Jervell and Lange-Nielsen Syndrome (JLNS) is a genetic disorder characterized by QT interval prolongation on the surface ECG and associated torsades de pointes arrhythmias, congenital bilateral sensory neural deafness, and predisposition to sudden cardiac death early in life (Schwartz et al. 2006). The JLNS is the most severe form of congenital long QT syndrome (LQTS), being autosomal recessive (vs the majority of LQTS being autosomal dominant). The disease was first described by Anton Jervell and Fred Lange-Nielsen in 1957 in a study of four children born with congenital deafness who all experienced recurrent syncope during exercise or emotion, and three of them suffered sudden death at ages 4, 5, and 9 years (Jervell and Lange-Nielsen 1957). Electrocardiographic studies in three of these cases revealed a marked prolongation of the QT interval with no other identifiable heart disease (Jervell and Lange-Nielsen 1957).