This case report illustrates the clinical presentation of Jervell and Lange-Nielsen syndrome in a pediatric patient with deafness, syncope, and prolonged Q-Tc interval.
Prompts ECG evaluation in deaf children with syncope; leaves open optimal therapies for Jervell and Lange-Nielsen syndrome.
An 8-year-old deaf-mute girl experienced syncopal attacks. Past medical history revealed cardiac arrhythmia which had developed following an abdominal surgery for a twisted ovarian cyst. Electrocardiogram showed a Q-Tc interval of 40/100 second. The diagnosis was Jervell and Lange-Nielsen syndrome. Etiology, physiopathology, clinical picture, and management of the disease are discussed.
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Mosavy et al. (1976) studied this question.
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