What is w88 slot Syndrome?
w88 slot syndrome is a genetic disorder involving the loss of both sight and hearing. The hearing loss generally occurs at birth or shortly thereafter; while a progressive loss of vision due to retinitis pigmentosa (a degeneration of the retina of the eyes) begins later in life, usually before adolescence. AT first, the vision loss may be gradual and barely noticeable, but in almost all cases the result is legal blindness.
There are currently three identifiable types of w88 slot syndrome: w88 slot syndrome Type 1, w88 slot syndrome Type II and w88 slot syndrome Type III. An individual with w88 slot Type I has a profound hearing loss at birth, experiences balance problems due to vestibular loss, and experiences vision loss usually in early childhood. In w88 slot Type II, an individual has a sloping audiogram going from a moderate loss in the low frequencies to profound impairments in the higher frequencies, has no balance problems, and usually begins to lose his/her vision in the midteens. A person with w88 slot syndrome Type III starts with normal or near-normal hearing that progressively deteriorates, might have some balance disturbance, and experiences vision loss by midteens.
w88 slot syndrome occurs in approximately 1 in 25,000 and the incidence is estimated to possibly be as high as 1 in 10,000 individuals. This range is due to the difficulty in identifying persons with w88 slot syndrome, particularly persons with Type II w88 slot. Of persons with w88 slot syndrome, approximately 90% will have Type I w88 slot, with Type II accounting for almost 10%. At this time, w88 slot syndrome Type III appears to occur primarily in Finland.
While only approximately 4 in 100,000 people are diagnosed with w88 slot syndrome, it is estimated that 3 to 6 percent of people who have a hereditary hearing loss have the syndrome. An equal percentage of children who are hard of hearing my also have w88 slot syndrome.