Congenital Inner Ear Malformations

Hearing impairment is defined as a person's hearing threshold being above the normal range of - 10 to 15 decibels. It has significant impact on a child's language, cognitive, socioemotional and behavioural development. Therefore early recognition of hearing impairment is of utmost importance. Congenital inner ear malformation is an important cause of sensorineural hearing loss in children. It consists of labyrinthine aplasia, cochlear aplasia, common cavity deformity, cystic cochleovestibular anomaly (incomplete partition type I), cochlear hypoplasia, Modini deformity (incomplete partition type II). The imaging manifestations of these conditions on computed tomography examination are discussed in this article. Semicircular canal malformation and internal auditory canal deformities, namely: atresia of the internal auditory canal and X-linked progressive hearing loss, are also illustrated.