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.
D000293:Adolescent; D001299:Audiometry; D001307:Auditory Perception / Q000502:physiology*; D001933:Brain Stem / Q000503:physiopathology*; D003056:Cochlear Nerve / Q000503:physiopathology; D005071:Evoked Potentials; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000235:genetics*; D011930:Reaction Time; D000159:Vestibulocochlear Nerve / Q000503:physiopathology*