{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T04:29:33Z","timestamp":1778041773244,"version":"3.51.4"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,7,9]],"date-time":"2020-07-09T00:00:00Z","timestamp":1594252800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hearing loss is frequently seen in mucopolysaccharidoses (MPS) patients. Although hematopoietic cell transplantation (HCT) increases overall survival, disease progression is observed in certain tissues. This study describes the course of hearing loss (HL) over time in transplanted MPS patients. Transplanted MPS patients between 2003 and 2018 were included and received yearly audiological evaluation, including auditory brainstem response (ABR) or pure tone audiometry (PTA). Twenty\u2010eight MPS\u20101 and four MPS\u20106 patients were analyzed with a median follow\u2010up of 5\u2009years (range 11\u2009months\u201316\u2009years). Air conduction threshold improved significantly over time (<jats:italic>P<\/jats:italic>\u2009&lt;\u2009.001) with a PTA 1\u2010year post\u2010HCT of 50\u2009\u00b1\u20090.7 dB to 23\u2009\u00b1\u200911\u2009dB 13\u2009years post\u2010HCT. Bone conduction threshold worsened with a PTA 1 year post\u2010HCT of 10\u2009\u00b1\u20097 dB to 18\u2009\u00b1\u20099 dB 13\u2009years post\u2010HCT (<jats:italic>P<\/jats:italic> = .34). The degree of HL varied from mainly mild\u2010severe early after HCT to normal\u2010mild at longer follow\u2010up. The type of HL consisted of mainly conductive in the first years post\u2010HCT in contrast to mainly sensorineural at longer follow\u2010up. MRIs of the cerebellopontine angle did not show abnormalities. HL is still seen in patients with MPS despite HCT and consists of a conductive type early after HCT in contrast to a sensorineural type at longer follow\u2010up in the majority of cases. Yearly follow\u2010up of HL is necessary to timely intervene, as hearing is important in the speech and language development of children and their academic achievements.<\/jats:p>","DOI":"10.1002\/jimd.12277","type":"journal-article","created":{"date-parts":[[2020,6,24]],"date-time":"2020-06-24T16:42:59Z","timestamp":1593016979000},"page":"1279-1287","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["<scp>Hearing loss in patients with mucopolysaccharidoses<\/scp>\u20101 <scp>and<\/scp> \u20106 <scp>after hematopoietic cell transplantation:<\/scp> A longitudinal analysis"],"prefix":"10.1002","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3208-0685","authenticated-orcid":false,"given":"Brigitte T. A.","family":"van den Broek","sequence":"first","affiliation":[{"name":"Sylvia Toth Center for Multidisciplinary Follow up after Hematopoietic Cell Transplantation Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"},{"name":"Pediatric Blood and Marrow Transplantation Program, Department of Child Health Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"},{"name":"Section of Metabolic Diseases, Department of Child Health Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adriana L.","family":"Smit","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology\u2014Head and Neck Surgery University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"},{"name":"Brain Center Rudolf Magnus University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jaap Jan","family":"Boelens","sequence":"additional","affiliation":[{"name":"Sylvia Toth Center for Multidisciplinary Follow up after Hematopoietic Cell Transplantation Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"},{"name":"Pediatric Blood and Marrow Transplantation Program, Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter M.","family":"van Hasselt","sequence":"additional","affiliation":[{"name":"Sylvia Toth Center for Multidisciplinary Follow up after Hematopoietic Cell Transplantation Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University  Utrecht The Netherlands"},{"name":"Section of Metabolic Diseases, Department of Child Health Wilhelmina Children's Hospital, University Medical Center 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