Usher syndrome is a family of genetic disorders characterized by early hearing loss and gradual vision loss. There are three distinct types of Usher Syndrome. The symptoms of each are listed below.
Hearing: Severe deafness in both ears from birth.
Vision: Slow vision loss starts with loss of night vision, usually in childhood.
Balance: Balance problems from birth.
Hearing: Moderate to severe hearing loss from birth, usually in higher frequencies
Vision: Slow vision loss starts with loss of night vision in late childhood or teens
Balance: No balance problems.
Hearing: No hearing loss at birth, but slow loss of hearing starting in childhood or teens.
Vision: Timing and severity of vision loss vary, but most often night vision loss begins in teens.
Balance: Minimal/no balance problems at birth, symptoms may get worse with age.
• Hearing problems are usually noticed and diagnosed within the first few years of life.
• Early on, vision loss will likely not be apparent, although subtle changes may be detected with vision testing.
• When vision loss becomes apparent, the first signs are usually a loss of night vision, followed by a gradual loss of peripheral vision.
As of today, there are no treatments for Usher Syndrome. The Foundation Fighting Blindness funds leading research into Usher Syndrome and encourages people living with this disease to enrol in the FFB Patient Registry, which will keep you updated on emerging clinical trials and opportunities to participate.
Usher Syndrome Fact Sheets