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Original Article

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

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Pages 293-298 | Published online: 07 Jul 2009
 

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.

Résumé

Chez 3 sujets souffrant de maladie de Norrie nous avons pratiqué un examen électro-cochléographique et recherché également les potentiels rapides évoqués au niveau du tronc cérébral. Nos résultats montrent qu'il existe dans cette maladie une atteinte cochléaire de l'audition sans pathologie rétro-labyrinthique. Il s'agit d'une affection héréditaire de type récessif liée au chromosome X ne touchant que les hommes.

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