Usher Syndrome – What is it, Causes and Treatments

Usher Syndrome – What it is, Causes and Treatments that many are still unaware of. Furthermore, Usher Syndrome is a rare genetic disorder that affects approximately 1 in 25,000 babies born and causes hearing and vision loss , often accompanied by balance problems,   Usher Syndrome accounts for 3-6% of all children. deaf.

Hearing loss usually presents first, although the speed of progression to deafness can vary greatly between individuals with Usher Syndrome . Retinitis pigmentosa initially causes problems with night vision and peripheral vision. Progressive retinal degeneration leads to reduced tunnel vision and eventual blindness in most cases.

Causes of Usher Syndrome: Usher Syndrome is inherited, which means that  Usher Syndrome  is passed from parents to their children through genes. Genes are located in almost every cell in the body.

Genes contain instructions that tell cells what to do. Each person inherits two copies of each gene, one from each parent. Sometimes genes are altered, or mutated. Mutated genes can cause cells to act differently than expected.

Usher syndrome is inherited in an autosomal recessive manner. The term autosomal means that the mutated gene is not located on any of the chromosomes that determine the person’s sex; in other words, both men and women can have Usher Syndrome and it can pass it on to a child. The recessive means that, to have Usher Syndrome , a person must receive a mutated form of the Usher Syndrome gene from each parent.

Types of Usher Syndrome:  There are three types of Usher Syndrome , check them out below:

Usher Syndrome Type-1: Usher Syndrome  Type -1  is characterized by profound deafness from birth and severe balance problems. Hearing aids often provide little benefit and many children affected by Usher Syndrome Type-1 need Usher Syndrome  to rely on sign language, which is best started as early as possible when the child is most receptive to cognitive learning.

As a result of difficulty with swinging, most children with Usher Syndrome  take longer to learn to sit, stand and walk. On average, independent movement begins in approximately 18 months.

Usher Syndrome Type-2 : Usher Syndrome Type-2  is probably the most common type of Usher Syndrome and children affected with this type are born with moderate to severe hearing loss . In most cases of Usher Syndrome , hearing aids offer a real benefit and can help children to communicate orally.

Vision loss usually presents and progresses more slowly than in  Usher Syndrome Type-1 , and many individuals do not notice the emergence of symptoms until their teen years.

Usher Syndrome Type-3: Usher Syndrome Type -3  does not carry any particular symptoms at birth, although they develop later on. Over time the senses of hearing and sight get worse, though the rate at which this occurs varies greatly.

It is common for individuals affected by Usher Syndrome Type-3 to require hearing aids and to suffer from significant vision loss or blindness in adulthood.

Usher Syndrome type indicates when symptoms initially present but do not necessarily match the severity of the illness.

Treatment of Usher Syndrome:  There is currently no cure for Usher Syndrome , although it may become a possibility in the future with genetic research pointing to replacement of the missing gene.

Useful links: 

Obs:  That’s why the importance of an early diagnosis because the Usher Syndrome compromises the two senses considered primordial (vision and hearing).

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