Auditory brainstem responses (ABR) in children with neurological disorders

Abstract

Auditory brainstem responses (ABR) were studied in 35 children with neurological disorders and 24 controls. Abnormal results were obtained in 16 patients. All 5 of the patients with metachromatic leukodystrophy had evidence of peripheral and/or central delay in transmission in keeping with evidence of demyelination of both peripheral (i.e. auditory nerve) and central (i.e. brainstem) pathways as occurs in this disorder. Two children with lead poisoning had delayed conduction in the peripheral pathways only and in these there was good correlation between the degree of delay and the ulnar nerve conduction velocity; both improved after chelation therapy. One infant with lead poisoning had central delay only. One infant with osteopetrosis manifested progressive damage to the auditory nerves. Delayed conduction was also found in one patient each with hydrocephalus, spinal muscular atrophy, and in 2 infants with cerebral palsy. No responses were obtained in one infant with congenital rubella, one deaf-mute and one child with an undiagnosed degenerative neurological disease. Auditory brainstem responses are of value in detecting disturbances of the auditory nerve or brainstem in children with various neurological disorders.