Álvarez De Linera-Alperi Marta, Dominguez Pablo, Blanco-Pareja Melissa, Menéndez Fernández-Miranda Pablo, Manrique-Huarte Raquel, Liaño Gloria, Pérez-Fernández Nicolas, Suárez-Vega Víctor
Department of Otorhinolaryngology, Clínica Universidad de Navarra, Madrid, Spain.
Department of Radiology, Clínica Universidad de Navarra, Pamplona, Spain.
Front Neurol. 2024 Dec 2;15:1477282. doi: 10.3389/fneur.2024.1477282. eCollection 2024.
The most common histopathological finding in Ménière's disease (MD) is endolymphatic hydrops (EH), which involves the dilation of the membranous labyrinth. The direct relationship between EH and MD is debated, although EH plays a crucial role in auditory and vestibular functional tests. MRI sequences such as 3D-FLAIR and 3D-real-IR are used to study EH, with the latter being more effective. This study aimed to examine whether the severity of EH detected by MRI is always more pronounced in the cochlea than in the vestibule, indicating a cochleocentric progression of the condition.
A retrospective longitudinal study was conducted at a tertiary care medical center from 2019 to 2023, involving patients diagnosed with unilateral Ménière's disease. All patients underwent MRI hydrops assessments (3D-REAL-IR sequences) using 3 Tesla magnets and gadobutrol contrast agent. EH was graded qualitatively and quantitatively for both ears using scales for cochlear endolymphatic hydrops (cEH) and vestibular endolymphatic hydrops (vEH). Volumetric measurements of the vestibule and endolymph were performed, and the vestibular endolymphatic ratio (vELR) was calculated. The degree of perilymphatic enhancement (PE) and endolymphatic herniation was also assessed. Patient data, including demographics, disease features, comorbidities, hearing loss, and vestibular function, were collected from medical records. Statistical analysis involved various tests to compare groups and evaluate correlations, using a significance level of < 0.05. The study aimed to classify the patients into cochleocentric (CC) or non-cochleocentric (NCC) groups based on the difference in the severity of EH in both compartments.
We included 137 patients, of whom 55 (40.15%) were classified as CC, and the remaining 82 (59.85%) were classified as NCC. The degree of vestibular EH (vEH) was more severe in the NCC group ( < 0.001), while cochlear EH (cEH) showed a moderate correlation with vEH. The mean vestibular endolymphatic ratio (vELR) was higher in the NCC group (80.5% ± 38%) compared to the CC group (55% ± 49.5%) ( < 0.0001). Vestibular herniation was more common in the NCC group, while vestibular perilymphatic enhancement was more prevalent in the CC group. Cardiovascular risk was associated with the CC group, while the NCC group reported more vestibular symptoms. Delayed Ménière's disease was linked to the CC group. The hearing loss and vestibular function tests did not show significant differences between the groups.
In conclusion, our study found that endolymphatic hydrops (EH) was more severe in the vestibule than in the cochlea in nearly 60% of the cases, with a clinical correlation to the initial symptoms. However, no significant differences were observed in the auditory or vestibular function tests during the follow-up.V Previous studies have indicated that vestibular EH occurs early in Ménière's disease (MD) and subsequently progresses to the cochlea, a finding that challenges the traditional cochleocentric progression theory supported by experimental and clinical otopathology. MRI techniques have enhanced the detection of EH, revealing that the relative amount of endolymph is slightly higher in the vestibule than in the cochlea, thereby supporting the study's findings. We considered the important technical limitations in the MRI visualization of EH and suggested that advanced imaging techniques and volumetric quantification could enhance the classification of cochleocentric and non-cochleocentric groups. The clinical findings revealed that cardiovascular risk factors and delayed MD phenotypes were more common in the cochleocentric group, while the non-cochleocentric group exhibited poorer vestibular MRI results and a higher incidence of endolymph herniation into the semicircular canals.
梅尼埃病(MD)最常见的组织病理学表现是内淋巴积水(EH),即膜迷路扩张。尽管EH在听觉和前庭功能测试中起关键作用,但EH与MD之间的直接关系仍存在争议。3D-FLAIR和3D-real-IR等MRI序列用于研究EH,后者更有效。本研究旨在探讨MRI检测到的EH严重程度在耳蜗中是否总是比在前庭中更明显,这表明该疾病以耳蜗为中心进展。
2019年至2023年在一家三级医疗中心进行了一项回顾性纵向研究,纳入诊断为单侧梅尼埃病的患者。所有患者均使用3特斯拉磁体和钆布醇造影剂进行MRI积水评估(3D-REAL-IR序列)。使用耳蜗内淋巴积水(cEH)和前庭内淋巴积水(vEH)量表对双耳的EH进行定性和定量分级。对前庭和内淋巴进行体积测量,并计算前庭内淋巴比率(vELR)。还评估了外淋巴强化(PE)程度和内淋巴疝。从病历中收集患者数据,包括人口统计学、疾病特征、合并症、听力损失和前庭功能。统计分析包括各种检验,以比较组间差异并评估相关性,显著性水平为<0.05。该研究旨在根据两个部位EH严重程度的差异将患者分为以耳蜗为中心(CC)或非以耳蜗为中心(NCC)组。
我们纳入了137例患者,其中55例(40.15%)被分类为CC组,其余82例(59.85%)被分类为NCC组。NCC组的前庭EH(vEH)程度更严重(<0.001),而耳蜗EH(cEH)与vEH呈中度相关。NCC组的平均前庭内淋巴比率(vELR)(80.5%±38%)高于CC组(55%±49.5%)(<0.0001)。前庭疝在NCC组中更常见,而前庭外淋巴强化在CC组中更普遍。心血管风险与CC组相关,而NCC组报告的前庭症状更多。迟发性梅尼埃病与CC组有关。两组之间的听力损失和前庭功能测试没有显著差异。
总之,我们的研究发现,近60%的病例中,前庭的内淋巴积水(EH)比耳蜗更严重,且与初始症状存在临床相关性。然而,随访期间听觉或前庭功能测试未观察到显著差异。先前的研究表明前庭EH在梅尼埃病(MD)早期出现,随后进展至耳蜗,这一发现挑战了实验和临床耳病理学支持的传统以耳蜗为中心的进展理论。MRI技术提高了EH的检测能力,显示前庭内淋巴的相对量略高于耳蜗,从而支持了本研究的结果。我们考虑了EH的MRI可视化中的重要技术局限性,并建议先进的成像技术和体积定量可以加强以耳蜗为中心和非以耳蜗为中心组的分类。临床结果显示,心血管危险因素和迟发性MD表型在以耳蜗为中心组中更常见,而非以耳蜗为中心组的前庭MRI结果较差,内淋巴疝入半规管的发生率较高。