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    Jervell and Lange-Nielsen Syndrome

    Important
    It is possible that the main title of the report Jervell and Lange-Nielsen Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • autosomal recessive long QT syndrome (LQTS)
    • Jervell and Lange-Nielsen (JLNS)
    • cardioauditory syndrome
    • cardioauditory syndrome of Jervell and Lange-Nielsen
    • deafness, congenital, and functional heart disease
    • surdocardiac syndrome

    Disorder Subdivisions

    • None

    General Discussion

    Jervell and Lange-Nielsen syndrome (JLNS) is a rare inherited disorder characterized by deafness present at birth (congenital) occurring in association with abnormalities affecting the electrical system of the heart. The severity of cardiac symptoms associated with JLNS varies from case to case. Some individuals may have no apparent symptoms (asymptomatic); others may develop abnormally increased heartbeats (tachyarrhythmias) resulting in episodes of unconsciousness (syncope), cardiac arrest, and potentially sudden death. Physical activity, excitement, fright, or stress may trigger the onset of these symptoms. Fainting during the aforementioned activities is also a classic sign of JLNS. JLNS is usually detected during early childhood and is inherited as an autosomal recessive genetic disorder. More than half of the untreated cases of JLNS result in death before the age of 15.

    Anton Jervell and Fred Lange-Nielsen provided the first complete description of the electrical conduction problem in the heart called long QT syndrome (LQTS) in 1957. LQTS refers to the QT-interval measured on the electrocardiogram that indicates that the heart muscle is taking longer than usual to recharge between beats. It predisposes those affected to tachyarrhythmias called torsade de pointes (TdP) which leads to syncope and may cause sudden cardiac death.

    Resources

    International Long QT Syndrome Registry
    Heart Research Follow-up Program
    Box 653
    University of Rochester Medical Center
    Rochester, NY 14642-8653
    Tel: (585)276-0016
    Fax: (585)273-5283
    Email: heartajm@heart.rochester.edu

    Hearing Health Foundation
    363 Seventh Avenue, 10th Floor
    New York, NY 10016-3904
    United States
    Tel: (212)257-6140
    Fax: (212)257-6139
    Tel: (866)454-3924
    TDD: (888)435-6104
    Email: info@hearinghealthfoundation.org
    Internet: http://hearinghealthfoundation.org/home

    American Heart Association
    7272 Greenville Avenue
    Dallas, TX 75231
    Tel: (214)784-7212
    Fax: (214)784-1307
    Tel: (800)242-8721
    Email: Review.personal.info@heart.org
    Internet: http://www.heart.org

    Sudden Arrhythmia Death Syndromes Foundation
    508 E. South Temple
    Suite 202
    Salt Lake City, UT 84102
    USA
    Tel: (801)531-0937
    Fax: (801)531-0945
    Tel: (800)786-7723
    Email: laura@sads.org
    Internet: http://www.sads.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    Cardiac Arrhythmias Research and Education Foundation, Inc. (C.A.R.E)
    427 Fulton Street
    P.O. Box 69
    Seymour, WI 54165
    USA
    Fax: (920)833-7005
    Tel: (800)404-9500
    Email: care@careforhearts.org
    Internet: http://www.longqt.org or http://www.careforhearts.org

    QTsyndrome.ch
    Ronnerweg 2
    Nidau, 2560
    Switzerland
    Tel: 0041794741535
    Email: info@qtsyndrome.ch
    Internet: http://www.qtsyndrome.ch/index.html

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Madisons Foundation
    PO Box 241956
    Los Angeles, CA 90024
    Tel: (310)264-0826
    Fax: (310)264-4766
    Email: getinfo@madisonsfoundation.org
    Internet: http://www.madisonsfoundation.org

    American Academy of Audiology
    11730 Plaza America Drive, Suite 300
    Reston, VA 20190
    Tel: (703)790-8466
    Fax: (703)790-8631
    Tel: (800)222-2336
    Email: infoaud@audiology.org
    Internet: http://www.audiology.org

    Fetal Hope Foundation
    9786 South Holland Street
    Littleton, CO 80127
    USA
    Tel: (303)932-0553
    Tel: (877)789-4673
    Email: info@fetalhope.org
    Internet: http://www.fetalhope.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 1/16/2013
    Copyright 1991, 1996, 1997, 2003, 2013 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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