What is Usher Syndrome?

Hearing loss is estimated to affect almost 50 million Americans. The most common causes of hearing loss include: aging, exposure to dangerous levels of noise or injury. The hearing loss can range from mild, where certain sounds are difficult, to profound.

Certain forms of hearing loss are genetic. Genes are the building blocks of every cell in our body, including those involved with our hearing. It’s estimated that roughly 30,000 genes are involved in every cell in our body.

Genetic Hearing Loss

In some cases, genes don’t behave as they are expected to. When this occurs, it’s known as a mutation. If genes involved with our hearing experience mutations, hearing loss may be the result.

This genetic hearing loss falls into two types: syndromic and non-syndromic.

According to the CDC, “About 70% of all mutations causing hearing loss are non-syndromic. This means that the person does not have any other symptoms. About 30% of the mutations causing hearing loss are syndromic. This means that the person has other symptoms besides hearing loss. For example, some people with hearing loss are also blind.”

One of the most common forms of syndromic hearing loss is Usher Syndrome.

What is Usher Syndrome?

Usher Syndrome is a genetic condition that is inherited. People with Usher syndrome have reported different experiences of the condition.

The primary symptoms of User syndrome are hearing loss and loss of vision that worsens over time. The U.S. National Library of Medicine notes: “The hearing loss is classified as sensorineural, which means that it is caused by abnormalities of the inner ear. The loss of vision is caused by an eye disease called retinitis pigmentosa (RP), which affects the layer of light-sensitive tissue at the back of the eye (the retina).”

To read more about sensorineural hearing loss, please click here.

Types of Usher Syndrome

There are three identified types of Usher syndrome. The distinguishing factors between the three types include the degree of hearing loss, whether balance is affected, and at what age symptoms first appeared.

The types are: 

  • Type 1 – Usher type 1 is characterized by profound hearing loss in both ears, generally present at birth. Problems with balance are a common symptom of type 1. The ability to sit and walk may be delayed in babies with Usher syndrome type 1.
    Nightblindness is commonly associated with type 1. This tends to occur in the first 10 years of childhood. Vision problems can worsen to additional sight problems associated with retinitis pigmentosa.
  • Type II – Usher type 2 is characterized by mild to severe hearing loss, generally present at birth. Sensorineural hearing loss is the typical form of hearing loss experienced. Balance problems are not associated with Usher syndrome type 2. People with Usher type 2 will generally develop RP in their teenage years or early 20’s.
  • Type III – Usher type 3 is characterized by gradual hearing and vision loss. It generally occurs later in life. Balance problems are generally associated with Usher type 3.

In some cases, some people with Usher Syndrome do not fit into these three types. Known as ‘Atypical Usher,’ it involves symptoms that make it difficult to diagnose a particular type of Usher syndrome.

Is Usher Syndrome Treatable?

There is currently no cure for Usher Syndrome. Rather, the focus is on managing the hearing loss, vision loss or balance problems.

In children, early detection can help with the development of bespoke educational programs. These can accommodate the severity of the child’s hearing or vision loss.

Treatment for the hearing loss may include: hearing aid devices, cochlear implants, assistive listening devices, auditory training. Learning sign language is another way of managing hearing loss, particularly when profound.

If you have further questions on Usher syndrome, read through the Usher Syndrome Coalition’s FAQs here.

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