AJR Integrative Imaging

LIFELONG LEARNING FOR RADIOLOGY

Radiological Reasoning: Congenital Sensorineural Hearing Loss

Nadja Kadom1, Raymond W. Sze

OBJECTIVE
A 12-year-old girl with an outside diagnosis of bilateral profound to severe sensorineural hearing loss came to the pediatric hearing and speech center for cochlear implant evaluation. CT evaluation showed bilateral marked vestibular aqueduct enlargement along with bilateral hypoplasia of the cochlea, minor dysplasia of the vestibules, and bilaterally underdeveloped lateral semicircular canals. The patient received a left cochlear implant and is doing well.

CONCLUSION
CT of the temporal bones is a valuable tool in the diagnosis and treatment of pediatric congenital sensorineural hearing loss. Key functions of the radiologist are design of optimal imaging studies for children and in-depth anatomicpathologic knowledge for pre- and postsurgical anatomy.

Case History
A 12-year-old girl presented to the pediatric hearing and speech center for cochlear implant evaluation. The patient had an outside diagnosis of bilateral profound sensorineural hearing loss. The patient was adopted from China, and no family history was available. The patient was referred for CT of the temporal bones to evaluate the inner ear anatomy.

CT
CT of the temporal bones showed bilateral hypoplasia of the cochlea (Fig. 1A) along with bilateral marked vestibular aqueduct enlargements (Fig. 1B), minor dysplasia of the vestibules (Fig. 1B), and bilaterally underdeveloped lateral semicircular canals (Fig. 1B).

Expert Discussion
Temporal bone CT is a well-established technique for evaluation of pediatric hearing loss, generally best suited for evaluation of trauma and nonacute congenital dysplasias of the otic capsule. For patients under consideration for cochlear implantation, the surgeon not only needs to identify congenital abnormalities (such as coexisting middle ear abnormalities, type of cochlear abnormality, pattern of cochlear ossification, and vestibular aqueduct abnormality) but also a variety of anatomic information, such as grade of mastoid
Keywords: cochlear implant, CT, dysplasia, sensorineural hearing loss DOI:10.2214/AJR.07.7120 Received August 18, 2008 accepted after revision March 14, 2009.
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bone aeration and internal auditory canal size and shape. The information helps in assessing the feasibility of cochlear implantation, side of implantation, modification of implantation technique, and anticipation of complications. To provide this information to the clinician, a high-resolution CT protocol needs to be applied. We perform direct axial-plane imaging through the temporal bone parallel to the hard palate using a bone algorithm, 512 512 matrix, and contiguous 0.625-mm sections. At our institution, direct coronal imaging is omitted for patients with sensorineural hearing loss to save radiation exposure. Instead, coronal reformatted images are generated. Sensorineural hearing loss is classified into congenital and acquired forms, the latter are further differentiated into sudden onset and asymmetric presentations and are best evaluated with MRI to detect retrocochlear abnormalities, such as tumors of the internal auditory canal or cerebellopontine angle, labyrinthitis, cerebellar infarcts, demyelinating disease, and pachymeningitis, to name a few [1]. About 50% of pediatric patients have a genetic cause for sensorineural hearing loss, such as congenital inner ear dysplasia or a syndrome associated with hearing impairment, such as CHARGE (coloboma, heart disease, atresia choanae, retarded growth and development, genital abnormalities, and ear anomalies). Between 20% and 40% of children develop sensorineural hearing loss as a consequence of prenatal injury, such as TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, and congenital syphilis) or postnatal injury, such as meningitis or trauma. In about 30%, the cause of sensorineural hearing loss remains unknown [2]. For optimal interpretation of high-resolution CT images, the radiologist must have comprehensive knowledge of the anatomy of the inner ear structures and the spectrum of pathology that can be encountered [2, 3]. Some inner ear structures require measurements. The most common congenital inner ear abnormality seen on CT is a large vestibular aqueduct (measuring > 1.5 mm in diameter) (Fig. 2). This is best measured at least 1 mm from the skull base opening (Fig. 3). Another reference for the maximum width of the vestibular aqueduct is the diameter of the semicircular canals. The vestibule should be smaller than or equal to

Both authors: Department of Radiology, Childrens National Medical Center, 111 Michigan Ave., Washington, DC 20010. Address correspondence to N. Kadom (nkadom@cnmc.org).

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Fig. 1Vestibulocochlear malformation, grade IP-II, in 10-year-old girl. A and B, High resolution CT images of temporal bone (A) and reformatted in plane perpendicular to petrous bone (B) in plane with lateral semicircular canal show lack of differentiation between middle and apical turn of cochlea (arrow, A) and associated large vestibule (long arrow, B), enlarged vestibular aqueduct (arrowhead, B), and hypoplastic lateral semicircular canal (short arrows, B).

Fig. 2Companion case in 11-year-old girl with enlarged vestibular aqueduct. High-resolution axial CT image of temporal bone shows vestibular aqueduct measures more than three times diameter of semicircular canal (? 2.7 vs ? 0.8 mm).

Fig. 3Companion case in healthy 3-year-old boy. High-resolution axial CT image of temporal bone shows normal anatomy. A = diameter of vestibular aqueduct, best measured away from skull base opening; B = diameter of semicircular canal, serves as maximum reference for vestibular aqueduct width; C = diameter of vestibule; D = width of bone between vestibule and lateral semicircular canal, serves as reference for maximum vestibular width; E = diameter of internal auditory canal, if less than 2 mm, this finding raises suspicion for cochlear nerve pathology.

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Congenital Sensorineal Hearing Loss

Fig. 4Companion case in 5-year-old girl with hypoplastic internal auditory canal. AC, Axial FIESTA (fast imaging employing steadystate acquisition) MR image (A) and sagittal reformations perpendicular to long axis of internal auditory canal (B and C). In axial FIESTA MR image, note abnormally small size of right internal auditory canal compared with left (1.8 vs 3.2 mm). Sagittal reformation on right (B) shows absence of cochlear nerve in expected anterior inferior location (arrow, B) as seen on contralateral side (long arrow, C). Note cranial nerve 7 normally visualized on left side in anterior superior portion of internal auditory canal (arrowhead, C) and normal superior and inferior vestibular nerves posteriorly (small arrows, C).

(but not larger than) the bone width between the lateral vestibular wall and the inner wall of the lateral semicircular canal. The internal auditory canal should be larger than 2 mm; a smaller diameter has been shown to adversely impact the long-term outcome with cochlear implantation. A small internal auditory canal can indicate absence or small size of the cochlear nerve, which is best further evaluated with MRI [4, 5] (Fig. 4). Some controversy exists about the classification of cochleovestibular malformations [6]. Especially the widespread use of the term Mondini malformation has been subject to scientific debate and has been differentiated into two types of incomplete partitioning (IP), IP-I and IP-II. The

following classification and definitions have been proposed by Sennaroglu and Saatci [6], listed here in descending order of severity: labyrinthine aplasia, Michel deformity, no inner ear structures have formed; cochlear aplasia, absence of cochlea; common cavity, single cavity of cochlea and vestibule; IP-I, cystic cochlea and cystic vestibule; cochlear hypoplasia and cochlea with less than 2.5 turns (Fig. 5); and IP-II, Mondini deformity, incomplete partition between the middle and apical turns of the cochlea forming a cystic apex, normal basal turn, large vestibular aqueduct, and enlarged vestibule (Fig. 1). The correct identification of inner ear pathology helps assessment for contraindications to cochlear implantation

Fig. 5Companion case in 2-year-old girl with hypoplastic cochlea. A, High-resolution CT image reformatted in coronal plane at level of cochlea shows hypoplastic right basal turn of cochlea (arrow); middle and apical turns have not formed. B, High-resolution CT image reformatted in coronal plane at level of cochlea in healthy 3-year-old boy is shown for comparison.

B
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and improves surgical planning. Successful cochlear implant function requires functionality of the cochlear nerve and at least some residual auditory cells. A small internal auditory canal seen on CT can be an indirect sign of cochlear nerve absence or hypoplasia, as confirmed by Glastonbury et al. [7] by MRI correlation. However, deficiency of the cochlear nerve can exist in the setting of a normal size internal auditory canal, probably due to an insult that occurs after formation is complete [7]. Some authors prefer high-resolution MRI over high-resolution CT because it simultaneously provides information about cochlear patency and presence and size of the cochlear nerve [4]. Absence of the cochlea and labyrinthine (Michel) aplasia are contraindications for cochlear implant surgery. Cochlear dysplasia, although it limits full insertion of the implant electrodes, still will yield benefit to the patient because of the residual auditory cells present. Precise radiologic information also affects surgical planning. For example, in the presence of a dysplastic cochlea there may also be a thinner barrier between the cochlea and subarachnoid space, which increases the risk of CSF leak, intracranial electrode insertion, and meningitis. However, this can be surgically addressed by choosing an appropriate electrode and imaging confirmation of correct placement. CSF leak requires packing of the cochleostomy site. Description of facial nerve position is important because an aberrant position may pose an increased risk of facial nerve injury and requires more careful surgical technique and facial nerve monitoring [8].

edge that bilateral cochlear implants are beneficial for development of 3D sound localization when implanted within the first 3 years of life [9]. High-resolution CT plays a vital role in the diagnosis of congenital sensorineural hearing loss and helps with surgical decision making and planning, as discussed earlier. However, additional imaging techniques are used in the pre- and postoperative evaluation of cochlear implant patients, and there are additional applications for CT of the temporal bones in this patient group. For example, it has been shown that preoperative functional studies of auditory response with MRI, PET, or SPECT can provide useful information on brain activity response to auditory stimuli. This, in turn, can help in deciding the side of a single cochlear implant and help predict functional outcome after implantation [4]. Given that most implant devices are not compatible with magnetic field exposure, the preferred methods for postoperative functional auditory assessment are PET and SPECT [4]. CT can play an additional role in postoperative assessment of the electrode position [10]. Modern imaging protocols, such as BrainLab (BrainLAB), can be used for 3D planning of minimally invasive surgery, using the imaging data to plan a drill path. Further studies using this technique are under way at the National Institutes of Health [11].
References
1. St. Martin MB, Hirsch BE. Imaging of hearing loss. Otolaryngol Clin North Am 2008; 41:157178 [vi-vii] 2. Lowe LH, Vzina LG. Sensorineural hearing loss in children. RadioGraphics 1997; 17:10791093 3. Som PM, Curtin HD. Head and neck imaging, 4th ed. St Louis, MO: Mosby, 2003 4. Pappas DG Jr, Cur JK. Diagnostic imaging. Otolaryngol Clin North Am 2002; 35:13171363 [ix] 5. Wu CC, Lee YC, Chen PJ, Hsu CJ. Predominance of genetic diagnosis and imaging results as predictors in determining the speech perception performance outcome after cochlear implantation in children. Arch Pediatr Adolesc Med 2008; 162:269276 6. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope 2002; 112:22302241 7. Glastonbury CM, Davidson HC, Harnsberger HR, Butler J, Kertesz TR, Shelton C. Imaging findings of cochlear nerve deficiency. AJNR 2002; 23:635643 8. Francis HW, Niparko JK. Cochlear implantation update. Pediatr Clin North Am 2003; 50:341361 [viii] 9. OLeary S, Chang A. Hearing impairment: technological advances and insights. Aust Fam Physician 2008; 37:322327 10. Verbist BM, Frijns JH, Geleijns J, van Buchem MA. Multisection CT as a valuable tool in the postoperative assessment of cochlear implant patients. AJNR 2005; 26:424429 11. Labadie RF, Noble JH, Dawant BM, Balachandran R, Majdani O, Fitzpatrick JM. Clinical validation of percutaneous cochlear implant surgery: initial report. Laryngoscope 2008; 118:10311039

Clinical Management
The audiology assessment showed bilateral moderate to profound sensorineural hearing loss, left worse than right. The patient received bilateral temporary hearing aids to bridge the time to cochlear implantation. Extensive speech and language testing was performed preoperatively and showed the patient had a good foundation for auditory skills. The patient received a left cochlear implant and postoperative follow-up implant mapping showed excellent acoustic benefit.

Commentary
The incidence of sensorineural hearing loss in children is about one in 1,000. Today, screening tests as part of newborn evaluations facilitate the early detection of congenital hearing impairment [9]. Cochlear implants bypass inner ear dysfunction from structural abnormalities by direct electrode activation of auditory nerve fibers that reside in the cochlea with an electrode, thus improving hearing and subsequent speech development [9]. There is emerging knowl-

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