Familial sensorineural hearing loss: a correlative study of audiologic, radiographic, and vestibular findings

A multidisciplinary approach to the study of a family with autosomal dominant sensorineural hearing loss is presented. The affected family members underwent extensive clinical and laboratory evaluation. They were found to have various degrees of bilateral congenital inner ear anomalies as imaged by computed tomography. The degree of structural abnormalities in the inner ears correlated with the severity of hearing impairment. Vestibular testing revealed nonspecific abnormalities generally correlating with audiologic and radiographic findings. This report presents a unique form of sensorineural hearing loss not previously described and is the first in-depth clinical study of nonsyndromal "Mondini dysplasia" occurring in a family.