Electrophysiological study of Norrie's disease. An X-linked recessive trait with hearing loss

3 patients with Norrie's diseas were examined with electrocochleography and brain-stem-evoked responses. The examination revealed a hearing loss of pure cochlear origin with no involvement of the brain stem. The disease is inherited as an X-linked recessive trait only affecting males with unaffected female carriers. The results are thus in agreement with previous investigations of the topical lesion in genetic hearing loss.