Open AccessPrevalence of Jervell-Lange Nielsen syndrome in children with congenital bilateral sensorineural hearing loss
Author(s) -
Yakup Ergül,
Hasan Kafalı,
Erman Çilsal,
Bekir Yükçü,
Ibrahim Yaman,
Filiz Cetinkaya Isik,
Alper Güzeltaş,
Mehmet Ertürk
Publication year - 2021
Publication title -
türk kardiyoloji derneği arşivi
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.2
H-Index - 24
ISSN - 1016-5169
DOI - 10.5543/tkda.2021.44890
Subject(s) - medicine , qt interval , long qt syndrome , pediatrics , family history , hearing loss , sudden cardiac death , short qt syndrome , sibling , sensorineural hearing loss , channelopathy , cardiology , audiology , psychology , developmental psychology
Long QT syndrome (LQTS) is an inherited cardiac ion channel disorder (channelopathy) that is characterized by prolonged QT intervals on the electrocardiography (ECG) and possess the risk of sudden cardiac death (SCD). Jervell-Lange Nielsen syndrome (JLNS) is a specific subtype of LQTS that is accompanied by congenital sensorineural hearing loss, inherited autosomal recessively, and higher risk of SCD. In this study, we aimed to investigate JLNS prevalence in deaf children attending special schools for hearing loss, located in our province.