SCOTCH PLAINS, NJ -- Mayor Al Smith and the Township Council at its Tuesday, Sept. 4 meeting proclaimed that Sept. 15 will be Usher Syndrome Awareness Day in Scotch Plains. Accepting were Gavin and Ethan Morrobel, who are national spokespersons for greater awareness of the rare genetic disorder.
Usher Syndrome is caused by a mutation in any one of the 10 genes resulting in a combination of hearing loss and visual impairment, and is a leading cause of combined deafness and blindness. More than 400,000 people are affected by this genetic disorder worldwide, with approximately 50,000 of them in the U.S.
Usher syndrome, which is presently incurable, impacts three major senses in the body:
- Visual: Vision loss in Usher syndrome is caused by a progressive vision disorder known as retinitis pigmentosa (RP). RP causes the light-sensing cells in the retina to gradually deteriorate, initially resulting in night blindness, followed by a narrowing of the visual field, commonly known as tunnel vision.
- Hearing: Children with Usher syndrome are born with or develop hearing loss. It is estimated that upward of ten percent of people with congenital bilateral, sensorineural hearing loss have Usher syndrome.
- Balance: Balance is achieved and maintained through input from one’s eyes, the vestibular organs in the inner ear and the sensory systems of the body, such as the skin, muscles and joints. Thus, people with Usher syndrome suffer from severe balance issues due to vestibular dysfunction;
There are three clinical types: Type 1, Type 2 and Type 3, which are distinguished by the severity and age when the signs and symptoms appear, and there are at least 11 different genetic types of Usher syndrome, as determined by the genes that are involved. One cannot determine the genetic type by clinical testing, as DNA testing is the only reliable way of determining the true genetic type.
Because of the limited public awareness, those affected with Usher syndrome may suffer with depression, anxiety, isolation and loss of independence. The third Saturday of September is designed as Usher Syndrome Awareness Day to promote worldwide awareness and education, and provide resources for affected families, medical professionals and the community at-large.
Click here to read TAPintoSPF's 2017 profile on the Morrobel brothers.