{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T23:42:35Z","timestamp":1773099755638,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,9,21]],"date-time":"2024-09-21T00:00:00Z","timestamp":1726876800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Hearing loss is considered common in children with 22q11.2 deletion syndrome (22q11.2DS), with a few prior studies reporting a 32%\u201378% prevalence; mild\u2013moderate conductive hearing loss has been reported most commonly. Overall, however, there remains a paucity of data regarding the frequency, type, age, and progression of hearing loss in children with 22q11.2DS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective chart review was completed, and data combined for two large 22q centers. Inclusion criteria were children with 22q11.2DS and a documented audiogram. Data extracted included a laboratory\u2010confirmed chromosome 22q11.2 deletion; co\u2010morbidities; results of all audiograms and radiologic temporal bone imaging; and otologic surgical procedures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One thousand seven hundred sixty\u2010nine charts were reviewed; 775 met inclusion criteria. Of these, 563 (73%) children had at least one abnormal audiogram demonstrating hearing loss. A total of 2,536 audiograms were reviewed; 74% of these showed abnormal hearing in at least one ear. Most of the hearing loss was conductive (right ear 76%; left ear 69%) and mild severity. For the children with SNHL, 90% of all follow\u2010up audiograms were stable without progression. Hearing loss was identified across all pediatric age ranges. Ear tube placement occurred in 39% of children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study confirms the high incidence of hearing loss for children with 22q11.2DS at some point in their childhood. In our cohort, hearing loss occurred in 73% of children and was most often conductive and mild in severity. The results highlight the importance of otolaryngology and audiology involvement in managing children with 22q11.2DS for timely diagnosis and treatment of hearing loss.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 135:929\u2013934, 2025<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.31777","type":"journal-article","created":{"date-parts":[[2024,9,21]],"date-time":"2024-09-21T12:09:35Z","timestamp":1726920575000},"page":"929-934","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Hearing Loss in Children with 22q11.2 Deletion Syndrome"],"prefix":"10.1002","volume":"135","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6527-1253","authenticated-orcid":false,"given":"Jill","family":"Arganbright","sequence":"first","affiliation":[{"name":"Division of Pediatric Otolaryngology, Children's Mercy Hospital  Kansas Missouri U.S.A."},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas Missouri U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Terrence Blaine","family":"Crowley","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Human Genetics Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meghan","family":"Tracy","sequence":"additional","affiliation":[{"name":"Division of Pediatric Otolaryngology, Children's Mercy Hospital  Kansas Missouri U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8171-6056","authenticated-orcid":false,"given":"Janelle","family":"Noel\u2010MacDonnell","sequence":"additional","affiliation":[{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas Missouri 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U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jamie","family":"Hamm","sequence":"additional","affiliation":[{"name":"Division of Hearing and Speech, Children's Mercy Hospital  Kansas Missouri U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernice","family":"Morrow","sequence":"additional","affiliation":[{"name":"Albert Einstein Collect of Medicine  Bronx New York U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hansoo","family":"Song","sequence":"additional","affiliation":[{"name":"Albert Einstein Collect of Medicine  Bronx New York U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Victoria","family":"Giunta","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Human Genetics Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel E.","family":"McGinn","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Human Genetics Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elaine H.","family":"Zackai","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Human Genetics Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Department of Pediatrics Perelman School of Medicine of the University of Pennsylvania  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beverly","family":"Emanuel","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Human Genetics Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Department of Pediatrics Perelman School of Medicine of the University of Pennsylvania  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Elden","sequence":"additional","affiliation":[{"name":"22q and You Center, Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."},{"name":"Department of Surgery Perelman School of Medicine of the University of Pennsylvania  Philadelphia Pennsylvania U.S.A."},{"name":"Division of Otolaryngology Children's Hospital of Philadelphia  Philadelphia Pennsylvania U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Srivats","family":"Narayanan","sequence":"additional","affiliation":[{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas Missouri 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