{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:20:40Z","timestamp":1790727640433,"version":"4.1.0"},"reference-count":14,"publisher":"Georg Thieme Verlag KG","issue":"03","license":[{"start":{"date-parts":[[2020,9,24]],"date-time":"2020-09-24T00:00:00Z","timestamp":1600905600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Int Arch Otorhinolaryngol"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Introduction\u2003Mucopolysaccharidosis (MPS) is a set of rare diseases caused by deficiency of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (GAG) in tissues and organs, which, in turn, is responsible for the multisystemic clinical, chronic, and progressive symptoms.<\/jats:p><jats:p>\n          Objective\u2003To describe the profile of the otorhinolaryngological clinical examination and audiology tests of patients with MPS disease.<\/jats:p><jats:p>\n          Methods\u2003The present study is a case series. The evaluation was performed, initially, in 24 patients with MPS types I, II, IIIA, IV and VI.<\/jats:p><jats:p>\n          Results\u2003The most common hearing complaint was hearing loss, which was confirmed by audiology tests in almost 100% of the patients, most of whom presented conductive hearing loss.<\/jats:p><jats:p>\n          Conclusions\u2003It is important to evaluate the complaints, physical examination, and audiology tests in patients with MPS. The otorhinolaryngologistshould be part of the group of professionals that follows these patients to better monitor their hearing and provide early hearing rehabilitation.<\/jats:p>","DOI":"10.1055\/s-0040-1712107","type":"journal-article","created":{"date-parts":[[2020,9,24]],"date-time":"2020-09-24T22:49:06Z","timestamp":1600987746000},"page":"e386-e391","source":"Crossref","is-referenced-by-count":4,"title":["Hearing Loss in Mucopolysaccharidosis"],"prefix":"10.1055","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2829-7646","authenticated-orcid":false,"given":"Cibele Gomes","family":"Bicalho","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universit\u00e1rio Professor Edgard Santos, Salvador, BA, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Em\u00edlia Katiane Embiru\u00e7u","family":"de Ara\u00fajo Le\u00e3o","sequence":"additional","affiliation":[{"name":"Medical Genetics Service, Hospital Universit\u00e1rio Professor Edgard Santos (HUPES), Salvador, BA, Brazil"},{"name":"Department of Life Sciences, Universidade Estadual da Bahia (UNEB), BA, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1lvaro Mui\u00f1os","family":"de Andrade","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universit\u00e1rio Professor Edgard Santos, Salvador, BA, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelina Xavier","family":"Acosta","sequence":"additional","affiliation":[{"name":"Medical Genetics Service, Hospital Universit\u00e1rio Professor Edgard Santos (HUPES), Salvador, BA, Brazil"},{"name":"Department of Pediatrics, Universidade Federal da Bahia, Salvador, BA, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,9,24]]},"reference":[{"issue":"S 05","key":"ref1","doi-asserted-by":"crossref","first-page":"v4","DOI":"10.1093\/rheumatology\/ker394","article-title":"Overview of the mucopolysaccharidoses","volume":"50","author":"J Muenzer","year":"2011","journal-title":"Rheumatology (Oxford)"},{"issue":"03","key":"ref2","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1136\/adc.72.3.263","article-title":"The mucopolysaccharidoses: a clinical review and guide to management","volume":"72","author":"J E Wraith","year":"1995","journal-title":"Arch Dis Child"},{"issue":"06","key":"ref3","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1007\/s10545-005-0112-z","article-title":"Cumulative incidence rates of the mucopolysaccharidoses in Germany","volume":"28","author":"F Baehner","year":"2005","journal-title":"J Inherit Metab Dis"},{"issue":"01","key":"ref5","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.ijporl.2009.09.042","article-title":"Role of the pediatric otolaryngologist in diagnosis and management of children with mucopolysaccharidoses","volume":"74","author":"S M Wold","year":"2010","journal-title":"Int J Pediatr Otorhinolaryngol"},{"issue":"01","key":"ref6","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.ymgme.2007.01.011","article-title":"The MPS I registry: design, methodology, and early findings of a global disease registry for monitoring patients with Mucopolysaccharidosis Type I","volume":"91","author":"G M Pastores","year":"2007","journal-title":"Mol Genet Metab"},{"issue":"05","key":"ref8","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/j.jpeds.2004.01.046","article-title":"Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase)","volume":"144","author":"J E Wraith","year":"2004","journal-title":"J Pediatr"},{"issue":"02","key":"ref9","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1007\/s10545-011-9378-5","article-title":"Hearing loss in patients with mucopolysaccharidosis II: data from HOS - 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