Jervell and Lange-Nielsen syndrome
Jervell and Lange-Nielsen syndrome (JLNS) is a rare type of long QT syndrome associated with severe, bilateral sensorineural hearing loss. Those with JLNS are at risk of abnormal heart rhythms called arrhythmias, which can lead to fainting, seizures, or sudden death. JLNS, like other forms of long QT syndrome, causes the cardiac muscle to take longer than usual to recharge between beats. It is caused by genetic variants responsible for producing ion channels that carry transport potassium out of cells. The condition is usually diagnosed using an electrocardiogram, but genetic testing can also be used. Treatment includes lifestyle measures, beta blockers, and implantation of a defibrillator in some cases. It was first described by Anton Jervell and Fred Lange-Nielsen in 1957.
| Jervell and Lange-Nielsen syndrome | |
|---|---|
| Other names | Long QT interval-deafness syndrome |
| The features of Jervell and Lange-Nielsen syndrome include a prolonged QT interval and sensorineural deafness | |
| Specialty | Cardiology |
| Symptoms | Blackouts, seizures, sensorineural deafness |
| Complications | Sudden death |
| Usual onset | Congenital |
| Causes | Genetic |
| Differential diagnosis | Other forms of Long QT syndrome |
| Treatment | Beta blockers, implantable cardioverter defibrillator |