Open AccessA rare case of type 3 usher syndrome with bilateral cystoid macular edema treated with topical dorzolamide
Author(s) -
Athul S Puthalath,
Ramanuj Samanta,
Neeraj Saraswat,
Ajai Agrawal,
Anupam Singh,
Mahsa Jamil
Publication year - 2021
Publication title -
taiwan journal of ophthalmology
Language(s) - English
Resource type - Journals
eISSN - 2211-5072
pISSN - 2211-5056
DOI - 10.4103/tjo.tjo_6_20
Subject(s) - medicine , retinitis pigmentosa , usher syndrome , fundus (uterus) , ophthalmology , visual acuity , macular edema , surgery , audiology , retinal
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.