Jervell and Lange-Nielsen syndrome (JLNS) is a rare inherited disorder characterised by congenital deafness and arrhythmia (disruption of the heart's normal rhythm). This condition is a form of long QT syndrome, which is a heart condition that causes the cardiac muscle to take longer than usual to recharge between beats. Since early childhood, these irregular heartbeats increase the risk of syncope and sudden death; however, some individuals remain asymptomatic. Physical activity, excitement or stress may trigger the onset of the symptoms. This disorder affects about 1.6 to 6 per 1 million people worldwide, but in Denmark and Norway, it has a higher prevalence where it affects at least 1 in 200,000 people. JLNS is genetically heterogeneous. JLNS1 is caused by homozygous or compound heterozygous mutations in the KCNQ1 gene and JLNS2 is associated with mutations in KCNE1 gene.