{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T20:27:54Z","timestamp":1786739274610,"version":"build-2736575974"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>A 30-year-old female presented with gradually progressive diminution of vision for 1 month, with night blindness for the past 5 years and difficulty in hearing for the past 10 years. Her developmental history and family history were unremarkable. Ocular examination revealed visual acuity of 6\/36 in both eyes. Fundus showed features of retinitis pigmentosa with bilateral macular edema. Audiometry revealed bilateral sensorineural hearing loss; although, her vestibular functions were preserved. Clinical diagnosis of Usher syndrome type 3 was made based on normal hearing at birth, delayed presentation of progressive visual and auditory impairment with normal vestibular function, and developmental milestones. Her macular edema resolved after 3 months of treatment with topical dorzolamide therapy. The unique feature of this case is the presence of bilateral macular edema in type 3 Usher syndrome, which is rarely reported in literature.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/tjo.tjo_6_20","type":"journal-article","created":{"date-parts":[[2020,4,20]],"date-time":"2020-04-20T05:10:41Z","timestamp":1587359441000},"page":"183-186","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["A rare case of type 3 usher syndrome with bilateral cystoid macular edema treated with topical dorzolamide"],"prefix":"10.4103","volume":"11","author":[{"given":"Athul","family":"Puthalath","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramanuj","family":"Samanta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neeraj","family":"Saraswat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajai","family":"Agrawal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anupam","family":"Singh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahsa","family":"Jamil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-12-20240830","first-page":"130","article-title":"On the inheritance of retinitis pigmentosa with notes of cases","volume":"19","author":"Usher","year":"1914","journal-title":"R Lond Ophthalmol Hosp Rep"},{"key":"R2-12-20240830","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1136\/bmj.323.7312.536","article-title":"Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: Questionnaire based ascertainment study","volume":"323","author":"Fortnum","year":"2001","journal-title":"BMJ"},{"key":"R3-12-20240830","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/jhg.2010.29","article-title":"Genetics and pathological mechanisms of Usher syndrome","volume":"55","author":"Yan","year":"2010","journal-title":"J Hum Genet"},{"key":"R4-12-20240830","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1288\/00005537-198901000-00014","article-title":"Usher syndrome: An otoneurologic study","volume":"99","author":"M\u00f6ller","year":"1989","journal-title":"Laryngoscope"},{"key":"R5-12-20240830","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1136\/bjo.81.1.46","article-title":"Usher syndrome in the city of Birmingham \u2013 Prevalence and clinical classification","volume":"81","author":"Hope","year":"1997","journal-title":"Br J Ophthalmol"},{"key":"R6-12-20240830","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1177\/000348948409300613","article-title":"Vestibular and auditory function in Usher syndrome","volume":"93","author":"Kumar","year":"1984","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"R7-12-20240830","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1097\/01.mlg.0000205167.08415.9e","article-title":"Audiologic performance and benefit of cochlear implantation in Usher syndrome type I","volume":"116","author":"Pennings","year":"2006","journal-title":"Laryngoscope"},{"key":"R8-12-20240830","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1016\/j.ijporl.2008.02.013","article-title":"Cochlear implantation in individuals with Usher type 1 syndrome","volume":"72","author":"Liu","year":"2008","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"R9-12-20240830","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1111\/j.1399-0004.1983.tb00082.x","article-title":"An unusual otological manifestation of Usher's syndrome in four siblings","volume":"24","author":"Karjalainen","year":"1983","journal-title":"Clin Genet"},{"key":"R10-12-20240830","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1017\/S0022215100096262","article-title":"Usher's syndrome type III: ENG findings in four affected and six unaffected siblings","volume":"99","author":"Karjalainen","year":"1985","journal-title":"J Laryngol Otol"},{"key":"R11-12-20240830","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1002\/ajmg.1320500107","article-title":"Clinical diagnosis of the Usher syndromes. 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