Brainstem auditory evoked responses in hereditary motor‐sensory neuropathy

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Abstract
In three patients with hereditary motor-sensory neuropathy (HMSN), type I, brainstem auditory evoked responses (BAERs) showed prolongation of the latency of all major components. Interleaf latency I-III was prolonged, but the III-V interval was normal, and separation of waves I and II accounted for the prolonged I-III interval. Prolongation of I-II interval in this condition, which affects the peripheral nerves, preservation of wave II after cerebral death, and preservation of only waves I and II in the leukodystrophies suggest that wave II may be generated in the intracranial extramedullary portion of the eighth nerve.

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