NF2相关听神经休息症（NF2-SWN）患者采用贝伐单抗维持治疗的前瞻性数据缺乏。在这项前瞻性多中心II期研究中，我们评估了贝伐单抗对NF2相关听神经休息症和前庭神经瘤（VS）导致听力损失的儿童和成人进行维持治疗的疗效、安全性和耐受性。在诱导治疗后，参与者每3周接受一次贝伐单抗5mg/kg，治疗至少持续18个月。参与者监测听力、肿瘤大小和生活质量（QOL）的变化以及不良反应。听力损失被定义为与基线研究相比单词识别分数（WRS）或纯音平均值的显著下降；肿瘤增长被定义为与基线相比体积增加>20%。20名NF2-SWN患者（中位年龄23.5岁；范围12.5-62.5岁）在目标耳部位（中位WRS 70%，范围2-94%）接受了维持治疗贝伐单抗。在48周后，目标耳部位无听力损失的自由度为95％，72周后为89％，98周后为70％。在48周后，目标VS未出现肿瘤增长的自由度为94％，在72周后为89％，在98周后为89％。NF2相关QOL保持稳定，耳鸣相关烦恼减少。维持治疗贝伐单抗的耐受性良好，有三名参与者（15％）因不良反应停止治疗。在18个月的后续观察中，维持治疗贝伐单抗（每3周5mg/kg）与高听力和肿瘤稳定率相关。在这个人群中，没有发现与贝伐单抗相关的新不良反应。© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.

Prospective data on maintenance therapy with bevacizumab for persons with NF2-related schwannomatosis (NF2-SWN) is lacking. In this prospective multicenter phase 2 study, we evaluated the efficacy, safety, and tolerability of bevacizumab for maintenance therapy in children and adults with NF2-SWN and hearing loss due to vestibular schwannomas (VS).Following induction therapy, participants received bevacizumab 5 mg/kg every 3 weeks for 18 months. Participants were monitored for changes in hearing, tumor size, and quality of life (QOL), and for adverse events. Hearing loss was defined as a statistically significant decline in word recognition score (WRS) or pure tone average compared to study baseline; tumor growth was defined as >20% increase in volume compared to baseline.Twenty participants with NF2-SWN (median age 23.5 years; range, 12.5-62.5 years) with hearing loss in the target ear (median WRS 70%, range 2-94%) received maintenance bevacizumab. Freedom from hearing loss in the target ear was 95% after 48 weeks, 89% after 72 weeks, and 70% after 98 weeks. Freedom from tumor growth in the target VS was 94% after 48 weeks, 89% after 72 weeks, and 89% after 98 weeks. NF2-related QOL remained stable for 98 weeks whereas tinnitus-related distress decreased. Maintenance bevacizumab was well tolerated, with three participants (15%) discontinuing treatment due to adverse events.Maintenance bevacizumab (5 mg/kg every 3 weeks) is associated with high rates of hearing and tumor stability during 18 months of follow-up. No new unexpected adverse events related to bevacizumab were identified in this population.© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.