Article
A dominantly inherited progressive deafness affecting distal auditory nerve and hair cells.
Journal of the Association for Research in Otolaryngology : JARO - 1 Dec 2004
Starr Arnold, Isaacson Brandon, Michalewski Henry J, Zeng Fan-Gang, Kong Ying-Yee, Beale Paula, Paulson George W, Keats Bronya J B, Lesperance Marci M
Abstract excerpt
We have studied 72 members belonging to a large kindred with a hearing disorder inherited in an autosomal dominant pattern. We used audiological, physiological, and psychoacoustic measures to characterize the hearing disorders. The initial phenotypic features of the hearing loss are of an auditory neuropathy (AN) with abnormal auditory nerve and brainstem responses (ABRs) and normal outer hair cell functions...
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