2名内耳不完全分隔Ⅲ型患者人工耳蜗植入效果分析  

Hearing Speech Rehabilitation of Two Patients with IP-ⅢInner Ear Malformation after Cochlear Implantation

作　　者：陈旭真[1] 李建红[1] 杜菲 周永青[1] CHEN Xu-zhen;LI Jian-hong;DU Fei;ZHOU Yong-qing

机构地区：[1]联勤保障部队第九八〇(白求恩国际和平)医院耳鼻咽喉头颈外科,石家庄050051

出　　处：《中国听力语言康复科学杂志》2023年第5期476-479,共4页Chinese Scientific Journal of Hearing and Speech Rehabilitation

摘　　要：内耳不完全分隔Ⅲ型(incomplete partition typeⅢ,IP-Ⅲ)是因POU3F4(DFNX2)基因突变导致的X连锁遗传性耳聋,表现为男性发病,女性携带。该病人工耳蜗植入手术难度大,易致严重并发症,如脑脊液漏、电极误入内听道等,术后听觉和言语康复疗效不确切。笔者科室分别于2014年和2022年收治2名IP-Ⅲ内耳畸形患儿,均顺利完成右侧人工耳蜗植入手术,无并发症,术后1个月开机,声场助听听阈分别为28.8 dB HL和31.7 dB HL,术后听力言语康复较好。Incomplete partition typeⅢ(IP-Ⅲ)is an X-linked genetic deafness caused by POU3F4(DFNX2)gene mutation.Clinical manifestation is male onset,female carrier.The patient with IP-Ⅲtype inner ear malformation is hard to be performed cochlear implantation because some serious complications could occur,such as cerebrospinal fluid otorrhea,electrode enter the internal auditory canal.Furthermore,the rehabilitation efficacies of postoperative hearing and speech are uncertain.Two boys with type IP-Ⅲinner ear malformation received cochlear implantation on their right ears in 2014 and 2022 in our Department,with no complications occurring.The electronic cochlears were smoothly started up one month after surgeries.The electronic cochlears have been working well until now.At present auditory thresholds of sound field hearing aid were 28.8 dB HL and 31.7 dB HL,respectively.Their hearing and speech rehabilitation efficacies were better than that in the preoperation.

关 键 词：不完全分隔Ⅲ型 内耳畸形 耳蜗植入 康复 疗效 

分 类 号：R764.9[医药卫生—耳鼻咽喉科]