Background Many patients with neurofibromatosis type 2 (NF2) suffer from sensorineural hearing loss, and associated cochlear nerve compromise in NF2 patients makes auditory brainstem implant (ABI) an attractive treatment option. The long-term outcomes and benefits of the device are still being explored. Methods A retrospective review was conducted for 11 ABI recipients at a single-institution tertiary center between November 2017 and August 2022. Patients diagnosed with NF2 undergoing resection for concurrent vestibular schwannoma (VS) were included. Pre- and postaudiometric assessments were reviewed. Evaluation included pure-tone audiometry and speech testing. Results Our cohort included 11 patients with a median age of 34 years. All patients underwent a translabyrinthine approach for implant placement with concurrent VS resection. Average tumor size of VS was 2.87 cm. Preoperatively, 8 patients had pure-tone averages with no response at 110 dB, 2 were within mild–moderate hearing loss (25–45 dB), and one patient had a PTA of profound loss (92 dB). Postoperatively, 9 (81%) patients had improvement in PTA. In total, seven patients reported mild side effects upon ABI activation which included dizziness (n = 2), tinnitus (n = 1), and abdominal and lower extremity tingling sensation (n = 3). Of the seven patients with early speech perception (ESP) scores, five had a score >75%, indicating the auditory ability to detect pattern perception upon auditory stimulation through the ABI alone. Conclusion Nine of 11 patients derived benefits from ABI placement. These findings demonstrate that ABI placement during concurrent VS resection can provide a significant hearing benefit for NF2 patients.

中文翻译：

---

NF2 患者前庭神经鞘瘤切除术中听觉脑干植入的听力结果

背景 许多 2 型神经纤维瘤病 (NF2) 患者患有感音神经性听力损失，并且 NF2 患者相关的耳蜗神经受损使得听觉脑干植入 (ABI) 成为一种有吸引力的治疗选择。该设备的长期结果和好处仍在探索中。方法 对 2017 年 11 月至 2022 年 8 月期间在单机构三级中心的 11 名 ABI 接受者进行回顾性分析。纳入诊断为 NF2 并因并发前庭神经鞘瘤 (VS) 接受切除术的患者。审查了测听前和测听后的评估。评估包括纯音听力测试和言语测试。结果 我们的队列包括 11 名患者，中位年龄为 34 岁。所有患者均接受经迷路入路种植体植入并同时进行 VS 切除。VS 的平均肿瘤大小为 2.87 cm。术前，8 名患者的纯音平均值在 110 dB 时无反应，2 名患者处于轻度至中度听力损失（25–45 dB）范围内，1 名患者的 PTA 严重丧失（92 dB）。术后，9 名（81%）患者 PTA 有所改善。总共有 7 名患者报告 ABI 激活后出现轻微副作用，包括头晕 (n = 2)、耳鸣 (n = 1) 以及腹部和下肢刺痛感 (n = 3)。在 7 名具有早期言语感知 (ESP) 评分的患者中，5 名患者的评分 >75%，表明仅通过 ABI 进行听觉刺激即可检测模式感知的听觉能力。结论 11 名患者中有 9 名从 ABI 植入中获益。这些发现表明，在同时 VS 切除期间放置 ABI 可以为 NF2 患者提供显着的听力益处。