{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T18:06:09Z","timestamp":1774548369938,"version":"3.50.1"},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T00:00:00Z","timestamp":1689033600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Laryngoscope Investig Oto"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>This systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness secondary to Waardenburg syndrome (WS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic review of the literature and narrative synthesis was performed in accordance with the PRISMA statement. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">ClinicalTrials.gov<\/jats:ext-link>. No limits were placed on language or year of publication.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Searches identified 186 abstracts and full texts. Of these, 16 studies met inclusion criteria reporting outcomes in 179 patients and at least 194 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 11 peri\/post\u2010operative complications were reported. The methodological quality of included studies was modest, mainly comprising noncontrolled case series with small cohort size. All studies were OCEBM grade III\u2013IV.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Cochlear implantation in congenitally deafened children with Waardenburg Syndrome is a well\u2010established intervention as a method of auditory rehabilitation. Due to the uncommon nature of the condition, there is a lack of large\u2010scale high\u2010quality studies examining the use of cochlear implantation in this patient group. However, overall outcomes following implantation are positive with the majority of patients demonstrating improved audiometry, speech perception and speech intelligibility supporting its use in appropriately selected cases.<\/jats:p><\/jats:sec>","DOI":"10.1002\/lio2.1110","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T12:29:33Z","timestamp":1689078573000},"page":"1094-1107","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Outcomes of Cochlear implantation in early\u2010deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9079-3902","authenticated-orcid":false,"given":"Amy","family":"Lovett","sequence":"first","affiliation":[{"name":"Royal Stoke University Hospital  Stoke on Trent UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2630-921X","authenticated-orcid":false,"given":"Michael","family":"Eastwood","sequence":"additional","affiliation":[{"name":"Royal Stoke University Hospital  Stoke on Trent UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8790-2722","authenticated-orcid":false,"given":"Chris","family":"Metcalfe","sequence":"additional","affiliation":[{"name":"Royal Stoke University Hospital  Stoke on Trent UK"},{"name":"University Hospitals Birmingham NHS Foundation Trust Queen Elizabeth Hospital Birmingham  Edgbaston UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3065-0269","authenticated-orcid":false,"given":"Jameel","family":"Muzaffar","sequence":"additional","affiliation":[{"name":"University Hospitals Birmingham NHS Foundation Trust Queen Elizabeth Hospital Birmingham  Edgbaston UK"},{"name":"Department of Clinical Neurosciences University of Cambridge  Cambridge UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7343-7105","authenticated-orcid":false,"given":"Peter","family":"Monksfield","sequence":"additional","affiliation":[{"name":"University Hospitals Birmingham NHS Foundation Trust Queen Elizabeth Hospital Birmingham  Edgbaston UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8050-3617","authenticated-orcid":false,"given":"Manohar","family":"Bance","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences University of Cambridge  Cambridge UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,7,11]]},"reference":[{"issue":"3","key":"e_1_2_8_2_1","first-page":"195","article-title":"A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness","volume":"3","author":"Waardenburg PJ","year":"1951","journal-title":"Am J Hum Genet"},{"key":"e_1_2_8_3_1","unstructured":"NIH Genetics Home Reference.Waardenburg Syndrome. 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