Bilateral internal auditory canal atresia and vestibulocochlear nerve aplasia
Presentation
Bilateral deafness. For preoperative assessment before cochlear implants.
Patient Data
Bilateral internal auditory canal atresia/stenosis with poor delineation (aplasia) of the vestibulocochlear nerves in the IAC on both sides. Instead, there are thin canals transmitting the facial nerves on both sides.
Normal inner ear structures on both sides.
Bilateral internal auditory canal atresia/stenosis with poor delineation (aplasia) of the vestibulocochlear nerves in the IAC on both sides. Instead, there are thin canals transmitting the facial nerves on both sides.
Normal inner ear structures on both sides.
Bilateral otomastoiditis.
Case Discussion
Internal auditory canal (IAC) stenosis is a rare entity. Hypoplasia of the vestibulocochlear nerves is a common association and an important consideration in the differential diagnosis of sensorineural hearing loss.
Radiographically, IAC stenosis is diagnosed when the IAC diameter is < 2 mm in vertical diameter on MRI or CT.
IAC stenosis is an important consideration in the differential diagnosis of sensorineural hearing loss, as it is a relative contraindication for cochlear implant placement.
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