人工耳蜗植入在Mondini畸形患者中的应用

目的探讨Mondini畸形患者人工耳蜗植入术特点及术后言语康复效果。方法回顾性分析2000～2008年于我科确诊的12例Mondini畸形行人工耳蜗植入患者的临床资料,所有患者均经乳突面隐窝进路行人工耳蜗植入术,并将此12例患者植入后听力情况与另12例耳蜗发育正常的人工耳蜗植入者进行比较。结果 12例Mondini畸形患者中10例术中出现井喷,2例术中耳蜗钻孔后见外淋巴液出现不同程度的搏动,但术中电极植入顺利,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1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。结论人工耳蜗植入可以作为伴有Mondini畸形的双侧极重度感音性聋患者恢复听觉功能的手段。

Cochlear implantation in patients with Mondini malformation

OBJECTIVE To explore thecharacteristics and postoperative speech rehabilitation of multi-channel cochlear implantation in patients with Mondini malformation. METHODS Multi-channel cochlear implantation was performed on 12 cases with Mondini malformation in our department from 2000 to 2008. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of 12 cases were compared with 12 cases with non-malformation cochlea as a control group. RESULTS Gusher appeared in 10 implantations, perilymph fluctuation were seen in other 2 cases. The electrodes were inserted into the cochlea successfully. There was no facial paralysis and cerebrospinal fluid leakage occurred after operation. The hearing threshold in this series was similar to that of the normal cochlear implantation after more than one year of speech rehabilitation. The abilities of speech discrimination and spoken language of all the patients were improved comparing with that before operation. CONCLUSION Cochlear implantation can be used as a rehabilitation maneuver in patients with Mondini malformation associated with very severe bilateral sensorineural hearing loss.