Electrophysiological study of Norrie's disease: an X-linked recessive trait with hearing loss

A Parving, C Elberling, M Warburg - Audiology, 1978 - Taylor & Francis
A Parving, C Elberling, M Warburg
Audiology, 1978Taylor & Francis
3 patients with Norrie's disease 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,
Abstract
3 patients with Norrie's disease 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,
Taylor & Francis Online