Sinnathuray Arasa Raj, Toner Joseph G, Geddis Andrea, Clarke-Lyttle Joanne, Patterson Christopher C, Hughes Anne E
Northern Ireland Regional Cochlear Implant Center, Belfast City Hospital, Belfast, United Kingdom.
Otol Neurotol. 2004 Nov;25(6):930-4. doi: 10.1097/00129492-200411000-00012.
Auditory perception and speech discrimination among pediatric cochlear implantees may vary because of underlying deafness etiology, including connexin 26 (GJB2) gene-related deafness.
Preliminary data suggest pathologic changes due to GJB2 mutations do not affect the spiral ganglion cells, which are stimulated by the cochlear implant. The survival of the spiral ganglion cells is believed to be an important determinant of outcome after surgery. Patients with GJB2-related deafness may therefore have enhanced prospects for good speech discrimination after implantation.
In an observational cohort study, GJB2 mutation analysis was performed using polymerase chain reaction amplification and direct sequencing on 31 prelingually deaf pediatric cochlear implantees, of which there were 30 with nonsyndromic deafness of unknown etiology, and one with keratitis-ichthyosis-deafness syndrome. Speech discrmination was assessed prospectively when they had reached postoperative year 3 using the IOWA Matrix Level B Sentences test and Glendonald Auditory Screening Procedure (GASP), with both patients and assessors blind to GJB2 status.
Eleven patients had GJB2-related deafness and 20 patients had GJB2-unrelated deafness. IOWA Matrix scores were higher in patients with GJB2-related deafness but did not reach statistical significance. However, GASP scores were statistically significantly higher in patients with GJB2-related deafness (median word score, 92%; median sentence score, 80%), compared with those of patients with GJB2-unrelated deafness (median word score, 63%; median sentence score, 45%; word score, p = 0.037; sentence score, p = 0.045). Ordinal logistic regression analysis on IOWA Matrix and GASP sentence scores found better statistically significant scores in patients with GJB2-related deafness (p < 0.05) after adjustment for confounding variables.
Pediatric cochlear implantees with GJB2-related deafness appear to have equal or better speech discrimination compared with a group of prelingually deaf children with deafness of unknown etiology.
儿童人工耳蜗植入者的听觉感知和言语辨别能力可能因潜在的耳聋病因不同而有所差异，其中包括连接蛋白26（GJB2）基因相关的耳聋。
初步数据表明，GJB2突变引起的病理变化不会影响人工耳蜗所刺激的螺旋神经节细胞。螺旋神经节细胞的存活被认为是手术后效果的一个重要决定因素。因此，患有GJB2相关耳聋的患者在植入后可能有更好的言语辨别前景。
在一项观察性队列研究中，对31名语前聋的儿童人工耳蜗植入者进行了GJB2突变分析，采用聚合酶链反应扩增和直接测序法，其中30名患有病因不明的非综合征性耳聋，1名患有角膜炎-鱼鳞病-耳聋综合征。当他们术后满3年时，使用爱荷华矩阵B级句子测试和格伦多纳德听觉筛查程序（GASP）对言语辨别能力进行前瞻性评估，患者和评估者均不知道GJB2状态。
11名患者患有GJB2相关耳聋，20名患者患有GJB2不相关耳聋。患有GJB2相关耳聋的患者的爱荷华矩阵得分较高，但未达到统计学显著性。然而，与患有GJB2不相关耳聋的患者相比（单词得分中位数为63%；句子得分中位数为45%；单词得分，p = 0.037；句子得分，p = 0.045），患有GJB2相关耳聋的患者的GASP得分在统计学上显著更高（单词得分中位数为92%；句子得分中位数为80%）。对爱荷华矩阵和GASP句子得分进行有序逻辑回归分析发现，在调整混杂变量后，患有GJB2相关耳聋的患者的得分在统计学上显著更好（p < 0.05）。
与一组病因不明的语前聋儿童相比，患有GJB2相关耳聋的儿童人工耳蜗植入者似乎具有同等或更好的言语辨别能力。
