Dr.Nikhila Sai Nallapati

Dr.Viswabharathi Pampana, Dr.Rishitha Srinidhi Boddu, Dr.Hema Sai Gongati

Abstract

Usher syndrome is a rare autosomal recessive disorder associated with retinitis pigmentosa, characterized by progressive retinal dystrophy, sensorineural
hearing loss and variable vestibular dysfunction. It affects approximately 4 to 17 per 1 lakh people. we report a case of 24 yr old male presented with
diminution of vision since 4 yrs and hearing loss since childhood. Anterior segment of both eyes were within normal limits. Indirect ophthalmoscopy revealed
bony spicule pigmentation in mid peripheral fundus. Gross cystoid edema was present over macula. Perimetry revealed visual field defects.