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March 1992

Upper Airway Function in Ondine's Curse

Author Affiliations

From the Department of Otolaryngology–Head and Neck Surgery, University of Michigan, Ann Arbor (Dr Olson); Division of Otolaryngology–Head and Neck Surgery (Dr Woodson) and Department of Pediatrics (Dr Heldt), University of California at San Diego; and the Veterans Affairs Medical Center, Head and Neck Surgery (Dr Woodson), San Diego, Calif.

Arch Otolaryngol Head Neck Surg. 1992;118(3):310-312. doi:10.1001/archotol.1992.01880030098020

• Congenital central alveolar hypoventilation (Ondine's curse) is a rare disorder with absence of automatic control of ventilation but preservation of voluntary breathing. Phrenic pacing, used to treat this condition, is usually not successful without a tracheotomy. We performed fiberoptic videoendoscopy on an affected infant airway. During wakefulness and ventilation by a negative pressure ventilator, the airway was normal, but with sleep onset, passive inspiration produced phasic epiglottic collapse. This case demonstrates the importance of central control in maintaining upper airway patency.

(Arch Otolaryngol Head Neck Surg. 1992;118:310-312)