Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome, and a review of literature

被引：15

作者：

Feng Yanmei ^{[1
]}

Wu Yaqin ^{[1
]}

Shi Haibo ^{[1
]}

Zhou Huiqun ^{[1
]}

Chen Zhengnong ^{[1
]}

Yu Dongzhen ^{[1
]}

Yin Shankai ^{[1
]}

机构：

[1] Shanghai Jiao Tong Univ, Peoples Hosp 6, Inst Otolaryngol, Dept Otolaryngol,Sch Med, Shanghai 200233, Peoples R China

来源：

INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY | 2008年 / 72卷 / 11期

基金：

中国国家自然科学基金;

关键词：

Cochlear implant; Jervell and Lange-Nielsen syndrome;

D O I：

10.1016/j.ijporl.2008.07.013

中图分类号：

R76 [耳鼻咽喉科学];

学科分类号：

100213 ;

摘要：

Jervell and Lange-Nielsen syndrome is characterized by a long QT interval in electrocardiography, syncopal attacks and congenital sensorineural hearing loss. Upon diagnosis, beta-blockers is recommended as the first choice of medication, which is considered to reduce the occurrence rate of syncopal attack and sudden death during life. Cochlear implantation is indicated for hearing rehabilitation in this syndrome with profound deafness. The auditory and language outcome after cochlear implantation in this syndrome is not worse than those in patients with non-syndromic sensorineural deafness. The life quality in this syndrome could be improved greatly with cochlear implantation only if the cardiologic event has been controlled effectively. (C) 2008 Elsevier Ireland Ltd. All rights reserved.

引用

页码：1723 / 1729

页数：7

共 33 条

[1] A novel mutation in KVLQT1 is the molecular basis of inherited long QT syndrome in a near-drowning patient's family [J].