神经纤维瘤病Ⅱ型的临床特点及听觉脑干植入

A Case Study Of Auditory Brainstem Implant in a Patient with Neurofibromatosis Type Ⅱ

目的探讨神经纤维瘤病Ⅱ型(neurofibromatosis type 2,NF2)的临床特点、治疗方法以及听觉脑干植入(auditory brainstem implant,ABI)后的效果。方法回顾性分析1例双侧听神经瘤(NF2)患者的诊疗经过及ABI术后6、9、12、24、36个月的听力言语康复效果。结果患者,男,13岁,因右耳听力下降及耳鸣3年伴步态不稳,声音嘶哑就诊,患者曾接受过腰椎椎管内神经纤维瘤切除病史,MRI检查示双侧桥小脑角区占位病变,临床诊断为NF2;经fishey入路分次切除双侧听神经瘤,术后病理诊断均为神经鞘膜瘤,术后2年行左耳ABI手术,ABI术后又先后接受过气管神经纤维瘤、腹膜后细胞性神经鞘瘤切除术。ABI术后开机调试时所有电极均能引出听觉反应,随着康复时间的延长,患者对自然环境声响、元音、单音节词的正确识别率均呈上升趋势,纯音听阈值逐渐降低,术后6、9、12、24、36个月元音的正确识别率分别为14%、18%、20%、24%、35%,单字词的正确识别率分别为5%、7%、10%、14%、20%;但双音节词及开放语句识别率均为0。结论 NF2多发生在青少年,治疗应遵循个体化原则,手术切除双侧听神经瘤仍是首选的方法,ABI对于双侧听神经瘤切除术后患者听力重建是一种很好的选择,术中准确定位耳蜗核是手术成功的关键。

Objective To study the clinical characteristics and treatment of neurofibromatosis type Ⅱ（neurofibromatosis type 2,NF2,bilateral acoustic neuroma）,and the effects of auditory brainstem implant for treating total deafness after bilateral acoustic neuroma resection.Methods One case of bilateral acoustic neuroma and all clinical data in terms of diagnosis,treatment and hearing-speech rehabilitation after surgery were retrospectively studied.Results The patient was a thirteen years old boy.His clinical symptoms were hearing loss on the right ear,tinnitus,hoarseness and gait instability three years.MRI showed space occupying lesion in the cerebellopontine angle.The postoperative pathological diagnosis was bilateral acoustic neuroma.The initial switch-on was peformed six weeks after the surgery,and confirmed that all electrodes generated listening responses.As the extension of recovery time,the correct recognition rate of patients on the natural environment sound,vowel,monosyllabic were on the rise and the pure tone hearing threshold gradually decreased.The vowel correct recognition rate of postoperative 6,9,12,24,and 36 months were 14%,18%,20%,24%,and 20%,respectively.The recognition rate of monosyllabic and open words at each postoperative rehabilitation stage were 0.Conclusion The clinical characteristics and treatment of bilateral acoustic neuroma were different from the unilateral acoustic neuroma.The individualized treatment should be followed.Auditory brainstem implant could be performed in patients with post- bilateral acoustic neuroma resection.The accurate location of the cochlear complex during the surgery was the crucial point for the success of ABI.