Diagnosis, Symptoms, And Treatment Of Aied

Jeannette | Posted on January 27, 2023 
autoimmune ear disease

AIED, shorthand for the autoimmune disease of the inner ear, is a rare disease with an occurrence rate of 15 for every 100,000 people. In an autoimmune disease, the body’s immune system mistakenly attacks itself, and in the case of autoimmune ear disease, the immune system attacks cochin, a protein in the inner ear.

Sometimes, an autoimmune ear disease occurs when the body is already suffering from another autoimmune disease, making a whole group of two or three autoimmune diseases. In most cases, however, the disease occurs on its own.


  • A major symptom of AIED is loss of hearing. It begins with one ear, and within a few weeks or months, spreads to the other. The hearing may become worse or improve randomly. The rate of hearing loss doesn’t need to be similar in both ears.
  • Patients may also feel hissing, ringing, buzzing, or roaring in their ears. That is medically known as tinnitus and described as sounds coming from the body rather than the outside.
  • Another significant symptom of autoimmune ear disease is dizziness. Around half the people who suffer from AIED report dizziness and disbalance as symptoms.


Early diagnosis is essential in treating autoimmune ear disease. The earlier the diagnosis, the greater the chances for treatment are. Since AIED occurs rarely, doctors may link its symptoms to some other disease. However, if hearing loss occurs in both ears over time, and the symptoms are unexplainable by any other disease, doctors may test for AIED.

The best way to check for the presence of an autoimmune disease is to check the autoantibody figures in the blood. That gets done through a blood test. Doctors may also test check for leukaemia or other hemolytic disorders through a complete blood count (CBC).

The main goal is to rule out the possibility of any other disease. So, doctors may also check for dyslipidemias through a lipid panel test, or syphilis through an FTA/ABS blood screening.

An ear, nose, or throat (ENT) specialist may test your hearing through serial audiograms or check the structures of your inner ear through imaging like MRIs. Your specialist will check for the presence of vascular lesions, and space-occupying lesions in your brain and cerebellopontine angle.

The vestibular portion of the ear controls the balance and composure of the body, and a vestibular test evaluates your hearing by checking this portion. Even after you get treated, you may have to get audiogram tests done regularly to keep track of your hearing. Hearing tests can also be done at home, so you may notice any changes and report to your doctor immediately.

Doctors may also administer steroids and conclude the disease in question by checking how the steroids improve symptoms.

Once you get diagnosed with AIED, do not feel too afraid. You at least know the exact cause of your hearing loss, and treatment is possible in most cases.

Autoimmune Specialist

Treatment for Autoimmune Ear Disease

Treatments for an autoimmune disease of the inner ear include the intake of medication to lessen the effect of symptoms or using hearing aids to reverse hearing.


Steroids like dexamethasone or prednisone are given on an initial trial period of four weeks and a dose of 60 mg or 1 mg/kg per day. Smaller doses are ineffective and increase the chances of relapse. An autoimmune disease causes inflammation and steroids reduce it. If the symptoms of autoimmune ear disease get better because of steroid treatment, the doctors conclude that the disease in the course was AIED indeed and giving you its symptoms.

Even though the results of corticosteroids vary from person to person, treatment is usually successful in 60% of people. Steroid treatment is affordable but has side effects such as increased blood pressure, mood swings, anxiety, irritability, ingestion, and headaches. The mode of administration for corticosteroids into the body includes injections administered directly into the eardrum. In case an emergency arises, doctors may also give the steroids orally.


Autoimmune ear disease can result due to antibodies attacking the body, allergies, or some other complex reason. In the case that AIED occurs due to antibodies, plasmapheresis proves as an effective, even though expensive, treatment. In this treatment, blood is taken out from the ear, and separated into its components, blood cells, and plasma.

Then technicians treat the blood by removing antibodies and then the blood is transferred back into the body. Another disadvantage of this treatment is that you will have to get the treatment every month. About 50% of the patients treated through plasmapheresis show successful results.

Gene, Cell Therapy, and Biologics

In gene therapy, new genes get introduced into old cells in the body, while in cell therapy; individual stem cells get introduced into the ear canal so they may reproduce.

If treatment with corticosteroids does not improve results, doctors may suggest biological drugs made from living organisms or some parts of living organisms that have proven effective. These drugs help with autoimmune ear disease treatment as well as people who get dependent on steroids. Examples of these drugs include golimumab and rituximab.

In biological treatment, either genes are combined to form a fusion gene to make fusion proteins, or monoclonal antibodies are used. A disadvantage of biologics is that they may increase the risk of bacterial infections.


Since the immune system attacking the body is the main characteristic of AIED, an obvious way to combat the disease is through immunosuppressants, through which the immune system’s response slows down. However, immunosuppressants may have a few side effects such as increased blood pressure, hair loss, acne, fatigue, mouth sores, and diabetes.

Auditory Devices

Hearing devices, like cochlear implants, help compensate for hearing loss by either intensifying sound or enhancing hearing. Cochlear implant surgery involves opening up the cochlea and inserting an electrode into it. The surgery lasts for two to four hours, is a safe procedure, and has only a few side effects, such as vertigo, numbness, and drying of the mouth.

Hearing aids are also a good option since the patient may remove them whenever he likes. However, they may not allow the sound to be clear.

Before assigning you treatment, doctors have to consider a variety of factors to understand what mode of treatment is best for you. Such factors include how old you are, your tolerance levels toward medication, how severe your symptoms and your overall lifestyle and health.

Treatment does not just end abruptly. If you are on some form of medication, you may have to continue it for some time so that the immune system does not begin to attack the ear again. However, the dosage of the medications is lessened over time.

See Also: The Diagnosis and Treatment of Autoimmune Encephalitis


You may risk complete hearing loss and lose your balance if you do not get treatment for autoimmune ear disease immediately. Hence, if you are already suffering from some other autoimmune disease, or face any of the symptoms of AIED, get yourself tested and treated. Our healthcare specialists at Family Medicine Austin are well-trained to test for autoimmune diseases, as well as treat them. If you suffer from hearing loss and cannot find any other reason to explain it, you may be suffering from AIED, so book a consultation now.

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