Important

It is possible that the main title of the report Jervell and Lange-Nielsen Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• Cardioauditory Syndrome of Jervell and Lange-Nielsen
• Cardioauditory Syndrome
• Deafness, Congenital, and Functional Heart Disease
• Surdocardiac Syndrome
• Autosomal Recessive Long QT Syndrome

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.