Jervell and Lange-Nielsen Syndrome (JLNS) is a rare genetic disorder characterized by congenital, bilateral, sensorineural hearing loss and a prolonged QT interval on an electrocardiogram (ECG). It is caused by mutations in the KCNQ1 and KCNE1 genes, which are responsible for the production of proteins that form the potassium channels in the heart. People with JLNS are at an increased risk of developing life-threatening arrhythmias, such as ventricular fibrillation and torsades de pointes. Treatment typically involves the use of medications to control the heart rate and rhythm, as well as hearing aids to improve hearing.

The most common symptoms of Jervell and Lange-Nielsen Syndrome (JLNS) include profound hearing loss, fainting spells (syncope), and an irregular heartbeat (arrhythmia). Other symptoms may include dizziness, fatigue, chest pain, shortness of breath, and palpitations. In some cases, people with JLNS may also have a heart murmur, an enlarged heart, or an abnormally slow heart rate.

Jervell and Lange-Nielsen Syndrome (JLNS) is a rare genetic disorder caused by a mutation in the KCNQ1 gene. This gene is responsible for producing a protein that helps control the electrical activity of the heart. When this gene is mutated, it can cause a prolonged QT interval on an electrocardiogram (ECG), which can lead to a higher risk of sudden cardiac death. Other symptoms of JLNS include hearing loss, fainting, and seizures.

The primary treatment for Jervell and Lange-Nielsen Syndrome is to manage the symptoms associated with the condition. This may include medications to control heart rate, medications to control blood pressure, and lifestyle modifications such as avoiding strenuous activity and avoiding triggers that can cause episodes of fainting. In some cases, a pacemaker may be recommended to help regulate the heart rate. Surgery may also be recommended to correct any structural abnormalities in the heart. Genetic counseling may also be recommended to help families understand the condition and the risks associated with it.

The primary risk factor for Jervell and Lange-Nielsen Syndrome is a family history of the disorder. Other risk factors include having a parent who is a carrier of the genetic mutation that causes the disorder, being of Northern European descent, and having a family history of long QT syndrome.

There is no cure for Jervell and Lange-Nielsen Syndrome, but medications can be used to help manage the symptoms. These medications include beta-blockers to help control the heart rate, ACE inhibitors to help reduce blood pressure, and anti-arrhythmic drugs to help control abnormal heart rhythms. In some cases, a pacemaker may be necessary to help regulate the heart rate.