Objective:To analyze the clinical characteristics of unilateral acoustic neuroma（AN） with normal hearing, so as to provide evidence for early identification AN. Methods:Clinical datas from 73 patients of unilateral AN with normal hearing of Otorhinolaryngology Head and Neck Surgery of Beijing Tiantan Hospital affiliated of Capital Medical University from August 2019 to April 2022 admitted to department were retrospectively analyzed. All patients underwent pure tone audiometry（PTA）, speech discrimination score（SDS）, auditory brainstem response（ABR）, distortion product otoacoustic emission（DPOAE） and head enhanced MRI. Results:The incidence of normal hearing among patients with AN was 10.7%. Male∶female=1∶2.2; the mean age of the patients was（37.3±9.4） years; the mean tumor size was（24.2±11.2） mm. Tinnitus was the most common reason for visit; the patients who had headache and dizziness had larger tumors. Surgery was the main treatment, and the patients who underwent surgery had larger tumors than those of follow-up. Heterogeneous tumors were the most common type of MRI, homogeneous tumors were smaller than heterogeneous and cystic tumors. The sensitivity of ABR in the diagnosis of AN with normal hearing was 95.9%, and that of ≥20 mm tumors was 100%; prolonged Ⅴ-waves were the most common, patients with Ⅴ-wave deletion had larger tumors than those with normal or prolonged Ⅴ-waves. Patients who had the longer the Ⅴ-wave and the longer difference between Ⅰ-Ⅴ wave had larger tumors. DPOAE was not elicited at full frequency in 11 patients. There was no statistically significant difference in age among patients with different symptoms, treatments, types of MRI, ABR and DPOAE. Conclusion:AN of normal hearing was most common in 30-39 years old women. Patients had different symptoms, phenotypes of MRI and ABR. Patients with normal hearing who had tinnitus, dizziness, headache, facial paraesthesia, and recovery after sudden haring loss can be further examination of ABR and DPOAE for early identification AN. The sensitivity of ABR in diagnosis of hearing normal AN was 95.9%, and the abnormal type of Ⅴ-wave is related to tumor size.
目的：分析听力正常单侧听神经瘤患者临床特征，以期为早期识别听神经瘤提供依据。 方法：回顾性分析2019年8月—2022年4月间首都医科大学附属北京天坛医院耳鼻咽喉头颈外科接诊资料完整、听力正常的73例单侧听神经瘤患者听力相关检查[纯音听阈测试、言语识别率、听性脑干反应（ABR）、畸变产物耳声发射（DPOAE）]和头部增强MRI检查结果。 结果：听力正常的听神经瘤患者占比为10.7%；男女比例为1：2.2；平均年龄（37.3±9.4）岁；肿瘤直径（24.2±11.2） mm。耳鸣是最常见的就诊原因；因头痛、头晕就诊者肿瘤较大。手术为主要治疗方式，手术疗法者肿瘤大于随诊者。MRI信号不均者最多见，信号均匀者肿瘤小于信号不均和囊性变者。ABR的敏感度为95.9%，≥20 mm肿瘤的敏感度为100%；Ⅴ波延长者最常见；Ⅴ波缺失者肿瘤大于Ⅴ波正常或延长者，Ⅴ波潜伏期和双耳Ⅰ～Ⅴ波间期差延长越多预示肿瘤越大。11例患者DPOAE未全频引出。不同就诊原因、不同治疗方式、不同MRI类型、不同ABR表型以及DPOAE是否全频引出患者间年龄差异无统计学意义。 结论：听力正常的听神经瘤患者多见于30~39岁女性，患者症状、MRI类型和ABR表型各异。伴有耳鸣、头晕、头痛或面部感觉异常以及突聋后痊愈等听力正常者，可行ABR和DPOAE等进一步检查早期识别听神经瘤。ABR诊断听力正常听神经瘤的敏感性为95.9%，Ⅴ波异常程度与肿瘤大小有关。.
Keywords: acoustic neuroma; auditory brainstem response; distortion product otoacoustic emission.
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