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Auditory brainstem implants in NF2 patients: Results and review of the literature

机译:NF2患者的听觉脑干植入物:结果和文献复习

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OBJECTIVE: Neurofibromatosis Type 2 (NF2) patients have multiple central nervous system tumors and, specifically, bilateral vestibular schwannomas (VSs) causing bilateral deafness. If the cochlear nerve is not preserved during tumor removal, the only hearing rehabilitation in these patients could be via an auditory brainstem implant (ABI). STUDY DESIGN: Retrospective case study and literature review. SETTING: Tertiary referral cranial base center. PATIENTS: In 24 NF2 patients, 25 ABIs were placed in the lateral recess of the fourth ventricle after VS surgery via a translabyrinthine approach. RESULTS: In this series, a large range of results are observed: from open speech and use of the telephone to no ABI use, because of the poor sound identification ability. Of the 24 patients, 19 use their ABI on a daily basis, 4 are nonusers, and 1 died of NF2 progression. A multivariate analysis did not reveal a good predictor for ABI outcome.In literature, the results of ABI in NF2 are difficult to compare, and the overall outcome was poor compared with cochlear implantation results. CONCLUSION: Auditory brainstem implantation in NF2 patients directly after tumor removal is a safe procedure and the best means of hearing rehabilitation if the cochlear nerve is not preserved. The results in NF2 cases in the literature and these series are poor compared with cochlear implantation. If a cochlear implant is possible, it has the preference over an ABI, also in NF2. Nevertheless, the majority of the patients have benefit of the ABI during daily life particularly in combination with lip reading.
机译:目的:2型神经纤维瘤病(NF2)患者患有多种中枢神经系统肿瘤,特别是双侧前庭神经鞘瘤(VSs)引起双耳聋。如果在切除肿瘤过程中未保留耳蜗神经,则这些患者的唯一听力康复可能是通过听觉脑干植入物(ABI)。研究设计:回顾性案例研究和文献综述。地点:三级转诊颅底中心。患者:24例NF2患者中,经透迷路方法将25例ABI放置在VS手术后第四脑室的侧凹处。结果:在这个系列中,观察到了很多结果:由于声音识别能力差,从开放语音和电话使用到不使用ABI。在24例患者中,每天有19例使用ABI,4例为非使用者,其中1例死于NF2进展。多变量分析不能很好地预测ABI的结果。在文献中,NF2中的ABI结果难以比较,与人工耳蜗植入结果相比,总体结果差。结论:在切除肿瘤后直接在NF2患者中进行听觉脑干植入是一种安全的方法,如果不保留耳蜗神经,则是听力康复的最佳方法。与人工耳蜗植入相比,文献中的NF2病例和这些系列的结果均较差。如果可以植入人工耳蜗,则其优先于ABI,同样在NF2中。尽管如此,大多数患者在日常生活中特别是与唇读结合使用ABI的好处。

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