{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T23:33:16Z","timestamp":1782516796197,"version":"3.54.5"},"reference-count":63,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,2,21]],"date-time":"2022-02-21T00:00:00Z","timestamp":1645401600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2022,2,21]],"date-time":"2022-02-21T00:00:00Z","timestamp":1645401600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100011852","name":"Paracelsus Medizinische Privatuniversit\u00e4t","doi-asserted-by":"publisher","award":["A\u201020\/01\/040\u2010WOS"],"award-info":[{"award-number":["A\u201020\/01\/040\u2010WOS"]}],"id":[{"id":"10.13039\/501100011852","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Hearing loss is a clinical symptom, frequently mentioned in the context of mitochondrial disease. With no cure available for mitochondrial disease, supportive treatment of clinical symptoms like hearing loss is of the utmost importance. The aim of this study was to summarize current knowledge on hearing loss in genetically proven mitochondrial disease in children and deduce possible and necessary consequences in patient care.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Systematic literature review, including Medline, Embase, and Cochrane library. Review protocol was established and registered prior to conduction (International prospective register of systematic reviews\u2014PROSPERO: CRD42020165356). Conduction of this review was done in accordance with MOOSE criteria.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 23 articles, meeting predefined criteria and providing sufficient information on 75 individuals with childhood onset hearing loss was included for analysis. Both cochlear and retro\u2010cochlear origin of hearing loss can be identified among different types of mitochondrial disease. Analysis was hindered by inhomogeneous reporting and methodical limitations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Overall, the findings do not allow for a general statement on hearing loss in children with mitochondrial disease. Retro\u2010cochlear hearing loss seems to be found more often than expected. A common feature appears to be progression of hearing loss over time. However, hearing loss in these patients shows manifold characteristics. Therefore, awareness of mitochondrial disease as a possible causative background is important for otolaryngologists. Future attempts rely on standardized reporting and long\u2010term follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Level of Evidence<\/jats:title>\n                    <jats:p>\n                      NA\n                      <jats:italic>Laryngoscope<\/jats:italic>\n                      , 132:2459\u20132472, 2022\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lary.30067","type":"journal-article","created":{"date-parts":[[2022,2,21]],"date-time":"2022-02-21T06:09:01Z","timestamp":1645423741000},"page":"2459-2472","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Mitochondrial Disease and Hearing Loss in Children: A Systematic Review"],"prefix":"10.1002","volume":"132","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3579-6435","authenticated-orcid":false,"given":"Sebastian","family":"Roesch","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"O'Sullivan","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery Paracelsus Medical University  Salzburg Austria"},{"name":"Insitute of Pathology Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georg","family":"Zimmermann","sequence":"additional","affiliation":[{"name":"Team Biostatistics and Big Medical Data, IDA Lab Salzburg Paracelsus Medical University  Salzburg Austria"},{"name":"Department of Research and Innovation Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alois","family":"Mair","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cvetka","family":"Lipu\u0161","sequence":"additional","affiliation":[{"name":"PMU University Library Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes A.","family":"Mayr","sequence":"additional","affiliation":[{"name":"University Children's Hospital Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saskia B.","family":"Wortmann","sequence":"additional","affiliation":[{"name":"University Children's Hospital Paracelsus Medical University  Salzburg Austria"},{"name":"Amalia Children's Hospital, Radboudumc  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4639-4044","authenticated-orcid":false,"given":"Gerd","family":"Rasp","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery Paracelsus Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,2,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2020.102730"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1603776"},{"key":"e_1_2_7_4_1","unstructured":"mitoNET. Accessed April 18 2021.https:\/\/www.mitonet.org"},{"key":"e_1_2_7_5_1","unstructured":"MetabERN. Accessed April 18 2021.https:\/\/metab.ern-net.eu"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00000673"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.cr.7310124"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2016.06.003"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.13347"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10540-007-9040-5"},{"key":"e_1_2_7_11_1","doi-asserted-by":"crossref","first-page":"2304","DOI":"10.3760\/cma.j.issn.0366-6999.20132799","article-title":"Audiological evaluation in Chinese patients with mitochondrial encephalomyopathies","volume":"127","author":"Liu Y","year":"2014","journal-title":"Chin Med J (Engl)"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0165-5876(94)90191-0"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200305000-00012"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.1.82"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.AUD.0000079802.82344.B5"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/119.3.741"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws340"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pmed.1000100"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.283.15.2008"},{"key":"e_1_2_7_20_1","unstructured":"PRISMA Statement. Accessed December 1 2019.https:\/\/www.prisma-statement.org\/"},{"key":"e_1_2_7_21_1","unstructured":"PROSPERO. Accessed December 1. 2019.https:\/\/www.crd.york.ac.uk\/prospero\/"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13643-019-1188-0"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2019.02.002"},{"key":"e_1_2_7_24_1","unstructured":"WellsGA SheaB O'ConnellD et al.The Newcastle\u2010Ottawa scale. Accessed December 1 2020.http:\/\/www.ohri.ca\/programs\/clinical_epidemiology\/oxford.asp"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjebm-2017-110853"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20681"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awm298"},{"key":"e_1_2_7_28_1","first-page":"169","article-title":"Kearns\u2010Sayre syndrome with facial and white matter extensive involvement: a (mitochondrial and nuclear gene related?) neurocristopathy?","volume":"39","author":"Berio A","year":"2017","journal-title":"Pediatr Med Chir"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-013-2166-5"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2011.08.019"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0044054"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2019.03.015"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0387-7604(00)00124-8"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.3109\/19401736.2014.905860"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2017.09.007"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2015.01.008"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2004.03.015"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000237781.10594.d1"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410430313"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348949710600414"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1258\/0022215011909224"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e3181e5c3a4"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2016.04.024"},{"key":"e_1_2_7_44_1","series-title":"Int J Clin Exp Med","first-page":"14457","volume-title":"Right auditory dysfunction during acute Leber's hereditary optic neuropathy harboring the 14484 mtDNA mutation: a case report","author":"Wang L","year":"2016"},{"key":"e_1_2_7_45_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-200103000-00024"},{"key":"e_1_2_7_46_1","first-page":"559","article-title":"Autosomal dominant optic atrophy with","volume":"25","author":"Maeda\u2010Katahira A","year":"2019","journal-title":"Mol Vis"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.ASN.0000080180.51098.02"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)92792-R"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.2337\/diab.43.6.746"},{"key":"e_1_2_7_50_1","unstructured":"HowickJ ChalmersI GlasziouP et al.Oxford Centre for evidence\u2010based medicine. Accessed April 18 2020.https:\/\/www.cebm.ox.ac.uk\/resources\/levels-of-evidence\/ocebm-levels-of-evidence"},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/120.10.1713"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.58.11.1885"},{"key":"e_1_2_7_53_1","article-title":"Mitochondrial sensorineural hearing loss: a retrospective study and a description of cochlear implantation in a MELAS patient","volume":"2012","author":"Scarpelli M","year":"2012","journal-title":"Genet Res Int"},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.24137"},{"key":"e_1_2_7_55_1","first-page":"15","article-title":"On renaming auditory neuropathy as auditory dys\u2010synchrony","volume":"13","author":"Berlin C","year":"2001","journal-title":"Audiol Today"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.3766\/jaaa.15050"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1080\/14992020802665959"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1097\/AUD.0b013e3181662c2a"},{"key":"e_1_2_7_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2017.02.008"},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-016-2813-8"},{"key":"e_1_2_7_61_1","doi-asserted-by":"publisher","DOI":"10.1097\/00003446-199906000-00006"},{"key":"e_1_2_7_62_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12070-014-0759-6"},{"key":"e_1_2_7_63_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2020.5030"},{"key":"e_1_2_7_64_1","first-page":"2677","article-title":"Establishment of normative data for monaural recordings of auditory brainstem response and its application in screening patients with hearing loss: a cohort study","volume":"7","author":"Chalak S","year":"2013","journal-title":"J Clin Diagn Res"}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.30067","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/lary.30067","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.30067","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T20:28:53Z","timestamp":1773001733000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.30067"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,21]]},"references-count":63,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["10.1002\/lary.30067"],"URL":"https:\/\/doi.org\/10.1002\/lary.30067","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,21]]},"assertion":[{"value":"2021-07-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-04","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}