Usher's Syndrome PT II

Tuesday, July 7, 2009
As promised, I wanted to briefly look again at Usher's Syndrome before moving on to the next disease in the Autosomal Recessive Disease Spectrum. The reason I delayed moving on is that I felt I needed two posts for Usher's in order to break up the basic overview and the three different types of the syndrome.

So, without further delay, here are the basics for each type, as well as the symptom:

Type I: Children with Type 1 Usher's are profoundly deaf at birth and receive practically zero assistance from hearing aids. This type of Usher's will also leave the child with significant vision problems by the time he/she is ten, many of whom will develop the sight problems earlier.

According to National Institute on Deafness and Other Communication Disorders, this type of Usher's also comes with significant balance issues. This makes sense, as unfortunate as it is, in light of the hearing loss it also spurs.

Type II: Although it makes sense that hearing loss equals at least some loss of balance in a patient with Usher's, that isn't always the case, as it the example of Type II Usher's, wherein the child is born with significant hearing loss, yet has little or no balance issues. Also unlike Type I, Type II doesn't result in sight problems until later in the teen years and patients can also benefit from hearing aids.

Type III: Breaking away from the other two types of Usher's, Type III doesn't manifest itself as a hearing problem at birth. In addition, the person is not usually legally blind until adulthood. As such, the patient has additional time to prepare and learn techniques that can help them later on in life.

As I've said before, I don't plan to make dissertations out of the Summer Knowledge Project. However, it is my hope that we can all benefit from the additional knowledge, whether we know someone who suffers from these diseases or not.