic neuroma. The
majority of patients with acoustic neuromas present with unilateral progressive
sensorineural hearing loss, often with
the discrimination score poorer than one
would predict by the pure-tone thresholds. However, the presenting symptoms of acoustic tumors are diverse, and
some patients will have sudden hearing loss, vestibular complaints, or even
trigeminal symptoms. Acoustic neuroma is not a rare or esoteric diagnosis.
It is the third most common intracranial tumor, and a recent study suggests
the overall prevalence in asymptomatic
people is 1 in 5000. Although audiometric
tests, including acoustic reflex decay
and auditory brain stem response, have
been used in the past for acoustic tumor

screening, gadolinium-enhanced MRI

is the gold standard for diagnosis, and
the recent reduction of the cost of
contrast makes MRI both the most
definitive and economical diagnostic
tool. Since everyone with a hearing
complaint cannot be scanned, an important dilemma is determining how
much asymmetry of hearing is sufficient to trigger imaging studies. Most
references on the subject suggest a
15-dB difference at three frequencies
or 20% asymmetry in discrimination
score as an indication for imaging. The
addition of vestibular complaints or distinct unilateral tinnitus may prompt
imaging of patients with less hearing
asymmetry.
Presbycusis refers to the almost inevitable decline of hearing with age. The

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141

" TINNITUS AND HEARING LOSS

KEY POINT:

142

Formal
audiometric
testing is the
gold standard
for determining
hearing loss.

etiology is almost certainly multifactorial and includes a lifetime of noise exposure, genetic predisposition, and as
yet unknown environmental factors.
Age-related hearing loss initially affects
the high frequencies, the frequency
range matching consonant sounds, making understanding of speech more difficult. Communication among elderly
people is particularly problematic because the hearing loss becomes prevalent just at the same time that vocal
strength becomes less robust.
HEARING SCREENING
The self-report of hearing loss and
accurate responses to questions about
hearing loss, particularly among the elderly population, are notoriously unreliable measures of either the presence
or severity of hearing loss. The individual frequently is unaware of what he or
she is missing in the environment and
often projects the communication difficulties onto others who are constantly
mumbling. The self-report of the presence of hearing impairment is slightly
more reliable than its denial.
Several hearing handicap questionnaires have been developed, including
the HHIE-S (Hearing Handicap for the
Elderly Screening Version) (Ventry and
Weinstein, 1982). This self-administered
questionnaire consisting of 10 questions
has demonstrated more accuracy in
identification of hearing-impaired individuals than open-ended questioning
(Table 7-3).
Office screening techniques include
whispered voice test, tuning forks, and
1
AudioScope . Although attempts have
been made to standardize the whispered voice test, it is at best a very crude
measure of hearing. Tuning-fork tests
are useful for identifying the side of
hearing loss and differentiating conductive from sensorineural hearing loss,
but tuning forks are uncalibrated and
are inadequate for general screening for
hearing loss.

The AudioScope
The AudioScope is a hand-held instrument that combines a miniature puretone screening audiometer with a visual
otoscope. It produces pure tones at
500 Hz, 1000 Hz, 2000 Hz, and 4000 Hz
at loudness levels of 20 dB, 25 dB, and
40 dB. The instrument is used to visualize the external auditory canal and
tympanic membrane to ensure that
there is no obstructive debris. For audiometric testing it is important that the
speculum has a tight seal with the ear
canal. The AudioScope then presents
tones at random loudness, and the patient indicates with a finger movement
when they can hear the tone. The
AudioScope has been shown to have
good interobserver reliability, and correlation to formal audiometric testing is
good (Lichtenstein et al, 1988).
Behavioral Audiometric Testing
Formal audiometric testing is the gold
standard for determining hearing loss.
To be accurate, audiometry must be
performed in a sound-treated room with
a well-calibrated audiometer by trained
and experienced personnel. Behavioral
audiometric tests are divided into puretone and speech audiometry.
Pure-tone audiogram tests determine
the individuals threshold for frequencies between 250 Hz and 8000 Hz presented at octave intervals. Louder and
softer sounds are presented to the
patient, who indicates when he or she
can hear the sound. Threshold is
defined as the loudness level at which
the subject responds correctly 50% of
the time.
Audiometric thresholds are measured on a logarithmic scale in decibels
hearing level (HL). HL is based on normative results obtained by testing young
individuals without previous history of
otological disease or noise exposure. A
hearing level of 0 dB HL is not the
absence of sound but is the threshold
for perfectly normal-hearing subjects,

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much the same as 20/20 is defined as

the normative value for normal vision.
In clinical practice, hearing is considered normal between 0 dB and 25 dB,
mild hearing loss between 21 dB and 40
dB, moderate loss between 41 dB and
55 dB, moderately severe between 56
dB and 70 dB, severe between 71 dB and
90 dB, and profound above 90 dB.
Stimuli for pure-tone tests are presented in two ways to test both air conduction and bone conduction (Figures
7-2 and 7-3). Air-conduction stimuli
are presented to the external auditory
canal with a speaker over the ear or
inserted in the external auditory canal.
Air-conduction thresholds measure the
performance of the entire auditory system from the ear canal to the higher

TABLE 7-3

auditory centers. Thus, hearing loss from

any cause is reflected in the air conduction pure-tone threshold.
Bone-conduction thresholds are determined by placing a bone oscillator
on the thin skin behind the ear and
applying the stimulus directly to the
skull. Bone-conduction stimuli bypass
the external auditory canal and middle ear conductive system and directly
stimulate the cochlea organ. The difference between air-conduction and boneconduction thresholds is designated the
air-bone gap and indicates the conductive or middle ear component of hearing
loss. Patients can have pure-conductive
hearing loss, pure-sensorineural hearing
loss, or a combination of both, commonly
termed a mixed loss.

KEY POINT:

A hearing level
of 0 dB is
not the absence
of sound but
is the threshold
for perfectly
normal-hearing
subjects, much
the same as
20/20 is
defined as
the normative
value for
normal vision.

Hearing Handicap Inventory for the Elderly (Screening)

Question

Yes (4)

Sometimes (2)

No (0)

Does a hearing problem cause you to feel

embarrassed when meeting new people?
Does a hearing problem cause you to feel
frustrated when talking to members of
your family?
Do you have difficulty hearing when
someone speaks in a whisper?
Do you feel handicapped by a hearing problem?
Does a hearing problem cause you difficulty
when visiting friends, relatives, or neighbors?
Does a hearing problem cause you to attend
religious services less often than you would like?
Does a hearing problem cause you to have
arguments with family members?
Does a hearing problem cause you difficulty
when listening to TV or radio?
Do you feel that any difficulty with your hearing
heavily limits or hampers your personal or social life?
Does a hearing problem cause you difficulty when
in a restaurant with relatives or friends?
Total score >8 suggests significant hearing impairment.
Ventry IM, Weinstein BE. Identification of elderly people with hearing problems. ASHA 1983;25:3742. Reprinted with permission.

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143

" TINNITUS AND HEARING LOSS

A complete audiogram also includes

suprathreshold tests, which evaluate
the performance of the auditory system
given sufficient stimulus levels. The
speech discrimination test determines
the ability of the individual to understand and correctly repeat an open set of
monosyllable words without any contextual cues. The words used are phonetically balanced monosyllables and
delivered to the patient from a standard
list. The speech determination score represents a simple percentage of correct
responses. Speech discrimination scores
out of proportion to what would be
predicted by the threshold audiogram
suggest problems with the auditory
nerve, especially if the score deteriorates
with higher levels of sound presentation
(normally louder presentation makes
the test easier and scores improve).

144

Audiogram showing sensorineural hearing

loss. Air conduction in the left ear (X) is
normal. Air conduction in the right ear (0)
shows a descending pattern, with a notch at 4k Hz, typical of
noise-induced hearing loss. The bone-conduction thresholds
for the right ear ([ ) are identical to the air-conduction
thresholds, reflecting a purely sensorineural hearing loss.

FIGURE 7-2

HL = hearing level; R = right; L = left.

Nonbehavioral Tests
Tympanometry is a means of determining the compliance of the tympanic
membrane and middle ear space. Normally the pressure within the middle
ear should be very close to atmospheric
pressure, allowing the tympanic membrane to vibrate freely. Negative pressure in the middle ear resulting from
inadequate ventilation through the
eustachian tube reduces the middle
ear compliance, which is easily measured with tympanometry. While not
strictly a test of hearing, tympanometry
can give a perspective on the status of
the middle ear, specifically indicating
whether it is air containing or fluid filled.
In addition, tympanometry allows testing of the acoustic reflex. The acoustic
reflex is a simple reflex arc starting with
the auditory nerve and ending with the
middle ear muscles, the tensor tympani
and stapedius. Normally these muscles
respond to loud sound and transiently
impede the movement of the tympanic
membrane, thus decreasing compliance
of the tympanic membrane. Absence of
the acoustic reflex is most commonly
caused by compromise of the auditory
nerve by lesions such as an acoustic
neuroma.
Two electrophysiological tests for
hearing do not depend on voluntary
behavioral responses and are therefore
useful when a patient is unwilling or
unable to give responses on the behavioral tests. The auditory brain stem
response (ABR) test is an abbreviated
electroencephalogram that records auditory brain stem waveforms in response
to tone bursts or clicks delivered to the
ear canal. The brain stem potentials
are very small and require the use of
a summating computer for their detection. The brain stem response is a series of five waves. The first wave results
from synchronous firing of the auditory nerve; the exact generators of later
waves are still controversial. ABR stimuli
are usually given with a speaker in the

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external canal; thus it is parallel to airconduction tests described above. The

stimulus can be delivered with a bone
oscillator to produce a bone-conduction
ABR. The ABR assesses the integrity of
the auditory system as far as the lower
brain stem nuclei but does not assess
whether hearing has any functional
meaning to the subject. Although the exact threshold of hearing cannot be determined with ABR, the threshold can
be approximated by subtracting 20 dB
from the lowest identifiable threshold.
Since click stimuli are primarily high frequency, ABR is an inadequate measure
of low-frequency hearing. ABR is useful
in identification of hearing loss in neonates and infants as well as when there
is a question of malingering or noncooperation on the part of the subject.
Cochlear emissions tests were recently
developed for evaluation of the integrity of the cochlea itself, separate
from the auditory nerve, upper brain
stem, and cortical auditory centers.
Cochlear emissions result from the motile activity of the outer hair cells. Under
the right conditions, this motile activity
of the outer hair cells can drive the
ossicular chain and tympanic membrane to produce sounds in the external
auditory canal, which can be amplified
and recorded. Otoacoustic emissions
can occur spontaneously or in response
to acoustic stimuli to the cochlea. Clinically the most commonly used form
of otoacoustic emission is produced by
paired frequencies that induce other
tones to be produced by the cochlea.
The most easily recorded frequency
equals 2 times the lower stimulus minus
the higher stimulus (2f1 f2). Continuous variation of the stimulus frequencies allows assessment of hair cell
function across the entire frequency
spectrum of the cochlea.
Otoacoustic emissions is the most
commonly used test for newborn infant
hearing screening because of the ease
and speed with which it can be admin-

Audiogram showing conductive hearing loss.

Air conduction in the left ear (X) is normal.
Air conduction in the right ear (O) shows a
hearing loss that is worst in the low frequencies and rises
through the mid frequencies. Bone-conduction responses in
the right ear ([ ) are normal. The difference between bone
and air conduction (air-bone gap) reflects the conductive
component of the hearing loss.

FIGURE 7-3

HL = hearing level; R = right; L = left.

istered. The test requires a quiet, but

not sedated, subject. Cochlear emissions
are also useful in evaluating subjects
who are unable or unwilling to cooperate with behavioral testing. Once again,
it is important to remember that cochlear emissions test only cochlear function and do not assess auditory nerve,
higher auditory centers, or cognition.
Cochlear emissions are important in the
diagnosis of auditory neuropathy, because emission testing will be normal
despite severe hearing impairment.
MANAGEMENT OF
HEARING LOSS
Hearing aids can always improve conductive hearing loss, and surgery can often produce a lasting benefit. Stapedotomy, the replacement of the fixed stapes
in otosclerosis, and tympanoplasty, the

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145

" TINNITUS AND HEARING LOSS

KEY POINTS:

146

Hearing
aids have
undergone
tremendous
improvement
even within
the last decade
and provide
benefit for
most types of
hearing loss.
Cochlear
implants have
revolutionized
the care of
the profoundly
deaf over
the past 2
decades.

repair of tympanic membrane perforation, both have predictably excellent results in experienced hands. The
results of reconstruction for other ossicular abnormalities are highly dependent on the extent of prior damage
and the geometry of remaining middle
ear structures. In chronic otitis media
the ability of the eustachian tube to
provide a permanently air-containing
middle ear space is the major determinant of surgical outcome.
Sensorineural hearing loss must be
managed with hearing aids, or with
cochlear implants in people with profound hearing loss. Hearing aids have
undergone tremendous improvement
even within the last decade and provide
benefit for most types of hearing loss.
Patients with relatively flat hearing loss
across the frequency spectrum and
relatively well-preserved discrimination
scores are especially good candidates
for amplification. Individuals with complex audiometric patterns, poor discrimination scores, and auditory neuropathy are more challenging to help with
hearing aids. In most instances bilateral sensorineural hearing loss patients
should be encouraged to seek binaural
amplification because binaural hearing
improves the signal-to-noise ratio and
assists the patient in picking out a desired conversation from background
noise. The results with modern digital
hearing aids are quite good, and individuals with a poor experience with
hearing aids a decade ago should be
encouraged to try them again. Even
adults have significant plasticity of the
central auditory system; therefore, it is
important that hearing-aid users wear
their aids consistently to allow thorough adaptation to them. Subjects who
take their aids in and out for different
situations generally do not do as well as
consistent hearing aid-wearers.
Cochlear implants have revolutionized the care of the profoundly deaf
over the past 2 decades. Cochlear im-

plants directly stimulate the auditory

nerve, bypassing the cochlear transducer completely. The auditory nerve
in most cases has a substantial proportion of residual fibers despite degeneration or absence of the peripheral end
organ. Three different brands of cochlear
implants are currently approved by
the US Food and Drug Administration
(USDFA). All have an internal implant
and an external processor. The internal
portion has a passive receiver under the
parietal scalp and an electrode array
inserted directly into the cochlea adjacent to the auditory nerve. The external
portion, typically an ear-level device
resembling a hearing aid, has a battery and sound processor that divides
speech into component frequency and
amplitude information and transmits it
to the internal device.
Two broad populations are candidates for cochlear implants. Postlingual
adults, those who have learned speech
with normal hearing and then lost hearing, are ideal because the speech areas
of the auditory cortex are fully developed and only need auditory input.
Congenitally deaf children are also
excellent candidates if the implant is
done during the time of normal development of the auditory cortex. Cochlear
implants are currently USFDA approved
down to the age of 12 months. Without
auditory stimulation, the auditory cortex
fails to develop and the window of
opportunity for useful hearing from the
implant closes sometime in late childhood. Overall, the success of implants
is outstanding. Communication without
the aid of lip reading (ie, over the telephone) is expected to be possible in
most postlingual adults. Most implanted
children, barring other developmental
issues, can be mainstreamed in hearing
schools during the primary years.
TINNITUS AND HYPERACUSIS
Tinnitus is the phantom sensation of
sound perceived in the ear. In most cases

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these sounds originate in the auditory

system, either the periphery or more
central auditory connections. Traditionally tinnitus has been divided between
subjective and objective tinnitus, and
the current International Classification
of Diseases, Ninth Revision (ICD-9)
coding system only recognizes these two
distinctions. The author feels strongly
that this is an inaccurate characterization because the differentiating factor
is whether the sound can be identified
by an observer, and this may be more
dependent on the fastidiousness of the
observer than the pathophysiology of
the patient. It is more useful to divide
tinnitus into that which originates from
the auditory system (sensorineural) and
that which derives from some somatic
structure around the ear, including vascular, temporal mandibular joint, and
the pharyngeal palatal musculature.
These sounds are accurately termed
somatosounds.

from middle ear effusion, chronic otitis

media, or otosclerosis. Either tinnitus
or hearing loss can occur alone, but
the two symptoms frequently occur
together. Tinnitus is an important corroborating symptom in Menie`res disease. Tinnitus associated with unilateral
hearing loss should prompt imaging
studies to exclude a cerebellopontine
angle lesion. In the absence of an identifiable cause for the hearing loss or
tinnitus, it is important to remember
that the presence of either does not
predict the development or worsening
of the other.
Sensorineural tinnitus can be characterized roughly by pitch-matching
and loudness-matching tests. Although
these tests are descriptive and are used
for monitoring patients, they are not
predictive or prognostic in any way.
Tinnitus loudness carries little relationship to the actual disturbance and
quality-of-life impact of tinnitus.

Sensorineural Tinnitus
So-called sensorineural tinnitus is described as a roaring, ringing, rushing
steam; crickets; or some similar sound
and is by far the most common type of
tinnitus. The tinnitus often has an unpredictable waxing and waning course
with abrupt increases or decreases, but
it does not have a regular rhythmic
quality suggestive of breathing or heartbeat. Within the category of sensorineural tinnitus the description of the
sound does not suggest etiology or
prognosis. To a certain degree tinnitus
is a normal phenomenon because most
people placed in an extremely quiet
environment (anechoic chamber) will
note some tinnitus.
The diagnostic workup of sensorineural tinnitus requires a thorough
otological examination to identify any
associated ear disease that may be
causing or exacerbating the tinnitus.
Tinnitus is a frequent complaint in patients who have conductive hearing loss

Somatosounds
The most common somatosounds are
vascular, and patients frequently complain of a rhythmic pulsing or whooshing sound in the ear. The patient
should be asked whether the pulsing
noise is coincident with the pulse; it is
remarkable how many patients have not
made this connection. The differential
diagnosis includes arterial and venous
bruits and occasional vascular tumors.
Arterial sources of pulsatile tinnitus include carotid bruits, carotid dissection,
dural arteriovenous malformations, and
carotid cavernous fistula. Venous bruits
are more common than arterial and can
arise from turbulence or stenosis in the
sigmoid or upper jugular vein or diverticulum of the sigmoid sinus. Idiopathic
intracranial hypertension may also present with unilateral or bilateral pulsatile
tinnitus, although headaches and visual
disturbances are more common. Like
other forms of venous tinnitus, the

KEY POINTS:

Tinnitus is
the phantom
sensation
of sound
perceived in
the ear. In
most cases
these sounds
originate in
the auditory
system, either
in the periphery
or more
central auditory
connections.
In the absence
of an
identifiable
cause for the
hearing loss
or tinnitus,
it is important
to remember
that the
presence of
either does not
predict the
development
or worsening
of the other.
The most
common
somatosounds
are vascular,
and patients
frequently
complain of
a rhythmic
pulsing or
whooshing
sound in
the ear.

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147

" TINNITUS AND HEARING LOSS

KEY POINT:

148

Patients with
tinnitus should
always be
counseled
to keep
environmental
noise around
them and avoid
silence. Sitting
in a quiet
environment
listening to
tinnitus only
promotes
individuals
ability to hear
their tinnitus.

sound is frequently eliminated by venous

compression (Sismanis, 2003). Glomus
tumors (chemodectomas) of the middle ear, jugular foramen, or carotid
bifurcation are less common causes of
pulsatile tinnitus.
On physical examination the physician should auscultate the neck, the
preauricular and postauricular areas, and
the scalp in a very quiet environment.
Differentiation between arterial and venous bruits is usually readily determined
by gentle supravenous compression
of the ipsilateral jugular vein, which
will obliterate venous, but not arterial,
sounds. The neck should be palpated
for masses and the ear examined for
the characteristic red mass behind the
tympanic membrane typical of glomus
tumors.
The evaluation of older individuals
with arterial bruits should start with a
carotid ultrasound. If the ultrasound
is negative, and in younger individuals
without carotid bruit, the diagnostic
test of choice is a computed tomographic angiogram and venogram, which
has higher sensitivity to small abnormalities of the vascular system and the
temporal bone than magnetic resonance angiography.
The complaint of a rapid clicking
in the ear suggests palatal myoclonus
causing rapid opening and closing of
the eustachian tube. Palatal myoclonus
is most reliably diagnosed with nasal
endoscopy because voluntary opening
of the mouth frequently inhibits the
myoclonus. Palatal myoclonus can be
an isolated phenomenon or can be a
manifestation of brain stem compression (Brown and Walsh, 2004) or supranuclear palsy (Suyama et al, 1997).
Therefore, brain stem imaging is critical
to rule out underlying central nervous
system disease. Isolated palatal myoclonus can be treated with botulinum toxin
(BoTox) injected into the muscles
around the nasopharyngeal opening of
the eustachian tube.

Lastly, some patients will complain of

fullness in the ear associated with a
roaring or rushing noise with breathing.
These patients will also complain of
autophony or excessive loudness of
their own voice in the ear. The complaints usually subside when the patient
is supine and return after the patient has
been erect for a short time. These are
symptoms of an abnormally patent eustachian tube. Normally the eustachian
tube is closed at rest and opens only
during swallowing or yawning. In some
individuals the eustachian tube remains
patent all of the time, allowing transmission of sounds from the pharynx
directly to the ear. An abnormally patent
eustachian tube can have a profound
effect on quality of life. The constant
and predictable annoyance of the autophony can cause social withdrawal. An
abnormally patent eustachian tube can
be treated with partial obliteration of
the pharyngeal opening of the tube with
cautery or cartilage grafts.
Management
The management of tinnitus is very
much dependent on the results of
the initial evaluation. When tinnitus
is associated with conductive hearing
loss from a defect in the external auditory canal, tympanic membrane, ossicular chain, or air compartment of
the middle ear, the treatment should
be directed toward the underlying
problem.
Management of sensorineural tinnitus is difficult. Most patients respond to
careful evaluation and reassurance that
there is no serious underlying pathology requiring treatment. Tinnitus truly
disturbs a small portion of patients.
Patients with tinnitus should always be
counseled to keep environmental noise
around them and avoid silence. Sitting
in a quiet environment listening to tinnitus only promotes individuals ability
to hear their tinnitus. Tinnitus with sensorineural hearing loss is appropriately

Copyright @ American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

treated with amplification, and amplification of environmental sounds frequently

makes the tinnitus less bothersome.
Other measures that have been used
to manage tinnitus include biofeedback, cognitive therapy, and tinnitus
retraining therapy (TRT). TRT uses a
combination of directive counseling and
sound stimulation to promote habituation to the tinnitus (Jastreboff et al,
1996; Jastreboff and Jastreboff, 2000).
Habituation to any stimulus occurs as
long as the stimulus is not perceived as
threatening or dangerous and other
stimuli are available. For instance, most
people will ignore the ticking of a clock,
but the same ticking connected to a
time bomb would be impossible to
ignore. Therefore, it is the significance
of the signal, not the signal itself, which
determines the emotional and physiological impact. The same analogy can be
made with tinnitus, and the difference
between individuals who are or are not
impacted by tinnitus is not the quality
of the tinnitus itself, but its interpretation on a conscious and subconscious
level. Through directive counseling and
the use of sound-generating devices,
TRT attempts to relegate the tinnitus
signal to the same status as other unimportant environmental sounds, for
instance, the noise of a refrigerator
or air-conditioning system. Although

not a panacea, TRT benefits a substantial number of patients with tinnitus

(Jastreboff and Jastreboff, 2000).
Hyperacusis is an underappreciated
symptom and occurs in a significant
proportion of individuals with hearing
loss and tinnitus. Hyperacusis is the
abnormal perception of the loudness of
sounds such that ordinary environmental sounds are uncomfortably or painfully loud. Hyperacusis can appear in
several different forms. True hyperacusis affects all sounds and frequencies
above a certain sound level. Other
patients have discomfort only from certain sounds or situations, termed misophonia. Hyperacusis appears to be a
disorder of central auditory processing.
It can be induced in normal individuals
by prolonged use of noise attenuation
devices (Formby et al, 2003). Therefore,
the natural inclination of patients with
hyperacusis to protect themselves from
noise by wearing earplugs or noise
cancellation devices is counterproductive and should be strongly discouraged. Hyperacusis can be treated with
the slow and general reintroduction
of sound (Formby et al, 2003; Jastreboff
et al, 1996).
ACKNOWLEDGMENT
The author thanks Anthony Mattox for
graphic assistance.

149
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