open access

Vol 23, No 1 (2016)
Original articles
Published online: 2015-09-23
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QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study

Agnieszka Sanecka, Elżbieta Katarzyna Biernacka, Małgorzata Szperl, Magdalena Sosna, Małgorzata Mueller-Malesińska, Urszula Kozicka, Rafał Baranowski, Agnieszka Kosiec, Hubert Łazarczyk, Henryk Skarżyński, Piotr Hoffman, Katarzyna Bieganowska, Ryszard Piotrowicz
DOI: 10.5603/CJ.a2015.0062
·
Pubmed: 26412604
·
Cardiol J 2016;23(1):34-41.

open access

Vol 23, No 1 (2016)
Original articles
Published online: 2015-09-23

Abstract

Background: The aim of the study was to determine, whether electrocardiogram (ECG) screening could reduce the risk of sudden cardiac death in patients with hearing loss through the early diagnosis of Jervell and Lange-Nielsen syndrome and the introduction of the therapy.

Methods: One thousand and eighty patients with hearing loss (aged 21.8 ± 19.9 years) underwent ECG. Additionally, all subjects were asked to complete a 3-question survey. Those who met, at least, one of the high-risk criteria underwent further cardiac assessment and genetic testing.

Results: QTc assessment was possible in 1,027 patients. Mean QTc measured 422.8 ± 23.7 ms in 313 women, 414.9 ± 27.7 ms in 273 men and 421.1 ± 21.5 ms in 441 children (individuals younger than 14 years). Abnormal QTc was found in 13 (4.1%) women, 20 (7.3%) men, and 72 (16.3%) children. In the studied group, no recessive mutation of KNCQ1 or KCNE1 was found. In 6 patients, other mutations were found: in KCNQ1 (n = 1), in KCNH2 (n = 3) and in SCN5A (n = 1), which were pathogenic for long-QT-syndromes (LQTS), and 2 mutations of unknown clinical significance in SCN5A. Overall, out of these 6 patients LQTS was diagnosed in 3 asymptomatic patients, but with abnormal QTc and in 2 patients with normal QTc, but who were previously treated for epilepsy.

Conclusions: Jervell and Lange-Nielsen syndrome is a very rare condition even in a population with hearing loss. In this population, the prevalence of prolonged QT interval is increased over the general population. Further investigations are necessary.  

Abstract

Background: The aim of the study was to determine, whether electrocardiogram (ECG) screening could reduce the risk of sudden cardiac death in patients with hearing loss through the early diagnosis of Jervell and Lange-Nielsen syndrome and the introduction of the therapy.

Methods: One thousand and eighty patients with hearing loss (aged 21.8 ± 19.9 years) underwent ECG. Additionally, all subjects were asked to complete a 3-question survey. Those who met, at least, one of the high-risk criteria underwent further cardiac assessment and genetic testing.

Results: QTc assessment was possible in 1,027 patients. Mean QTc measured 422.8 ± 23.7 ms in 313 women, 414.9 ± 27.7 ms in 273 men and 421.1 ± 21.5 ms in 441 children (individuals younger than 14 years). Abnormal QTc was found in 13 (4.1%) women, 20 (7.3%) men, and 72 (16.3%) children. In the studied group, no recessive mutation of KNCQ1 or KCNE1 was found. In 6 patients, other mutations were found: in KCNQ1 (n = 1), in KCNH2 (n = 3) and in SCN5A (n = 1), which were pathogenic for long-QT-syndromes (LQTS), and 2 mutations of unknown clinical significance in SCN5A. Overall, out of these 6 patients LQTS was diagnosed in 3 asymptomatic patients, but with abnormal QTc and in 2 patients with normal QTc, but who were previously treated for epilepsy.

Conclusions: Jervell and Lange-Nielsen syndrome is a very rare condition even in a population with hearing loss. In this population, the prevalence of prolonged QT interval is increased over the general population. Further investigations are necessary.  

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Keywords

long-QT syndrome, hearing loss, Jervell and Lange-Nielsen syndrome, electrocardiogram

About this article
Title

QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study

Journal

Cardiology Journal

Issue

Vol 23, No 1 (2016)

Pages

34-41

Published online

2015-09-23

DOI

10.5603/CJ.a2015.0062

Pubmed

26412604

Bibliographic record

Cardiol J 2016;23(1):34-41.

Keywords

long-QT syndrome
hearing loss
Jervell and Lange-Nielsen syndrome
electrocardiogram

Authors

Agnieszka Sanecka
Elżbieta Katarzyna Biernacka
Małgorzata Szperl
Magdalena Sosna
Małgorzata Mueller-Malesińska
Urszula Kozicka
Rafał Baranowski
Agnieszka Kosiec
Hubert Łazarczyk
Henryk Skarżyński
Piotr Hoffman
Katarzyna Bieganowska
Ryszard Piotrowicz

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