KD is an acute, self-limited vasculitis of the pediatric age group that must be diagnosed and treated in a timely manner. This presents a unique dilemma for the clinician: the disease may be difficult to recognize, there is no diagnostic laboratory test, there is an extremely effective therapy, and there is a 25% chance of serious cardiovascular damage if the therapy is not administered early in the course of the disease. Children with KD may be referred to the otolaryngologist for a variety of signs and symptoms including cervical adenitis, hoarseness, airway obstruction, torticollis, and retropharyngeal soft-tissue swelling accompanied by fever and failure to respond to appropriate antibiotic therapy. Clinical signs of KD should be sought through a careful history and physical examination in these patients, and cardiac echocardiography should be considered as an adjunct to establishing the diagnosis. Sensorineural hearing loss is a rare but potentially devastating sequela of the acute vasculitis of KD. A history of an antecedent illness compatible with KD should be sought in all pediatric patients evaluated for idiopathic sensorineural hearing loss.

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