What is Usher syndrome?
Usher syndrome is the most common genetic cause of combined deafness and blindness. As a progressive condition, vision and hearing will deteriorate over time.
What causes vision loss in Usher syndrome?
The vision loss component of Usher syndrome is caused by retinitis pigmentosa (RP). A progressive disorder, RP causes the cells in the retina to deteriorate over time. Night blindness is the first stage of RP, followed by a narrowing of the visual field into tunnel vision, and, finally, total blindness. The speed of the progression varies significantly.
What causes hearing loss in Usher syndrome?
The inner ear contains hair cells that receive sound. Usher syndrome causes a genetic mutation that results in the abnormal development of these crucial sound receptor cells.
Three Types of Usher Syndrome
There are three clinical types: Type 1, Type 2 and Type 3. Each type is distinguished by the age when the symptoms appear, as well as the severity of the symptoms that include hearing, vision and sometimes balance. DNA testing is available to confirm the type.
Type I
- Hearing: profound deafness in both ears from birth
- Vision: decreased night vision before age 10
- Balance: problems from birth
Type 2
- Hearing: moderate to severe hearing loss from birth
- Vision: decreased night vision begins in late childhood or teens
- Balance: normal
Type 3
- Hearing: profound deafness in both ears from birth
- Vision: decreased night vision before age 10
- Balance: problems from birth
Is there a cure for Usher syndrome?
Currently, there is no cure for Usher syndrome, however, researchers are hard at work seeking out treatments to potentially slow, stop, or even reverse vision loss and hearing.
How can I help find a cure?
There are two great ways you can help! The first is by simply spreading awareness about Usher syndrome and being a supporter. Be sure to follow Wanderlight Moments and sign up for my newsletter join my community of amazing supporters. The more people who know about Usher syndrome, the closer we are to a cure.
Second, you can donate to the Usher Syndrome Society, a non-profit that uses the arts, educational events, and collaboration to raise awareness and funds for treatments and a cure for Usher syndrome.
