Congenital sensorineural hearing loss with anomalous vestibular nerve complex: A rare radiological finding

Asma Binti Abdullah, Faizah Binti Mohd Zaki, Luqman Bin Rosla

Indian Journal of Otology 2018 24(1):71-73

Radiological assessment is an essential tool for preoperative assessment of cochlear implant (CI) candidates with congenital sensorineural hearing loss (SNHL). It gives crucial information of the inner ear, vestibulocochlear nerve, and brain. Hypoplasia of the cochlear nerve bony canal is commonly associated with profound SNHL, whereby 88% of this abnormality was found in 95% of CI candidates. This finding may be associated with internal auditory canal hypoplasia or other cranial nerve abnormalities. We describe a rare case of a patient diagnosed with congenital SNHL exhibiting an interesting radiological finding of an anomalous vestibular nerve course alongside hypoplasia of the cochlear bony canal which could be postulated by the complex embryological development of the inner ear.

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