Sinnathuray Arasa Raj, Toner Joseph G, Clarke-Lyttle Joanne, Geddis Andrea, Patterson Christopher C, Hughes Anne E
Northern Ireland Regional Cochlear Implant Center, Belfast City Hospital, Belfast, UK.
Otol Neurotol. 2004 Nov;25(6):935-42. doi: 10.1097/00129492-200411000-00013.
Speech intelligibility in children after cochlear implantation may depend on their deafness cause, including connexin 26 (GJB2) gene-related deafness.
There is significant variability in the degree of intelligibility, or clarity, of children's speech after cochlear implantation. GJB2 gene-related deafness may be a factor, as preliminary data suggest that pathologic changes do not affect the spiral ganglion cells, which are the neural elements stimulated by the implant, thus favoring better results.
In an observational retrospective cohort study of pediatric cochlear implantees, 38 patients with nonsyndromic deafness of unknown cause and 1 with keratitisichthyosis-deafness syndrome underwent GJB2 mutation analysis using polymerase chain reaction amplification and direct sequencing. The primary outcome measure assessed was Speech Intelligibility Rating score from postoperative Year 1 (n = 39) to Year 5 (n = 17). Educational setting was considered as a secondary outcome measure. Statistical analysis was double-blinded, with patients and assessors of outcome unaware of GJB2 status.
Fourteen patients had GJB2-related deafness and 25 had GJB2-unrelated deafness. Comparisons at Year 3 (n = 31) revealed intelligible speech achieved by 9 of 11 with GJB2-related deafness, compared with only 6 of 20 with GJB2-unrelated deafness (p = 0.017). Ordinal logistic regression analysis on Speech Intelligibility Rating scores found statistically significantly better scores in children with GJB2-related deafness (p < 0.05) both before and after adjustment for confounding variables. A larger proportion with GJB2-related deafness also attended mainstream school (p = 0.01).
In pediatric cochlear implantees, GJB2-related deafness is a predictor of good speech intelligibility.
儿童人工耳蜗植入后的言语可懂度可能取决于其致聋原因，包括与连接蛋白26（GJB2）基因相关的耳聋。
儿童人工耳蜗植入后的言语可懂度或清晰度存在显著差异。GJB2基因相关的耳聋可能是一个因素，因为初步数据表明病理变化不会影响螺旋神经节细胞，而螺旋神经节细胞是植入物刺激的神经元件，因此可能会带来更好的效果。
在一项针对儿科人工耳蜗植入者的观察性回顾性队列研究中，38例病因不明的非综合征性耳聋患者和1例角膜炎-鱼鳞病-耳聋综合征患者采用聚合酶链反应扩增和直接测序进行GJB2突变分析。评估的主要结局指标是术后第1年（n = 39）至第5年（n = 17）的言语可懂度评分。教育环境被视为次要结局指标。统计分析采用双盲法，患者和结局评估者均不知道GJB2状态。
14例患者为GJB2相关耳聋，25例为GJB2非相关耳聋。在第3年（n = 31）进行比较时发现，11例GJB2相关耳聋患者中有9例实现了可懂言语，而20例GJB2非相关耳聋患者中只有6例实现了可懂言语（p = 0.017）。对言语可懂度评分进行有序逻辑回归分析发现，在调整混杂变量前后，GJB2相关耳聋儿童的评分在统计学上均显著更高（p < 0.05）。就读主流学校的GJB2相关耳聋患者比例也更高（p = 0.01）。
在儿科人工耳蜗植入者中，GJB2相关耳聋是言语可懂度良好的一个预测指标。
