Electrophysiological study of norrie's disease: An X-Linked Recessive Trait with Hearing Loss

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