{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T05:19:36Z","timestamp":1792300776255,"version":"4.4.0"},"reference-count":14,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2014,3,25]],"date-time":"2014-03-25T00:00:00Z","timestamp":1395705600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2014,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Girls with Turner syndrome are prone to cholesteatoma, a serious suppurative middle ear disease. We aimed to confirm its high prevalence in Turner syndrome, identify risk factors and suggest possible strategies for earlier detection.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We reviewed 179 girls with Turner syndrome between 1989 and 2012 to identify cases of cholesteatoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seven girls (3.9%) had cholesteatoma (index girls) and each was compared with three age\u2010matched girls without cholesteatoma (comparison girls). All the index girls had either the 45,X or 45,X\/46X,i(Xq) karyotypes. Nine ears were initially affected, with three recurrences in two girls. Median age at first cholesteatoma presentation was 11.9\u00a0years (range: 7.5\u201315.2), with otorrhoea for three (range: one to seven) months in all 12 affected ears. Index girls had a significantly higher proportion of previous recurrent acute (p\u00a0=\u00a00.007) and chronic otitis media (p\u00a0=\u00a00.008), chronic perforation (p\u00a0=\u00a00.038) aural polyps (p\u00a0&lt;\u00a00.0001) and tympanic membrane retraction (p\u00a0=\u00a00.0001) than comparison girls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Cholesteatoma has a high prevalence in Turner syndrome. Risk factors include 45,X and 46,XiXq karyotypes; a history of chronic otitis media, tympanic membrane retraction and persistent otorrhoea; and older age. Earlier recognition of ear disease is needed and otoscopy training for paediatricians caring for Turner syndrome patients may be beneficial.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.12622","type":"journal-article","created":{"date-parts":[[2014,3,8]],"date-time":"2014-03-08T10:15:35Z","timestamp":1394273735000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Cholesteatoma has a high prevalence in Turner syndrome, highlighting the need for earlier diagnosis and the potential benefits of otoscopy training for paediatricians"],"prefix":"10.1111","volume":"103","author":[{"given":"DBN","family":"Lim","sequence":"first","affiliation":[{"name":"Section of Child Health School of Medicine University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"EJ","family":"Gault","sequence":"additional","affiliation":[{"name":"Section of Child Health School of Medicine University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H","family":"Kubba","sequence":"additional","affiliation":[{"name":"Department of Ear, Nose and Throat Surgery Royal Hospital for Sick Children Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MSC","family":"Morrissey","sequence":"additional","affiliation":[{"name":"Department of Ear, Nose and Throat Surgery Royal Hospital for Sick Children Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"DM","family":"Wynne","sequence":"additional","affiliation":[{"name":"Department of Ear, Nose and Throat Surgery Royal Hospital for Sick Children Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MDC","family":"Donaldson","sequence":"additional","affiliation":[{"name":"Section of Child Health School of Medicine University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,3,25]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0030-6665(02)00052-X"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2003.035907"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-5955(98)00113-0"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2008.09.022"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2010.12.007"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.118571"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100057996"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.42.5.460"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-5955(99)00162-8"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejo\/cjs097"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2273.1992.tb01689.x"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20765"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348941112000408"},{"key":"e_1_2_10_15_1","unstructured":"Diabetic Retinopathy Screening Centre[Internet].United Kingdom: Heart of England NHS Foundation Trust; c2005\u20132013. 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