Imagine suddenly losing hearing in one ear... and then, after a few weeks or months, losing hearing in the other ear as well. At the same time, you feel dizzy, and a constant ringing sound comes from inside your ear... If something like this happens, anyone would be very scared, wouldn't they? Today we're going to talk about a condition that causes these symptoms, which is a bit rare, but very important to know about. That's Autoimmune Inner Ear Disease, or AIED for short.
Simply put, what is AIED?
To understand this, let's first talk a little about our body's defense system. Our body has an immune system . It's like an army that protects a country. The main duty of the soldiers (immune cells) of this army is to find, capture, and destroy enemies like germs, viruses, and bacteria that enter our body.
But, sometimes our own army goes a little wrong. They start attacking some of our own healthy, good cells, thinking they're "enemies." This is what we call an 'autoimmune' condition.
So, that's what happens in this disease called AIED. Our immune system mistakes the delicate cells in our inner ear for enemies and attacks and damages them. This is a very rare condition. Less than 1% of people with hearing loss have this condition. It is especially common in middle-aged women.
What are the main symptoms of this?
Because the symptoms of AIED are so common, they can sometimes be confused with other conditions. But it's important to be aware of these symptoms.
| Symptom | Its nature |
|---|---|
| Hearing loss | This is the main symptom. It usually starts in one ear and spreads to the other ear within a few weeks or months. |
| Dizziness | Since the inner ear also helps control the body's balance, damage to it can cause a feeling of dizziness. |
| Hearing a ringing in the ears (Tinnitus) | A continuous ringing, roaring, or humming sound coming from inside the ears. |
| Vertigo | Severe dizziness that feels like you or the things around you are spinning, even if you are just standing still. |
| A feeling of fullness in the ear. | A feeling of heaviness, as if something is full inside the ear. |
Relationship with other autoimmune diseases
This is also very important. About 30% of people with AIED may have another autoimmune condition that affects the whole body. For example:
- Rheumatoid Arthritis
- Lupus
- Scleroderma
- Ulcerative Colitis
- Sjogren's Syndrome (dry eye disease)
This means that the immune system may be attacking not only the ear, but also other organs in the body.
How do you diagnose this disease?
To be honest, it can be a bit difficult to diagnose AIED, as the symptoms mentioned above can also be seen in other more common conditions, such as ear infections. In many cases, it may not be suspected until the hearing loss starts in one ear and spreads to the other ear.
Therefore, if you have these symptoms, see a doctor immediately. It is best to see an ear, nose, and throat specialist (ENT Surgeon).
Your doctor will do the following to diagnose the disease:
1. They will ask you questions: They will ask you a lot of details, such as your health history, whether anyone in your family has had this disease, and how your symptoms started.
2. Performs a physical examination: The ears, nose, and throat are thoroughly examined.
3. Hearing Test: A special test will be performed to see how well you can hear.
4. Balance Test: This can check how well your inner ear communicates with your brain.
5. Blood Work: There is no blood test that can confirm AIED. However, these tests can give you some idea of whether you are experiencing an autoimmune reaction.
In most cases, even if the diagnosis cannot be 100% confirmed, the doctor may decide to start treatment based on the symptoms and test results. This is because if treatment is delayed, the damage to the ear may not be reversible. For some people, it is confirmed that it is AIED when symptoms improve after treatment is started.
What do you do for treatment?
The main goal of treating AIED is to control the immune system's malfunction and prevent further damage to the inner ear.
- Steroids: Your doctor may first give you a high dose of an anti-inflammatory medication like steroids. These have been shown to be effective for AIED. However, they can have side effects, so they should not be used for more than a few weeks.
- Immunosuppressant drugs: After stopping steroids, other drugs that "calm down" your immune system (e.g., Azathioprine, Methotrexate) may be given long-term.
- Hearing Aids: If you have hearing loss, you can use a hearing aid to compensate for it.
- Cochlear Implant: In cases of severe hearing loss, your doctor may recommend a cochlear implant. This is a small electronic device that is surgically implanted under the skin behind the ear. It stimulates the nerves in the inner ear to convert sound into signals and sends them to the brain.
As medical science advances, it is possible that more effective drugs with fewer side effects and modern treatments such as gene therapy will emerge for AIED in the future.
Take-Home Message
- AIED is a rare condition in which your own immune system attacks your inner ear.
- If you experience symptoms such as sudden hearing loss (especially if it affects both ears), dizziness, or ringing in the ears, don't ignore them.
- If you have these symptoms, see a doctor, especially an ENT doctor, as soon as possible. Prompt treatment can prevent permanent damage.
- Diagnosing the disease can be complicated, so it is essential to seek medical advice for appropriate testing and treatment.
- AIED is not a single disease, but may also be a symptom of another autoimmune disease in your body.
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