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In 70% of affected patients, bi-allelic pathogenic variants in ERCC6 are identified. Although the role of ERCC6 in DNA damage repair has been studied, little is known about the mechanism for defective ERCC6 function in clinical findings, particularly hearing loss. To identify the mechanism of disease caused by pathogenic variants in ERCC6, we developed a zebrafish (Danio rerio) ercc6 loss of function model. We assessed survival after UV and metronidazole exposure, measured basal respiration rates, and evaluated mechanoelectrical transduction function and counts of lateral line hair cells. We found that UV exposure significantly reduces ercc6\u2212\/\u2212 larval viability. Metronidazole treatment results in complete lethality; wildtype controls show nearly complete survival. ercc6\u2212\/\u2212 embryos have significantly increased oxygen consumption, suggesting abnormal mitochondrial function. Phalloidin staining of lateral line hair cells with and without UV treatment shows no difference in hair cell counts per neuromast between treatment groups. Mechanoelectrical transduction function after UV exposure, measured by FM1\u201343 uptake, is reduced. Metronidazole lethality is reduced, oxygen consumption rates are restored, and mechanoelectrical transduction function is preserved by treatment with Mn(III)tetrakis(4-benzoic acid)porphyrin Chloride (MnTBAP), a superoxide dismutase mimetic. We propose that defective mitochondrial function and increased reactive oxygen species levels provide a mechanism for hair cell dysfunction in this model of Cockayne Syndrome. These results provide a foundation for further experiments to explore disease mechanisms and treatment modalities for this premature aging condition.<\/jats:p>","DOI":"10.1093\/hmg\/ddaf203","type":"journal-article","created":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T12:38:07Z","timestamp":1767098287000},"source":"Crossref","is-referenced-by-count":1,"title":["<i>ercc6<\/i>\n                    deficient zebrafish exhibit UV and metronidazole sensitivity, increased oxygen consumption, and impaired hair cell mechanoelectrical transduction which can be restored by the superoxide dismutase mimetic MnTBAP"],"prefix":"10.1093","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-2908-6744","authenticated-orcid":false,"given":"Gabriel A","family":"Hernandez Herrera","sequence":"first","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4324-7658","authenticated-orcid":false,"given":"Joseph A","family":"Dugdale","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2245-7295","authenticated-orcid":false,"given":"Jasmine G","family":"Wallace","sequence":"additional","affiliation":[{"name":"Summer Research Fellowship (SRF) Program, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7798-7443","authenticated-orcid":false,"given":"Ryan P","family":"Cotter","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2504-1493","authenticated-orcid":false,"given":"Alyssa M","family":"Jolliffe","sequence":"additional","affiliation":[{"name":"Robert and Arlene Kogod Center on Aging, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9637-0967","authenticated-orcid":false,"given":"Karl J","family":"Clark","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 First Street SW, Rochester, MN 55905 ,","place":["United States"]},{"name":"Department of Animal Science, Texas A&M University , 474 Olsen Blvd, College Station, TX 77843 ,","place":["United 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