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Brainstem auditory evoked responses in hereditary motor-sensory neuropathy

Site of origin of wave II

Department of Neurology, Indiana University School of Medicine, Indianapolis, IN.

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.

Address correspondence and reprint requests to Dr. Garg, Department of Neurology, Indiana University School of Medicine, 1100 W. Michigan, Indianapolis, IN 46223.

Presented in part at the annual meeting of the Central Association of Electroencephalographers, March 1980, Madison, WI.

Accepted for publication February 24, 1982.

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