Abstract
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.