平均纯音听阈正常或轻度听力损失听神经病患者的临床特征研究  

Study on the clinical characteristics of auditory neuropathy patients with normal pure tone average or mild hearing loss

作　　者：施韬 王洪阳 李进 谢林怡 熊芬 兰兰 王大勇 王秋菊 Shi Tao;Wang Hongyang;Li Jin;Xie Linyi;Xiong Fen;Lan Lan;Wang Dayong;Wang Qiuju(Department of Audio-Vestibular Medicine,Senior Department of Otolaryngology-Head and Neck Surgery,the Sixth Medical Center of Chinese PLA General Hospital,Beijing 100048,China;State Key Laboratory of Hearing and Balance Science,National Clinical Research Center for Otolaryngologic Diseases,Beijing 100853,China)

机构地区：[1]中国人民解放军总医院第六医学中心耳鼻咽喉头颈外科医学部耳鼻咽喉内科,北京100048 [2]听觉与平衡觉全国重点实验室、国家耳鼻咽喉疾病临床医学研究中心,北京100853

出　　处：《中华耳鼻咽喉头颈外科杂志》2024年第5期439-446,共8页Chinese Journal of Otorhinolaryngology Head and Neck Surgery

基　　金：国家重点研发计划(2023YFC2509800,2023YFC2508400,2023YFF1203504);国家自然科学基金优秀青年基金项目(82222016)。

摘　　要：目的:探究纯音测听气导平均听阈(pure-tone average,PTA)正常或轻度听力损失的听神经病(auditory neuropathy,AN)患者的临床特征及其听力变化规律。方法:研究对象来自听神经病临床诊断和干预多中心研究(注册号:ChiCTR2100050125),AN诊断标准依照2022年中国听神经病临床实践指南。筛选首诊纯音测听平均听阈(500、1000、2000、4000 Hz)正常或存在轻度听力损失的AN患者,分为纯音平均听阈正常组(PTA-Normal组,即PTA N组,平均听阈<20 dBHL)和轻度听力损失组(PTA-Mild hearing loss组,即PTA M组,平均听阈20~35 dBHL),观察记录患者的听力学特征、临床表现和伴随症状以及随访结果,采用GraphPad Prism 8及SPSS 20.0软件进行数据分析。结果:共纳入纯音平均听阈正常或轻度听力损失的AN患者75例(150耳),其中PTA N组19例(38耳),男12例、女7例,发病年龄(16.9±4.5)岁(均数±标准差,下同)、检测年龄为(22.1±5.8)岁;PTA M组共56例(112耳),男29例、女27例,发病年龄(16.2±7.9)岁、检测年龄(23.9±9.0)岁。两组患者首诊低频(125~500 Hz)平均听阈显著升高;86.0%(126/150)的患耳听性脑干反应(ABR)波形缺失;PTA N组言语识别率为71.8%±22.4%,PTA M组言语识别率为58.1%±29.3%;PTA N组耳蜗电图-SP/AP为0.98±0.47,PTA M组为1.07±0.63。75例AN患者中40例(53.3%)存在耳鸣,部分患者还伴有其他系统功能异常。29例患者完成随访,其中PTA N组10例,PTA M组19例。短期随访(<3年),29例患者听阈均未发生明显变化;随着病程延长(>3年),PTA N组趋向于低频听力先下降,而PTA M组则表现为高频听力先下降,PTA N组250 Hz与PTA M组4000 Hz听阈均显著升高。结论:纯音平均听阈正常或轻度听力损失的AN患者存在ABR、耳蜗电图、言语识别率等听力学检查结果异常,临床实践中应组合运用各项听力学检查对其进行综合评估;随着病程延长,这部分患者的听力逐渐下降,应动态评估其听力改变。Objective The purpose of this study was to analyze the clinical characteristics of auditory neuropathy(AN)patients with normal hearing or mild hearing loss.Methods Data from Multicenter Study on Clinical Diagnosis and Intervention of Acoustic Neuropathy(registration number:ChiCTR2100050125).According to the Chinese clinical practice guideline of auditory neuropathy(version 2022),these patients divided into two groups:the normal hearing group(PTA Normal,PTAN group,the average hearing threshold<20 dB HL)and the mild hearing loss group(PTA Mild hearing loss,PTAM group,the average hearing threshold between 20-35 dBHL).The audiology characteristics,clinical features,and follow-up were analyzed.Data analysis was conducted using GraphPad Prism 8 and SPSS 20.0 software.Results A total of 75 AN with normal hearing or mild hearing loss were included in this study.The PTAN group consisted of 19 patients(38 ears),including 12 males and 7 females.The average onset age was(16.9±4.5)years old,while the test age was(22.1±5.8)years old for PTAN group.The PTAM group consisted of 56 patients(112 ears),including 29 males and 27 females.The average onset age was(16.2±7.9)years old,while the test age was(23.9±9.0)yeas old for PTAM group.The average hearing threshold of low frequency(0.125-0.5 kHz)was significantly decreased.ABR disappeared in 86.00%(126/150)of the patients.The speech recognition rate was 71.80±22.44%in the PTAN group and 58.08±29.28%in the PTAM group.-SP/AP was 0.98±0.47 in the PTAN and 1.07±0.63 in PTAM group;40(53.33%)patients had tinnitus.29 patients(58 ears)were followed up,including 10 patients(20 ears)in the PTAN group and 19 patients(38 ears)in the PTAM group.There was no significant change in hearing threshold in short-term follow-up(<3 years).With the extension of the disease duration(>3 years),the PTAN group tended to decrease at low frequency,and the PTAM group decreased at high frequency first.The hearing threshold at 0.25 kHz in the PTAN group and 4 kHz in the PTAM group decreased significantly.Co

关 键 词：听神经病 测听法 纯音 测听法 言语 诱发电位 听觉 脑干 

分 类 号：R764[医药卫生—耳鼻咽喉科]