Cardiology · Cardiology / Cardiac electrophysiology
Jervell and Lange-Nielsen syndrome
also: cardioauditory syndrome; JLNS
Autosomal recessive long QT syndrome with congenital bilateral sensorineural deafness.
- Specialty
- Cardiology
- Subspecialty
- Cardiology / Cardiac electrophysiology
- Sex & age distribution
- Male: affected; Female: affected equally; Children: yes, deafness congenital and syncope in early childhood
- Gene(s) involved
- KCNE1, KCNQ1
- Alternative names & eponyms
- cardioauditory syndrome; JLNS
Etiology & mechanism
Biallelic loss of KCNQ1 or KCNE1 impairs the IKs current in the heart and the endolymph potassium secretion in the inner ear.
Gene(s) involved (2)
Symptoms & signs (6)
In plain terms, someone with Jervell and Lange-Nielsen syndrome may experience the following — click any to explore related syndromes:
Same gene(s)
Other syndromes that involve one of this syndrome's genes.
Overlapping symptoms
Other syndromes that share symptoms with this one.
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Shares symptoms with
4 shared
Long QT syndrome
prolonged qt interval, seizure-like episodes, syncope, torsades de pointes
2 shared
PRKAG2 syndrome
sudden death, syncope
1 shared
Anomalous left coronary artery from pulmonary artery
sudden death
1 shared
Brugada syndrome
syncope
1 shared
Early repolarization syndrome
syncope
1 shared
Eisenmenger syndrome
syncope
1 shared
Heyde syndrome
syncope
1 shared
Naxos disease
syncope
1 shared
Portopulmonary hypertension syndrome
syncope
1 shared
Postural orthostatic tachycardia syndrome
syncope
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