Abnormal auditory evoked potentials in hereditary motor-sensory neuropathy.

From a kinship with a dominantly inherited motor-sensory neuropathy, we studied 2 brothers with brainstem auditory evoked potentials and behavioral audiometric tests. They had abnormal prolongation of I-III interpeak intervals. Wave V was poorly developed. Conventional audiometric tests did not reveal a peripheral hearing loss. It is probable that their auditory nerves and spiral ganglia are undergoing a pathophysiological process analogous to that of their peripheral nerves.