Diseases

Jervell-Lange Nielsen Syndrome

A form of long QT syndrome that is associated with congenital deafness. It is characterized by abnormal cardioelectrophysiology involving the VOLTAGE-GATED POTASSIUM CHANNEL. It results from mutation of KCNQ1 gene (Subtype 1 or JLN1) or the KCNE1 gene (Subtype 2 or JLN2).

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A form of long QT syndrome that is associated with congenital deafness. It is characterized by abnormal cardioelectrophysiology involving the VOLTAGE-GATED POTASSIUM CHANNEL. It results from mutation of KCNQ1 gene (Subtype 1 or JLN1) or the KCNE1 gene (Subtype 2 or JLN2).

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Synonyms

19 entry terms
  • Cardio-Auditory-Syncope Syndrome
  • Cardioauditory Syndrome of Jervell and Lange-Nielsen
  • Deafness, Congenital, and Functional Heart Disease
  • Jervell and Lange-Nielsen Syndrome
  • Prolonged QT Interval in EKG and Sudden Death
  • Surdo-Cardiac Syndrome
  • Cardio Auditory Syncope Syndrome
  • Cardio-Auditory-Syncope Syndromes
  • Cardioauditory Syndrome of Jervell and Lange Nielsen
  • Jervell Lange Nielsen Syndrome
  • Jervell and Lange Nielsen Syndrome
  • Surdo Cardiac Syndrome
  • Surdo-Cardiac Syndromes
  • Syndrome, Cardio-Auditory-Syncope
  • Syndrome, Jervell-Lange Nielsen
  • Syndrome, Surdo-Cardiac
  • Syndromes, Cardio-Auditory-Syncope
  • Jervell And Lange-Nielsen Syndrome 1
  • Jervell And Lange Nielsen Syndrome 1

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Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2002; use LONG QT SYNDROME 1986-2001

MeSH Record

Previous Indexing

  • Deafness (1969-1985)
  • Long QT Syndrome (1986-2001)

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AMA Style

References

  1. National Library of Medicine. Jervell-Lange Nielsen Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D029593. http://id.nlm.nih.gov/mesh/2026/D029593
  2. Jervell-Lange Nielsen Syndrome. In: Wikipedia. https://en.wikipedia.org/wiki/Jervell_and_Lange-Nielsen_syndrome
  3. Jervell-Lange Nielsen Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q3304152