Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?

Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?

Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. We present the first case in literature of bilateral cochlear implantation (CI) in a patient with XP-D with neurodegen...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Journal of surgical case reports 2022-01, Vol.2022 (1), p.rjab594-rjab594
Hauptverfasser: Woodun, H, Vedachalam, R Vetrivel, Fassihi, H, Achar, P
Format: Artikel
Sprache:eng
Schlagworte:
Surgical Technique
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page rjab594
container_issue 1
container_start_page rjab594
container_title Journal of surgical case reports
container_volume 2022
creator Woodun, H
Woodun, H
Vedachalam, R Vetrivel
Fassihi, H
Achar, P
description Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. 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 (0–10 μW/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus®-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation. This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.
doi_str_mv 10.1093/jscr/rjab594
format Article
fullrecord <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_8759561</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><oup_id>10.1093/jscr/rjab594</oup_id><sourcerecordid>2621658442</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3249-8f7088c9f61e1da263d3f191e42a4cc783cfc9062781bd59bcaa0a192ec6cd3c3</originalsourceid><addsrcrecordid>eNp9kcFu1DAQhi1ERau2N87IN4pEWttxEvsCggItUiU4gMTNmnUmu14ldmonLb3xBlx4Qp4Er3apygVfbI0__f_M_IQ85eyUM12erZONZ3ENi0rLR-RAMCkKpbh4_OC9T45TWrN8pOZc1U_Iflkx2fBGHZCfb10PE0boqQ121SNE6oaxBz_B5IKnzlOgd2H2SzrmCvqJ3rppRb9jDC3GAejolkMuhzQP9OTb5-LdCwq-pWMMy4gpuRukCX0K0XmcN0arbOKyXh9S-v3j12W4pVOgbaBuen1E9jroEx7v7kPy9cP7L-eXxdWni4_nb64KWwqpC9U1TCmru5ojb0HUZVt2XHOUAqS1jSptZzWrRaP4oq30wgIw4FqgrW1b2vKQvNrqjvNiwNbmAXJrZoxugHhnAjjz7493K7MMN0Y1la5qngVOdgIxXM-YJjO4ZLHPm8MwJyNqwetKSSky-nKL2pgnjtjd23BmNjGaTYxmF2PGnz1s7R7-G1oGnm-BMI__l_oDFnCs6A</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2621658442</pqid></control><display><type>article</type><title>Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?</title><source>Oxford Journals Open Access Collection</source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><creator>Woodun, H ; Woodun, H ; Vedachalam, R Vetrivel ; Fassihi, H ; Achar, P</creator><creatorcontrib>Woodun, H ; Woodun, H ; Vedachalam, R Vetrivel ; Fassihi, H ; Achar, P</creatorcontrib><description>Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. 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 (0–10 μW/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus®-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation. This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.</description><identifier>ISSN: 2042-8812</identifier><identifier>EISSN: 2042-8812</identifier><identifier>DOI: 10.1093/jscr/rjab594</identifier><identifier>PMID: 35047178</identifier><language>eng</language><publisher>England: Oxford University Press</publisher><subject>Surgical Technique</subject><ispartof>Journal of surgical case reports, 2022-01, Vol.2022 (1), p.rjab594-rjab594</ispartof><rights>Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2022. 2022</rights><rights>Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2022.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3249-8f7088c9f61e1da263d3f191e42a4cc783cfc9062781bd59bcaa0a192ec6cd3c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8759561/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8759561/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,885,1603,27922,27923,53789,53791</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/35047178$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Woodun, H</creatorcontrib><creatorcontrib>Woodun, H</creatorcontrib><creatorcontrib>Vedachalam, R Vetrivel</creatorcontrib><creatorcontrib>Fassihi, H</creatorcontrib><creatorcontrib>Achar, P</creatorcontrib><title>Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?</title><title>Journal of surgical case reports</title><addtitle>J Surg Case Rep</addtitle><description>Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. 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 (0–10 μW/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus®-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation. This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.</description><subject>Surgical Technique</subject><issn>2042-8812</issn><issn>2042-8812</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>TOX</sourceid><recordid>eNp9kcFu1DAQhi1ERau2N87IN4pEWttxEvsCggItUiU4gMTNmnUmu14ldmonLb3xBlx4Qp4Er3apygVfbI0__f_M_IQ85eyUM12erZONZ3ENi0rLR-RAMCkKpbh4_OC9T45TWrN8pOZc1U_Iflkx2fBGHZCfb10PE0boqQ121SNE6oaxBz_B5IKnzlOgd2H2SzrmCvqJ3rppRb9jDC3GAejolkMuhzQP9OTb5-LdCwq-pWMMy4gpuRukCX0K0XmcN0arbOKyXh9S-v3j12W4pVOgbaBuen1E9jroEx7v7kPy9cP7L-eXxdWni4_nb64KWwqpC9U1TCmru5ojb0HUZVt2XHOUAqS1jSptZzWrRaP4oq30wgIw4FqgrW1b2vKQvNrqjvNiwNbmAXJrZoxugHhnAjjz7493K7MMN0Y1la5qngVOdgIxXM-YJjO4ZLHPm8MwJyNqwetKSSky-nKL2pgnjtjd23BmNjGaTYxmF2PGnz1s7R7-G1oGnm-BMI__l_oDFnCs6A</recordid><startdate>20220101</startdate><enddate>20220101</enddate><creator>Woodun, H</creator><creator>Woodun, H</creator><creator>Vedachalam, R Vetrivel</creator><creator>Fassihi, H</creator><creator>Achar, P</creator><general>Oxford University Press</general><scope>TOX</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20220101</creationdate><title>Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?</title><author>Woodun, H ; Woodun, H ; Vedachalam, R Vetrivel ; Fassihi, H ; Achar, P</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3249-8f7088c9f61e1da263d3f191e42a4cc783cfc9062781bd59bcaa0a192ec6cd3c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Surgical Technique</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Woodun, H</creatorcontrib><creatorcontrib>Woodun, H</creatorcontrib><creatorcontrib>Vedachalam, R Vetrivel</creatorcontrib><creatorcontrib>Fassihi, H</creatorcontrib><creatorcontrib>Achar, P</creatorcontrib><collection>Oxford Journals Open Access Collection</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of surgical case reports</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Woodun, H</au><au>Woodun, H</au><au>Vedachalam, R Vetrivel</au><au>Fassihi, H</au><au>Achar, P</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?</atitle><jtitle>Journal of surgical case reports</jtitle><addtitle>J Surg Case Rep</addtitle><date>2022-01-01</date><risdate>2022</risdate><volume>2022</volume><issue>1</issue><spage>rjab594</spage><epage>rjab594</epage><pages>rjab594-rjab594</pages><issn>2042-8812</issn><eissn>2042-8812</eissn><abstract>Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. 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 (0–10 μW/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus®-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation. This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.</abstract><cop>England</cop><pub>Oxford University Press</pub><pmid>35047178</pmid><doi>10.1093/jscr/rjab594</doi><oa>free_for_read</oa></addata></record>
fulltext fulltext
identifier ISSN: 2042-8812
ispartof Journal of surgical case reports, 2022-01, Vol.2022 (1), p.rjab594-rjab594
issn 2042-8812
2042-8812
language eng
recordid cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_8759561
source Oxford Journals Open Access Collection; EZB-FREE-00999 freely available EZB journals; PubMed Central
subjects Surgical Technique
title Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-10T03%3A48%3A47IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Bilateral%20cochlear%20implantation%20in%20a%20young%20patient%20with%20xeroderma%20pigmentosum%20(XP-D)%20and%20progressive%20sensorineural%20hearing%20loss%E2%80%94How%20to%20do%20it?&rft.jtitle=Journal%20of%20surgical%20case%20reports&rft.au=Woodun,%20H&rft.date=2022-01-01&rft.volume=2022&rft.issue=1&rft.spage=rjab594&rft.epage=rjab594&rft.pages=rjab594-rjab594&rft.issn=2042-8812&rft.eissn=2042-8812&rft_id=info:doi/10.1093/jscr/rjab594&rft_dat=%3Cproquest_pubme%3E2621658442%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2621658442&rft_id=info:pmid/35047178&rft_oup_id=10.1093/jscr/rjab594&rfr_iscdi=true