Li Guo, Liu Li, Yang Ting, Lang Chunmei, Ma Xiuli, Zhao Liping, Tang Xianchao, Wang Xiang, Zhang Tiesong, Ma Jing
Department of Otolaryngology Head and Neck Surgery，Kunming Children's Hospital（Yunnan Children's Medical Center，Children's Hospital Affiliated to Kunming Medical University），Kunming，650100，China.
Lijiang Special Education School Resource Center.
Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2022 May;36(5):347-352. doi: 10.13201/j.issn.2096-7993.2022.05.005.
By comparing the hearing and speech rehabilitation effects of cochlear implantation （CI） in children with Waardenburg syndrome （WS） and children with common deafness genes （, ） in the Chinese population, and the hearing and speech rehabilitation effects of bilateral CI and unilateral CI in children with WS, to provide a reference for clinical CIin children with WS. Follow up and return visit 72 pedestrian cochlear implant children with severe and above sensorineural hearing loss and clear gene mutation type diagnosed by Kunming Children's Hospital from 2017 to 2019, including 24 cases in the WS group, 24 cases in the control group （ deafness group and deafness group）. All enrolled children were evaluated for auditory and speech ability 12 months after startup. The hearing aid threshold, the correct recognition rate of speech recognition ability evaluation, IT-MAIS / MAIS score rate, CAP score, SIR score, there was no significant difference（>0.05）. The correct recognition rates of IT-MAIS / MAIS score, SIR score, natural environment sound recognition, vowel recognition, tone recognition, monosyllabic word recognition, disyllabic word recognition and short sentence recognition in children with WS bilateral CI were significantly higher than those in children with WS unilateral CI （<0.05）. There was no significant difference in CAP score, initial recognition and correct recognition rate of trisyllabic words between children with WS bilateral CI and children with WS unilateral CI （>0.05）. Common deafness genes in children with WS and Chinese population （, ） the effect of cochlear implantation on hearing and speech rehabilitation of sick children is equivalent. For children with severe and above sensorineural hearing loss associated with this syndrome, CI can be used clinically to improve their hearing and speech ability. WS bilateral CI has advantages in some hearing and speech abilities compared with unilateral CI, so those whomeet the conditions should be encouraged bilateral implantation.
通过比较中国人群中患有瓦登伯革综合征（WS）的儿童和患有常见耳聋基因的儿童人工耳蜗植入（CI）后的听力和言语康复效果，以及WS儿童双侧CI和单侧CI的听力和言语康复效果，为WS儿童临床CI提供参考。随访2017年至2019年在昆明儿童医院确诊的72例重度及以上感音神经性听力损失且基因突变类型明确的人工耳蜗植入患儿，其中WS组24例，对照组（耳聋组和耳聋组）24例。所有入组患儿开机12个月后进行听觉和言语能力评估。助听器阈值、言语识别能力评估正确识别率、IT-MAIS/MAIS评分率、CAP评分、SIR评分，差异无统计学意义（>0.05）。WS双侧CI患儿IT-MAIS/MAIS评分、SIR评分、自然环境声音识别、元音识别、声调识别、单音节词识别、双音节词识别和短句识别的正确识别率均显著高于WS单侧CI患儿（<0.05）。WS双侧CI患儿与WS单侧CI患儿CAP评分、三音节词初始识别及正确识别率差异无统计学意义（>0.05）。WS患儿与中国人群中的常见耳聋基因（，）人工耳蜗植入对患病儿童听力和言语康复的效果相当。对于患有与该综合征相关的重度及以上感音神经性听力损失的儿童，临床上可采用CI改善其听力和言语能力。WS双侧CI在某些听力和言语能力方面比单侧CI具有优势，因此符合条件的应鼓励双侧植入。
