先天性内耳畸形患者人工耳蜗植入的临床体会

目的 探讨先天性内耳畸形重度或极重度感音神经性聋患者人工耳蜗植入方法及术后言语康复效果.方法 回顾性分析1998-2007年于我科行人工耳蜗植入295例患者的临床资料,其中前庭水管扩大25例,Modini畸形9例,共同腔畸形5例.所有患者均采用澳大利亚Nucleus24型人工耳蜗.对大前庭水管综合症患者,4例采用Nucleus 24R(ST)型植入体.8例采用Contuor型24R,10例采用Contuor Advance 24R.其余病例均采用Nucleus24M型直电极植入体.结果 25例大前庭水管综合征患者有3例术中出现井喷,15例术中耳蜗钻孔后见外淋巴液出现不同程度的波动;另有4例Mondini畸形及2例Common cavity畸形亦出现严莺井喷,但所有内耳畸形患者术中电极插入均顺利,术后无面瘫及脑脊液漏现象发生,术后听阈与耳蜗结构正常植入患者无明显区别.经过半年以上的言语康复训练,所有患者的听力及言语能力均有不同程度提高.结论 人工耳蜗适用于内耳畸形患者,但对耳蜗未发育或内听道听神经缺失患者除外,术前全面的听力学及影像学评估至关重要.

Abstract：

Objective To summarize the clinical experience with multi-channel cochlear implantation in patients with inner ear malformations and evaluate and the outcomes of speech rehabilitation. Methods A retrospective study was conducted in 295 patients receiving cochlear implantation from 1998 to 2007, including 25 patients with large vestibular aqueduct syndrome (LVAS), 9 with Modini malformation, and 5 with common cavity deformity. All the patients received the Nucleus24 cochlear implants. In LVAS cases, 4 had Nucleus 24R (ST) implants, 8 had Contuor implants, 10 had Contuor Advance, and the remaining cases used Nucleus24(M) straight-electrode implants. Results Severe gusher appeared in 3 cases of LVAS, and perilymph fluctuation were seen in aother 15 cases. Four patients with Mondini malformation and 2 with common cavity malformation also experienced severe gusher, but the electrodes were inserted smoothly in all the patients without postoperative facial paralysis or cerebrospinal fluid leakage. The hearing threshold in these patients was similar to that in patients with normal cochlear structure. After speech rehabilitation for over 6 months, the abilities of speech discrimination and spoken language improved in all the cases in comparison with the preoperative lingual functions. Conclusion Multi-channel cochlear implantation can be performed in patients with inner ear malformation, but should not be attempted in patients with poor cochlear and cochlear nerve development. A comprehensive pre-operative radiographic and audiological evaluation is essential.

Cochlear implant in patients with congenital malformation of the inner ear

Objective To summarize the clinical experience with multi-channel cochlear implantation in patients with inner ear malformations and evaluate and the outcomes of speech rehabilitation. Methods A retrospective study was conducted in 295 patients receiving cochlear implantation from 1998 to 2007, including 25 patients with large vestibular aqueduct syndrome (LVAS), 9 with Modini malformation, and 5 with common cavity deformity. All the patients received the Nucleus24 cochlear implants. In LVAS cases, 4 had Nucleus 24R (ST) implants, 8 had Contuor implants, 10 had Contuor Advance, and the remaining cases used Nucleus24(M) straight-electrode implants. Results Severe gusher appeared in 3 cases of LVAS, and perilymph fluctuation were seen in aother 15 cases. Four patients with Mondini malformation and 2 with common cavity malformation also experienced severe gusher, but the electrodes were inserted smoothly in all the patients without postoperative facial paralysis or cerebrospinal fluid leakage. The hearing threshold in these patients was similar to that in patients with normal cochlear structure. After speech rehabilitation for over 6 months, the abilities of speech discrimination and spoken language improved in all the cases in comparison with the preoperative lingual functions. Conclusion Multi-channel cochlear implantation can be performed in patients with inner ear malformation, but should not be attempted in patients with poor cochlear and cochlear nerve development. A comprehensive pre-operative radiographic and audiological evaluation is essential.