{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T11:49:01Z","timestamp":1793015341268,"version":"4.5.0"},"reference-count":36,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2013,2,12]],"date-time":"2013-02-12T00:00:00Z","timestamp":1360627200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:sec><jats:title>Objectives\/Hypothesis<\/jats:title><jats:p>Osteogenesis imperfecta (OI) is an autosomal\u2010dominant connective\u2010tissue disorder, predominantly characterized by bone fragility. Conductive hearing loss develops in half of the OI patients and often progresses to mixed loss. Findings of computed tomography (CT) and magnetic resonance (MR) imaging of the temporal bone in the largest series of OI patients to date are presented and correlated with the audiograms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>Retrospective case series.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>CT images and audiograms of 17 hearing\u2010impaired OI patients, aged 9 to 67 years, were analyzed retrospectively. In four patients, MR imaging was performed as well. Imaging abnormalities were correlated with type and severity of hearing loss deduced from the audiograms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>CT revealed fenestral hypodense foci in the fissula ante fenestram (25 of 33 ears), oval window (23 of 33 ears), and round window (20 of 33 ears). Retrofenestral hypodensities were observed, affecting the cochlear turns (16 of 33 ears), facial nerve canal (10 of 33 ears), or semicircular canals (6 of 33 ears), or appearing like the fourth turn of the cochlea (11 of 33 ears). The site of hypodensities corresponded to the type of hearing loss in 72.2% of the OI ears. The air\u2010bone gap and bone\u2010conduction thresholds showed significant positive associations with the number of affected fenestral (<jats:italic>P<\/jats:italic> &lt; .05) and retrofenestral structures (<jats:italic>P<\/jats:italic> &lt; .01), respectively. Gadolinium\u2010enhanced MR images demonstrated active lesions in three patients with mixed hearing loss or deafness.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The site of hypodensities on temporal bone CT images in OI corresponds to presence and type of hearing loss determined by audiometry. The more severe the hearing loss, the more affected temporal bone structures in OI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4. Laryngoscope, 123:1988\u20131995, 2013<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.23963","type":"journal-article","created":{"date-parts":[[2013,2,12]],"date-time":"2013-02-12T16:58:45Z","timestamp":1360688325000},"page":"1988-1995","source":"Crossref","is-referenced-by-count":28,"title":["Temporal bone imaging in osteogenesis imperfecta patients with hearing loss"],"prefix":"10.1002","volume":"123","author":[{"given":"Freya K. R.","family":"Swinnen","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology Ghent University Hospital Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan W.","family":"Casselman","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging Sint\u2010Jan Hospital Bruges Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Els M. R.","family":"De Leenheer","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Ghent University Hospital Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cor W. R. J.","family":"Cremers","sequence":"additional","affiliation":[{"name":"FC Donders Institute for Neurosciences Radboud University Nijmegen Medical Centre Department of Otorhinolaryngology Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingeborg J. M.","family":"Dhooge","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Ghent University Hospital Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,2,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.16.2.101"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrendo.2011.81"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20071"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.3109\/01050398409043042"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1991.tb19594.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100088344"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mao.0000172410.85992.21"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1982.00790560005002"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1990.01870030081014"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200309000-00004"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20155"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348940211101014"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.3766\/jaaa.19.8.3"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1962.00740040501002"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1969.00770030006004"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198502000-00014"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348947508400311"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348948109000301"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348948809700603"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-005-0128-7"},{"key":"e_1_2_7_22_1","first-page":"661","article-title":"CT densitometry of the cochlear capsule","volume":"6","author":"Valvassori GE","year":"1985","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-438X(04)95535-1"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1159\/000336211"},{"key":"e_1_2_7_25_1","first-page":"1106","article-title":"Osteogenesis imperfecta of the temporal bone: CT and MR imaging in Van der Hoeve\u2010de Kleyn syndrome","volume":"25","author":"Alkadhi H","year":"2004","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.175.1.2315478"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.3109\/00016489309135874"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2241001707"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.156.3.4023229"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348949610500813"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.156.3.4023230"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/0720-048X(90)90098-V"},{"key":"e_1_2_7_33_1","first-page":"317","article-title":"Tomographic and CT features of the petrous bone in Lobstein's disease. One case and a review of the literature","volume":"12","author":"Jardin C","year":"1985","journal-title":"J Neuroradiol"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1159\/000111779"},{"key":"e_1_2_7_35_1","first-page":"253","article-title":"Cochlear implantation in osteogenesis imperfecta","volume":"52","author":"Szilvassy J","year":"1998","journal-title":"Acta Otorhinolaryngol Belg"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otohns.2004.12.012"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0196-0709(98)90090-4"}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.23963","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.23963","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T23:27:58Z","timestamp":1696375678000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.23963"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,12]]},"references-count":36,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2013,8]]}},"alternative-id":["10.1002\/lary.23963"],"URL":"https:\/\/doi.org\/10.1002\/lary.23963","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,12]]}}}