Autoimmune Inner Ear Disease (AIED) is a rare autoimmune disorder in which your body’s immune system attacks your inner ear. It causes balance issues and hearing loss that become progressively more severe over time. This disease is quite rare; only about 1% of hearing loss cases are due to AIED. Let’s discuss this complex, rare disease more in-depth.
The Inner Ear Under Attack
Inside the inner ear, there are two organs: the cochlea, which is the main organ in the auditory (hearing) system, and the main organs of the vestibular (balance) system. Put together, these organs resemble a snail, with the cochlea making up the shell and the vestibular system making up the snail’s body. The cochlea and the vestibular system both collect information (sound information in the case of the cochlea, movement and balance information in the case of the vestibular system) and send it to the brain.
With AIED, the immune system mistakenly attacks the inner ear. This damages blood vessels, tissues and sensory cells in the organs and causes inflammation at the site of the attack, making it harder for the inner ear to transmit sound and balance information to the brain.
Symptoms of Autoimmune Inner Ear Disease
Symptoms for AIED often come in “flares” that last a few hours or days. During these flares, it’s common to notice or feel:
- Diminished hearing ability (e.g., sounds appear quieter or muffled)
- Tinnitus (ringing in the ears)
- Dizziness
- Vertigo
- A feeling of fullness or pressure inside your ear
These symptoms often develop quickly and worsen rapidly. Hearing loss may start in one ear and then spread to the other.
Hearing loss from AIED is most often sensorineural (occurring in the inner ear), which makes sense, given that the disease causes direct damage to the inner ear. AIED can also cause conductive hearing loss (occurring in the outer or middle ear) due to inflammation, which may obstruct the ear canal and prevent sound waves from reaching the inner ear.
Diagnosing Autoimmune Inner Ear Disease
There is no specific test for AIED, which means it is not always easy to diagnose. But some tests look for evidence of an immune system problem, such as high levels of certain antibodies or white blood cells. These tests can help to diagnose an autoimmune disorder or rule one out. AIED differentiates itself from other autoimmune disorders and inner ear dysfunctions by how rapidly hearing ability declines.
A hearing test will also be part of diagnosing AIED and determining its severity. A hearing test can also help determine if hearing loss is sensorineural, conductive or both.
Treating Autoimmune Inner Ear Disease
Once a diagnosis of AIED is obtained, a treatment plan of corticosteroids to reduce inflammation and immunosuppressants to manage the immune system may be used to prevent AIED attacks from happening.
Hearing devices, such as hearing aids or cochlear implants, may also be used to help with the damaged inner ear. Hearing aids can amplify pitches and tones that the damaged cochlea can no longer hear, and cochlear implants can send sound signals directly to the nerve pathway, bypassing the damaged cochlea.
You can learn more about AIED, hearing loss and hearing devices by calling us at San Diego Hearing Center.