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散发性前庭神经鞘瘤与神经纤维瘤病 2 型相关患者的人工耳蜗植入结果。

Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2-Associated Vestibular Schwannoma.

机构信息

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN.

Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN.

出版信息

Otol Neurotol. 2023 Sep 1;44(8):791-797. doi: 10.1097/MAO.0000000000003963. Epub 2023 Jul 18.

DOI:10.1097/MAO.0000000000003963
PMID:37464449
Abstract

OBJECTIVE

Compare cochlear implant (CI) performance between patients with ipsilateral sporadic vestibular schwannoma (VS) and NF2-related schwannomatosis (NF2). Compare CI performance according to VS management modality.

STUDY DESIGN

Historical cohort.

SETTING

Tertiary academic center.

PATIENTS

Forty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28).

INTERVENTIONS

CI ipsilateral to VS.

MAIN OUTCOME MEASURES

Auditory thresholds, consonant-nucleus-consonant (CNC) word scores, and AzBio sentences in quiet scores.

RESULTS

Among all patients, median post-CI pure tone average was 28 dB HL (interquartile range [IQR], 21-38), CNC word score was 39% (IQR, 6-62), and AzBio sentences in quiet score was 60% (IQR, 11-83) at a median of 12.5 months postimplantation. Despite the NF2 cohort having larger tumors, when comparing patients with sporadic versus NF2-associated VS, there were no statistically significant differences in CNC word (49% [30-70] vs. 31% [0-52]) or AzBio sentences in quiet (66% [28-80] vs. 57% [5-83]) scores. Regardless of NF2 status, all patients managed with observation, and radiosurgery achieved open-set speech. In patients who underwent microsurgery, 6 (46%) of 13 with NF2 achieved open-set speech recognition compared with 4 (67%) of 6 with sporadic disease.

CONCLUSION

Select patients with VS achieve successful hearing rehabilitation with a CI. In this cohort, tumor management strategy significantly influenced CI performance, whereas differences in NF2 status exhibited less effect. Specifically, all patients managed with observation or radiosurgery achieved open-set speech perception, whereas approximately half of people with NF2-related VS and two-thirds of people with sporadic VS achieved this outcome after tumor microsurgery. When disease permits, observation and radiosurgery should be considered in patients who may later pursue a CI.

摘要

目的

比较同侧散发性前庭神经鞘瘤(VS)和 NF2 相关神经鞘瘤病(NF2)患者的人工耳蜗植入(CI)效果。根据 VS 管理方式比较 CI 效果。

研究设计

历史队列研究。

设置

三级学术中心。

患者

49 例(52 耳)在同侧散发性(n = 21)或 NF2 相关 VS(n = 28)的情况下接受人工耳蜗植入。

干预措施

VS 同侧的 CI。

主要观察指标

听觉阈值、辅音-核-辅音(CNC)词得分和安静时 AzBio 句子得分。

结果

在所有患者中,CI 后中位纯音平均听阈为 28 dB HL(四分位距[IQR],21-38),CNC 词得分 39%(IQR,6-62),安静时 AzBio 句子得分 60%(IQR,11-83),中位数为植入后 12.5 个月。尽管 NF2 组肿瘤较大,但比较散发性 VS 与 NF2 相关 VS 患者时,CNC 词(49%[30-70]与 31%[0-52])或 AzBio 句子在安静时(66%[28-80]与 57%[5-83])得分无统计学差异。无论 NF2 状态如何,所有接受观察和放射外科治疗的患者均获得开放式言语识别。在接受显微镜手术的患者中,13 例 NF2 患者中有 6 例(46%)获得开放式言语识别,而 6 例散发性疾病患者中有 4 例(67%)。

结论

选择合适的 VS 患者可通过 CI 获得成功的听力康复。在本队列中,肿瘤管理策略显著影响 CI 效果,而 NF2 状态的差异影响较小。具体来说,所有接受观察或放射外科治疗的患者均获得开放式言语感知,而大约一半的 NF2 相关 VS 患者和三分之二的散发性 VS 患者在肿瘤显微镜手术后获得该结果。在疾病允许的情况下,对于可能以后需要接受 CI 的患者,应考虑观察和放射外科治疗。

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引用本文的文献

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Cochlear implantation in vestibular schwannoma: A game changer? A narrative review.前庭神经鞘瘤的人工耳蜗植入:是变革之举?一篇叙述性综述。
Braz J Otorhinolaryngol. 2025 Feb 5;91(3):101563. doi: 10.1016/j.bjorl.2025.101563.