Around 20% of congenital sensorineural hearing loss have detectable imaging findings¹.

This case illustrates an isolated, non syndromic unilateral congenital cochlear nerve and cochlear aperture aplasia, in the presence of a normal cochlea. Images acquired on 1.5 T machine, distinction between hypoplastic and aplastic nerve may be difficult, however there seems to be no cochlear aperture. The lack of cochlear aperture in also in favor of congenital and not acquired hearing loss, as normal size IAC may raise this issue ².

Sagittal-oblique images should be systematically reviewed in cases of congenital SNHL even when there is no abnormal IAC diameter, nor structural cochlear/vestibular anomalies.

Possible treatments include rerouting auditory stimuli to contralateral healthy ear. Cochlear implants are rarely successful ³.