Audiometric Profile in an Indian Family with Alport's Syndrome

Abstract

An audiometric profile of some of the members of an Indian family with Alport's syndrome has been presented. The findings of audiometric and vestibular testing suggest an end-organ lesion. It has been suggested that an ingerent weakness of ectodermal derivatives in this syndrome, which is mediated through an inborn error of metabolism, accounts for the end-organ hearing loss, cataract and kidney involvement in such cases.

Key Words:

• Auditory genetics
• Sensorineural hearing loss
• Caloric test