Brain-stem auditory response in Ondine's syndrome.

Abstract

Brain-stem auditory evoked responses were measured during sleep in four infants with congenital central alveolar hypoventilation syndrome (Ondine's syndrome) and four controls matched for age and sex. Delays in peak latencies p III and interpeak latencies p I-III were consistently seen in these patients but not in the control children. These abnormalities were reproducible and suggested disruption in the normal auditory pathways at the level of the mid to upper brain stem through which fibers pass close to the area of respiratory control. These abnormalities, both electrophysiologic and metabolic, imply a functional disturbance of brain-stem control of ventilation during sleep in infants and children suffering from Ondine's syndrome.