{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T08:21:38Z","timestamp":1780561298477,"version":"3.54.1"},"reference-count":0,"publisher":"Caleo","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Int Adv Otol."],"abstract":"<jats:p>BACKGROUND: Sotos syndrome is a rare genetic disorder characterized by neurodevelopmental delay and excessive childhood growth including macrocephaly. In this study, we present our experience of children with Sotos syndrome and cholesteatoma.\nMETHODS: Retrospective case note review and cross-referencing with hospital picture archive and communication systems or cases identified from a prospective database of consecutive cholesteatoma surgeries.\nRESULTS: A total of 400 children underwent surgery for acquired cholesteatoma and 5 (1%) had Sotos syndrome (1 bilaterally). In comparison, 42(11%) had cleft palate which is around 10 times more common than Sotos syndrome, 5 (1%) had Down syndrome, and 3 (1%) had Turner syndrome. The median age at primary surgery was 8 years old (3.5-10.9 years), 124 children with Sotos syndrome were identified in picture archive and communication systems (4% with cholesteatoma) of which temporal bone imaging was available in 86 (70%) at the median age of 9 years (0-17.2), and 33\/86 (38%) had normal ears bilaterally on all imaging. Changes consistent with fluid or inflammation were present in 9\/30 (30%) computed tomography and 24\/72 (33%) magnetic resonance imaging scans. Development of mastoid pneumatization was impaired in 20\/30 (67%) computed tomography and 8\/72 (11%) magnetic resonance imaging scans. At 5 years, children with Sotos syndrome (33%) had greater recidivism than those with cleft palate (15%) (Kaplan\u2013Meier log-rank analysis, P=.001)\nCONCLUSION: Children with Sotos syndrome appear to be at increased risk of developing acquired cholesteatoma. Impaired temporal bone pneumatization is a common incidental finding in Sotos syndrome in keeping with this risk. Further study of this previously unreported association may improve the understanding of pathogenetic mechanisms in cholesteatoma.\nCite this article as: Leonard CG, Ranguis S, Lynn Cushing S, Blaser S, James A. Cholesteatoma in children with sotos syndrome. J Int Adv Otol. 2022;18(2):139-144.<\/jats:p>","DOI":"10.5152\/iao.2022.21309","type":"journal-article","created":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T03:26:50Z","timestamp":1649302010000},"page":"139-144","source":"Crossref","is-referenced-by-count":3,"title":["Cholesteatoma in Children with Sotos Syndrome"],"prefix":"10.65717","volume":"18","author":[{"given":"Colin","family":"Gerald Leonard","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Ranguis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Lynn Cushing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Blaser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian","family":"James","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"55248","published-online":{"date-parts":[[2022,3,30]]},"container-title":["The Journal of International Advanced Otology"],"original-title":[],"link":[{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1616\/1614","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1591\/1588","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1591\/1588","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T10:38:08Z","timestamp":1769078288000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/view\/1591"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,30]]},"references-count":0,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2022,3,30]]}},"URL":"https:\/\/doi.org\/10.5152\/iao.2022.21309","relation":{},"ISSN":["2148-3817"],"issn-type":[{"value":"2148-3817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,30]]}}}