Jervell and Lange-Nielsen syndrome (MONDO_0002441) is an autosomal recessive inherited syndrome caused by mutations in the KCNE1 and KCNQ1 genes. It is characterized by congenital hearing loss and arrhythmia. It is a form of long QT syndrome. Also known as: Jervell Lange-Nielsen syndrome, Jervell and Lange Nielsen syndrome, Jervell and Lange-Nielson syndrome, Jervell-Lange Nielsen syndrome, long QT interval-deafness syndrome.