{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T07:05:06Z","timestamp":1791270306738,"version":"4.1.0"},"reference-count":34,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2010,11,6]],"date-time":"2010-11-06T00:00:00Z","timestamp":1289001600000},"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":["European Journal of Ophthalmology"],"published-print":{"date-parts":[[2011,1]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>TO evaluate the retinal function, with emphasis on phenotype and rate of progression, in infants and children with different genotypes of Usher syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Fourteen children (2\u201310 years of age) with retinitis pigmentosa and hearing impairment were examined with full-field electroretinography (ERG) during general anesthesia, ophthalmologic examination, and genetic analysis. Five children were repeatedly examined (follow-up 5\u201310 years) with full-field ERG under local anesthesia and in 2 children multifocal ERG and optical coherence tomography (OCT) were performed. These results were compared to full-field ERG data from 58 children without retinal eye disorder.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six children were genotyped as Usher 1B, 2A, and 3A. Full-field ERG demonstrated early alterations corresponding to a rod-cone dystrophy in all children. A remaining rod function could be verified in the majority of the children up to 4 years of age. After 4 years of age, there was a further deterioration of the rod function; the progress was severe in Usher types 1 and 2 and moderate in Usher type 3. In all children, the cone function was moderately reduced, in a few cases almost normal. The results from the 58 children without retinal disorder confirm that full-field ERG during general anesthesia is reliable. Multifocal ERG confirmed a preserved central cone function and in OCT there were discrete structural alterations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Full-field ERG during general anesthesia in children with Usher syndrome demonstrates variable phenotypes and different degrees in rate of progression during childhood.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.5301\/ejo.2010.5433","type":"journal-article","created":{"date-parts":[[2010,11,18]],"date-time":"2010-11-18T04:59:29Z","timestamp":1290056369000},"page":"30-38","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":26,"title":["Alteration of Rod and Cone Function in Children with Usher Syndrome"],"prefix":"10.1177","volume":"21","author":[{"given":"Eva","family":"Malm","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Lund University Hospital, Lund - Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vesna","family":"Ponjavic","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Lund University Hospital, Lund - Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claes","family":"M\u00f6ller","sequence":"additional","affiliation":[{"name":"Department of Audiology\/Swedish Institute of Disability Research, University Hospital \u00d6rebro, \u00d6rebro - Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William J.","family":"Kimberling","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology and Visual Science, Iowa University Carver School of Medicine, Iowa City, Iowa - USA"},{"name":"Usher Syndrome Center, BoysTown National Research Hospital, Omaha, Nebraska - USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edwin S.","family":"Stone","sequence":"additional","affiliation":[{"name":"Usher Syndrome Center, BoysTown National Research Hospital, Omaha, Nebraska - USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sten","family":"Andr\u00e9asson","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Lund University Hospital, Lund - Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2010,11,6]]},"reference":[{"key":"bibr1-EJO.2010.5433","first-page":"250","volume":"4","author":"Von Graefe A.","year":"1858","journal-title":"Von Graefes Arch Ophthalmol"},{"key":"bibr2-EJO.2010.5433","first-page":"53","volume":"13","author":"Liebreich R.","year":"1861","journal-title":"Dtsch Arch Klin Med"},{"key":"bibr3-EJO.2010.5433","first-page":"130","volume":"19","author":"Usher C.","year":"1914","journal-title":"R Lond Ophthalmol Hosp Rep"},{"key":"bibr4-EJO.2010.5433","first-page":"1","volume-title":"The Treasury of Human Inheritance.","volume":"2","author":"Bell J.","year":"1922"},{"key":"bibr5-EJO.2010.5433","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1111\/j.1651-2227.1959.tb05398.x","volume":"34","author":"Hallgren B.","year":"1959","journal-title":"Acta Psychiatr Scand Suppl"},{"key":"bibr6-EJO.2010.5433","first-page":"63","volume":"6","author":"Kimberling W.J.","year":"1995","journal-title":"J Am Acad Audiol"},{"key":"bibr7-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.81.1.46"},{"key":"bibr8-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1997.tb02480.x"},{"key":"bibr9-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-002-0485-8"},{"key":"bibr10-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9924(02)00090-4"},{"key":"bibr11-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1159\/000091090"},{"key":"bibr12-EJO.2010.5433","first-page":"87","volume-title":"Fifth International Conference on Birth Defects.","author":"Davenport S.L.H.","year":"1977"},{"key":"bibr13-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(90)90546-7"},{"key":"bibr14-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.genom.2.1.271"},{"key":"bibr15-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1080\/16513860310003058"},{"key":"bibr16-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.2003.00109.x"},{"key":"bibr17-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1016\/j.exer.2005.11.010"},{"key":"bibr18-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1016\/j.visres.2007.08.015"},{"key":"bibr19-EJO.2010.5433","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19990924)89:3<158::AID-AJMG6>3.0.CO;2-#"},{"key":"bibr20-EJO.2010.5433","unstructured":"Online Mendelian Inheritance in Man. 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