{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:14:45Z","timestamp":1790835285171,"version":"4.1.0"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,3,18]],"date-time":"2024-03-18T00:00:00Z","timestamp":1710720000000},"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":["Br J Haematol"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Despite numerous studies, the true scenario of hearing loss in beta\u2010thalassaemia remains rather nebulous.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and methods<\/jats:title><jats:p>Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5\u2009\u00b1\u200916.6\u2009years, 204 females, 66 non\u2010transfusion\u2010dependent) and 139 healthy controls (mean age 37.6\u2009\u00b1\u200917.7\u2009years, 81 females) were collected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patient and control groups did not differ for age (<jats:italic>p<\/jats:italic>\u2009=\u20090.59) or sex (<jats:italic>p<\/jats:italic>\u2009=\u20090.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.00001), correlated with male sex (32.6% in males vs. 21.8% in females; <jats:italic>p<\/jats:italic>\u2009=\u20090.01) and it was sensorineural in 79\/100. Hypoacusis rate correlated with increasing age (<jats:italic>p<\/jats:italic>\u2009=\u20090.0006) but not with phenotype (13\/66 non\u2010transfusion\u2010dependent vs. 87\/310 transfusion\u2010dependent patients; <jats:italic>p<\/jats:italic>\u2009=\u20090.16). Sensorineural\u2010notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (<jats:italic>p<\/jats:italic>\u2009=\u20090.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non\u2010significantly lower compared to chelation\u2010treated patients while it was significantly higher compared to controls (<jats:italic>p<\/jats:italic>\u2009=\u20090.003).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Sensorineural hypoacusis rate is high in beta\u2010thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural\u2010notch in beta\u2010thalassaemia appears questionable.<\/jats:p><\/jats:sec>","DOI":"10.1111\/bjh.19401","type":"journal-article","created":{"date-parts":[[2024,3,19]],"date-time":"2024-03-19T07:27:50Z","timestamp":1710833270000},"page":"2016-2024","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Hearing loss in beta\u2010thalassaemia: An Italian multicentre case\u2013control study"],"prefix":"10.1111","volume":"204","author":[{"given":"Renzo","family":"Manara","sequence":"first","affiliation":[{"name":"Neuroradiology, Neurosciences Department University of Padova  Padova Italy"},{"name":"DIMED University of Padova  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Brotto","sequence":"additional","affiliation":[{"name":"Section of Otorhinolaryngology, Neurosciences Department University of Padova  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"Barillari","sequence":"additional","affiliation":[{"name":"Division of Phoniatrics and Audiology, Department of Mental and Physical Health and Preventive Medicine University of Campania \"Luigi Vanvitelli\"  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Costa","sequence":"additional","affiliation":[{"name":"Division of Phoniatrics and Audiology, Department of Mental and Physical Health and Preventive Medicine University of Campania \"Luigi Vanvitelli\"  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa Valentina","family":"Villani","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica Universit\u00e0 degli Studi della Campania \"Luigi Vanvitelli\"  Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmine","family":"Perna","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica Universit\u00e0 degli Studi della Campania \"Luigi Vanvitelli\"  Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brunella","family":"Ziello","sequence":"additional","affiliation":[{"name":"UOSD Malattie Rare del Globulo Rosso, Dipartimento di oncoematologia e pneumologia AORN Cardarelli  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"di Salle","sequence":"additional","affiliation":[{"name":"Dipartimento di Medicina e Chirurgia, Scuola Medica Salernitana Universit\u00e0 di Salerno  Fisciano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cantone","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatologic Sciences, Unit of Ear, Nose and Throat \"Federico II\" University  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Pasanisi","sequence":"additional","affiliation":[{"name":"Centro della Microcitemia \u201cA. Quarta\u201d UOC di Ematologia con trapiannto Ospedale Perrino  Brindisi Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"De\u00a0Michele","sequence":"additional","affiliation":[{"name":"Medicina Trasfusionale AUO \"San Giovanni di Dio e Ruggi D'Aragona\"  Salerno Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Ciancio","sequence":"additional","affiliation":[{"name":"Unit\u00e0 Operativa Ematologia\u2014Day Hospital di Talassemia, Ospedale \u201cMadonna delle Grazie\u201d  Matera Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"D'Urzo","sequence":"additional","affiliation":[{"name":"Dipartimento di Pediatria Ospedale \"Umberto I\"  Nocera Inferiore Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasqualina","family":"Valentino","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica Universit\u00e0 degli Studi della Campania \"Luigi Vanvitelli\"  Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silverio","family":"Perrotta","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica Universit\u00e0 degli Studi della Campania \"Luigi Vanvitelli\"  Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Ricchi","sequence":"additional","affiliation":[{"name":"UOSD Malattie Rare del Globulo Rosso, Dipartimento di oncoematologia e pneumologia AORN Cardarelli  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1278-2372","authenticated-orcid":false,"given":"Immacolata","family":"Tartaglione","sequence":"additional","affiliation":[{"name":"Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica Universit\u00e0 degli Studi della Campania \"Luigi Vanvitelli\"  Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,3,18]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11010102"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022\u20103476(05)80556\u20106"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165\u20105876(94)90008\u20106"},{"key":"e_1_2_12_5_1","unstructured":"Report of the informal working group on prevention of deafness and hearing impairment programme planning Geneva 18\u201321 June Geneva World Health Organization.1991[cited 2019 April 1]. 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