We have all heard about autoimmune diseases and probably recognize some of them by name. Lupus and rheumatoid arthritis are two of the most common. The label ‘autoimmune’ is used because the body’s immune system begins to attack healthy cells, mistaking them for an infection.
Another, rarer condition known as Autoimmune Inner Ear Disease (AIED) attacks the inner ear. Individuals with this disease experience loss of hearing and balance.
Unlike typical hearing loss, which can develop over time, AIED can cause hearing to change rapidly over the course of weeks rather than years.
AIED attacks the cochlea and vestibular system in the inner ear. While this disease is considered quite rare, accounting for less than 1% of hearing loss cases, the impact can be significant.
AIED Symptoms
While various symptoms can vary from person to person, the most common are listed below:
- Progressive hearing loss in one or both ears
- Ringing in the ears, also known as tinnitus
- Dizziness due to involvement of the vestibular system.
- Ear pressure, which can be mistaken for fluid buildup.
- Intermittent hearing changes making it harder to detect early.
While these hearing issues can appear similar to other conditions, a hearing health professional can determine whether it is AIED or not.
Causes of AIED
While the exact cause is not completely known, researchers have suggested it happens when the immune system misidentifies proteins in the inner ear, leading to damage and inflammation.
AIED may occur on its own (primary AIED) or as part of another autoimmune disease (secondary AIED). Some related conditions of secondary AIED can include the following:
- Rheumatoid arthritis
- Lupus
- Multiple sclerosis
- Inflammatory bowel disease
Women who are between 20 and 50 years of age have a much higher risk of developing AIED and should watch for symptoms. If you suspect you may have AIED, contact a hearing health professional.
How AIED Is Diagnosed
Sometimes diagnosing AIED can be challenging. This is due to the fact that symptoms can resemble other hearing health issues. No single test can confirm the presence of AIED. Hearing health professionals typically use a battery of tests to determine if you have AIED.
- Standard hearing tests to track changes in hearing over time.
- Blood tests to determine if autoimmune markers are in the bloodstream.
- Steroid treatment as a testing protocol. If hearing improves with the treatment, it can be an indicator of autoimmune disorders.
If you suspect you have AIED, see a hearing health professional for a thorough hearing test. Early treatment can improve outcomes.
Treatment Options
Curing AIED might not be possible; however, treatments can slow its progression and help you manage the associated symptoms.
- Corticosteroids – Often the first line of treatment, steroids reduce inflammation and may temporarily improve hearing.
- Immunosuppressive medications – For patients who cannot tolerate long-term steroid use, these drugs help control the immune response.
- Hearing aids – If hearing loss continues, modern hearing aids can provide clarity and amplification.
- Cochlear implants – For severe or late-stage hearing loss, cochlear implants may restore access to sound.
- Balance therapy – Vestibular rehabilitation may be recommended to address dizziness or balance challenges.
Early treatment increases the chance of preserving hearing, making timely care vital.
Finding Balance with AIED
Living with AIED can certainly be challenging; however, there are available supports and resources to help:
- Regular follow-up appointments with a hearing health professional can help to adjust available treatments to minimize your symptoms.
- Protecting hearing health by avoiding situations where you’ll be exposed to loud noises can help maintain your hearing.
While the occurrences of Autoimmune Inner Ear Disease are rare, it is a serious health condition that requires medical intervention. Although it cannot be reversed in most situations, it is important to recognize that there are treatments to help with the symptoms that occur.
By staying informed and proactive, individuals with AIED can manage the condition effectively and maintain strong connections to the world of sound.