{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T18:22:06Z","timestamp":1777918926718,"version":"3.51.4"},"reference-count":30,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2010,10,7]],"date-time":"2010-10-07T00:00:00Z","timestamp":1286409600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Vet Pathol"],"published-print":{"date-parts":[[2011,5]]},"abstract":"<jats:p>Mucopolysaccharidosis (MPS) types I and VII are inborn errors of metabolism caused by mutation of enzymes involved in glycosaminoglycan catabolism, which leads to intralysosomal accumulation of glycosaminoglycans. In children, severe forms of MPS I and VII are characterized by somatic and neurologic manifestations, including a poorly understood hearing loss. The purpose of this study is to describe the age-related histopathologic changes of the ear in spontaneous canine models of MPS I and VII. Pathologic changes in the ear were assessed in MPS I and VII dogs ranging from 1.6 to 9.3 months of age. Paraffin-embedded sections of the whole ear and Epon-embedded semithin sections of the cochlea were examined. The following lesions were blindly scored in the middle and inner ear: inflammation, cells vacuolization, thickening of osseous and membranous structures, perivascular vacuolated macrophages infiltration, and bone resorption. All dogs had lysosomal storage within cells of tympanic membrane, ossicles, tympanic bone and mucosa, cochlear bone, spiral ligament, limbus, and stria vascularis. The MPS I dogs mainly had progressive cochlear lesions. The MPS VII dogs had severe and early middle ear lesions, including chronic otitis media and bone resorption. The MPS I dog only partially recapitulates the pathology seen in humans; specifically, the dog model lacks inflammatory middle ear disease. In contrast, the MPS VII dog has severe inflammatory middle ear disease similar to that reported in the human. In conclusion, the canine MPS VII model appears to be a good model to study MPS VII\u2013related deafness.<\/jats:p>","DOI":"10.1177\/0300985810384413","type":"journal-article","created":{"date-parts":[[2010,10,7]],"date-time":"2010-10-07T22:53:38Z","timestamp":1286492018000},"page":"616-626","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["Histopathologic Changes of the Ear in Canine Models of Mucopolysaccharidosis Types I and VII"],"prefix":"10.1177","volume":"48","author":[{"given":"J.","family":"Hordeaux","sequence":"first","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Deniaud","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Bemelmans","sequence":"additional","affiliation":[{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Bertrand","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Moreau","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Amiaud","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Wyers","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Cherel","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.-A.","family":"Colle","sequence":"additional","affiliation":[{"name":"UMR 703, INRA (Institut National de la Recherche Agronomique), Nantes, France"},{"name":"Department of Veterinary Pathology, ONIRIS Nantes-Atlantic National College of Veterinary Medicine, Food Science and Engineering, Nantes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2010,10,7]]},"reference":[{"key":"bibr1-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-0112-z"},{"key":"bibr2-0300985810384413","first-page":"438","volume":"71","author":"Berry CL","year":"1994","journal-title":"Lab Invest"},{"key":"bibr3-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2007.00211.x"},{"key":"bibr4-0300985810384413","first-page":"1553","volume":"138","author":"Haskins ME","year":"1991","journal-title":"Am J Pathol"},{"key":"bibr5-0300985810384413","first-page":"980","volume":"18","author":"Haskins ME","year":"1984","journal-title":"Pediatr Res"},{"key":"bibr6-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100065993"},{"key":"bibr7-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5876(97)00176-6"},{"key":"bibr8-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/S0196-0709(87)80047-9"},{"key":"bibr9-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32781"},{"key":"bibr10-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2008.00965.x"},{"key":"bibr11-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1001\/jama.281.3.249"},{"key":"bibr12-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.03.005"},{"key":"bibr13-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.34.21439"},{"key":"bibr14-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5876(00)00320-7"},{"key":"bibr15-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2007.135772"},{"key":"bibr16-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050641"},{"key":"bibr17-0300985810384413","first-page":"3421","volume-title":"The Metabolic and Molecular Basis of Inherited Disease","author":"Neufeld EF","year":"2001"},{"key":"bibr18-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-5955(02)00341-6"},{"key":"bibr19-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1126\/science.1646484"},{"key":"bibr20-0300985810384413","doi-asserted-by":"publisher","DOI":"10.3109\/00016489109137402"},{"key":"bibr21-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2006.09.016"},{"key":"bibr22-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1177\/000348948409300115"},{"key":"bibr23-0300985810384413","first-page":"487","volume":"114","author":"Shull RM","year":"1984","journal-title":"Am J Pathol"},{"key":"bibr24-0300985810384413","first-page":"244","volume":"109","author":"Shull RM","year":"1982","journal-title":"Am J Pathol"},{"key":"bibr25-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2005.01.017"},{"key":"bibr26-0300985810384413","first-page":"239","volume":"10","author":"Sly WS","year":"1974","journal-title":"Birth Defects Orig Artic Ser"},{"key":"bibr27-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(73)80162-3"},{"key":"bibr28-0300985810384413","first-page":"653","volume-title":"Greenfield\u2019s Neuropathology","author":"Suzuki K","year":"2002","edition":"7"},{"key":"bibr29-0300985810384413","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-009-0967-y"},{"key":"bibr30-0300985810384413","author":"Wold SM","journal-title":"Int J Pediatr Otorhinolaryngol"}],"container-title":["Veterinary Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/0300985810384413","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/0300985810384413","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/0300985810384413","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T18:50:02Z","timestamp":1777661402000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/0300985810384413"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,10,7]]},"references-count":30,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2011,5]]}},"alternative-id":["10.1177\/0300985810384413"],"URL":"https:\/\/doi.org\/10.1177\/0300985810384413","relation":{},"ISSN":["0300-9858","1544-2217"],"issn-type":[{"value":"0300-9858","type":"print"},{"value":"1544-2217","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,10,7]]}}}