Autoimmune inner ear disease, known as A.I.E.D., is a rare condition distinguished by the immune system’s inappropriate attack on the inner ear. This disorder can result in various symptoms, including hearing loss, dizziness, and tinnitus (ringing in the ears). AIED is estimated to affect less than one percent of the almost 30 million Americans with hearing loss.
Autoimmune Inner Ear Disease: What Is It and How to Treat
Common Symptoms
Individuals diagnosed with Autoimmune Inner Ear Disease (AIED) typically experience hearing loss that initially starts in one ear before gradually progressing to the other. This development may occur over several weeks or extend over a few months. Other symptoms include:
• Tinnitus
• Dizziness and vertigo
• Feeling clogged or stuffed up in one or both ears
• Hearing loss in one or both ears
Common Causes
The immune system plays a crucial role in protecting the body from pathogens. However, there are instances when immune cells may mistakenly identify the cells of the inner ear as foreign invaders. This misidentification leads to an autoimmune reaction, wherein the immune system threatens to damage its own tissues and organs. Research indicates that approximately one-third of individuals diagnosed with Autoimmune Inner Ear Disease (AIED) also present with another autoimmune condition that impacts the entire body. These conditions may include rheumatoid arthritis, scleroderma, ulcerative colitis, lupus, or Sjögren’s syndrome (dry eye syndrome).
Getting Diagnosed
Due to the nature of the symptoms associated with AIED, accurate diagnosis can be challenging. Frequently, these symptoms are attributed to an ear infection until the hearing loss progresses to the second ear. To accurately diagnose AIED, a physician will inquire about the patient’s health and medical history, conduct a thorough physical examination, and administer a hearing test. In addition, the physician will assess the patient’s balance, which can provide insight into the communication between the inner ear and the brain. Blood tests may also be required to aid in the diagnostic process. Currently, no definitive test is available to confirm the presence of AIED. However, test results may indicate an autoimmune response. Given the potential for hearing damage that may be irreversible, your physician may initiate treatment even in the absence of a conclusive diagnosis. Many individuals are not formally diagnosed with AIED until they commence treatment and experience an improvement in their symptoms. In such cases, consulting with an otolaryngologist, preferably one with expertise in autoimmune disorders, is advisable.
Treatment Options
A healthcare provider will typically prescribe medication to help manage symptoms. High doses of corticosteroids have demonstrated efficacy in addressing Autoimmune Inner Ear Disease; however, use generally is limited to a few weeks due to potential side effects. The healthcare provider may recommend additional medications to reduce the immune response following the corticosteroid treatment. Agents such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and methotrexate are among some of the options available. Hearing aids can be a practical solution for individuals adjusting to hearing loss; however, a healthcare professional may recommend a cochlear implant in cases of severe impairment. This device works by stimulating the nerves in the inner ear, which in turn send signals to the brain, allowing for sound perception. The cochlear implant consists of two components: one positioned behind the ear and the other surgically implanted beneath the skin.
As research advances our understanding of AIED, a broader range of treatment options may emerge. These could include medications with enhanced effectiveness and fewer side effects and innovative approaches such as gene therapy.