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Electrocardiogram (EKG) Screening (Jervell and Lange-Nielsen Syndrome)

Routine electrocardiogram (EKG) screening of all infants with hearing loss is not recommended. However, some physicians routinely perform EKGs for infants with profound bilateral hearing loss to rule out Jervell and Lange-Nielsen syndrome – a syndromic hearing loss associated with prolonged QTc, which can result in sudden death. An EKG is indicated in individuals with hearing loss and syncope or a family history of prolonged QT interval, syncope, or sudden cardiac death. [Bitner-Glindzicz: 2000], [O'Neill: 2004], [Tranebjaerg: 2002]

Authors & Reviewers

Initial publication: April 2009; last update/revision: July 2013
Current Authors and Reviewers:
Author: Medical Home Team

Page Bibliography

Bitner-Glindzicz M, Tranebjaerg L.
The Jervell and Lange-Nielsen syndrome.
Adv Otorhinolaryngol. 2000;56:45-52. PubMed abstract

O'Neill MJF, Brennan P, McKusick VA.
Jervell and Lange-Nielsen Syndrome – OMIM.
McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University (Baltimore, MD) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, MD); (2004) http://omim.org/entry/220400. Accessed on 02/03/05.
From the Online Medelian Inheritance in Man collection; provides a detailed medical description of the syndrome, including clinical features, molecular genetics, and history.

Tranebjaerg L, Samson R, Green GE.
Jervell and Lange-Nielsen Syndrome.
Gene Reviews, University of Washington, National Library of Medicine; (2002) http://www.ncbi.nlm.nih.gov/books/NBK1405/. Accessed on 7/27/13.
Authoritative compilation of information addressing the diagnosis, clinical characteristics, and management of Jervell and Lange-Nielsen Syndrome.