Usher Syndrome is a rare genetic disorder that affects both hearing and vision. It is estimated to occur in approximately 1 in every 25,000 to 50,000 people worldwide. This makes it one of the leading causes of deaf-blindness. Usher Syndrome is classified into three types: type 1, type 2, and type 3, each with varying degrees of severity and age of onset.
Type 1 is the most severe form, typically causing profound hearing loss from birth and vision loss that starts in childhood. It accounts for about 10-20% of Usher Syndrome cases.
Type 2 is less severe, with moderate to severe hearing loss from birth and vision loss that usually begins in adolescence or early adulthood. It is the most common type, accounting for approximately 80% of cases.
Type 3 is the rarest form, with progressive hearing loss and vision loss that starts in adolescence or adulthood. It accounts for a small percentage of cases.
Usher Syndrome is inherited in an autosomal recessive pattern, meaning that both parents must carry a mutated gene for their child to be affected. Genetic testing and counseling are crucial for individuals with a family history of Usher Syndrome or those experiencing symptoms. Early diagnosis and intervention can help manage the condition and improve quality of life for those affected.