Q: What is Jervell and Lange-Nielsen syndrome?

Answer: The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive form of Long QT Syndrome (LQTS) with associated congenital deafness.

Clinical Significance: if undiagnosed or untreated, about 50 percent die by the age of 15 years due to ventricular arrhythmias.