{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,10]],"date-time":"2026-02-10T16:46:14Z","timestamp":1770741974930,"version":"3.49.0"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,6,30]],"date-time":"2020-06-30T00:00:00Z","timestamp":1593475200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Outcomes following cochlear implantation in children with X\u2010linked deafness\u20102 are variable, resulting in challenges in appropriate preoperative counseling. To address this uncertainty, we performed a systematic review and synthesis of the literature on audiologic and speech outcomes after cochlear implantation in these patients to inform prognostic counseling.<\/jats:p><\/jats:sec><jats:sec><jats:title>Data Sources<\/jats:title><jats:p>PubMed, Embase, and Cochrane Library were queried for articles published between January 2000 and July 2019.<\/jats:p><\/jats:sec><jats:sec><jats:title>Review Methods<\/jats:title><jats:p>We performed a systematic review of all studies published between 2000 and 2019 that reported on (1) children with confirmed X\u2010linked deafness\u20102 undergoing cochlear implantation and (2) formal assessment of hearing and\/or speech capabilities postimplantation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Our initial database search yielded 313 articles. Fourteen articles met inclusion criteria. These studies reported on 61 children with X\u2010linked deafness\u20102 who underwent implantation at a wide age range (1\u201029 years) for severe\u2010profound sensorineural hearing loss of prelingual onset. The mean follow\u2010up duration after implant activation was 32\u00a0months (range, 12\u201061). Outcome domains assessed at follow\u2010up were heterogeneous, though each study employed at least 1 assessment of hearing (eg, pure tone audiometry), speech perception (eg, Early Speech Perception Test), or auditory perception (eg, Categories of Auditory Perception scores). In 10 of 14 studies, cochlear implantation afforded significant improvement in hearing and speech capabilities relative to preoperative performance or as compared with age\u2010matched, normal\u2010hearing controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The majority of studies demonstrate that cochlear implantation provides improvements in hearing and speech performance in patients with X\u2010linked deafness\u20102. This information is valuable for decision making regarding cochlear implantation in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1177\/0194599820932138","type":"journal-article","created":{"date-parts":[[2020,6,30]],"date-time":"2020-06-30T04:11:07Z","timestamp":1593490267000},"page":"19-26","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Systematic Review of Outcomes After Cochlear Implantation in Children With X\u2010Linked Deafness\u20102"],"prefix":"10.1002","volume":"164","author":[{"given":"Joshua D.","family":"Smith","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery University of Michigan Medical School Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nour","family":"El\u2010Kashlan","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Feinberg School of Medicine Northwestern University Chicago Illinois USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Owen A. F.","family":"Darr","sequence":"additional","affiliation":[{"name":"Department of Pediatric Otolaryngology Children\u2019s Hospital Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc C.","family":"Thorne","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery University of Michigan Medical School Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,6,30]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2014-306099"},{"key":"e_1_2_8_3_1","unstructured":"University of Antwerp.Hereditary hearing loss. 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