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       XXVII Annual Congress of the Iranian Society of Ophthalmology        بـیــست و هفتمین کنــگــره سـالیـانه انـجـمـن چـشـم پـزشـکی ایـــران
مقاله Abstract


Title: Usher syndrome associated with Fuchs’ heterochromic uveitis: a rare case report
Author(s): Leila Rezaei,Firuzeh Alizadeh,Jalil Omidian,Farshid Ramezani
Presentation Type: Poster
Subject: Posterior Segment
Others:
Presenting Author:
Name: Leila Rezaei
Affiliation :(optional) Emam khomeini eye research center,Kermanshah university of medical science
E mail: leyla_rezaei60@yahoo.com
Phone: 08337278759
Mobile: 09188370655
Purpose:

We report a rare case of Usher syndrome in association with unilateral Fuchs’ heterochromic uveitis.

Methods:

A 25-year-old patient referred to us with progressive bilateral visual diminution.Systemic examination demonstrated no remarkable findings, except for sensorineural hearing loss. Visual acuity of both eyes was 20/100. Slit lamp examination of the left eye revealed endothelial stellate keratic precipitates, mild anterior chamber reaction, iris hetetochromia and mild posterior subcapsular cataract. Her intraocular pressures were 14 mmHg in the right eye and 18 mmHg in the left eye

Results:

Funduscopy demonstrated waxy pallor optic nerve, marked arterial narrowing and retinal bone spicule pigment formation with a normal macula. The pattern electroretinogram displayed a reduced response. A threshold hearing test showed total bilateral hearing loss.

Conclusion:

The association of Usher syndrome and Fuchs’ heterochromic uveitis is rare and has only been reported twice in the literature.

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