The Basics of Williams Syndrome

Williams syndrome

Williams syndrome is a rare congenital disorder that affects little over 0.01 percent of people. It occurs as the result of a deletion of up to 28 genes from chromosome 7. Half of affected parents will pass on the condition to their children. In some cases, children can develop Williams syndrome even if there is no family history of the disorder.

Williams Syndrome and Hearing Loss

Seeing as it’s a developmental disorder, Williams syndrome has many effects on people who suffer from it. It affects both their physical appearance, as well as their bodily functions. Research shows that the condition can also affect people’s hearing.

Children with Williams syndrome are more likely to suffer from high-frequency hearing loss. What’s more, their hearing loss is progressive. This means that, as they age, its degree will become more severe. But the condition can also impact their hearing in a different way.

Hyperacusis

Hyperacusis is a disorder characterized by very high sensitivity to sound. In otherwise healthy people, it can occur as a result of some ear injury. But in people with Williams syndrome, it can develop on its own.

There is still no known cause of this disorder. Experts believe it has to do with the brain’s impaired ability to interpret everyday sounds. People who suffer from it will thus hear common sounds much more loudly. For kids, this condition can be very painful. It can also be hard to diagnose. If your child covers their ears and cries when they hear loud sounds, it could be the sign of hyperacusis.

What You Can Do

If your child has Williams syndrome, it is vital to check their hearing on a regular basis. ENTs will most often use sound therapy to treat hyperacusis. Apart from this, they may also recommend counseling. You could also use headphones to help your child better cope with noise. But make sure not to use them too much. If you do, their sensitivity may in fact increase.