de Cos Víctor, Gibson Madeline, Li Vivienne, La Monte Olivia, Moshtaghi Omid, Dixon Peter, Khan Usman, Friedman Rick, Schwartz Marc S
University of California San Diego School of Medicine, La Jolla, California, United States.
Department of Otolaryngology-Head and Neck Surgery, UC San Diego Medical Center, San Diego, California, United States.
J Neurol Surg B Skull Base. 2024 Jan 29;86(1):6-12. doi: 10.1055/a-2236-0113. eCollection 2025 Feb.
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. 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. 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 ( = 2), tinnitus ( = 1), and abdominal and lower extremity tingling sensation ( = 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. 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.
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损伤使听觉脑干植入物（ABI）成为一种有吸引力的治疗选择。该设备的长期效果和益处仍在探索中。
对2017年11月至2022年8月期间在一家单机构三级中心接受ABI植入的11名患者进行了回顾性研究。纳入诊断为NF2并因同时性前庭神经鞘瘤（VS）接受切除术的患者。回顾了术前和术后听力评估。评估包括纯音听力测定和言语测试。
我们的队列包括11名患者，中位年龄为34岁。所有患者均采用经迷路入路进行植入物放置并同时切除VS。VS的平均肿瘤大小为2.87厘米。术前，8名患者的纯音平均值在110分贝时无反应，2名患者处于轻度至中度听力损失（25 - 45分贝），1名患者的纯音平均听阈为重度损失（92分贝）。术后，9名（81%）患者的纯音平均听阈有所改善。总共有7名患者在ABI激活后报告了轻微副作用，包括头晕（n = 2）、耳鸣（n = 1）以及腹部和下肢刺痛感（n = 3）。在有早期言语感知（ESP）评分的7名患者中，5名患者的评分>75%，表明仅通过ABI进行听觉刺激时能够检测模式感知的听觉能力。
11名患者中有9名从ABI植入中获益。这些发现表明，在同时切除VS期间植入ABI可为NF2患者提供显著的听力益处。
