{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T16:47:36Z","timestamp":1787503656748,"version":"build-2736575974"},"reference-count":26,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T00:00:00Z","timestamp":1649808000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objectives: To determine whether temporal bone computed tomography (CT) features are linked to the presence and type of hearing loss in osteogenesis imperfecta (OI) when considering hearing-impaired OI patients and normally hearing (NH) OI ones. A secondary objective was to assess whether other factors influence CT features in a large sample: age, type of mutation, or bone mineral density (BMD). Methods: A total of 41 adults with OI underwent CTs and pure-tone audiometry in 82 ears. Hearing thresholds were normal in 64 out of 82 ears, and most had not been operated on for stapedectomy or stapedotomy. Ossicle density, footplates, oval and round windows, retrofenestral peri- and endolabyrinths, and temporal pneumatization were analyzed twice by an experienced radiologist. CT features were compared to hearing, age, collagen mutations, and bone mineral density. Results: Unexpectedly a high prevalence of footplate, ossicle, and otic capsule anomalies was observed, even in NH ears. Footplate hypodensity or thickening was mostly found in ears without conductive hearing loss. There were significantly more retrofenestral anomalies or window obstruction in ears with a sensorineural hearing loss component than in ears without. Age was significantly higher in ears with middle layer hypodensity than in ears without. Patients with mutations were expected to have reduced collagen quantity and had significantly more footplate or retrofenestral anomalies than those with qualitative mutations. BMD was significantly higher in ears without temporal hyperpneumatization. Conclusion: Temporal bone CT features in OI are present in a large proportion of patients, had they hearing loss or not, and might be determined more by collagen mutation type than by age or BMD.<\/jats:p>","DOI":"10.3390\/jcm11082171","type":"journal-article","created":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T21:33:42Z","timestamp":1649885622000},"page":"2171","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["High Heterogeneity of Temporal Bone CT Aspects in Osteogenesis Imperfecta Is Not Linked to Hearing Loss"],"prefix":"10.3390","volume":"11","author":[{"given":"A\u00efcha","family":"Ltaief-Boudrigua","sequence":"first","affiliation":[{"name":"Department of Radiology, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevieve","family":"Lina-Granade","sequence":"additional","affiliation":[{"name":"Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"Centre de Comp\u00e9tence Maladies Rares en ORL, Hospices Civils de Lyon, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1769-8623","authenticated-orcid":false,"given":"Eric","family":"Truy","sequence":"additional","affiliation":[{"name":"Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"Centre de Comp\u00e9tence Maladies Rares en ORL, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"INSERM U1028, CNRS UMR5292, Lyon Neuroscience Research Center, Equipe IMPACT, 69675 Bron, France"},{"name":"Claude Bernard University Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1920-6450","authenticated-orcid":false,"given":"Ruben","family":"Hermann","sequence":"additional","affiliation":[{"name":"Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"Centre de Comp\u00e9tence Maladies Rares en ORL, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"INSERM U1028, CNRS UMR5292, Lyon Neuroscience Research Center, Equipe IMPACT, 69675 Bron, France"},{"name":"Claude Bernard University Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Chevrel","sequence":"additional","affiliation":[{"name":"Department of SAMU 69-Emergency, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"},{"name":"Department of Rheumatology and Bone Diseases, Edouard Herriot Hospital, Hospices Civils de Lyon, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,13]]},"reference":[{"key":"ref_1","first-page":"1470","article-title":"Osteogenesis Imperfecta: Clinical Diagnosis, Nomenclature and Severity Assessment","volume":"164A","author":"Sillence","year":"2014","journal-title":"Am. 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