Related Experiment Videos
Brain-stem auditory response in Ondine's syndrome
Insights
Infants with Ondine's syndrome show brain-stem auditory evoked response abnormalities during sleep. These findings suggest impaired auditory pathways near respiratory control centers in the brain stem.
Area of Science:
- Neuroscience
- Pediatrics
- Sleep Medicine
Background:
- Congenital central alveolar hypoventilation syndrome (Ondine's syndrome) affects respiratory control during sleep.
- Brain-stem function is critical for regulating breathing and processing auditory information.
Purpose of the Study:
- To investigate brain-stem auditory evoked responses (BAERs) in infants with Ondine's syndrome during sleep.
- To identify potential electrophysiological markers of brain-stem dysfunction in this condition.
Main Methods:
- BAERs were recorded during sleep in four infants diagnosed with Ondine's syndrome.
- A control group of four age- and sex-matched healthy infants was included for comparison.
- Analysis focused on peak latencies (p III) and interpeak latencies (p I-III).
Main Results:
- Infants with Ondine's syndrome exhibited consistent delays in p III and p I-III latencies.
- These auditory pathway abnormalities were not observed in the control group.
- The findings were reproducible across the patient cohort.
Conclusions:
- Abnormalities in BAERs suggest disruption of auditory pathways in the mid to upper brain stem in infants with Ondine's syndrome.
- These electrophysiological findings correlate with impaired brain-stem control of ventilation during sleep.
- The study implies a functional disturbance in brain-stem regulatory mechanisms 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.