Do you know about this strange disease (Susac Syndrome) that affects your eyes, ears, and brain at the same time?

Imagine if you suddenly had a severe headache, blurred vision, and hearing loss? You might think that these are three unrelated problems. But today we are talking about a very rare condition that combines all three of these symptoms, but is not widely discussed in society. This is called "Susac Syndrome." This name may be new to you. But it is very important to be aware of this.

Simply put, what is Susac Syndrome?

Susac Syndrome is a condition in which our own body's defense system, the immune system, mistakenly attacks our own body. We call this an autoimmune condition. Imagine an army defending a country that mistakenly attacks its own people.

In this disease, our immune system targets the tiny blood vessels in three very important places in our body. Those three places are:

1. Brain

2. Retina

3. Inner ear (Cochlea)

When this happens, the inner walls of these blood vessels swell. Just as a water pipe becomes clogged, the flow of water through these blood vessels is reduced, and sometimes completely blocked. This deprives the brain, eyes, and ears of the oxygen and nutrients they need. That's when the symptoms of this disease begin to appear.

What are the main symptoms of this disease?

In Susac Syndrome, symptoms related to these three main parts may appear together or separately. But usually all three parts are affected. Let's see what these symptoms are.

Why does this happen and who is most at risk?

As we discussed earlier, the exact cause of this has not yet been found . The only thing that is known is that it is an autoimmune condition. It is not a hereditary disease . That means there is no evidence that children will develop it if their parents had it.

However, certain factors can identify people who are at risk of developing this disease:

• Gender: Women are about three times more likely to develop this disease than men.
• Age: These symptoms most often begin to appear in people between the ages of 20 and 40 .

How do doctors find this?

Susac Syndrome can be difficult to diagnose because its symptoms are similar to those of many other diseases. For example, the symptoms are very similar to those of diseases such as Multiple Sclerosis (MS), stroke, and brain infection (encephalitis). Therefore, a doctor may mistakenly think that it is a different disease.

Therefore, several special tests are performed to make an accurate diagnosis.

• Physical exam
• Neurological exam
• Ophthalmological exam

In addition, these special tests are very important to confirm the disease.

What is the treatment?

The main goal of treating this disease is to control our immune system, which is malfunctioning. That is, to suppress its activity. We call the drugs given for this purpose immunosuppressants .

These treatments may need to be taken long-term. Some of the most commonly used medications are:

• Corticosteroids - e.g. prednisone
• Intravenous immunoglobulin (IVIG)
• Mycophenolate
• Azathioprine
• Cyclophosphamide
• Rituximab

There are several ways to give these medications. Some are given as pills, others are given as injections, and some medications are given intravenously (IV) as a saline solution.

Like all medications, this one can have side effects. So talk openly with your doctor about it before starting treatment. If any side effects occur, inform them immediately.

How can we have hope for the future?

Although the name Susac Syndrome may sound scary, there are very good treatments for this condition. Especially if the disease is diagnosed and treated early, the outcomes are very good. Early treatment can prevent permanent damage to the brain, eyes, and ears.

However, if treatment is delayed, sometimes vision and hearing can be permanently lost. However, this is very rare.

This disease is very rarely life-threatening. That is, it does not shorten life expectancy. However, it can affect the quality of daily life. When things like vision and hearing, which were previously good, change, as well as changes in the way you think and remember, it can have a very psychological impact. Therefore, talking to a mental health counselor after a diagnosis like this also brings relief to many people.

When should I see a doctor?

If you or someone you know experiences one or more of the following changes at the same time, see a doctor without delay .

• Sudden changes in vision (black spots, feeling like you are being covered by a curtain).
• Sudden loss of hearing , dizziness, or ringing in the ears.
• Difficulty thinking or remembering (changes in brain function).
• Unexplained changes in behavior or personality .
• If these symptoms are interfering with your daily life .

Your doctor can diagnose the exact cause of these symptoms and provide you with the necessary treatment, which can help prevent long-term complications.