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profound congenital deafness and a prolonged QTc interval (greater than 500 milliseconds) in the ECG waveforms. The prevalence of JLNS is about 1\/1000000 to 1\/200000 around the world. However, exceed 25% of JLNS patients suffered sudden cardiac death with kinds of triggers containing anesthesia. Approximately 90% of JLNS cases are caused by <jats:italic>KCNQ1<\/jats:italic> gene mutations. Here, using next-generation sequencing (NGS), we identified a compound heterozygosity for two mutations c.1741A&gt;T (novel) and c.477+5G&gt;A (known) in <jats:italic>KCNQ1<\/jats:italic> gene as the possible pathogenic cause of JLNS, which suggested a high risk of cardiac events in a deaf child. The hearing of this patient improved significantly with the help of cochlear implantation (CI). But life-threatening arrhythmias occurred with a trigger of anesthesia after the end of the CI surgery. Our findings extend the <jats:italic>KCNQ1<\/jats:italic> gene mutation spectrum and contribute to the management of deaf children diagnosed with JLNS for otolaryngologists (especially cochlear implant teams).<\/jats:p>","DOI":"10.1155\/2020\/3569359","type":"journal-article","created":{"date-parts":[[2020,5,16]],"date-time":"2020-05-16T23:31:08Z","timestamp":1589671868000},"page":"1-8","source":"Crossref","is-referenced-by-count":8,"title":["Jervell and Lange-Nielsen Syndrome due to a Novel Compound Heterozygous <i>KCNQ1<\/i> Mutation in a Chinese Family"],"prefix":"10.1155","volume":"2020","author":[{"given":"Yue","family":"Qiu","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sen","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Union 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