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隐匿性大前庭导水管(EVA)综合征酷似耳硬化症的病例:鉴别诊断的临床特征及高分辨率计算机断层扫描在术前评估中的作用

Insidious Cases of Enlarged Vestibular Aqueduct (EVA) Syndrome Resembling Otosclerosis: Clinical Features for Differential Diagnosis and the Role of High-Resolution Computed Tomography in the Pre-Operative Setting.

作者信息

Motta Giovanni, Allosso Salvatore, Castagna Ludovica, Trifuoggi Ghita, Di Meglio Tonia, Testa Domenico, Mesolella Massimo, Motta Gaetano

机构信息

ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania "Luigi Vanvitelli", 80121 Naples, Italy.

Otorhinolaryngology-Head and Neck Surgery Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University of Naples Federico II, 80138 Naples, Italy.

出版信息

Audiol Res. 2024 Jun 27;14(4):593-601. doi: 10.3390/audiolres14040050.

DOI:10.3390/audiolres14040050
PMID:39051194
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11270216/
Abstract

BACKGROUND

Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air-bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors' studies and seems to be related to a third mobile window (TMW) phenomenon. This can lead to misdiagnosis and inappropriate treatment. Given that it would be inappropriate and harmful to perform CT scans in all patients with a clinical diagnosis of otosclerosis, this study aims to highlight some clinical features useful for the differential diagnosis between otosclerosis and these rare cases of EVA presenting with an ABG, thus enabling the identification of suspected cases to be tested with CT scans.

METHODS

Between April and May 2024, a narrative review was conducted focusing on the differential diagnosis between some rare cases of EVA and otosclerosis. Clinical, audiological, and radiologic features of both conditions were investigated.

RESULTS

This review demonstrates the diagnostic challenge in differentiating atypical cases of EVA from otosclerosis in a subset of patients. Clinical and audiological features are important for differential diagnosis, but may not always be sufficient. Therefore, high-resolution computed tomography (HRCT) of the temporal bone plays a pivotal role in definitive diagnosis.

CONCLUSIONS

In some specific cases, pre-operative imaging assessment using HRCT emerges as an essential tool for differentiating these two conditions and avoiding unnecessary stapes surgery.

摘要

背景

扩大的前庭导水管(EVA)综合征在成人中可类似耳硬化症,表现为气骨导间距(ABG)甚至镫骨肌反射消失。内耳疾病中的ABG目前是几位作者研究的对象,似乎与第三活动窗(TMW)现象有关。这可能导致误诊和不恰当的治疗。鉴于对所有临床诊断为耳硬化症的患者进行CT扫描既不合适也有害,本研究旨在突出一些有助于耳硬化症与这些罕见的伴有ABG的EVA病例进行鉴别诊断的临床特征,从而能够识别出需要进行CT扫描检查的疑似病例。

方法

在2024年4月至5月期间,进行了一项叙述性综述,重点关注一些罕见的EVA病例与耳硬化症之间的鉴别诊断。研究了这两种疾病的临床、听力学和放射学特征。

结果

本综述表明,在一部分患者中,将非典型EVA病例与耳硬化症区分开来存在诊断挑战。临床和听力学特征对鉴别诊断很重要,但可能并不总是足够的。因此,颞骨高分辨率计算机断层扫描(HRCT)在明确诊断中起着关键作用。

结论

在某些特定情况下,使用HRCT进行术前影像评估成为区分这两种疾病和避免不必要的镫骨手术的重要工具。

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Insidious Cases of Enlarged Vestibular Aqueduct (EVA) Syndrome Resembling Otosclerosis: Clinical Features for Differential Diagnosis and the Role of High-Resolution Computed Tomography in the Pre-Operative Setting.隐匿性大前庭导水管(EVA)综合征酷似耳硬化症的病例:鉴别诊断的临床特征及高分辨率计算机断层扫描在术前评估中的作用
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J Pers Med. 2024 Mar 27;14(4):349. doi: 10.3390/jpm14040349.
2
Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up-Is Ménière's Disease the Sole Cause of Vertigo and Fluctuating Hearing Loss?模拟其他内耳疾病的耳囊裂:特征、临床概况及随访——梅尼埃病是眩晕和波动性听力损失的唯一原因吗?
Audiol Res. 2024 Apr 12;14(2):372-385. doi: 10.3390/audiolres14020032.
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A Large Vestibular Aqueduct With Conductive Hearing Loss as the Only Manifestation.
Ear Nose Throat J. 2023 Jul 18:1455613231183539. doi: 10.1177/01455613231183539.
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Stapedotomy Removing Only the Stapes Head and Not the Entire Stapes Superstructure: Long-term Results.仅切除镫骨头而非整个镫骨超结构的镫骨切除术:长期结果
Otol Neurotol. 2021 Aug 1;42(7):e844-e848. doi: 10.1097/MAO.0000000000003163.
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Conductive hearing loss in large vestibular aqueduct syndrome -clinical observations and proof-of-concept predictive modeling by a biomechanical approach.大前庭水管综合征的传导性听力损失-基于生物力学方法的临床观察和概念验证预测模型。
Int J Pediatr Otorhinolaryngol. 2021 Jul;146:110752. doi: 10.1016/j.ijporl.2021.110752. Epub 2021 Apr 29.
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The Third Mobile Window Effects in Otology/Neurotology.耳科学/神经耳科学中的第三次移动窗口效应。
J Int Adv Otol. 2021 Mar;17(2):156-161. doi: 10.5152/JIAO.2021.8632.
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Cerebrospinal Fluid Leak During Stapes Surgery: The Importance of Temporal Bone CT Reconstructions in Oblique Anatomically Oriented Planes.镫骨手术期间的脑脊液漏:在斜位解剖定向平面进行颞骨 CT 重建的重要性。
Ear Nose Throat J. 2023 Apr;102(4):227-230. doi: 10.1177/0145561320984569. Epub 2021 Feb 24.
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