University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital Birmingham, Bigmingham, UK.
Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK.
J Int Adv Otol. 2020 Dec;16(3):456-462. doi: 10.5152/iao.2020.9025.
Establish outcomes following cochlear implantation (CI) in patients with Jervell and Lange-Nielsen Syndrome (JLNS). Methods Systematic review and narrative synthesis. Databases searched on Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 63 abstracts and 19 full texts. Of these, 9 studies met inclusion criteria reporting outcomes in 66 patients with at least 72 implants. Hearing outcomes were generally good. Mortality secondary to cardiac complications within the follow up period occurred in at least five cases (7.6%), though three of these were thought to be unrelated to surgery. Potentially dangerous arrhythmias without associated morbidity were also noted in at least five patients. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade IV. One study contributed 41/66 patients (62%). Hearing outcomes following CI in JLNS are generally good with the majority of patients experiencing useful hearing improvement. Significant peri-operative cardiac risks exist and should be discussed with the patient and family during pre-operative counselling and prompt thorough investigation, pre-operative optimisation and peri-operative monitoring.
研究 Jervell 和 Lange-Nielsen 综合征 (JLNS) 患者耳蜗植入 (CI) 后的结局。方法 系统评价和叙述性综合。在 Medline、Pubmed、Embase、Web of Science、Cochrane Collection 和 ClinicalTrials.gov 数据库中进行检索。未对语言或出版年份设置限制。按照 PRISMA 声明进行审查。检索共确定了 63 篇摘要和 19 篇全文。其中，9 项研究符合纳入标准，报告了至少 72 例植入物的 66 例患者的结局。听力结果通常较好。在随访期间，至少有 5 例（7.6%）发生了心脏并发症导致的死亡，但其中 3 例被认为与手术无关。至少有 5 例患者也出现了无相关发病率的潜在危险心律失常。纳入研究的方法学质量中等，主要由病例报告和非对照病例系列组成，患者人数较少。所有研究均为 OCEBM 四级。一项研究贡献了 41/66 例患者（62%）。JLNS 患者 CI 后的听力结果通常较好，大多数患者听力改善明显。围手术期存在显著的心脏风险，应在术前咨询期间与患者和家属讨论，并进行及时彻底的检查、术前优化和围手术期监测。
