Congenital Anomaly of Cochlea: A Case Report

Abstract

Congenital malformations of inner ear are rare anomalies. These occur due to arrested development at different stages of embryogenesis. One rare anomaly of inner ear is Mondini dysplasia. Possibly it occurs due to arrested development of cochlea in its embryonic stage at about sixth week of gestation. In this anomaly only basal turn of cochlea is developed. There is deficient interscalar septum for distal one & half turn. Thus bony cochlea is restricted to 1.5 turns only. This causes sensorineural hearing loss. One such anomaly was recognized clinically in a 4yrs old girl who presented with congenital deafness. Thorough systemic & specific examination was done.Radiological examination (Computed Tomography) revealed, that there was defect in the turn of cochlea i.e. cochlea was only one half turn.