Autoimmune Inner Ear Disease (AIED)
AIED occurs when the body’s immune system mistakenly attacks the cells of the inner ear
Autoimmune inner ear disease (AIED) is an uncommon disorder affecting roughly 15 out of every 100,000 people. During AIED, the body’s immune system mistakenly attacks the cells of the inner ear, resulting in progressive hearing loss. Currently, it is still not fully understood what causes this errant autoimmune reaction but we do know that it primarily affects adults (men and women but skews slightly to women), typically between the ages of 20 and 50. It is rare in children but not impossible.

Symptoms of AIED
Autoimmune inner ear disease is characterized by progressive sensorineural hearing loss occurring in both ears, though the loss can be asymmetrical. The rate of loss can also be variable – some people experience a rapid progression of hearing loss over a few weeks, while others find their loss is slightly slower over the course of months. Knowing how quickly the hearing loss began can help doctors discriminate AIED from sudden sensorineural hearing loss (which develops within 3 days) or age-related hearing loss (which is gradually progressive and occurs in older age). The hearing loss associated with AIED is also known to fluctuate in its severity.
Other common symptoms of AIED can include:
- Vertigo and dizziness (about 50% of cases)
- Tinnitus, which is a ringing, buzzing, clicking or other sound in the ear (25-50% of cases)
- A sensation of fullness in the ear
Although AIED can be a primary condition, meaning only the inner ear is affected, it can also be secondary to another autoimmune disease that affects other parts of the body. About 30% of AIED cases are due to a systemic autoimmune condition such as Sjogren’s syndrome, rheumatoid arthritis, or systemic lupus erythematosus. In these situations, individuals will also experience additional systemic symptoms such as fever, muscle aches, and fatigue, alongside their hearing loss.
Diagnosis and Treatment of AIED
Identifying AIED can be challenging as there are no specific tests that can diagnose it. The symptoms of AIED are not unique, and many early cases are mistaken for an ear infection. Other conditions such as Meniere’s disease may also mimic early AIED.
Diagnosing AIED is based on:
- Clinical symptoms and a history that rules out other more likely causes of sensorineural hearing loss
- Tests that show there is an autoimmune reaction occurring
- A positive response to immunosuppressive treatment
The first treatment for autoimmune inner ear disease is with corticosteroid medications to manage the overreaction of the immune system. An otolaryngologist (ear, nose, throat specialist) may recommend beginning corticosteroid treatment early even before a definitive diagnosis of AIED has been confirmed, as this can prevent further hearing loss. For most patients with AIED, other healthcare professionals including a rheumatologist and audiologist will also be involved in managing the condition.
However, not all people with AIED are responsive to treatment with steroids, especially in the mid- to longer term. In these situations, alternative immunosuppressive medications, including methotrexate and ciclosporin A, or biologics such as rituximab, have demonstrated some usefulness in treating AIED, though the evidence is limited.
Living with Hearing Loss in AIED
After consultation with an otolaryngologist or audiologist, individuals with sensorineural hearing loss may be fitted with hearing aids to help them adjust to their new level of hearing ability. However, if the hearing loss is profound, the best treatment option is a cochlear implant.

While a hearing aid amplifies sounds picked up by the ear, if the structures of the ear are too damaged to transmit these signals, hearing aids are ineffective. In these cases, a cochlear implant is used to bypass these structures and connect directly to the auditory nerve, which can then carry the signals to the brain for interpretation.
At the moment, there is much more still to learn about autoimmune inner ear disease. With advances in research and understanding, more effective treatment options may become available in the future.