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目的探讨异常结构耳蜗人工耳蜗植入的有关问题。方法对2002年1月至2005年12月行人工耳蜗植入的10例内耳畸形和耳蜗骨化的资料进行分析。10例中耳蜗畸形7例,其中大前庭导水管综合征3例,共同腔畸形2例,Pendred综合征(甲状腺肿耳聋)伴小耳蜗畸形1例,内听道狭窄1例；耳蜗完全骨化1例,：不完全骨化2例。结果手术在显微镜下完成。3例大前庭导水管综合征中,1例术中可见外淋巴液波动,另1例出现井喷,但均顺利完全插入电极并用颞肌筋膜成功封闭鼓阶小窗,阻止脑脊液外漏；2例共同腔畸形只能分别插入15和17个电极；1例Pendred综合征并小耳蜗畸形患儿在治疗甲状腺功能低下后接受手术并顺利完全插入电极；1例内听道狭窄患儿有残余听力,顺利接受了植入手术,术中术后未发生脑脊液漏；2例耳蜗不完全骨化可完全插入电极(其中1例为经前庭阶插入)．另1例完全骨化,术中与其家长与语训老师讨论后放弃手术。全部病例均无并发症,成功插入电极者均成功开机,所有病例均有听觉反应。电极阻抗、阈值与舒适值与其他正常耳蜗患儿相似,植入后6个月MAIS得分也与同期植入的正常耳蜗病例相仿。结论对于异常结构耳蜗患儿,人工耳蜗植入术前必须详细了解病史,仔细分析影像学资料,深入与患儿家长讨论病情,规范进行术中操作都是非常重要的。
Objective To investigate the related problems of cochlear implant in abnormal structure cochlea. Methods From January 2002 to December 2005, 10 cases of cochlear implants and cochlear ossification were analyzed. 10 cases of middle-cochlear malformations in 7 cases, including large vestibular aqueduct syndrome in 3 cases, common cavity deformity in 2 cases, Pendred syndrome (goiter deformity) with cochlear malformation in 1 case, stenosis in 1 case; cochlear complete ossification 1 case, 2 cases of incomplete ossification. Results The surgery was done under a microscope. In 3 cases of large vestibular aqueduct syndrome, one case of peripheral lymph fluid fluctuations can be seen in the other cases blowout, but were successfully inserted fully electrode and the temporal fascia successfully closed the window of the drum order to prevent leakage of cerebrospinal fluid; Cases of common cavity deformity can only be inserted into 15 and 17 electrodes; 1 case of Pendred syndrome and cochlear malformation in children after surgery for hypothyroidism and surgery fully inserted into the electrode; 1 case of children with residual hearing stenosis , Successfully accepted the implantation operation, no postoperative cerebrospinal fluid leakage occurred during surgery; 2 cases of incomplete ossification of the cochlea could be completely inserted into the electrode (one of them was inserted through the vestibular cavity). The other 1 case of complete ossification, surgery and their parents after the discussion with the language teacher to give up surgery. No complications were found in all the cases. Successful successful insertion of the electrodes led to an auditory reaction in all cases. Electrode impedance, threshold and comfort values were similar to those in other children with normal cochlear implants, with a similar MAIS score 6 months after implantation as for normal cochlear implants during the same period. Conclusions For children with abnormal structure cochlear implant, it is necessary to understand the history before cochlear implantation. Careful analysis of the imaging data, in-depth discussions with parents of children about the condition, and specification of intraoperative procedures are very important.