{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T17:17:38Z","timestamp":1781111858697,"version":"3.54.1"},"reference-count":16,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,1,7]],"date-time":"2020-01-07T00:00:00Z","timestamp":1578355200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Margaret and Leo Meyer and Hans M. Hirsch Foundation"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Laryngoscope Investig Oto"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Describe the histopathology of the temporal bones in MELAS (myopathy, encephalopathy, lactic acidosis, and stroke\u2010like episodes) syndrome. The syndrome results from a known point mutation in mitochondrial DNA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Histopathology analysis of a pair of temporal bones from the oldest surviving MELAS syndrome temporal bone donor. Histopathologic findings were correlated with known premortem clinical data.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The inner ears showed severe but incomplete atrophy of the stria vascularis for the length of the cochleae. In contrast, the organ of Corti and inner hair cells appeared intact with some loss of outer hair cells. Other than moderate loss at the basal turn, spiral ganglion cells numbers were normal. The vestibular neuroepithelium was mostly normal with the exception of moderate degeneration of the macula sacculi and partial collapse of the saccular wall on the right. The cerebral cortex had infarct\u2010like lesions with adjacent gliosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is an analysis of the oldest patient with MELAS syndrome to date, an addition to only two previously published patients. It supports the notion that hearing loss is a result of dysfunction of the stria vascularis and not loss of hair cells or neurons. Patterns of vestibular pathology are in agreement to in\u2010vivo measurements. These findings support auditory rehabilitation with cochlear implants and may be relevant to hearing loss due to other mitochondrial mutations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of evidence<\/jats:title><jats:p>4<\/jats:p><\/jats:sec>","DOI":"10.1002\/lio2.344","type":"journal-article","created":{"date-parts":[[2020,1,7]],"date-time":"2020-01-07T17:56:37Z","timestamp":1578419797000},"page":"152-156","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Temporal bone histopathology in MELAS syndrome"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6905-3034","authenticated-orcid":false,"given":"Ophir","family":"Handzel","sequence":"first","affiliation":[{"name":"Cochlear Implant Center, Department of Otolaryngology\/Head, Neck &amp; Maxillofacial Surgery Tel\u2010Aviv Sourasky Medical Center, Sackler Faculty of Medicine  Tel\u2010Aviv University Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omer J.","family":"Ungar","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\/Head, Neck &amp; Maxillofacial Surgery Tel\u2010Aviv Sourasky Medical Center, Sackler Faculty of Medicine  Tel\u2010Aviv University Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan J.","family":"Lee","sequence":"additional","affiliation":[{"name":"Otopathology Laboratory, Department of Otolaryngology\u2010Head and Neck Surgery, Massachusetts Eye and Ear Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4033-3104","authenticated-orcid":false,"suffix":"Jr","given":"Joseph B.","family":"Nadol","sequence":"additional","affiliation":[{"name":"Otopathology Laboratory, Department of Otolaryngology\u2010Head and Neck Surgery, Massachusetts Eye and Ear Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,1,7]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2007.00800.x"},{"key":"e_1_2_6_3_1","first-page":"233","volume-title":"Genetic neuromuscular disorders: a case based approach","author":"Corrado A","year":"2014"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-018-0770-1"},{"key":"e_1_2_6_5_1","first-page":"29","volume-title":"Schuknecht's pathology of the ear","author":"Merchant SN","year":"2010"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tips.2012.03.010"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-00263-z"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.73.1.97"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2016.04.024"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537-200308000-00018"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5876(01)00546-8"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.3109\/00016480903148282"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1177\/2326409817697399"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-004-0817-x"},{"key":"e_1_2_6_15_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_6_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.71.2.281"},{"issue":"2","key":"e_1_2_6_17_1","first-page":"187","article-title":"Successful cochlear implantation in a patient with MELAS syndrome","volume":"20","author":"Rosenthal EL","year":"1999","journal-title":"Am J Otol"}],"container-title":["Laryngoscope Investigative Otolaryngology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flio2.344","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lio2.344","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/lio2.344","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lio2.344","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,7]],"date-time":"2023-09-07T07:59:27Z","timestamp":1694073567000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lio2.344"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1,7]]},"references-count":16,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["10.1002\/lio2.344"],"URL":"https:\/\/doi.org\/10.1002\/lio2.344","archive":["Portico"],"relation":{},"ISSN":["2378-8038","2378-8038"],"issn-type":[{"value":"2378-8038","type":"print"},{"value":"2378-8038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,1,7]]},"assertion":[{"value":"2019-10-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-12-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-01-07","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}