Autoimmune Hearing Loss
Autoimmune Inner Ear Disease (AIED) is a syndrome of rapidly fluctuating hearing loss over a period of weeks to months caused by antibodies that attack the inner ear. 50% of people with this type of hearing loss can also experience vertigo or dizziness. Tinnitus is also very common due to the decline in hearing which can occur over several months.
Allergy and autoimmune diseases can have a direct impact on the inner ear. There are several autoimmune diseases that can be associated with AIED which include: Wegener’s Granulomatosis, Sjogren’s Syndrome, Ankylosing Spondylitis, Lupus, Rheumatoid Arthritis, Scleroderma, and Bechet’s Disease.
AIED is uncommon and accounts for 1% of incidents of hearing loss and vertigo.
AIED is diagnosed based on the clinical picture of patient presentation, immune tests, and response to steroids. Oftentimes, the patient will present with bilateral nerve hearing loss with poor speech discrimination scores that arise over weeks or months. Most patients experience bilateral (both ears) hearing loss, but for some the hearing loss may lateralize to one ear. Fluctuations in hearing that can vary from day to day (sometimes better, sometimes worse) are also a common symptom of this ear disease. Half of the patients will also complain of problems with balance and will describe vertigo (spinning sensation), disequilibrium, or motion intolerance.
Labwork may be obtained to check for evidence of systemic and ear specific antibodies. These labs are not definitive for AIED, but can help lead to a diagnosis in certain situations.
An important feature of AIED is that hearing loss will often have a positive response to corticosteroids. Steroids reduce inflammation and are given orally or via intratympanic injection. However, 50% of patients will not achieve improvement in their hearing despite taking the steroids. Some patients cannot take steroids due to contraindications with diseases or interactions with medications; therefore, some patients are offered an alternative immunosuppressant such as Methotrexate. In addition, patients that have a positive response to corticosteroids with improved hearing may benefit from long term immunosuppressant treatment, which is often managed by a Rheumatologist.