• 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.
Grimes AM, Elks ML, Grunberger G, Pikus AM. Auditory Brain-Stem Responses in Adrenomyeloneuropathy. Arch Neurol. 1983;40(9):574–576. doi:10.1001/archneur.1983.04050080074015
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