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?

IF 2.1 Q1 AUDIOLOGY & SPEECH-LANGUAGE PATHOLOGY
Joan Lorente-Piera, C. Prieto-Matos, R. Manrique-Huarte, O. Garaycochea, Pablo Dominguez, Manuel Manrique
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Abstract

INTRODUCTION We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière's disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. MATERIALS AND METHODS A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. RESULTS Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18-73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière's disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. CONCLUSIONS Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière's disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation.
模拟其他内耳疾病的耳囊开裂:特征、临床特征和随访--梅尼埃病是眩晕和波动性听力损失的唯一原因吗?
简介:我们介绍了六个病例,这些病例的临床表现为第三移动窗机制的听觉前庭表现,与梅尼埃病和耳硬化症有一定的相似之处。这些病例在如此短的时间内出现,促使人们重新审视其发病的根本原因。了解第三活动窗综合征的病理生理学,并将这些病例纳入眩晕和听力损失伴轻微气骨间隙的鉴别诊断中,对于理解这类病症至关重要。结果在 2014 年至 2024 年期间,本中心记录的 84 例裂隙病例中,78 例属于上半规管裂隙,6 例属于其他耳囊裂隙。这六名患者的平均年龄为 47.17 岁(18-73 岁不等),所有患者都有某种形式的耳囊开裂,并伴有听觉和/或前庭影响,通过听力和前庭测试进行测量,六名患者中有五人的结果出现异常。其中两人被诊断为梅尼埃病(MD)。另外两人患有耳蜗前庭水肿,但不符合梅尼埃病的诊断标准。有两个病例的耳囊开裂诊断是由术中涌水现象引起的并发症造成的,而有一个病例则是意外的放射学发现。结论:耳囊裂是一种相对较新且陌生的疾病,当临床上提示梅尼埃病,但辅助检查结果不一致或治疗效果不佳时,应考虑梅尼埃病。虽然存在高灵敏度和特异性的听觉前庭测试,但仍有必要通过影像学检查(尤其是岩骨 CT 扫描)完成研究,以确定导致临床表现的耳膜缺损的位置和特征。
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来源期刊
Audiology Research
Audiology Research AUDIOLOGY & SPEECH-LANGUAGE PATHOLOGY-
CiteScore
2.30
自引率
23.50%
发文量
56
审稿时长
11 weeks
期刊介绍: The mission of Audiology Research is to publish contemporary, ethical, clinically relevant scientific researches related to the basic science and clinical aspects of the auditory and vestibular system and diseases of the ear that can be used by clinicians, scientists and specialists to improve understanding and treatment of patients with audiological and neurotological disorders.
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