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This case highlights the importance of considering temporal bone abnormalities in all children with CSS or any dysmorphia, when they may require mastoid procedures. Reviewing the management of this case provides relevant learning opportunities for both primary, secondary and tertiary care institutions.<\/jats:p>","DOI":"10.1136\/bcr-2020-236139","type":"journal-article","created":{"date-parts":[[2021,1,18]],"date-time":"2021-01-18T11:57:34Z","timestamp":1610971054000},"page":"e236139","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Temporal bone dysplasia in Coffin-Siris syndrome"],"prefix":"10.1136","volume":"14","author":[{"given":"Jessica","family":"Wauchope","sequence":"first","affiliation":[{"name":"Department of Ear, Nose and Throat, The Royal Belfast Hospital for Sick Children, Belfast, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colin","family":"Leonard","sequence":"additional","affiliation":[{"name":"Department of Ear, Nose and Throat, The Royal Belfast Hospital for Sick Children, Belfast, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven","family":"McKinstry","sequence":"additional","affiliation":[{"name":"Radiology, The Royal Belfast Hospital for Sick Children, Belfast, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keith","family":"Trimble","sequence":"additional","affiliation":[{"name":"Department of Ear, Nose and Throat, The Royal Belfast Hospital for Sick Children, Belfast, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,1,18]]},"reference":[{"key":"2025082804055434000_14.1.e236139.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otohns.2009.04.008"},{"key":"2025082804055434000_14.1.e236139.2","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A4735"},{"key":"2025082804055434000_14.1.e236139.3","unstructured":"Som PM , Curtin HD . 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