Cochlear Apalsia and Cochlear Hypoplasia Incidental CT Finding A Case Report

Abstract

The etiology of profound hearing loss in children is complex. Congenital inner ear malformation is an important cause of SNHL in children. It consists of labrynthine aplasia, cochlear aplasia, common cavity deformity, cystic cochleovestibular anomaly, cochlear hypoplasia and Modini's deformity. We report one patient who was born deaf and was being evaluated in our service for possible cochlear implantation. CT scan evaluation shows cochlear aplasia on one side and hypoplasia.

Cochlear anomaly with preserved hearing - A Clinical Paradox: Our Experience with Two cases

“…However, a normal waveform pattern on BERA merits some explanation. Mohammed AS et al in 2017 reported a case of a two-year old with incidentally detected cochlear hypoplasia on one side and cochlear nerve aplasia on the other (10). The authors in their description of cochlear hypoplasia mention that hearing in such ears is variable and maybe remarkably good.…”