Hereditary Unilateral Deafness with Malformation of the Internal Ear

Abstract

Three cases in a family with hereditary unilateral deafness with malformations of the internal ear is reported. The proband is a 12-year-old girl, and her audiogram revealed unilateral deafness of the right ear. Her mother (38-year-old) has also deafness of the right ear. Caloric vestibular tests of the mother and daughter showed normal response. The patient's maternal grandfather has been deaf in the right ear since his youth. There was a consanguineous marriage, and the maternal grandfather was cousin of grandmother.
X-ray of the temporal bones of the mother and daughter showed stenosis of the internal auditory canal and aplasia of the cochlea.