Although steroids are effective for many patients with autoimmune inner ear disease (AIED), there are still challenges in diagnosing the condition, mysteries behind what happens to the inner ear, and controversy over whether intratympanic injections help.
Explore this issue:December 2007
AIED is a rapidly-progressive bilateral sensorineural hearing loss. Usually, hearing loss occurs in one ear first, then progresses to the second ear. It is often not caught until there is a decline in hearing in the second ear. The condition tends to be steroid-responsive, but there are some controversies and uncertainties in their use for some types of AIED.
ENToday spoke to two thought leaders in the field: Jeffrey Harris, MD, PhD, Professor and Chief of Otolaryngology at the University of California, San Diego, and Steven Rauch, MD, Associate Professor of Otology and Laryngology at Harvard Medical School. Both agreed that, generally, steroids are effective for AIED, but because of side effects with long-term use there is a limit to their use. Steroids have a number of actions believed to help AIED and its various causes. They have anti-inflammatory action, are immunosuppressive, and can target glucocortcicoid receptors in the inner ear.
We don’t really know which of those effects are most prevalent in autoimmune treatment, Dr. Harris said.
AIED Classification System
Dr. Harris has developed a classification system for AIED and AIED-like conditions that breaks the condition into six key categories.
The first is rapidly progressive bilateral sensorineural hearing loss where there is no evident systemic autoimmune disease. This can occur in people of any age, but only about 50% of these patients respond to high-dose steroids.
The second form of AIED usually occurs with a flare-up of an existing autoimmune condition, such as lupus, ulcerative colitis, or rheumatoid arthritis. Serological studies will confirm the illness. We usually maintain these patients with drugs that are effective for their rheumatic condition as well-so we start with high-dose steroids but we convert them to other drugs that manage their rheumatic diseases, Dr. Harris said.
Third is an immune-mediated Méniàre’s disease, which consists of bilateral, fluctuating sensorineural hearing loss. Vertigo may predominate as a symptom. The condition is steroid-responsive, but may require long-term immunosuppression.
A fourth type is rapidly progressive bilateral sensorineual hearing loss that accompanies inflammatory disease such as chronic otitis, Lyme disease, otosyphilis, or serum sickness. There can be hearing loss even if the infective agent is treated. This too is steroid-responsive.