Jervell and Lange-Nielsen syndrome

Jervell and Lange-Nielsen syndrome is a condition that causes profound hearing loss from birth and a disruption of the heart's normal rhythm (arrhythmia). This disorder is a form of long QT syndrome, which is a heart condition that causes the heart (cardiac) muscle to take longer than usual to recharge between beats. Beginning in early childhood, the irregular heartbeats increase the risk of fainting (syncope) and sudden death.

Other names

Autosomal recessive long QT syndrome (LQTS); Cardio-auditory-syncope syndrome; Cardioauditory syndrome of Jervell and Lange-Nielsen; Deafness, congenital, and functional heart disease; Jervell-Lange Nielsen syndrome; JLNS; Prolonged QT interval in EKG and sudden death; Surdo-cardiac syndrome

Inheritance pattern

Autosomal recessive

Related genes

KCNQ1; KCNE1

Source: MedlinePlus Genetics, U.S. National Library of Medicine.