Usher Syndrome A Report Study of A Jordanian Family

Usher syndrome includes Retinitis Pigmentation (Pigmentosa) accompanied by hearing loss. The hearing loss is congenital , stable, and usually quite severe, although severity can vary in different individuals. Usher syndrome is a recessively inherited condition. One of the greatest difficulties experienced by sufferers is the increased isolation as the restrictions on both spoken and visual communication increase Our study includes a family of six individuals, three of them were found affected with Usher. Two of them were found homozygous type ll with moderate hearing loss, the third was found homozygous type I with profound hearing loss. The three affected children were audiologically rehabilitated with suitable hearing aids, accompanied with regular visual examination. The study recommends the application of global hearing screening program for school children which will help in early identification of hearing disability, and its early management.