What is Susac Syndrome?

Almost 50 million Americans are affected by a hearing impairment. While the most common causes include natural aging (presbycusis), dangerous noise levels, injury to the ear, or medication. In some cases, a hearing impairment is caused by an underlying condition.

Susac syndrome is a reasonably rare autoimmune condition that can impact your ears. Today, we’re taking a closer look at this rare disorder, including causes and treatment options.  It causes the network of small blood vessels in your inner ear, retina and brain to become blocked. This in turn affects the organs.

What is Susac Syndrome?

The National Institutes of Health defines Susan syndrome as: “an autoimmune condition that affects the very small blood vessels in the brain, retina, and inner ear (cochlea). The condition is characterized by three main symptoms: brain disease (encephalopathy), hearing loss, and vision loss.”

For many of us, our immune system works as it is expected to. It helps to protect us from germs, and fights off any foreign invaders. An autoimmune condition, however, is where your immune system mistakenly attacks your own body.

Susac syndrome causes your immune system to attack the network of small blood vessels in your inner ear, retina and brain. This can result in decreased blood flow.

Symptoms of Susac Syndrome

The symptoms and severity of Susac syndrome vary from person to person. Given that it affects three areas of your body (brain, eyes and ears), symptoms may include one or all three areas.

The disease may not immediately present in all three areas. It may take years for all three areas to be affected. Some patients report only experiencing symptoms in two areas. Below are the common symptoms in correlation to the area of the body involved.

Brain: 

  • Headaches, often severe.
  • Difficulty thinking, including: confusion, short-term memory loss, reduced ability to problem solve.
  • Unable to remain focused or stay alert
  • Slurred speech
  • Personality changes
  • Depression, anxiety, withdraw or aggression

Eyes:

  • A dark area in your visual field
  • The feeling of a dark curtain drawn over your eyes
  • Loss of peripheral vision

Inner Ear: 

How is Susac Syndrome Diagnosed?

Your doctor may suspect Susac syndrome after a clinical evaluation and detailed medical history. Generally, diagnosis requires at least two areas of your body to be affected by reported symptoms.

The symptoms of Susac syndrome can mimic diseases, including multiple sclerosis or chronic encephalitis. Because of this, researchers are unable to accurately determine how common Susac syndrome is in the general population.

Diagnosis generally involves a neurological exam and specialized tests. These may include  magnetic resonance imaging (MRI). An MRI can reveal changes in your brain caused by Susac syndrome.

A thorough eye examination is also required. The exam will “include a fluorescein angiography, which measures the blood flow through the retina.” (source)

A hearing exam will help to establish if the patient is experiencing a hearing loss.

Is Susac Syndrome Treatable?

Early detection of Susac syndrome increases the potential benefits from treatment. Treatment includes medications that suppresses the immune system, such as steroids. The objective of treatment is to help suppress the immune system, to avoid further attacks on the blood vessels in your brain, eyes and ears.

If you have further questions on Usher syndrome, read through the National Organization for Rare Disorders database here.

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