{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T14:59:41Z","timestamp":1774277981089,"version":"3.50.1"},"reference-count":0,"publisher":"Caleo","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Int Adv Otol"],"abstract":"<jats:p>Cornelia de Lange syndrome (CdLS) is a rare congenital multisystem syndrome characterized by a distinctive craniofacial appearance, developmental delay, intellectual disability, limb abnormalities, and hypertrichosis. Hearing loss is one of the common manifestations in CdLS. This study presents a 6-year-old child with CdLS, who exhibited developmental delay, intellectual disability, and displayed autistic features, and experienced challenges during right cochlear implant (CI) surgery at the age of 5 due to inner ear abnormality. After the CI, the sound field hearing test showed minimal response levels of 60-70 dB HL at post-CI 12 months. He obtained scores of 2%, 14%, 16%, and 27% on the PEACH rating scale at pre-CI, post-CI 3 months, post-CI 6 months, and post-CI 12 months, respectively. Nevertheless, for CdLS patients with severe-to-profound hearing loss, CI can offer potential improvements in auditory skills and social connections, even though the benefits may be limited. It remains a viable solution.<\/jats:p>","DOI":"10.65717\/iao.2026.252138","type":"journal-article","created":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T14:09:08Z","timestamp":1774274948000},"page":"1-4","source":"Crossref","is-referenced-by-count":0,"title":["Cochlear Implantation in a Child with Cornelia de Lange Syndrome: Challenges and Prospect"],"prefix":"10.65717","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-6908-1641","authenticated-orcid":false,"given":"I-Hsiu","family":"Chen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-4413-0753","authenticated-orcid":false,"given":"Pei-Hsuan","family":"Ho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-8613-5860","authenticated-orcid":false,"given":"Mei-Chi","family":"Chiu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9014-8986","authenticated-orcid":false,"given":"Chia-Huei","family":"Chu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4711-7818","authenticated-orcid":false,"given":"Pey-Yu","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8698-4212","authenticated-orcid":false,"given":"Hung-Ching","family":"Lin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"55248","published-online":{"date-parts":[[2026,3,5]]},"container-title":["The Journal of International Advanced Otology"],"original-title":[],"link":[{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1962\/1949","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/download\/1962\/1949","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T14:09:08Z","timestamp":1774274948000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.advancedotology.org\/index.php\/pub\/article\/view\/1962"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,5]]},"references-count":0,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,2,24]]}},"URL":"https:\/\/doi.org\/10.65717\/iao.2026.252138","relation":{},"ISSN":["2148-3817"],"issn-type":[{"value":"2148-3817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,5]]}}}