先天性内耳畸形的人工耳蜗植入

目的 探讨先天性内耳畸形引起重度感音神经性聋者人工耳蜗植入的有关问题。方法 2001年1月至2003年4月间对内耳畸形引起极重度感音神经性聋18例进行了人工耳蜗植入术。结果 18例中前庭水管11例，Waardenburg综合征3例，Mondini畸形3例，Usher综合征1例。全部病例采用Nucleus24型人工耳蜗，其中对前庭水管5例采用Contour植入体，其余病例采用直电极植入体。术中发现前庭水管11例开骨窗后仅有外淋巴搏动，但无井喷(脑脊液漏)，电极植入顺利。Waardenburg综合征3例和Mondini畸形3例中各有1例伴发圆窗骨性封闭畸形。结论 前庭水管者人工耳蜗植入手术顺利，术后效果与耳蜗发育正常者相同。如Mondini和Common Cavity等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形，要充分估计手术难度和避免术后可能出现的脑脊液耳鼻漏及其颅内感染。

Cochlear implant in patients with congenital malformation of inner ear]

OBJECTIVE: To study surgical difficulty and key of the cochlear implant in patients with congenital malformation of inner ear. METHODS: The cochlear implantations were performed in our department from Jan. 2001 to Apr. 2003 for 18 patients with the malformation of inner ear. RESULTS: In this series, there were 11 cases of large vestibular aqueduct syndrome (LVAS), 3 cases of Waardenberg syndrome, 3 cases of Mondini malformation, and 1 case of Usher syndrome. All 18 patients accepted the Nucleus 24-channel cochlear implantations, including Nucleus straight electrode in 13 cases but Contour implantation in 5 cases of LVAS. During operations, leakage of perilymph but not cerebrospinal fluid (CSF) from the open of scala tympani occurred in 11 cases of LVAS, however, the electrode was inserted successfully. The abnormalities of round window occurred in one of 3 cases of Waardenberg syndrome and 3 cases of Mondini malformation, respectively. CONCLUSION: The cochlear implant could be conducted successfully for the LVAS, and the postoperative effect was same as the ones for the deafness persons with normal development of inner ear. However, for the patients with Mondini syndrome and common cavity, it is important to accurately assess the extent of abnormalities in the inner ear and accompanied malformation before operation, and to evaluate the full extent of difficulties of the operation in order to minimize the risk of CSF leakage and meningitis.