Unveiling a rare cause of congenital bilateral sensorineural hearing loss

Congenital anomalies of the cochlea are important cause of congenital sensorineural hearing loss in children. Cochlear anomalies are also known to be associated with vestibulocochlear nerve aplasia or hypoplasia. Such children are ideal candidates for cochlear implan¬tation surgery. We report here a rare case of a 5year old female child who presented with bilateral hearing loss and was planned for cochlear implantation. She was referred for pre-operative imaging evaluation of temporal bone. HRCT temporal bone and MRI inner ear were performed, which revealed in complete partition type I with cochlear nerve aplasia on the right side and common cavity cochlear malformation with normal vestibulocochlear nerve on the left side. It is vital that cochlear and associated nerve anomalies must be recognized prior to surgery as this would predict the feasibility and outcome of the cochlear implantation.