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鼓室内应用甲基泼尼松龙和庆大霉素治疗自身免疫性膜迷路积水
目的:了解和证实自身免疫性膜迷路积水(AIEH)患者鼓室内注射甲基泼尼松龙(MP)或庆大霉素(GM)的治疗效果和不良反应.方法:诊断AIEH 62例,随机分为MP组29例和GM组33例.经鼓室内置入临时导管,隔日注射MP 2周或隔日注射GM 4次(出现听损者立即停止).治疗前和治疗后1周分别进行听觉和前庭功能测定.结果:依据眩晕控制和听损改善评定标准,MP组和GM组有效率分别为69%和64%.52例膜迷路积水试验转变成阴性.两组优势偏向发生率明显减少,MP组管麻痹发生率亦明显减少.结论:鼓室内应用MP治疗AIEH具有与GM相似的效果.作为针对内耳自身免疫的病因学治疗药物,局部应用MP不仅能够较好地控制临床症状,而且有助于内耳生理功能的恢复.
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迟发性膜迷路积水的诊断
目的:探讨迟发性膜迷路积水(DEH)的诊断手段及意义.方法:15例DEH患者,分别行纯音听阈及耳蜗电图检查、前庭双温试验和前庭诱发的肌源性电位检查(VEMP),用以诊断及判断DEH的侧别和病变累及范围.结果:15例患者均为中～重度以上感音神经性聋.积水与听力下降同侧10例,对侧4例,双侧1例.水平半规管和球囊均有积水5例,水平半规管积水6例,球囊积水2例.VEMP异常者7例,其中1例患侧p13-n23振幅消失,2例p13潜伏期延长,4例患侧p13-n23低振幅.结论:DEH的诊断除了纯音测听外,耳蜗电图检查、前庭双温试验和VEMP检查是重要的实验室检查,应列为诊断常规.
关键词： 迟发性膜迷路积水 聋 前庭诱发的肌源性电位 耳蜗电图 -
半规管填塞及人工耳蜗植入共同治疗迟发性膜迷路积水1例
A 46-year-old female with profound sensorineural hearing loss in her left ear from childhood developed tinnitus and fluctuating hearing loss on the right side 8 years ago.Four years later,paroxysmal episodes of rotatory vertigo occurred with gradually increased frequency,lasting from half an hour to 2 hours and accompanied with nausea and vomiting.Audiometric test revealed severe sensorineural hearing loss in both of her ears.The tympanograms showed type A on both sides.The threshold of auditory brainstem response was 97 dBnHL in her right ear while no waves could be educed on her left side.Weakened right-sided vestibular function was confirmed on caloric testing and vestibular-evoked myogenic potential.A normal cochlear morphology and clearness cerebellopontine angle were shown in the MR imaging scan.She was diagnosed with Delayed Endolymphatic Hydrops.
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迟发性膜迷路积水的研究现状
Delayed endolymphatic hydrops (DEH) is a disease entity first described by Kamei and named by Schuknecht,defined as profound sensorineural deafness at early stage and after several years started to appear clinical feature of endolymphatic hydrops such as vertigo,aural fullness like Meniere's disease or fluctuating hearing loss in the contralateral ear.DEH can be classified into ipsilateral type,contralateral type and bilateral type.Although DEH has low incident rate,there are many kinds of etiology and audiology and vestibular tests.Up to now,a lot of literatures about etiology,diagnose,clinical manifestation,relevant examination and therapy of DEH were published abroad.In this review,we will make a summary of research status of DEH.
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迟发性膜迷路积水的临床研究现状
迟发性膜迷路积水(delayed endolymphatic hydrops,DEH)是类似梅尼埃病但又有明确病因的疾病.1975年Nadol等首次报道,1978年Schuknecht 对其分类和命名进行了全面阐述,DEH指患者原有一侧耳极重度感音神经性聋,以后又发生同侧或对侧膜迷路积水,从耳聋发生到以后出现膜迷路积水症状的间隔期可长达数十年.
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内耳显影对迟发性膜迷路积水诊断的意义
目的：明确鼓室内钆造影剂注射后内耳显影对迟发性膜迷路积水（delayed endolymphatic hydrops ， DEH）诊断的临床价值，探讨迟发性膜迷路积水的病理机制。方法10例DEH患者行双鼓室钆造影剂注射24小时后行三维快速液体衰减反转恢复（three dimensional fluid－attenuated inversion recovery ，3D －FLAIR）序列和三维实时反转恢复（three dimensional real inversion recovery ，3D－real IR）序列扫描成像，分析患者内淋巴积水的范围及严重程度。结果10例DEH患者中9例为同侧型，1例为对侧型，均显示单侧或双侧内淋巴积水征象，其中9例同侧型DEH患者中仅1例（11．1％）患侧耳前庭内淋巴显示轻度积水，其余8例（88．9％）均显示患侧前庭内淋巴重度积水；8例（88．9％）同侧型DEH患者患侧耳蜗内淋巴积水，而1例（11．1％）同侧型DEH患者患侧耳蜗内淋巴无积水，9例同侧DEH患者对侧耳均未见前庭、耳蜗内淋巴积水。1例对侧型DEH患者的双侧前庭内淋巴重度积水，右侧耳蜗内淋巴轻度积水，而左侧耳蜗未见积水。结论内淋巴积水是DEH的主要病理因素，鼓室内钆注射后内耳显影能直观地反映出DEH患者内淋巴积水的范围及严重程度。
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迟发性膜迷路积水的诊断与治疗
目的 探讨迟发性膜迷路积水的诊断和治疗.方法 回顾性分析37例(40耳)迟发性膜迷路积水患者的临床资料,包括纯音测听、前庭诱发的肌源性电位(vestibular evoked myogenic potentials,VEMP)、高刺激ABR、前庭双温试验等结果.主要予以利尿剂、血管扩张剂等药物治疗.结果 37例患者均为中、重度以上感音神经性聋.膜迷路积水与听力下降同侧25例,对侧9例,双侧3例.水平半规管和球囊均有积水10例,水平半规管积水9例,球囊积水15例,VEMP和双温试验均正常3例.VEMP正常12例,异常者25例,其中6例患侧p13-n23振幅消失,5例p13潜伏期延长,14例患侧p13-n23低振幅.37例患者中一周内完全控制眩晕发作18例;眩晕明显减轻、二周后完全控制17例;2例行化学迷路切除后眩晕未再发作.结论 迟发性膜迷路积水的诊断应依据纯音测听、耳蜗电图、前庭双温试验和前庭诱发肌源性电位等检查结果综合判定;治疗以药物治疗为主,利尿剂和血管扩张剂是主要治疗药物.
