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第三移动窗口综合征:不同解剖位置的临床谱——特征、治疗反应及对内淋巴积水发展的影响

The Third Mobile Window Syndrome: A Clinical Spectrum of Different Anatomical Locations-Characterization, Therapeutic Response, and Implications in the Development of Endolymphatic Hydrops.

作者信息

Lorente-Piera Joan, Manrique-Huarte Raquel, Pérez Fernández Nicolás, Calavia Gil Diego, Jiménez Vázquez Marcos, Domínguez Pablo, Manrique Manuel

机构信息

Department of Otorhinolaryngology, Clínica Universidad de Navarra, 31008 Pamplona, Spain.

Department of Otorhinolaryngology, Clínica Universidad de Navarra, 28027 Madrid, Spain.

出版信息

J Clin Med. 2024 Nov 28;13(23):7232. doi: 10.3390/jcm13237232.

Abstract

Multiple dehiscences of the otic capsule can exhibit behavior similar to Ménière's disease, not only from a clinical perspective but also in the results of audiovestibular tests. The main objective of this study is to characterize third mobile window etiologies from an audiovestibular perspective, while also evaluating the therapeutic response to four different treatment protocols. Furthermore, we aim to explore a potential association with the development of radiologically defined endolymphatic hydrops (EH). : This is a retrospective cohort study conducted from 2017 to 2024 at a tertiary-level otology and otoneurology unit. All patients underwent pure tone audiometry, vHIT, cVEMP, and oVEMP. Some of these patients, selected under rigorous inclusion criteria based on clinical and audiometric findings, were subjected to a 4-h delayed intravenous gadolinium-enhanced 3D-FLAIR MRI. : We obtained a sample of 86 patients, with a mean age of 52.2 ± 7.64 years: 62.76% were female (n = 54) and 37.21% were male (n = 32); 88.37% (n = 76) were diagnosed with superior semicircular canal dehiscence syndrome (SSCDS), while 11.62% (n = 10) had other forms of otic capsule dehiscence. The most common symptom observed was unsteadiness (44%). While surgery is the only curative treatment, other medical treatments, such as acetazolamide, also helped reduce symptoms such as autophony, falls, instability, and vertigo attacks, with a relative risk reduction (RRR) exceeding 75% (95% CI, < 0.05). The results of the MRI in EH sequences indicate that 7.89% of the patients diagnosed with SSCDS also developed radiological EH, compared to 40.00% of the patients with other otic capsule dehiscences, a difference that was statistically significant ( = 0.0029. : Otic capsule dehiscences are relatively unknown conditions that require clinical diagnosis. Although VEMP testing is useful, imaging studies are necessary to localize and characterize the defect, most commonly found in the superior semicircular canal. We should consider these dehiscences in cases where there is a suspicion of EH development. Further research, including in vivo neuroimaging studies using hydrops sequences, is required to better understand their relationship to potential Ménière's disease.

摘要

耳囊多处裂开不仅在临床方面,而且在听前庭测试结果上都可能表现出与梅尼埃病相似的症状。本研究的主要目的是从听前庭角度对第三活动窗病因进行特征描述,同时评估对四种不同治疗方案的治疗反应。此外,我们旨在探索与放射学定义的内淋巴积水(EH)发展之间的潜在关联。 :这是一项于2017年至2024年在三级耳科和耳神经科进行的回顾性队列研究。所有患者均接受了纯音听力测定、视频头脉冲试验(vHIT)、颈肌源性前庭诱发肌源性电位(cVEMP)和眼肌源性前庭诱发肌源性电位(oVEMP)检查。其中一些根据临床和听力检查结果严格纳入标准选择的患者,接受了4小时延迟静脉注射钆增强三维液体衰减反转恢复序列(3D-FLAIR)磁共振成像(MRI)检查。 :我们获得了86例患者的样本,平均年龄为52.2±7.64岁:62.76%为女性(n = 54),37.21%为男性(n = 32);88.37%(n = 76)被诊断为上半规管裂综合征(SSCDS),而11.62%(n = 10)有其他形式的耳囊裂开。观察到的最常见症状是不稳(44%)。虽然手术是唯一的治愈性治疗方法,但其他药物治疗,如乙酰唑胺,也有助于减轻诸如自听过强、跌倒、不稳和眩晕发作等症状,相对风险降低(RRR)超过75%(95%可信区间,<0.05)。EH序列的MRI结果表明,诊断为SSCDS的患者中有7.89%也出现了放射学上的EH,而其他耳囊裂开患者中这一比例为40.00%,差异具有统计学意义(=0.0029)。 :耳囊裂开是相对不为人知的疾病,需要临床诊断。虽然VEMP测试有用,但成像研究对于定位和描述缺陷是必要的,该缺陷最常见于上半规管。在怀疑有EH发展的病例中,我们应考虑这些裂开情况。需要进一步的研究,包括使用积水序列的体内神经影像学研究,以更好地了解它们与潜在梅尼埃病的关系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a85f/11642179/ad3a429a2207/jcm-13-07232-g001.jpg

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