{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T11:51:01Z","timestamp":1779105061198,"version":"3.51.4"},"reference-count":9,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"publisher","award":["P30GM114736"],"award-info":[{"award-number":["P30GM114736"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["SAGE Open Medical Case Reports"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:p> Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12\u2009months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12\u2009months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process. <\/jats:p>","DOI":"10.1177\/2050313x19873791","type":"journal-article","created":{"date-parts":[[2019,8,31]],"date-time":"2019-08-31T06:00:15Z","timestamp":1567231215000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["Cochlear implantation in a patient with mucopolysaccharidosis IVA"],"prefix":"10.1177","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0917-6603","authenticated-orcid":false,"given":"Kyoko","family":"Nagao","sequence":"first","affiliation":[{"name":"Nemours Biomedical Research, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"},{"name":"Department of Linguistics and Cognitive Science, University of Delaware, Newark, DE, USA"},{"name":"Communication Sciences and Disorders, College of Health Sciences, University of Delaware, Newark, DE, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cassidy","family":"Walter","sequence":"additional","affiliation":[{"name":"Department of Linguistics and Cognitive Science, University of Delaware, Newark, DE, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William J","family":"Parkes","sequence":"additional","affiliation":[{"name":"Otolaryngology, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"},{"name":"Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Teixido","sequence":"additional","affiliation":[{"name":"Otolaryngology, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"},{"name":"Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA"},{"name":"Otology, Christiana Care Health System, Wilmington, DE, USA"},{"name":"Department of Otorhinolaryngology, University of Pennsylvania, Philadelphia, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary C","family":"Theroux","sequence":"additional","affiliation":[{"name":"Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA"},{"name":"Department of Anesthesiology and Perioperative Medicine, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stacy","family":"Szymkowski","sequence":"additional","affiliation":[{"name":"Department of Audiology, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thierry","family":"Morlet","sequence":"additional","affiliation":[{"name":"Nemours Biomedical Research, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"},{"name":"Department of Linguistics and Cognitive Science, University of Delaware, Newark, DE, USA"},{"name":"Communication Sciences and Disorders, College of Health Sciences, University of Delaware, Newark, DE, USA"},{"name":"Osborne College of Audiology, Salus University, Elkins Park, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shunji","family":"Tomatsu","sequence":"additional","affiliation":[{"name":"Nemours Biomedical Research, Nemours\/Alfred I. duPont Hospital for Children, Wilmington, DE, USA"},{"name":"Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2019,8,30]]},"reference":[{"key":"bibr1-2050313X19873791","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2013.04.002"},{"key":"bibr2-2050313X19873791","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0529-7"},{"key":"bibr3-2050313X19873791","doi-asserted-by":"publisher","DOI":"10.1186\/s13052-018-0561-2"},{"key":"bibr4-2050313X19873791","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1977.00780260048003"},{"key":"bibr5-2050313X19873791","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2018.02.002"},{"key":"bibr6-2050313X19873791","first-page":"59","volume":"15","author":"Lavery C","year":"2015","journal-title":"JIMD 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