Jervell and Lange-Nielsen Syndrome

Jervell and Lange Nielsen syndrome (JLNS) is a rare autosomal recessive disorder characterized by bilateral sensorineural hearing loss and a prolonged QTc interval (usually more than 500 msec), which can lead to Torsades de pointes and sudden cardiac death. It is a form of an inherited long QT syndrome (LQTS). The disease was first described in 1957 by Anton Jervell and Fred Lange-Nielsen in a study of 4 children born with congenital deafness that all suffered from syncope. There was a marked prolongation of the QT interval on electrocardiographic studies with no other identifiable cause for the patient’s fainting spells.