{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:12:54Z","timestamp":1760145174522,"version":"build-2065373602"},"reference-count":23,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2024,6,27]],"date-time":"2024-06-27T00:00:00Z","timestamp":1719446400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Audiology Research"],"abstract":"<jats:p>Background: Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air\u2013bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors\u2019 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.<\/jats:p>","DOI":"10.3390\/audiolres14040050","type":"journal-article","created":{"date-parts":[[2024,6,28]],"date-time":"2024-06-28T06:51:53Z","timestamp":1719557513000},"page":"593-601","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["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"],"prefix":"10.3390","volume":"14","author":[{"given":"Giovanni","family":"Motta","sequence":"first","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4312-4711","authenticated-orcid":false,"given":"Salvatore","family":"Allosso","sequence":"additional","affiliation":[{"name":"Otorhinolaryngology-Head and Neck Surgery Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University of Naples Federico II, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ludovica","family":"Castagna","sequence":"additional","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ghita","family":"Trifuoggi","sequence":"additional","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tonia","family":"Di Meglio","sequence":"additional","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7176-3652","authenticated-orcid":false,"given":"Domenico","family":"Testa","sequence":"additional","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5081-1552","authenticated-orcid":false,"given":"Massimo","family":"Mesolella","sequence":"additional","affiliation":[{"name":"Otorhinolaryngology-Head and Neck Surgery Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University of Naples Federico II, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaetano","family":"Motta","sequence":"additional","affiliation":[{"name":"ENT Unit-Department of Mental, Physical Health and Preventive Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.amjoto.2013.07.015","article-title":"Enlarged vestibular aqueduct syndrome mimicking otosclerosis in adults","volume":"34","author":"Wieczorek","year":"2013","journal-title":"Am. J. Otolaryngol."},{"key":"ref_2","first-page":"178","article-title":"Misdiagnosis of otosclerosis in a patient with enlarged vestibular aqueduct syndrome: A case report","volume":"2","author":"Miller","year":"2012","journal-title":"J. Med. Case Rep."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1017\/S0022215100128695","article-title":"Large vestibular aqueduct syndrome and stapes fixation","volume":"108","author":"Shirazi","year":"1994","journal-title":"J. Laryngol. Otol."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Yang, N., and Yang, B.B. (2023). A Large Vestibular Aqueduct with Conductive Hearing Loss as the Only Manifestation. Ear Nose Throat J.","DOI":"10.1177\/01455613231183539"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"110752","DOI":"10.1016\/j.ijporl.2021.110752","article-title":"Conductive hearing loss in large vestibular aqueduct syndrome-clinical observations and proof-of-concept predictive modeling by a biomechanical approach","volume":"146","author":"Pang","year":"2021","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1016\/j.otc.2017.11.017","article-title":"Controversies in the Evaluation and Management of Otosclerosis","volume":"51","author":"McElveen","year":"2018","journal-title":"Otolaryngol. Clin. N. Am."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/j.otc.2017.11.005","article-title":"Impact of Imaging in Management of Otosclerosis","volume":"51","author":"Wolfovitz","year":"2018","journal-title":"Otolaryngol. Clin. N. Am."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"372","DOI":"10.3390\/audiolres14020032","article-title":"Otic Capsule Dehiscences Simulating Other Inner Ear Diseases: Characterization, Clinical Profile, and Follow-Up- Is M\u00e9ni\u00e8re\u2019s Disease the Sole Cause of Vertigo and Fluctuating hearing Loss?","volume":"14","author":"Garaycochea","year":"2024","journal-title":"Audiol. Res."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1097\/MAO.0b013e318161ab24","article-title":"Conductive hearing loss caused by third-window lesions of the inner ear","volume":"29","author":"Merchant","year":"2008","journal-title":"Otol. Neurotol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1177\/000348940711600709","article-title":"Clinical investigation and mechanism of air-bone gaps in large vestibular aqueduct syndrome","volume":"116","author":"Merchant","year":"2007","journal-title":"Ann. Otol. Rhinol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1002\/lary.1978.88.7.1178","article-title":"Stapes gusher and Klippel-Feil syndrome","volume":"88","author":"Daniilidis","year":"1978","journal-title":"Laryngoscope"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1179\/1754762810Y.0000000006","article-title":"Cochlear implantation in children with Jervell and Lange-Nielsen syndrome-a cautionary tale","volume":"13","author":"Broomfield","year":"2012","journal-title":"Cochlear Implants Int."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1161\/CIRCULATIONAHA.105.592899","article-title":"The Jervell and Lange-Nielsen syndrome: Natural history, molecular basis, and clinical outcome","volume":"113","author":"Schwartz","year":"2006","journal-title":"Circulation"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1179\/146701011X12950038111819","article-title":"Cochlear implantation in Pendred syndrome","volume":"12","author":"Kontorinis","year":"2011","journal-title":"Cochlear Implants"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2305","DOI":"10.1007\/s00405-020-06333-9","article-title":"Enlarged vestibular aqueduct and Mondini Malformation: Audiological, clinical, radiologic and genetic features","volume":"278","author":"Lazzerini","year":"2021","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"156","DOI":"10.5152\/JIAO.2021.8632","article-title":"The Third Mobile Window Effects in Otology\/Neurotology","volume":"17","author":"Ota","year":"2021","journal-title":"J. Int. Adv. Otol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1159\/000489813","article-title":"Stapes Surgery for Otosclerosis in Patients Presenting with Mixed Hearing Loss","volume":"23","author":"Salmon","year":"2018","journal-title":"Audiol. Neurootol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e844","DOI":"10.1097\/MAO.0000000000003163","article-title":"Stapedotomy Removing Only the Stapes Head and Not the Entire Stapes Superstructure: Long-term Results","volume":"42","author":"Malafronte","year":"2021","journal-title":"Otol. Neurotol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1177\/014556131809700302","article-title":"Schwartze sign","volume":"97","author":"Peng","year":"2018","journal-title":"Ear Nose Throat J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.anl.2007.11.002","article-title":"Minimizing the dead ear in otosclerosis surgery","volume":"35","author":"Vital","year":"2008","journal-title":"Auris Nasus Larynx"},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Motta, G., Massimilla, E.A., Allosso, S., Mesolella, M., De Luca, P., Testa, D., and Motta, G. (2024). Critical Steps and Common Mistakes during Temporal Bone Dissection: A Survey among Residents and a Step-by-Step Guide Analysis. J. Pers. Med., 14.","DOI":"10.3390\/jpm14040349"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.1007\/s00405-019-05538-x","article-title":"Gusher in stapes surgery: A systematic review","volume":"276","author":"Molinari","year":"2019","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1177\/0145561320984569","article-title":"Cerebrospinal Fluid Leak During Stapes Surgery: The Importance of Temporal Bone CT Reconstructions in Oblique Anatomically Oriented Planes","volume":"102","author":"Re","year":"2023","journal-title":"Ear Nose Throat J."}],"container-title":["Audiology Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2039-4349\/14\/4\/50\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:06:37Z","timestamp":1760108797000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2039-4349\/14\/4\/50"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,27]]},"references-count":23,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2024,8]]}},"alternative-id":["audiolres14040050"],"URL":"https:\/\/doi.org\/10.3390\/audiolres14040050","relation":{},"ISSN":["2039-4349"],"issn-type":[{"type":"electronic","value":"2039-4349"}],"subject":[],"published":{"date-parts":[[2024,6,27]]}}}