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第三窗综合征:病因、诊断及治疗的最新系统综述

Third Window Syndrome: An Up-to-Date Systematic Review of Causes, Diagnosis, and Treatment.

作者信息

Lazarou Ilias, Sideris Giorgos, Papadimitriou Nikolaos, Delides Alexander, Korres George

机构信息

2nd Otolaryngology Department, School of Medicine, National and Kapodistrian University of Athens, "Attikon" University Hospital, Athens, Greece.

出版信息

J Audiol Otol. 2025 Apr;29(2):86-94. doi: 10.7874/jao.2024.00696. Epub 2025 Apr 18.

DOI:10.7874/jao.2024.00696
PMID:40296471
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12046203/
Abstract

Third window syndrome (TWS) is an inner ear condition caused by an additional compliant point in the otic capsule that disrupts auditory and vestibular functions. Superior semicircular canal dehiscence is the most common cause, presenting with hearing loss, vertigo, and autophony, significantly impairing quality of life. This study evaluated the pathophysiology, diagnostics, treatments, and recent advancements in TWS while identifying research gaps. Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, 70 studies from Embase, MEDLINE, Cochrane, and UpToDate databases were analyzed. TWS affects inner ear mechanics, enhancing bone conduction and reducing air conduction. Diagnosis involves clinical evaluations, high-resolution imaging, and functional tests such as vestibular evoked myogenic potentials, which are known for their high sensitivity and specificity. Management strategies range from vestibular rehabilitation and pharmacotherapy to surgical interventions, including transmastoid and middle cranial fossa approaches, which achieve over 75% success. Emerging minimally invasive techniques, such as underwater endoscopic ear surgery and round window reinforcement, show promise but carry risks like cerebrospinal fluid leakage and inconsistent symptom relief. Advancements in TWS management have improved outcomes, yet gaps remain, particularly in terms of false-positive imaging and long-term efficacy. Future studies should prioritize predictive models and minimally invasive techniques. A multidisciplinary approach is essential to improve patient care.

摘要

第三窗综合征(TWS)是一种内耳疾病,由耳囊内额外的顺应性点引起,会破坏听觉和前庭功能。上半规管裂是最常见的病因,表现为听力损失、眩晕和自听增强,严重损害生活质量。本研究评估了TWS的病理生理学、诊断方法、治疗手段及最新进展,同时找出研究空白。按照系统评价和Meta分析的首选报告项目指南,对来自Embase、MEDLINE、Cochrane和UpToDate数据库的70项研究进行了分析。TWS会影响内耳力学,增强骨传导并降低气传导。诊断包括临床评估、高分辨率成像以及诸如前庭诱发肌源性电位等功能测试,这些测试以其高灵敏度和特异性而闻名。管理策略包括前庭康复、药物治疗以及手术干预,其中手术干预包括经乳突和中颅窝入路,成功率超过75%。新兴的微创技术,如水下内镜耳手术和圆窗强化术,显示出前景,但存在脑脊液漏和症状缓解不一致等风险。TWS管理方面的进展改善了治疗效果,但仍存在空白,尤其是在假阳性成像和长期疗效方面。未来的研究应优先考虑预测模型和微创技术。多学科方法对于改善患者护理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b374/12046203/bdb40dcfa4b3/jao-2024-00696f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b374/12046203/bdb40dcfa4b3/jao-2024-00696f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b374/12046203/bdb40dcfa4b3/jao-2024-00696f1.jpg

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本文引用的文献

1
Superior semicircular canal dehiscence postoperative outcomes: a case series of 350 repairs.上半规管裂术后结局:350 例修复的病例系列。
Acta Neurochir (Wien). 2024 May 24;166(1):230. doi: 10.1007/s00701-024-06115-w.
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Large Vestibular Aqueduct-Associated Symptoms: Endolymphatic Duct Blockage as a Surgical Treatment.大前庭导水管相关症状:以内淋巴管阻塞作为手术治疗方法
Audiol Res. 2024 Mar 18;14(2):304-316. doi: 10.3390/audiolres14020027.
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Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.
水肺潜水导致的内耳病变:上半规管和鼓室天盖缺损的影像学表现。
Am J Case Rep. 2024 Jan 2;25:e941558. doi: 10.12659/AJCR.941558.
4
Hearing Results after Transmastoid Superior Semicircular Canal Plugging for Superior Semicircular Canal Dehiscence: A Meta-Analysis.经乳突上半规管阻塞术治疗上半规管裂的听力结果:一项荟萃分析
Audiol Res. 2023 Oct 8;13(5):730-740. doi: 10.3390/audiolres13050065.
5
Superior canal dehiscence syndrome: A review.上半规管裂综合征:综述。
Auris Nasus Larynx. 2024 Feb;51(1):113-119. doi: 10.1016/j.anl.2023.08.004. Epub 2023 Aug 26.
6
X-Linked Gusher Disease DFNX2 in Children, a Rare Inner Ear Dysplasia with Mixed Hearing and Vestibular Loss.儿童X连锁突发性疾病DFNX2,一种罕见的内耳发育异常,伴有混合性听力和前庭功能丧失。
Audiol Res. 2023 Aug 4;13(4):600-614. doi: 10.3390/audiolres13040052.
7
Ankle Audiometry: A Clinical Test for the Enhanced Hearing Sensitivity for Body Sounds in Superior Canal Dehiscence Syndrome.踝音测听:用于检测上半规管裂综合征患者对体声听力敏感度的临床测试。
Audiol Neurootol. 2023;28(3):219-229. doi: 10.1159/000528407. Epub 2023 Jan 12.
8
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Otol Neurotol. 2022 Mar 1;43(3):368-375. doi: 10.1097/MAO.0000000000003461.
9
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World Neurosurg. 2021 Dec;156:e229-e234. doi: 10.1016/j.wneu.2021.09.038. Epub 2021 Sep 20.
10
The evaluation of superior semicircular canal bone thickness and radiological patterns in relation to age and gender.上半规管骨厚度及其与年龄和性别的放射影像学特征评估。
Surg Radiol Anat. 2021 Nov;43(11):1839-1844. doi: 10.1007/s00276-021-02797-4. Epub 2021 Jul 9.