{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T08:16:44Z","timestamp":1778141804978,"version":"3.51.4"},"reference-count":26,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T00:00:00Z","timestamp":1767312000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Laryngol. Otol."],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec id=\"S0022215125104209_sec1a\">\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This study aimed to report a 10-year single-centre experience of cochlear implantation in children with CHARGE syndrome (manifests as coloboma, heart defects, atresia of the choanae, growth retardation and genital and ear anomalies) and evaluate surgical strategies and outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"S0022215125104209_sec2b\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Children with CHARGE syndrome who underwent cochlear implantation between 2014 and 2024 were retrospectively reviewed. Patient characteristics and post-operative auditory outcomes were analysed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"S0022215125104209_sec3c\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six patients (nine ears) were included. Most had airway anomalies, one-third required post-operative intensive care. Temporal bone malformations were present in 78 per cent of ears. Standard posterior tympanotomy was feasible in one-third; the remainder required transcanal or combined approaches. Full-electrode insertion was achieved in all ears. Median follow-up was 3.4 years; aided thresholds ranged 25 to 50 dB hearing level. Three patients developed reliable open-set speech perception, two achieved closed-set recognition and one demonstrated environmental sound awareness.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"S0022215125104209_sec4d\">\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Cochlear implantation provides meaningful auditory benefit in selected children with CHARGE syndrome. Pre-operative cochlear nerve imaging, flexible surgical planning and coordinated peri-operative airway management are essential.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1017\/s0022215125104209","type":"journal-article","created":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T09:15:21Z","timestamp":1767345321000},"page":"374-380","source":"Crossref","is-referenced-by-count":0,"title":["Cochlear implantation in patients with CHARGE syndrome: a 10-year institutional experience and literature review"],"prefix":"10.1017","volume":"140","author":[{"given":"Nanoka","family":"Sugamo","sequence":"first","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyoko","family":"Shirai","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-9918-0930","authenticated-orcid":false,"given":"Ryota","family":"Tomioka","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keitaro","family":"Miyake","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sayaka","family":"Arai","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Teruhisa","family":"Yano","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuhiro","family":"Nishiyama","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyoaki","family":"Tsukahara","sequence":"additional","affiliation":[{"name":"Tokyo Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2026,1,2]]},"reference":[{"key":"S0022215125104209_ref12","doi-asserted-by":"publisher","DOI":"10.1002\/lary.23682"},{"key":"S0022215125104209_ref18","doi-asserted-by":"publisher","DOI":"10.1177\/01945998211008911"},{"key":"S0022215125104209_ref26","doi-asserted-by":"publisher","DOI":"10.1097\/MLG.0b013e31806009c9"},{"key":"S0022215125104209_ref3","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-013-2424-1"},{"key":"S0022215125104209_ref1","doi-asserted-by":"publisher","DOI":"10.1002\/ohn.808"},{"key":"S0022215125104209_ref25","unstructured":"25 CHARGE Syndrome Foundation. Foundation supported research. In: https:\/\/www.chargesyndrome.org\/overview\/research\/foundation-supported-research\/ [ August 2025 ]"},{"key":"S0022215125104209_ref4","doi-asserted-by":"publisher","DOI":"10.1080\/14670100.2019.1634857"},{"key":"S0022215125104209_ref10","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0000000000000915"},{"key":"S0022215125104209_ref17","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.3002786"},{"key":"S0022215125104209_ref11","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0000000000001488"},{"key":"S0022215125104209_ref20","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31591"},{"key":"S0022215125104209_ref15","first-page":"1663","article-title":"Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings","volume":"27","author":"Morimoto","year":"2006","journal-title":"AJNR Am J Neuroradiol"},{"key":"S0022215125104209_ref21","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2024.1348439"},{"key":"S0022215125104209_ref16","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-020-02382-3"},{"key":"S0022215125104209_ref22","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215122001438"},{"key":"S0022215125104209_ref8","doi-asserted-by":"publisher","DOI":"10.1177\/000992289803700302"},{"key":"S0022215125104209_ref23","doi-asserted-by":"publisher","DOI":"10.1067\/mhn.2000.108203"},{"key":"S0022215125104209_ref5","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0000000000001528"},{"key":"S0022215125104209_ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.bjorl.2023.101383"},{"key":"S0022215125104209_ref24","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2024.111875"},{"key":"S0022215125104209_ref9","first-page":"396","article-title":"Expert consensus on auditory intervention and language rehabilitation in CHARGE syndrome","volume":"39","year":"2025","journal-title":"Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi"},{"key":"S0022215125104209_ref19","unstructured":"19 OrphanAnesthesia Expert Group. CHARGE syndrome: Anaesthesia recommendations. OrphanAnesthesia 2019 In: https:\/\/www.orphananesthesia.eu\/rare-diseases\/published-guidelines\/charge-syndrome\/630-charge-syndrome-en-2019\/file.html [ November 2025 ]"},{"key":"S0022215125104209_ref7","first-page":"198","article-title":"Perioperative management of cochlear implantation for CHARGE syndrome","volume":"36","author":"Lin","year":"2022","journal-title":"Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi"},{"key":"S0022215125104209_ref2","doi-asserted-by":"publisher","DOI":"10.1159\/000500659"},{"key":"S0022215125104209_ref6","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000018283"},{"key":"S0022215125104209_ref13","doi-asserted-by":"publisher","DOI":"10.1177\/0194599817718798"}],"container-title":["The Journal of Laryngology &amp; Otology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0022215125104209","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T08:04:26Z","timestamp":1778141066000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0022215125104209\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,2]]},"references-count":26,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["S0022215125104209"],"URL":"https:\/\/doi.org\/10.1017\/s0022215125104209","relation":{},"ISSN":["0022-2151","1748-5460"],"issn-type":[{"value":"0022-2151","type":"print"},{"value":"1748-5460","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,2]]}}}