中耳肌阵挛性耳鸣56例临床分析

Diagnosis and treatment strategies of 56 cases of middle ear myoclonus

目的分析中耳肌阵挛性耳鸣的临床特征及治疗方法。方法回顾性分析2019年9月至2021年8月山东大学山东省耳鼻喉医院收治的中耳肌阵挛性耳鸣患者56例, 其中男23例, 女33例;年龄6~75岁, 中位年龄35岁;单侧耳鸣47例, 双侧耳鸣9例;耳鸣时间20 d至8年。记录耳鸣的声音特点、诱发因素、性质(频率等)、耳鸣时鼓膜情况、听力学相关检查(长时程声导抗、镫骨肌声反射、纯音听阈、短增量敏感测试、重振试验、响度不适阈)结果、前庭功能检查、面肌电图、影像学检查。采用口服卡马西平和/或手术治疗, 随访6~24个月, 观察治疗后耳鸣变化。结果耳鸣声音多样, 有"踩雪声、节律性击鼓声、白噪声"等。诱发因素包括外部声音、体位变化、触摸面部及耳周皮肤、讲话及咀嚼、眨眼等。单一因素诱发44例;2种及以上因素诱发9例;无明确诱发因素1例;1例耳鸣伴癫痫;1例外伤性面神经麻痹行面神经减压术后耳鸣患者于耳廓活动时可诱发患侧耳鸣。耳镜检查可见与耳鸣频率一致的鼓膜扇动12例, 长时程声导抗可见与耳鸣频率一致的波形;耳镜检查未见鼓膜扇动, 长时程声导抗亦可见与耳鸣频率一致波形者7例, 但波形改变速率较可见鼓膜扇动者相比更快。56例患者听力均无改变。1例耳鸣合并癫痫的6岁患儿, 予抗癫痫药物(托吡酯)后耳鸣消失;1例外伤性面神经麻痹行面神经减压术后耳鸣患者, 未行特殊治疗。54例口服卡马西平治疗的患者中症状完全控制10例;症状减轻23例;无效21例。卡马西平治疗无效的21例患者中采用手术治疗8例, 7例术后耳鸣消失, 1例先后行3次手术, 第3次手术后随访6个月, 未再出现耳鸣;余13例因个人原因拒绝手术治疗。结论中耳肌阵挛性耳鸣患者的耳鸣声音多样化、诱发因素多样化, 口服卡马西平等镇静类药物部分患者有效, 无效者可行手术治疗。

Objective To analyze the clinical characteristics and treatment of middle ear myoclonus.Methods Fifty-six cases of middle ear myoclonus were enrolled in Shandong Provincial ENT Hospital,Shandong University from September 2019 to August 2021,including 23 males and 33 females.The age ranged from 6 to 75 years,with a median age of 35 years;Forty-seven cases were unilateral tinnitus,nine cases were bilateral tinnitus.The time of tinnitus ranged from 20 days to 8 years.The voice characteristics,inducing factors,nature(frequency)of tinnitus,tympanic membrane conditions during tinnitus,audiological related tests,including long-term acoustic tympanogram,stapedius acoustic reflex,pure tone auditory threshold,short increment sensitivity test,alternate binaural loudness balance test,loudness discomfort threshold,vestibular function examination,facial electromyography,and imaging examination were recorded.Oral carbamazepine and/or surgical treatment were used.The patients were followed up for 6-24 months and the tinnitus changes were observed.Results Tinnitus was diverse,including stepping on snow liking sound,rhythmic drumming,white noise,and so on.The inducing factors included external sound,body position change,touching the skin around the face and ears,speaking,chewing and blinking,etc.Forty-four cases were induced by single factor and 9 cases were induced by two or more factors.There was no definite inducing factor in 1 case.One patient had tinnitus with epilepsy.One case of traumatic facial paralysis after facial nerve decompression could induce tinnitus on the affected side when the auricle moved.Tympanic membrane flutter with the same frequency as tinnitus was found in 12 cases by otoscopy,and the waveform with the same frequency as tinnitus was found by long-term tympanogram examination.There were 7 patients with no tympanic membrane activity by otoscopy,the 7 cases also with the same frequency of tinnitus by long-term tympanogram examination,but the change rate of the waveform was faster than that of the patients with tympanic membrane flutter.All patients with tinnitus had no change in hearing.One case of tinnitus complicated with epilepsy(a 6-year-old child)was treated with antiepileptic drug(topiramate)and tinnitus subsided.One case suffered from tinnitus after facial nerve decompression for traumatic facial paralysis was not given special treatment.Fifty-four cases were treated with oral drug(carbamazepine),of which 10 cases were completely controlled and 23 cases were relieved;21 cases were invalid.Among the 21 patients with no effect of carbamazepine treatment,8 patients were treated by surgery,7 patients had no tinnitus after surgery,1 patient received three times of operation,and the third operation was followed up for 6 months,no tinnitus occurred again.The other 13 cases refused the surgical treatment due to personal reasons.Conclusions Middle ear myoclonus tinnitus and the inducing factors manifestate diversity.Oral carbamazepine and other sedative drugs are effective for some patients,and surgical treatment is feasible for those who are ineffective for medication.