To demonstrate diagnostic imaging of an extremely rare presentation of bilateral narrow duplication of the internal auditory canal.

An adolescent boy with profound sensorineural hearing loss presented for hearing rehabilitation. Imaging studies (i.e. multidetector computed tomography and magnetic resonance imaging) clearly demonstrated bilateral duplication of the internal auditory canals, with narrowing of the lower canals, unilateral cochlear and vestibular dysplasia, bilateral superior semicircular canal malformation, and bilateral absence of the posterior semicircular canals.

To our knowledge, this is only the third such case described in the literature. Considering that the vestibulocochlear nerve has been unable to be demonstrated in almost all cases of duplicated internal auditory canal (unilateral and bilateral), our case supports the hypothesis that vestibulocochlear nerve aplasia or hypoplasia leads to internal auditory canal stenosis. We consider this rare presentation of bilateral narrow duplication of the internal auditory canal to represent a contraindication for cochlear implantation.

We report a young man with bilateral enlarged internal auditory canals who developed sudden sensorineural hearing loss following weight-lifting exercise.

We present a detailed clinical history, including the patient's high resolution computed tomography and magnetic resonance imaging scans.

The patient reported left-sided hearing loss immediately following weight-lifting exercise. He had no vestibular disturbance. He was treated with a combined regimen of steroids and an antiviral drug, but his profound hearing loss did not resolve. During another session of weight-lifting exercise, he suffered another episode of sudden hearing loss.

To the best of our knowledge, this is the first report of a patient with patulous internal auditory canals, with no other anomalies, who developed bilateral sudden hearing loss after weight-lifting exercise. Although no definitive conclusions can be drawn, close surveillance and lifestyle warnings should be considered in such patients, even if they are clinically asymptomatic.

We report the use of bilateral surgical decompression of the internal auditory canals to treat hyperostosis cranialis interna in an eight-year-old girl presenting with bilateral facial palsy due to hyperostosis cranialis interna.

Using a middle fossa craniotomy approach, both internal auditory canals were unroofed and cranial nerves VII and VIII were decompressed, with a one-year interval between sides. The mimic function recovered. One year post-operatively, the right and left facial sides had been restored to House–Brackmann grades I and II, respectively.

This is the first report of the use of surgical decompression of the internal auditory canal in a case of hyperostosis cranialis interna. Surgical decompression of the internal auditory canal is recommended therapeutically, but may also be performed prophylactically in younger patients with hyperostosis cranialis interna.