Kaneshiro Shinsuke, Hiraumi Harukazu, Shimamoto Kirito, Sasamori Kaori, Kobayashi Yumiko, Sato Hiroaki
Department of Otolaryngology-Head & Neck Surgery, Iwate Medical University, 19-1, Uchimaru, Morioka, Iwate 020-8505, Japan.
Department of Otolaryngology-Head & Neck Surgery, Iwate Medical University, 19-1, Uchimaru, Morioka, Iwate 020-8505, Japan.
Auris Nasus Larynx. 2018 Aug;45(4):890-893. doi: 10.1016/j.anl.2017.11.017. Epub 2018 Mar 24.
Jervell and Lange-Nielsen syndrome (JLNS), a rare autosomal recessive congenital QT prolongation syndrome, is characterized by cardiac arrhythmias, syncopal episodes, and profound deafness. A cochlear implant (CI) for patients with JLNS is expected to result in hearing improvement. Sometimes, defibrillation is required if a patient experiences lethal arrhythmia. In this paper, we report a pediatric patient with JLNS who received defibrillation after CI surgery in his right ear at the age of 2 years. With intensive care, the post-operative course was uneventful, and the patient acquired satisfactory speech and hearing abilities. Five years after the surgery, he underwent defibrillation because of the incidence of syncopal attack. Thereafter, arrhythmic syncope recurred three times, which necessitated defibrillation therapy. To prevent recurrence of cardiac arrhythmia, he underwent ICD (implantable cardioverter-defibrillator) implantation at the age of 11 years. At present, CI works well and provides good hearing, while syncopal attack is prevented by ICD. From the experience of this case, electronic circuit of CI is thought to tolerate emergency countershock if the speech processor is removed.
杰韦尔和朗格-尼尔森综合征（JLNS）是一种罕见的常染色体隐性先天性QT间期延长综合征，其特征为心律失常、晕厥发作和重度耳聋。对于JLNS患者，人工耳蜗植入（CI）有望改善听力。有时，如果患者发生致命性心律失常，则需要进行除颤。在本文中，我们报告了一名JLNS儿科患者，该患者在2岁时接受了右耳CI手术后接受了除颤治疗。经过重症监护，术后过程顺利，患者获得了令人满意的言语和听力能力。手术后五年，他因晕厥发作而接受了除颤治疗。此后，心律失常性晕厥复发了三次，这需要进行除颤治疗。为防止心律失常复发，他在11岁时接受了植入式心律转复除颤器（ICD）植入。目前，CI运行良好并提供良好的听力，同时ICD可预防晕厥发作。从这个病例的经验来看，如果移除言语处理器，CI的电子电路被认为能够耐受紧急电击。
