Synonyms
Cardioauditory Syndrome of Jervell and Lange-Nielsen
Cardioauditory Syndrome
Deafness, Congenital, and Functional Heart Disease
Surdocardiac Syndrome
Autosomal Recessive Long QT Syndrome
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Disorder Subdivisions
None

General Discussion
Jervell and Lange-Nielsen syndrome is a rare inherited disorder characterized by deafness present at birth (congenital) occurring in association with abnormalities affecting the electrical system of the heart. The severity of cardiac symptoms associated with Jervell and Lange-Nielsen syndrome varies from case to case. Some individuals may have no apparent symptoms (asymptomatic); others may develop abnormally increased heartbeats (tachyarrhythmias) resulting in episodes of unconsciousness (syncope), cardiac arrest, and potentially sudden death. Physical activity, excitement or stress may trigger the onset of these symptoms. Jervell and Lange-Nielsen syndrome is usually detected during early childhood and is inherited as an autosomal recessive trait.