Jervell and Lange-Nielsen Syndrome

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Silvia Borkoski B.
Daniel Pérez P.
Juan Carlos Falcón G.
Ángel Ramos M.

Keywords

Long QT syndrome, Jervell and Lange-Nielsen Syndrome

Abstract

The Jervell and Lange-Nielsen (JLNS) is an uncommon form of long QT syndrome. His inheritance is autosomal recessive and manifests as a sensorineural deafness. We review the case of a 7 year old girl bilateral cochlear implanted. After a syncope episode, a long QT syndrome was confirmed by genetic study . We recommend electrocardiogram (ECG) to all children with severe hearing loss in order to rule out this syndrome.

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