目的·评估蜗神经发育不良（cochlear nerve deficiency，CND）先天性耳聋儿童植入人工耳蜗（cochlear implant，CI）后听觉及言语能力和社会生活能力改善的长期效果及其影响因素。方法·选取上海交通大学医学院附属第九人民医院随访的21例植入CI的CND患儿作为CND组，植入年龄、CI使用时间与CND组相匹配的20例内耳结构正常的极重度感音神经性聋CI植入者作为对照组，采用助听听阈值检测、婴幼儿有意义听觉整合量表（Infant-toddler Meaningful Auditory Integration Scale，IT-MAIS）、汉语普通话版小龄儿童听觉发展问卷（LittlEARS Auditory Questionnaire，LEAQ）、扩展版听觉能力分级问卷（Categories of Auditory Performance-Ⅱ，CAP-Ⅱ）、有意义使用言语量表（Meaningful Use of Speech Scale，MUSS）、言语可懂度分级标准（Speech Intelligibility Rating，SIR）、闭合式和开放式双音节言语识别测试、语音清晰度测试对受试者的听觉和言语能力进行层级评估，采用婴儿-初中生社会生活能力量表（Infant-junior Middle School Student′s Social Life Ability Scale，S-M量表）评定受试者的社会生活能力。结果·CND组各频率点平均助听听阈值低于对照组，差异有统计学意义（均P<0.05）。CND组90.5%的患儿各频率助听听阈值均进入中文香蕉图。CND组听觉和言语能力差于对照组，其中2组的IT-MAIS、CAP-Ⅱ、MUSS、SIR、闭合式和开放式双音节言语识别率、语音清晰度结果差异有统计学意义（均P<0.05），2组LEAQ得分的差异无统计学意义。CND组社会生活能力差于对照组，且差异有统计学意义（P<0.05）。植入年龄、CI使用时间、康复时间与CND组听觉及言语效果间无相关性。结论·CI对CND患儿是一种有效的听觉干预方法，但其平均听觉言语及社会生活能力明显落后于内耳结构正常的CI植入者。应注重对患儿采用个性化的交流策略和康复训练方法，加强其社会生活能力培养和心理指导。

Objective ·To evaluate the long-term performance and influencing factors of auditory and speech abilities, and social-life abilities in congenital deaf children with cochlear nerve deficiency (CND) after cochlear implant (CI) surgery. Methods ·Twenty-one CND children with CI implantation in Shanghai Ninth People′s Hospital, Shanghai Jiao Tong University School of Medicine were assigned to CND group, and twenty children with extremely severe sensorineural hearing loss with normal inner ear structure matching implantation age and CI use time of the CND group were selected as control group. The aided hearing threshold, Infant-toddler Meaningful Auditory Integration Scale (IT-MAIS), LittlEARS Auditory Questionnaire (LEAQ), Categories of Auditory Performance-Ⅱ (CAP-Ⅱ), Meaningful Use of Speech Scale (MUSS), Speech Intelligibility Rating (SIR), closed and open disyllabic speech recognition test, and speech intelligibility test were used to hierarchically evaluate the subjects′ auditory and verbal abilities, and the Infant-junior Middle School Student′s Social Life Ability Scale (S-M Scale) was used to assess the subjects′ social-life ability. Results ·The average aided hearing threshold of each frequency point in the CND group was significantly worse than that of the control group (all P<0.05). In the CND group, 90.5% of children′s aided hearing threshold of all frequencies entered the Chinese banana map. The auditory and speech abilities of the CND group were worse than those of the control group, and the significant differences between the two groups were found in the results of IT-MAIS, CAP-Ⅱ, MUSS, SIR, and closed and open disyllabic speech recognition (all P<0.05), but not in LEAQ. The social-life ability of the CND group was worse than that of the control group, and the difference was statistically significant (P<0.05). There was no correlation between implantation age, usage time of CI, auditory and language rehabilitation time, and auditory and speech ability of the CND group. Conclusion ·CI is an effective auditory intervention method for children with CND, but their average auditory and speech abilities and social-life abilities are significantly behind those of CI users with normal inner ear structure. Therefore, for CND children, individualized communication strategies and rehabilitation training methods should be emphasized, and the cultivation of social-life ability and psychological guidance should be strengthened.