II型神经纤维瘤病疾病特征与人工耳蜗植入

付林玲; 王淑为; 王丹丹; 洪梦迪; 庄博翔; 杜海侨; 李佳楠

doi:10.3969/j.issn.1672-4933.2024.05.010

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II型神经纤维瘤病疾病特征与人工耳蜗植入

临床研究 | 更新时间：2024-09-05

- 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
- 中国听力语言康复科学杂志 2024年22卷第5期页码：492-494
- 作者机构：
  
  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
- 作者简介：
  
  付林玲硕士住院医师；研究方向：耳科、听力学
  李佳楠，E-mail：lijianan2005@126.com
- 基金信息：
  
  国家重点研发计划(2021YFF0702302)
- DOI：10.3969/j.issn.1672-4933.2024.05.010
  中图分类号： R764.43
- 纸质出版日期：2024-09-15，
  
  收稿日期：2024-07-02，
- 稿件说明：
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付林玲,王淑为,王丹丹等.II型神经纤维瘤病疾病特征与人工耳蜗植入[J].中国听力语言康复科学杂志,2024,22(05):492-494.

FU Lin-ling,WANG Shu-wei,WANG Dan-dan,et al.Disease Characteristics of Neurofibromatosis Type II and Cochlear Implant[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2024,22(05):492-494.
付林玲,王淑为,王丹丹等.II型神经纤维瘤病疾病特征与人工耳蜗植入[J].中国听力语言康复科学杂志,2024,22(05):492-494. DOI： 10.3969/j.issn.1672-4933.2024.05.010.

FU Lin-ling,WANG Shu-wei,WANG Dan-dan,et al.Disease Characteristics of Neurofibromatosis Type II and Cochlear Implant[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2024,22(05):492-494. DOI： 10.3969/j.issn.1672-4933.2024.05.010.

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。

Abstract

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

Keywords

Neurofibromatosis type IIInner ear schwannomaHearing rehabilitationCochlear implantation

references

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Ahronowitz I, Xin W, Kiely R, et al. Mutational spectrum of the NF2 gene: a meta-analysis of 12 years of research and diagnostic laboratory findings[J]. Hum Mutat, 2007, 28(1): 1-12.

左文静, 纵亮, 杨仕明. 神经纤维瘤病Ⅱ型发病机理的研究现况. 中华耳科学杂志[J], 2018, 16(4): 488-492.

中国抗癌协会神经肿瘤专业委员会. 2型神经纤维瘤病神经系统肿瘤多学科协作诊疗策略中国专家共识[J]. 中华神经外科杂志, 2021, 37(7): 663-668.

王兴朝, 李仕维, 李朋, 等. 2型神经纤维瘤病的手术策略及疗效分析[J]. 中华神经外科杂志, 2023,39(6): 546-550.

Gao F, Li M, Wang Z, et al. Efficacy and Safety of Gamma Knife Radiosurgery for Meningiomas in Patients with Neurofibromatosis Type 2: A Long-Term Follow-Up Single-Center Study[J]. World Neurosurg, 2019,125:e929-e936.

Ghalavand MA, Asghari A, Farhadi M, et al. The genetic landscape and possible therapeutics of neurofibromatosis type 2[J]. Cancer Cell Int, 2023,23:99-126.

Lu VM, Ravindran K, Graffeo CS, et al. Efficacy and safety of bevacizumab for vestibular schwannoma in neurofibromatosis type 2: a systematic review and meta-analysis of treatment outcomes[J]. J Neurooncol, 2019, 144(2): 239-248.

Goldbrunner R, Weller M, Regis J, et al. EANO guideline on the diagnosis and treatment of vestibular schwannoma[J]. Neuro Oncol, 2020, 22(1): 31-45.

吴皓, 贾欢. 关注人工听觉脑干植入[J]. 中华医学杂志, 2021, 101(2): 92-96.

Tadokoro K, Bartindale MR, El-Kouri N, et al. Cochlear Implantation in Vestibular Schwannoma: A Systematic Literature Review[J]. J Neurol Surg B Skull Base, 2021, 82(6): 643-651.

Peng KA, Lorenz MB, Otto SR, et al. Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2[J]. Laryngoscope, 2018, 128(9): 2163-2169.

Laborai A, Ghiselli S, Cuda D. Cochlear Implant in Patients with Intralabyrinthine Schwannoma without Tumor Removal[J]. Audiol Res, 2022, 12(1): 33-41.

Eitutis ST, Jansen T, Borsetto D, et al. Cochlear Implantation in NF2 Patients Without Intracochlear Schwannoma Removal[J]. Otol Neurotol, 2021, 42(7): 1014-1021.

火子榕, 张治华, 黄琦, 等. 唯一听力耳听神经瘤患者人工耳蜗植入疗效分析 [J]. 中华耳鼻咽喉头颈外科杂志, 2016, 51(6): 408-413.

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