梅尼埃病内淋巴积水影像学测定

目的采用经咽鼓管鼓室内导入造影剂钆喷酸葡胺稀释液、内耳三维快速液体衰减反转恢复磁共振扫描（three dimensional fluid attenuated inversion recovery magnetic resonance imaging,3D-FLAIR MRI）,探讨梅尼埃病内淋巴液截面积的测定方法。方法 5例梅尼埃病患者和10名健康受试者经咽鼓管鼓室内导入钆喷酸葡胺稀释液、24h后行内耳3D-FLAIR MRI。耳蜗部采用斜矢位蜗轴截取平面,测量耳蜗底转内淋巴间隙和总淋巴间隙面积;前庭部采用冠状位前庭长轴垂直截取平面,测量前庭部内淋巴间隙（椭圆囊和球囊的面积之和）和总淋巴间隙。结果 5例梅尼埃病患者单侧患耳和10名健康受试者受试耳的内耳3D-FLAIR MRI显示内耳的耳蜗、前庭和（或）半规管的外淋巴间隙广泛可见造影剂钆增强区影,可以区分内、外淋巴间隙的边界和清晰显像内淋巴间隙的情况。5例梅尼埃病患者的耳蜗和前庭部的内淋巴间隙评定值分别为0.36±0.08和0.40±0.12;全组梅尼埃病患者（5例）和健康受试者（10名）之间耳蜗的内淋巴间隙评定值比较有统计学差异（P〈0.05）。结论经咽鼓管鼓室内导入造影剂钆、内耳3D-FLAIR MRI,采用客观统一的内淋巴间隙核磁采集和测定方法可以评定梅尼埃病内淋巴积水的情况。     

梅尼埃病内淋巴积水机理研究进展

目的梅尼埃病（Ménière’s disease,MD）的病因至今尚未完全阐明,近年来人们在梅尼埃病内淋巴积水(endolymphatic hydrops,ELH)机理方面有了更加深入的研究。本文从内淋巴清除率、内淋巴转运障碍、激素影响、内耳压力调节障碍、血流动力学失衡、过敏机制、自身免疫机制、病毒感染及遗传倾向等几个方面对其进行了综述。方法结果结论     

链霉素迷路灌注术治疗梅尼埃病和迟发性内淋巴积水

目的：研究链霉素迷路灌注术（ＳＰＬ）治疗梅尼埃病的临床疗效。方法：对１３例梅尼埃病和３例迟发性内淋巴积水患者用ＳＰＬ进行治疗。结果：随访５￣７年,平均６．３年,眩晕完全控制９例,基本控制４例,部分控制２例,无效１例;听力提高１例,无变化５例,下降１０例;耳鸣改善３例,不变５例,加重８例。结论：链霉素迷路灌注术是一种安全、疗效肯定的治疗梅尼埃病的方法,但部分患者听力可能受损,耳鸣可能加重。     

梅尼埃病内淋巴积水程度与临床特征的相关性分析

目的 探讨梅尼埃病(meniere’s disease,MD)患者内淋巴积水(endolymphatic hydrops,EH)程度与临床特征的相关性。方法 研究对象为2020年12月—2022年3月就诊北京清华长庚医院的31例MD患者,所有患者均行纯音测听(pure tone audiometry,PTA)、耳蜗电图(electrocochleogram,ECochG)、耳鸣分级、眩晕残障程度评定量表(dizziness handicap inventory,DHI)评分及内耳钆造影MRI扫描。据内耳MRI显像,采用目测4分法,以3D FLAIR序列评估前庭积水程度,以3D real IR序列评估耳蜗积水程度,采用Spearman秩相关检验和Fisher精确检验分析EH程度与患者临床特征的相关性。结果 不同EH程度患者的性别、年龄、侧别及病程比较,差异无统计学意义(P>0.05);Spearman相关性分析显示,耳蜗积水程度与耳鸣分级、低频听阈、平均听阈及疾病分期呈正相关(r_s=0.517、0.438、0.439、0.378,P<0.05),与ECo...     

关于梅尼埃病内淋巴积水定位诊断的文献综述

梅尼埃病(Meniere's Disease,MD)的临床表现为反复发作的旋转性眩晕、波动性听力下降、耳鸣和耳闷胀感,主要病理改变为内淋巴积水.目前MD诊断比较困难,主要依赖临床症状,辅以听力学及一系列前庭功能检查.本文就MD内淋巴积水的定位诊断展开综述.     

梅尼埃病的历史

法国人Prosper Meniere于1861年因首先报告内耳疾病引起眩晕、耳鸣、耳聋而声名远扬.具有眩晕、耳鸣、耳聋等症状的这类疾病被冠以Meniere的名字.自从Meniere这一名字传入中国之后,一直被翻译为美尼尔,以Meniere的名字命名的疾病被称为美尼尔氏病或美尼尔氏综合症.1989年,中国国家科学技术名词审定委员会认为,法国人名Meniere翻译为梅尼埃更为贴切,因此,目前统一译为梅尼埃病.     

内淋巴囊引流术治疗梅尼埃病

报告以内淋巴囊引流术治疗２４例梅尼埃病的结果，其中１４例随访２－３年，眩晕控制１０例，好转３例，无效１例。详细介绍了内淋巴囊引流术的手术方法，并对手术治疗的机理，适应证选择，以及并发症的以预防进行讨论。     

梅尼埃病与内淋巴的内环境失衡

由离子渗透压所形成的容积渗克分子浓度梯度和由内淋巴囊糖蛋白所形成的胶体渗透压是驱使内淋巴液纵向流动至内淋巴囊的动力，这种动态平衡状态是由许多内耳酶的调节所维持。一旦平衡被破坏，就可能产生膜迷路积水。本就近年来关于内淋巴液流变学变化与梅尼埃闰膜迷路积水的关系进行综述和探讨。     

内淋巴管重吸收功能与梅尼埃病相关性研究进展

梅尼埃病基本的病理改变是内淋巴积水。关于内淋巴积水的发生有多种推测,任何因素使内淋巴液的生成超过重吸收均可能导致内淋巴积水,内淋巴囊一直被认为是内淋巴液的主要吸收部位。然而,越来越多的研究显示内淋巴管在内淋巴液重吸收中的作用可能比内淋巴囊更重要。本文将内淋巴管的结构、功能及其在梅尼埃病发病机制和治疗中的作用做一综述。     

内淋巴囊神经分布与梅尼埃病相关性

梅尼埃病(Memem’s disese，MD)的基本病理改变为膜迷路积水。能够引起内淋巴囊分泌和吸收功能障碍的许多因素都可能引起膜迷路积水，而其中的一个重要因素就是局部血流的调节，它可被自主神经所影响。因此，自主神经功能紊乱自然就成为膜迷路积水或梅尼埃病的可能原因之一。     

Correlation of semi-quantitative findings of endolymphatic hydrops in MRI with the audiometric findings in patients with Meniere's disease

Purpose: To investigate the correlation between vestibular hydrops (VH), cochlearhydrops (CH), vestibular aqueduct non-visibility (VANV), and visually increased perilymphatic enhancement (VIPE) with the findings of pure-tone audiometry (PTA) in Meniere’s disease (MD) patients.Methods: In this cross-sectional study, 53 ears belonging to 48 patients were divided into two groups and evaluated. In group “MD patients,” there were 24 ears of 19 patients diagnosed with the definite MD (14 patients with unilateral and 5 patients withbilateral involvements). The “control group” consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma. All the patients underwent 2 sessions of temporal bone MRI using the same 3T system: an unenhanced axial T1, T2, and 3D-FLAIR MRI, an intravenous gadolinium-enhanced axial T1 fat-sat, and 4 h after the injection, an axial 3D-T2 cube and 3D-FLAIR session. VH, CH, VANV, and VIPE were assessed. Subsequently, the correlation between EH indices and PTA findings (in three frequency domains of low, middle, and high) were evaluated, and the predictive value of MRI was calculated.Results: VH was significantly correlated with the hearing threshold in the low, middle, and high-frequency domains. CH was also correlated with the hearing threshold in the low and middle domains. Contrarily, VIPE was not associated with hearing thresholds, and VANV was only correlated with the hearing threshold in low frequencies.Conclusion: The grade of VH, CH, and VANV were significantly correlated with the hearing thresholds in PTA.     

经鼓室钆注射内耳成像磁共振检查在梅尼埃病中的应用

目的 对梅尼埃病(Meniere's disease)患者采用经鼓膜穿刺鼓室内注入对比剂钆、内耳三维快速液体衰减反转恢复磁共振(three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging,3D-FLAIR MRI)扫描,将影像学显示的膜迷路积水情况与临床听力及前庭功能检查结果进行比较,探讨经鼓室钆注射内耳成像磁共振在梅尼埃病诊断中的应用价值.方法 32例经临床确诊的单侧梅尼埃病患者,双侧耳均经鼓膜穿刺鼓室内注入对比剂钆喷酸葡胺稀释液,24 h后行3D-FLAIR MRI和三维平衡快速梯度回波磁共振扫描,观察双侧耳蜗、前庭和半规管外淋巴间隙的增强显影,对耳蜗底转鼓阶和前庭阶分别进行评分,并对前庭外淋巴间隙显影范围进行测量.患者常规行纯音测听、冷热试验、前庭诱发肌源性电位(vestibular evoked myogenic potential,VEMP)和耳蜗电图检查.将患侧耳蜗底转前庭阶评分及前庭外淋巴间隙显影范围分别按纯音测听、冷热试验、VEMP、耳蜗电图的检查结果分组进行比较,采用SPSS17.0统计软件进行数据分析.结果 全部患者内耳3D-FLAIR MRI均显示对比剂钆广泛分布于耳蜗、前庭和半规管的外淋巴间隙,可清晰对比显示内淋巴间隙.患侧耳蜗底转前庭阶评分值与健侧比较,差异具有统计学意义(Z=4.309,P＜0.05);患侧与健侧前庭外淋巴间隙显影范围(-x±s)分别为(6.04±2.89)mm2和(8.28±3.04) mm2,二者比较差异具有统计学意义(t =3.322,P ＜0.05).VEMP异常组患者与VEMP正常组比较,患侧前庭显影范围明显缩小,差异具有统计学意义(F=11.96,P＜0.05).耳蜗电图异常组患者与正常组比较,耳蜗底转前庭阶评分明显偏低,差异具有统计学意义(Z=3.17,P＜0.05).患侧耳蜗前庭阶评分及前庭显影范围与患者听力水平及冷热试验结果无明显相关.结论 经鼓室钆注射内耳3 D-FLAIRMRI可以区分内、外淋巴间隙的边界,显示膜迷路积水情况,可为梅尼埃病的诊断提供影像学参考.VEMP和耳蜗电图检查结果可能与前庭和耳蜗积水程度有一定相关性.     

Visualization of endolymphatic hydrops in patients with Meniere's disease

OBJECTIVE: Recently, there have been many reports of intratympanic gentamicin therapy for the treatment of intractable Meniere's disease. Intratympanic administration of steroids has also been used to treat sudden sensorineural hearing loss. We attempted to visualize how the intratympanically administered drug enters the inner ear. METHODS: Gadolinium hydrate diluted eightfold with saline was injected intratympanically through the tympanic membrane using a 23 G needle in nine patients with inner ear diseases. With a 3 Tesla magnetic resonance imaging (MRI) unit, three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) imaging was performed. RESULTS: 3D-FLAIR MRI clearly revealed that the gadolinium entered the perilymphatic space and delineated the perilymphatic and endolymphatic spaces of the inner ear. In patients with endolymphatic hydrops, the perilymphatic space surrounding the endolymph was small or had disappeared. Gadolinium appeared first in the scala tympani of the basal turn of the cochlea and the perilymphatic space of the vestibule. One day after the intratympanic injection of gadolinium, the gadolinium was observed in almost all parts of the perilymph. Six days after the intratympanic injection, the gadolinium had almost disappeared from the inner ear. CONCLUSION: We reported the first visualization of endolymphatic hydrops in patients with Meniere's disease. The relationship between the image of the endolymphatic space and functional tests, such as electrocochleography and vestibular-evoked myogenic potential, must be examined in the near future. It is important for the development of intratympanic drug therapies for inner-ear diseases to investigate how the drugs enter and leave the inner ear.     

Changes in endolymphatic hydrops in a patient with Meniere's disease observed using magnetic resonance imaging

We describe a case report of a patient with Meniere's disease whose changes in endolymphatic hydrops were observed using magnetic resonance imaging (MRI). Gadolinium was injected intratympanically through the tympanic membrane, and MRI scans performed with a 3-T MRI unit revealed endolymphatic hydrops inside the perilymphatic space filled with gadolinium. We evaluated the relationship between the image findings and hearing level. The correlation between the degree of endolymphatic hydrops observed by MRI and hearing level in patients with Meniere's disease offers a promising new method to study the progression of Meniere's disease.     

Visualization of endolymphatic hydrops and correlation with audio-vestibular functional testing in patients with definite Meniere's disease

Objectives

Visulization of endolymphatic hydrops has been reported using magnetic resonance imaging (MRI) in patients with Meniere's disease. However, the relationship between the endolymphatic hydrops visualized by MRI and audio-vestibular functional tests have not been sufficiently investigated, such as pure tone audiometry (PTA), electrocochleography (EcoG), and vestibular evoked myogenic potential (VEMP).

Methods

Here we attempted to visualize endolymphatic hydrops in patients with Meniere's disease using 3 Tesla (3 T)-MRI following intratympanic gadolinium-diethylene-triamnie petaaetic acidbis (Gd-DTPA) injection and correlated the presence of hydrops with auditory testing. Following intratympanic Gd-DTPA contrast injections in 26 patients diagnosed as having definite Meniere's disease, 3D-fluid-attenuated inversion recovery (3D-FLAIR) sequence imagings were performed. The PTA, EcoG, and VEMP testing was done bilaterally. Patients had typical results of auditory testing for Meniere's disease.

Results

The 3D-FLAIR MRI clearly demonstrated endolymphatic hydrops with signal voids in the cochlea (81%) and saccule (69%) in the definite Meniere's disease group. Auditory tests verified that the visualized cochlear hydrops (basal turn) was correlated with a high tone hearing threshold in PTA and an abnormal EcoG. Though the absence of VEMP did not correlate with vestibular hydrops on MRI, large vestibular hydrops were detected by imaging. This study demonstrates that endolymphatic hydrops could be visualized by 3D-FLAIR MRI in Meniere's disease patients.

Conclusion

Cochlea hydrops and vestibular (saccular) hydrops are readily visualized using these techniques. Hydrops, as visualized on MRI, may be a reliable means to diagnosis Meniere's disease; this is supported by appropriate correlations with auditory vestibular functional testing.     

用4.7特斯拉磁共振成像活体内观测豚鼠实验性内淋巴积水

目的探讨用4.7特斯拉试验用磁共振成像系统能否在豚鼠中检测内淋巴积水.方法20只白色或者杂色豚鼠用于该研究.5只正常豚鼠作为对照组,15只豚鼠用于制作内淋巴积水模型.9只内淋巴囊破坏组中的5只和6只内淋巴囊完整组(与乙状窦游离)动物采用gadolinium(Gd)-DTPA-BMA增强MRI检测内淋巴积水.结果由于Gd-DTPA-BMA主要进入鼓阶和前庭阶,耳蜗的三个阶可在所有动物中由MRI清晰显示.在内淋巴囊完整组,内淋巴囊手术后6天MRI即可检测到内淋巴积水,并且由组织学证实.在内淋巴囊破坏组中的1只动物,因内耳屏障的严重破坏而使Gd-DTPA-BMA快速漏入中阶,MRI可检测到该变化,其听力损失为60dB.结论用Gd-DTPA-BMA增强的高分辨MRI可检测出内淋巴积水,有可能对积水程度进行定量测试.在Gd-DTPA-BMA的帮助下,内耳屏障损伤或可能的膜破裂可以被检出.     

Contralateral type of delayed endolymphatic hydrops may consist of two phenotypes based on a magnetic resonance imaging preliminary study

Objective: This study was performed to determine the volume distribution of EH in contralateral DEH.

Patients and methods: Five contralateral DEH patients (age range?=?21–77 years) and one ipsilateral DEH patient. Visualization of in vivo EH using 3T magnetic resonance (MR) imaging was performed by intravenous administration of gadolinium. The EH volume was determined quantitatively by adding the area of EH in consecutive MR images.

Results: The total EH volume of the patients with contralateral DEH varied from 24.2 to 56.6?μL in the first ear and 20.2 to 35.7?μL in the contralateral ear. EH was dominantly developed in the first ear for Patient no. 3 and 4, and not dominantly developed in the first ear for Patient no. 1, 2, and 5.

Conclusions: The volume distribution of endolymphatic hydrops (EH) in contralateral delayed endolymphatic hydrops (DEH) was classified into two phenotypes. This suggests that contralateral DEH may consist of two etiologies.