Ushers Syndrome
Eye Conditions & Care | Eye Conditions | Ushers Syndrome
Ushers Syndrome is an inherited disorder. Moderate to profound hearing loss, which is present at birth or shortly after, and progressive vision loss due to Retinitis Pigmentosa (RP), a degeneration of the retina are the characteristics of Ushers Syndrome. It is the major cause of deaf blindness.
There are three forms of Usher Syndrome:
Type 1 - People with Ushers Syndrome Type 1 are born profoundly deaf and experience problems with balance. In adolescence, they usually begin to exhibit the first signs of RP in early adulthood.
Type 2 - Individuals with Ushers Syndrome Type II experience moderate to severe hearing impairment at birth. Symptoms of RP develop later in adolescence.
Type 3 - With Ushers Syndrome Type III, hearing loss and vision loss due to RP are both progressive - meaning that people lose their hearing and sight over a period of time.
Researchers believe that the hearing loss in Ushers Syndrome is due to a problem with the sensory cells in the cochlea, a structure within the inner ear that is necessary for transmission of sound to the brain. The vision loss is caused by a degeneration of the retina. Ushers Syndrome is passed to succeeding family generations through the autosomal recessive inheritance pattern. Please see Inheritance Patterns for more information about this.