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Article
September 1983

Auditory Brain-Stem Responses in Adrenomyeloneuropathy

Author Affiliations

From the National Institutes of Health (Mss Grimes and Pikus), the National Heart, Lung, and Blood Institute (Dr Elks), and the National Institute of Arthritis, Diabetes, and Digestive and Kidney Diseases (Dr Grunberger), Bethesda, Md.

Arch Neurol. 1983;40(9):574-576. doi:10.1001/archneur.1983.04050080074015
Abstract

• We studied three patients with adrenomyeloneuropathy. Complete audiologic assessment was obtained: two patients showed unimpaired peripheral hearing and one showed a mild high-frequency hearing loss. Auditory brain-stem responses were abnormal in both ears of all subjects, with one subject showing no response above wave I, and the other two having significant wave I to III and wave III to V interval prolongations. We concluded that auditory brain-stem response testing provides a simple, valid, reliable method for demonstrating neurologic abnormality in adrenomyeloneuropathy even prior to evidence of clinical signs.

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