The Clinical Manifestations of Usher Syndrome in Korea.

Han, Sang Beom; Kim, Seong Joon; Kim, Jeong Hun; Choung, Ho Kyung; Yu, Young Suk

Original Article

Journal of the Korean Ophthalmological Society 2007;48(7):950-956.
Published online July 31, 2007.

The Clinical Manifestations of Usher Syndrome in Korea.

Sang Beom Han, Seong Joon Kim, Jeong Hun Kim, Ho Kyung Choung, Young Suk Yu

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.

한국인에서 발생한 어셔증후군의 임상양상

한상범¹,김성준^1,2,김정훈^1,2,정호경^1-3,유영석^1,2

Department of Ophthalmology, Seoul National University College of Medicine¹, Seoul, Korea Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute², Seoul, Korea Department of Ophthalmology, Seoul National University Boramae Hospital³, Seoul, Korea

Correspondence: 김성준

Abstract

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.

Key Words: Usher syndrome;Retinitis Pigmentosa;Sensorineural hearing loss