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Rare auditory-electophysiology finding in Wilson's disease

Hardin, M.F.; Barker, M.; Neis, P.

Journal of the Arkansas Medical Society 101(9): 281-283

2005

ISSN/ISBN: 0004-1858
PMID: 15787078
Document Number: 594498
Wilson's disease is a rare genetic disease involving the malabsorption of copper by the body. The most common characteristic sign is the presence of Kayser-Fleischner ring surrounding the cornea. Other systemic and motor signs have been documented as well as MRI changes within the brain and brainstem. This rare case illustrates the potential importance of audiometric assessment for patients with Wilson's disease who complain of hearing loss, tinnitus and intra-aural pressure. Unilateral findings were significant for retrocochlear neural transmission delays.

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