Yanmei Feng, Yaqin Wu, Haibo Shi, Huiqun Zhou, Zhengnong Chen, Dongzhen Yu, Shankai Yin
Department of Otolaryngology, The Sixth People's Hospital, Shanghai JiaoTong University, Institute of Otolaryngology, Medicine School of Shanghai JiaoTong University, Shanghai 200233, China.
Int J Pediatr Otorhinolaryngol. 2008 Nov;72(11):1723-9. doi: 10.1016/j.ijporl.2008.07.013. Epub 2008 Sep 20.
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.
杰韦尔和朗格-尼尔森综合征的特征是心电图显示QT间期延长、晕厥发作和先天性感音神经性听力损失。一经诊断，推荐使用β受体阻滞剂作为首选药物，其被认为可降低一生中晕厥发作和猝死的发生率。对于该综合征导致的重度耳聋，建议进行人工耳蜗植入以恢复听力。该综合征患者人工耳蜗植入后的听觉和语言效果并不比非综合征性感音神经性耳聋患者差。只有在心脏事件得到有效控制的情况下，人工耳蜗植入才能极大地改善该综合征患者的生活质量。
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