{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:04:47Z","timestamp":1753877087691,"version":"3.41.2"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2022,1,14]],"date-time":"2022-01-14T00:00:00Z","timestamp":1642118400000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss.<\/jats:p>\n               <jats:p>We present the first case in literature of bilateral cochlear implantation (CI) in a patient with XP-D with neurodegeneration. Multi-disciplinary team members (national XP team, dermatologist, anaesthetist, theatre team, biophysicists) were involved. UV exposure from equipment and areas where the 14-year-old patient would track was measured. Maximum possible surgery was performed under operating headlights to limit higher-UV microscope exposure. Its bulb light intensity was reduced to achieve safe UV level (0\u201310\u00a0\u03bcW\/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus\u00ae-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation.<\/jats:p>\n               <jats:p>This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.<\/jats:p>","DOI":"10.1093\/jscr\/rjab594","type":"journal-article","created":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T20:12:58Z","timestamp":1639167178000},"source":"Crossref","is-referenced-by-count":0,"title":["Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss\u2014How to do it?"],"prefix":"10.1093","volume":"2022","author":[{"given":"H","family":"Woodun","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology, Queen\u2019s Medical Centre, Nottingham NG7 2UH, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H","family":"Woodun","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Lincoln County Hospital, Lincoln LN2 5QY, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R Vetrivel","family":"Vedachalam","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Queen\u2019s Medical Centre, Nottingham NG7 2UH, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H","family":"Fassihi","sequence":"additional","affiliation":[{"name":"Consultant Dermatologist and Clinical Lead for National XP Service, St John\u2019s Institute of Dermatology, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 7EP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Achar","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Queen\u2019s Medical Centre, Nottingham NG7 2UH, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,1,14]]},"reference":[{"key":"2022011417084943700_ref1","article-title":"Otopathology of xeroderma pigmentosum. NIDCD national temporal bone","volume":"21","author":"Kraemer","year":"2013","journal-title":"Hearing Balance Pathol Res Regist"},{"key":"2022011417084943700_ref2","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1136\/jmg.2010.083022","article-title":"Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair","volume":"48","author":"Bradford","year":"2011","journal-title":"J Med Genet"},{"key":"2022011417084943700_ref3","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1093\/brain\/aws317","article-title":"Auditory analysis of xeroderma pigmentosum 1971-2012: hearing function, sun sensitivity and DNA repair predict neurological degeneration","volume":"136","author":"Totonchy","year":"2013","journal-title":"Brain"},{"key":"2022011417084943700_ref4","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1097\/MAO.0b013e31829795e9","article-title":"Histopathology of the inner ear in patients with xeroderma pigmentosum and neurologic degeneration","volume":"34","author":"Viana","year":"2013","journal-title":"Otolo Neurotol"},{"key":"2022011417084943700_ref5","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1007\/978-981-10-6722-8","volume-title":"DNA Repair Disorders","author":"Nishigori","year":"2019"},{"key":"2022011417084943700_ref6","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1097\/MAO.0000000000002784","article-title":"Cochlear implant outcomes following vestibular schwannoma resection: systematic review","volume":"41","author":"Wick","year":"2020","journal-title":"Otol Neurotol"},{"key":"2022011417084943700_ref7","first-page":"18","article-title":"Adult-onset xeroderma pigmentosum neurological disease\u2013observations in an autopsy case","volume":"21","author":"Robbins","year":"2002","journal-title":"Clin Neuropathol"},{"key":"2022011417084943700_ref8","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1097\/MLG.0b013e3180325106","article-title":"Cochlear implantation in Cockayne syndrome: our experience of two cases with different outcomes","volume":"117","author":"Morris","year":"2007","journal-title":"Laryngoscope"},{"key":"2022011417084943700_ref9","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1186\/s13023-017-0616-2","article-title":"Xeroderma pigmentosum-Cockayne syndrome complex","volume":"12","author":"Natale","year":"2017","journal-title":"Orphanet J Rare Dis"},{"key":"2022011417084943700_ref10","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.ijporl.2017.12.029","article-title":"Cochlear implantation in pediatric patients with Cockayne syndrome","volume":"106","author":"Van Wyhe","year":"2018","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"2022011417084943700_ref11","doi-asserted-by":"crossref","first-page":"4293","DOI":"10.1111\/bph.14557","article-title":"Xeroderma pigmentosum: overview of pharmacology and novel therapeutic strategies for neurological symptoms","volume":"176","author":"Abeti","year":"2019","journal-title":"Br J Pharmacol"}],"container-title":["Journal of Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2022\/1\/rjab594\/42195511\/rjab594.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2022\/1\/rjab594\/42195511\/rjab594.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,14]],"date-time":"2022-01-14T17:47:15Z","timestamp":1642182435000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jscr\/article\/doi\/10.1093\/jscr\/rjab594\/6507364"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,1]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,1,1]]}},"URL":"https:\/\/doi.org\/10.1093\/jscr\/rjab594","relation":{},"ISSN":["2042-8812"],"issn-type":[{"type":"electronic","value":"2042-8812"}],"subject":[],"published-other":{"date-parts":[[2022,1]]},"published":{"date-parts":[[2022,1,1]]},"article-number":"rjab594"}}