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

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

doi:10.3390/audiolres14040050

Audiology Research (Jun 2024)

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

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

Affiliations

Giovanni Motta: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy
Salvatore Allosso: Otorhinolaryngology-Head and Neck Surgery Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University of Naples Federico II, 80138 Naples, Italy
Ludovica Castagna: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy
Ghita Trifuoggi: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy
Tonia Di Meglio: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy
Domenico Testa: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy
Massimo Mesolella: Otorhinolaryngology-Head and Neck Surgery Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University of Naples Federico II, 80138 Naples, Italy
Gaetano Motta: ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania “Luigi Vanvitelli”, 80121 Naples, Italy

DOI: https://doi.org/10.3390/audiolres14040050
Journal volume & issue: Vol. 14, no. 4
pp. 593 – 601

Abstract

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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.

Published in Audiology Research

ISSN: 2039-4349 (Online)
Publisher: MDPI AG
Country of publisher: Switzerland
LCC subjects: Medicine: Otorhinolaryngology
Website: https://www.mdpi.com/journal/audiolres

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