Ⅱ型神经纤维瘤病疾病特征与人工耳蜗植入  

Disease Characteristics of Neurofibromatosis Type II and Cochlear Implant

作　　者：付林玲 王淑为 王丹丹[1,2] 洪梦迪 庄博翔[1,2] 杜海侨 李佳楠 FU Lin-ling;WANG Shu-wei;WANG Dan-dan;HONG Meng-di;ZHUANG Bo-xiang;DU Hai-qiao;LI Jia-nan

机构地区：[1]解放军总医院第六医学中心耳鼻咽喉头颈外科医学部,北京100048 [2]国家耳鼻咽喉疾病临床医学研究中心/听觉与平衡觉全国重点实验室,北京100853

出　　处：《中国听力语言康复科学杂志》2024年第5期492-494,509,共4页Chinese Scientific Journal of Hearing and Speech Rehabilitation

基　　金：国家重点研发计划(2021YFF0702302)

摘　　要：目的 分析1例表现较为复杂的Ⅱ型神经纤维瘤病(neurofibromatosis type Ⅱ,NF2)患者的临床表现、听力康复方法和效果,明确一种特殊类型的NF2听神经瘤的听力重建方法及术后康复效果。方法 整理该患者临床表现及既往史,术前检查,临床手术方法及术后随访听力康复效果。结果 患者双侧听神经瘤,听力表现为双耳极重度感音神经性听力下降。外周血基因检测未见治病基因突变,考虑为嵌合体。患者右耳为内耳神经鞘膜瘤,且双眼视力极差,右耳行带瘤人工耳蜗植入术,术后通过为期2个月的以家庭为主的听力语言康复,听觉能力分级(CAP)由0分提高到5分。结论 NF2临床表现复杂,临床预后差。带有微小的内耳神经鞘膜瘤的NF2患者可以通过带瘤植入人工耳蜗重建听力。Objective To analyze the clinical manifestation,hearing rehabilitation method,and effect in a patient with complicated neurofibromatosis type II(NF2).To study the hearing reconstruction method and postoperative rehabilitation effect of a special type of NF2.Methods The clinical manifestations,past history,preoperative examination,clinical surgical method,and follow-up hearing rehabilitation effect were summarized.Results The patient had bilateral acoustic neuroma and severe sensorineural hearing loss in both ears.A peripheral blood test did not detect any related gene mutations and she was considered a chimera.She had an inner ear schwannoma(IES)on right side and very poor vision in both eyes.Cochlear implantation was performed in the right ear.Home-based hearing and speech rehabilitation lasted for two months after the operation,and the auditory ability grade(categorization of auditory performance,CAP)increased from 0 to 5 points.Conclusion NF2 has complex clinical manifestations and a poor clinical prognosis.Hearing can be reconstructed in NF2 patients with small IES by cochlear implantation with tumor survival.

关 键 词：Ⅱ型神经纤维瘤病 内耳听神经瘤 听力重建 人工耳蜗植入 

分 类 号：R764.43[医药卫生—耳鼻咽喉科]