Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2.
Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2.
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2 型神经纤维瘤病中小体积耳蜗前庭神经鞘瘤的听力学自然史。
DOI：
10.1097/mao.0000000000001690
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发表时间：
2018
期刊：
影响因子：
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通讯作者：
Kim,HungJeffrey
中科院分区：
文献类型：
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作者：
deTorres,AlvinT;Brewer,CarmenC;Zalewski,ChrisK;King,KellyA;Walker,Robert;Scott,GretchenC;Asthagiri,AshokR;Chittiboina,Prashant;Kim,HungJeffrey
目的：描述2型神经纤维瘤病（NF 2）中小体积（<1，000 mm 3）、未经治疗的耳蜗前庭神经鞘瘤（CVS）患者耳听力自然进展的特征。研究设计：前瞻性纵向队列研究。设置：第四医学研究所。患者：2006年7月至2016年7月观察的71例小型、未经治疗的CVS患者的111耳。干预：系列听力测试，包括纯音测听、言语测听和磁共振成像（MRI）。结果测量：记录0.5、1、2和4 kHz的四频纯音平均值（4f-PTA）和单词识别评分（WRS）。将其变化与CVS体积随时间的MRI变化进行比较。时间显着听力损失（10分贝损失在4f-PTA）和WRS的基础上95%的临界differentiation.Results：线性回归分析显示，基线听力水平（4f-PTA）和内听道（IAC）肿瘤体积显着相关性与年听力下降率（AHDR）（p= 0.003，p= 0.0004）。基线时的听力水平和肿瘤体积与AHDR相关，而肿瘤体积增长率则不相关。双向方差分析发现，基于基线听力水平，AHDR、显著听力损失风险和WRS关键差异风险存在显著差异（异常或正常）和IAC肿瘤体积（大于或小于200 mm 3）。基线听力正常和小IAC肿瘤成分的受试者的AHDR较低，显著听力损失的风险较低，可能需要保守治疗，而基线听力损失和大IAC体积的存在导致ADHR较高，进一步的风险较高。听力损失，并可能受益于早期治疗干预。
Objective:To characterize the audiometric natural progression in patient-ears with small volume (< 1,000 mm 3), treatment-naïve cochleovestibular schwannomas (CVSs) in Neurofibromatosis Type 2 (NF2).Study Design:Prospective, longitudinal cohort study.Setting:Quaternary medical research institute.Patients:One hundred eleven ears in 71 NF2 patients with small, treatment-naïve CVSs observed from July 2006 to July 2016.Intervention:Serial audiometric testing, including pure tone audiometry, speech audiometry, and magnetic resonance imaging (MRI).Outcome Measures:Four-frequency pure tone average (4f-PTA) of 0.5, 1, 2, and 4 kHz and word recognition score (WRS) were recorded. Their changes were compared with MRI changes in CVS volume over time. Times to significant hearing loss (10 dB loss in 4f-PTA) and WRS based on 95% critical difference were measured.Results:Linear regression analysis showed a significant correlation with baseline hearing level (4f-PTA) and internal auditory canal (IAC) tumor volume to annual hearing decrease rate (AHDR)(p= 0.003, p= 0.0004). Hearing level at baseline and tumor volume correlate with AHDR while tumor volume growth rate does not. Two-way analysis of variance found significant differences in AHDR, risk of significant hearing loss, and risk of critical difference in WRS based on baseline hearing level (abnormal or normal) and IAC tumor volume (greater or less than 200 mm 3).Conclusion:Subjects with normal baseline hearing and small IAC tumor component had a low AHDR and low risk of significant hearing loss and may warrant conservative management while the presence of baseline hearing loss and large IAC volume resulted in higher ADHR and greater risk for further hearing loss and may benefit from early treatment interventions.