Jervell and Lange-Nielsen syndrome
ORPHA:90647· ICD-10 I45.8
Definition
A rare severe familial long QT syndrome characterized by congenital profound bilateral sensorineural hearing loss, a long QT interval on electrocardiogram and life-threatening ventricular tachyarrhythmias.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Autosomal recessive
- Age of onset
- Neonatal