J Korean Ophthalmol Soc.  1998 Apr;39(4):784-789.

A Case of Type 1 Ushers Syndrome with Bilateral Cataract

• Lee SJ
• Lee JH
• Choi YJ
• Kim JW

Abstract

Ushers syndrome is an autosomal recessively inherited entity which is characterized by a retinitis pigmentosa and congenital sensorineural hearing loss. This syndrome represents different clinical features according to its subtypes. We experienced one case of type 1 Ushers syndrome, who had congenital hearing loss, visual field loss, visual loss of early childhood onset. We performed bilateral cataract extraction. Fundus examination after operation showed characteristic findings of retinitis pigmentosa. ERG was non-recordable ERG. Her audiogram confirmed the bilateral sensorineural hearing loss.

Keyword

Autosomal recessive; Total cataract; Retinitis pigmentosa; Ushers syndrome

MeSH Terms

Cataract Extraction
Cataract*
Hearing Loss
Hearing Loss, Sensorineural
Retinitis Pigmentosa
Usher Syndromes*
Visual Fields