{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T20:28:33Z","timestamp":1775593713783,"version":"3.50.1"},"reference-count":52,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,9,11]],"date-time":"2018-09-11T00:00:00Z","timestamp":1536624000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01HL129241"],"award-info":[{"award-number":["R01HL129241"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sickle cell disease (SCD) may cause injury to any organ, including the auditory system. Although the association of SCD and hearing loss has been described, the nature of this complication is unknown. We sought to establish the prevalence and nature of hearing loss in a referred cohort of children with SCD and to identify correlating disease\u2010 or treatment\u2010associated factors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>We conducted a retrospective review of patients with SCD\u00a0&lt;\u00a022 years of age who had hearing evaluations between August 1990 and December 2014. Demographics, audiograms, and disease and treatment variables were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two hundred and ten audiograms among 81 patients were reviewed, and 189 were evaluable. Seventy\u2010two children constituted the referred cohort. Fourteen (19.4%) had hearing loss documented on at least one audiogram. Seven (9.7%) patients had only conductive hearing loss, and the loss persisted for up to 10.3 years. The median age of first identification was eight years. Six (8.3%) patients had hearing loss that was at least partially sensorineural. One patient's hearing loss was ambiguous. All sensorineural hearing losses were unilateral and 4\/6 patients had prior documented normal hearing, indicating acquired loss. No correlations were identified.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Both conductive and sensorineural hearing losses are more prevalent in our study population than those observed in the general pediatric population. In children with SCD, sensorineural hearing loss appears to be acquired and unilateral. Conductive hearing loss was identified in older children and can persist. Serial screening is needed for early detection and more prompt intervention in this population.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.27457","type":"journal-article","created":{"date-parts":[[2018,9,12]],"date-time":"2018-09-12T05:54:45Z","timestamp":1536731685000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Prevalence and nature of hearing loss in a cohort of children with sickle cell disease"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8744-2618","authenticated-orcid":false,"given":"Alison S.","family":"Towerman","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics, Washington University School of Medicine St. Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susan S.","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Audiology St. Louis Children's Hospital St. Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert J.","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics, Washington University School of Medicine St. Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8933-0046","authenticated-orcid":false,"given":"Monica L.","family":"Hulbert","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics, Washington University School of Medicine St. Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,9,11]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcomdis.2005.02.007"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.21374"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348948709600304"},{"key":"e_1_2_9_5_1","first-page":"235","article-title":"Hearing loss in children with sickle cell disease","volume":"55","author":"Koussi A","year":"2001","journal-title":"Acta Otorhinolaryngol Belg"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2004.05.009"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2008.01.024"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2018.01.020"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/10245332.2018.1478494"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-198823000-00006"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1288\/00005537-198804000-00005"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/00003446-199110000-00007"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100124442"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.1996.641"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5876(98)90152-5"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0196-0709(00)85001-2"},{"key":"e_1_2_9_17_1","first-page":"21","article-title":"Sensorineural hearing loss in adults with sickle cell anemia","volume":"31","author":"Onakoya PA","year":"2002","journal-title":"Afr J Med Med Sci"},{"key":"e_1_2_9_18_1","first-page":"1135","article-title":"Hearing thresholds in sickle cell anemia patients: emerging new trends","volume":"97","author":"Aderibigbe A","year":"2005","journal-title":"J Natl Med Assoc"},{"key":"e_1_2_9_19_1","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1017\/S0022215106001678","article-title":"Sensorineural hearing loss with brainstem auditory evoked response changes in homozygote and heterozygote sickle cell patients in Guadeloupe (France)","volume":"120","author":"Jovanovic\u2010Batemen L","year":"2006","journal-title":"J Laryngol Otol"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)71047-3"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12916-015-0366-8"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2009-1997"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-2333"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1177\/10598405060220060401"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbi.2008.08.010"},{"key":"e_1_2_9_26_1","unstructured":"Centers for Disease Control and Prevention.Summary of 2009 national CDC EHDI data.https:\/\/www.cdc.gov\/ncbddd\/hearingloss\/2009-data\/2009_ehdi_hsfs_summary_508_ok.pdf. 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