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Jervell and Lange-Nielsen syndrome

Disease definition

Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive variant of familial long QT syndrome (see this term) characterized by congenital profound bilateral sensorineural hearing loss, a long QT interval on electrocardiogram and ventricular tachyarrhythmias.


Classification level: Disorder
  • Synonym(s):
    • Long QT interval-deafness syndrome
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: I45.8
  • OMIM: 220400  612347
  • UMLS: C0022387
  • MeSH: D029593
  • GARD: 3048
  • MedDRA: 10057936

Detailed information


Additional information

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