Autoimmune Inner Ear Disease (AIED)
What Causes AIED?
The causes of AIED can include:
- The body’s uncontrolled immune system attacks the inner ear protein, forming immune complexes and antibodies and causing progressive hearing loss in both ears.
- Cochlin is a protein located in the inner ear that is attacked by the immune system.
- Endolymphatic sac, a structure of the inner ear, can become dilated as the immune response of the inner ear.
What Are the Treatment Options?
If you are having hearing loss that is getting worse in both ears over weeks to months, you should see an ENT specialist who can make a diagnosis after reviewing your hearing tests and imaging scans. If your doctor suspects that you may have AIED, you may respond well to medical therapy—steroid and immunosuppressive medication—if started early.
Corticosteroid is the main treatment to suppress the immune response to reduce inner ear swelling and inflammation. Early detection of AIED and prompt steroid treatment may help reverse your SNHL. If you cannot tolerate steroid treatment, there are alternative medications, such as cyclophosphamide, methotrexate, azathiorprine, and rituximab.
Your doctor may also evaluate you for a hearing aid or other assistive listening devices. If hearing loss is substantial, your doctor may recommend a hearing device called a cochlear implant. Speak with your doctor about other specific treatment options.