J Korean Ophthalmol Soc.
2007 Jul;48(7):950-956.
The Clinical Manifestations of Usher Syndrome in Korea
- Affiliations
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- 1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea. ophjun@hananet.net
- 2Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea.
- 3Department of Ophthalmology, Seoul National University Boramae Hospital, Seoul, Korea.
Abstract
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PURPOSE: To obtain useful information for counseling patients with Usher syndrome by analyzing the characteristics of its ophthalmologic manifestations.
METHOD: The records of eight patients were reviewed for visual acuity, refractive error, Goldmann perimetry, electroretinogram, and other abnormalities.
RESULTS
The best corrected visual acuity was 20/30 or better, bilaterally in six patients. All patients showed retinal findings characteristic of of retinitis pigmentosa. All seven patients who received Goldmann perimetry showed visual field defect. Furthermore, electroretinograms taken from seven patients were all extinguished. Six patients had myopic astigmatism, with one showing high myopia. Two patients showed exotropia. No correlation was found between the severity of the ophthalmologic problems and the level of the hearing loss or vestibular dysfunction.
CONCLUSIONS
Visual acuity was relatively preserved in most patients. Usher syndrome can be accompanied by strabismus, myopia, and even high myopia.
