The symptoms of Usher III may not be present at birth. Individuals generally have normal hearing, which gradually recedes into hearing loss. The exact onset and severity of hearing and vision loss can vary from person to person.
Hearing Loss in Usher III:
For people with Type 3 Usher syndrome, hearing loss usually begins in adolescence. By the time these individuals reach mid-to-late adulthood, using hearing aids will be necessary and a progression up to deafness cannot be ruled out.
Individuals with Usher III often start experiencing night blindness during adolescence and may notice blind spots in their field of vision by their late teens or early twenties. This vision impairment often progresses to legal blindness by midlife.
Balance and Usher III:
One key characteristic difference between Type 3 Usher syndrome and other types is the effect on balance. Individuals with Usher III generally experience normal to near-normal balance, unlike the pronounced balance problems associated with Type 1 and Type 2.
Identifying Usher III involves a comprehensive medical history and testing for hearing, vision, and balance impairments. However, only a genetic test can provide definitive proof of the disorder.
Sadly, there currently is no effective cure for Usher Syndrome. The treatments available can only focus on managing symptoms. For example, using hearing aids or glasses helps for the moment but doesn’t stop the disease from progressing.
Because Usher III is a much lesser-known subtype of Usher syndrome, there is close to no research being done. That’s why we need to drive for a change in Usher III and orphan drug development. Remember, knowledge is power. The more we know and understand about Usher III, the closer we get to the goal of finding effective treatments, and hopefully one day, a cure.