Treatments For Usher Syndrome: Are These Really Effective?

Is it possible for a condition to affect not just one, but two senses? Well, yeah it is possible. In fact, there is this condition called as the Usher syndrome that affects both the sense of vision and hearing. This condition is often characterized by vision impairment and loss of hearing. Stay right wherever you are, reading this article because you will be enlightened more about this condition. This article will not just give you various insights about the condition, but it will also supply you with the possible treatments for Usher syndrome.

Usher Syndrome: What is this condition?

The Usher syndrome is actually named after Dr. Charles Usher, who first defined the condition in 1914. Usher syndrome is actually a rare and hereditary illness, which causes plodding vision loss and deafness. Furthermore, it may affect the balance as well. The loss of hearing is usually present at birth or soon from then on. This is mainly because of an impaired ability of the auditory nerves to convey sensory input towards the brain. This is what they call sensorineural hearing loss.

On the other hand, the vision loss or what they call Retinitis Pigmentosa initiate later in childhood, commonly after the age of ten. The condition slowly becomes worse over the course of time. Furthermore, during the teen years, the loss of vision is described by night blindness and the loss of peripheral vision.

Approximately about 3-6 percentage of all the children whoa re deaf and about 3-6 percent of youngsters who have a hearing impairment have the condition.

Here are some other terms for the condition:

  • Retinitis pigmentosa-dysacusis syndrome
  • Dystrophia retinae dysacusis syndrome
  • Usher-Hallgren syndrome
  • Hallgren syndrome

Moreover, the Usher syndrome is a disorder or a disease that has more than one feature or symptom. Commonly, the symptoms are the loss of hearing and retinitis pigmentosa. There is also some that experience some severe balance problems while experiencing Usher syndrome. Furthermore, there are three clinical types of Usher syndrome, type 1, type 2, and type 3. The different types of the condition are actually divided by the degree of vision and hearing loss, the age it occurred, how it quickly progresses, and whether the problems in balance are present or not.

Causes of Usher Syndrome

Experts state that condition is hereditary, meaning it is handed from parents to their children. Furthermore, it is also caused by various changes, known as gene mutations. Genes are actually the instructions in cells that are chemically coded, which tell the cells to do what it needs to do. Furthermore, the body has about 20,000-25,000 genes. And each person has about two replicas of each gene one from the mother and one from the father.

The illness is a type of autosomal declining disorder. Meaning:

  • A child who acquires the gene from his or her parents will have the condition.
  • A male and a female may both get affected by Usher syndrome.

What causes the vision damage in the condition is the mutation of the gene, which affects the light-identifying cells in the retina. The cause of the hearing loss, on the other hand, is the same –the mutation of the gene, which affects the cochlear nerve cells.

Symptoms of Usher Syndrome

The major symptoms of the Usher syndrome are vision loss and hearing loss. However, some people may also experience balance problems because of inner ear conditions. The signs and symptoms of each type of the condition, include the following:

Type 1

  • Blindness
  • Vision loss begins at the age of 10
  • Slow to sit without any support
  • Severe balance problems
  • Deaf at birth and receive little to no benefits form hearing aids

Type 2

  • Vision loss begins in the teenage years
  • No balance problems
  • Born with moderate to severe loss of hearing, and may benefit from hearing aids

Type 3

  • Vision loss starts in the early teenage years and leads to blindness in adulthood
  • Born with near-normal balance
  • Born with normal hearing, which gets worse in teenage and leads to loss of hearing in adulthood

Treatments for Usher Syndrome

Currently, there are no known treatments for Usher syndrome yet. However, the possible treatments for Usher syndrome may involve early diagnosis. This is for some educational programs to begin as soon as possible, reliant on the severity of the vision loss and the ability and the age of the patient.

The treatments for Usher syndrome may include:

  • Learning to use the low-vision techniques and devices
  • Instruction on reading with Braille

Furthermore, some researchers also show that vitamin A may help in slowing down the progress of various forms of retinitis pigmentosa. Your own ophthalmologist may advise you about the possible risks as well as the benefits of vitamin A. always remember that too much of everything is bad.

Usher syndrome can be a serious condition, so treating it as soon as possible is a must.


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