A businessman in his 50s who had normal hearing began experiencing dizzy spells and tinnitus with episodes of severe imbalance. He came to the House Clinic for answers. Was it a tumor? A mystery infection? Too much loud music? “No,” Dr. M. Jennifer Derebery, told him. He was suffering from a rare disorder called autoimmune inner ear disease (AIED).

AIED is a complex of symptoms that can cause rapidly progressing sensorineural hearing loss as well as tinnitus, vertigo and dizziness. It ultimately affects both ears, although it can start on one side. Problems tend to worsen over weeks or months as opposed to the sudden hearing loss associated with a virus or the years of progressive loss that come with aging. “The key thing to watch for is the hearing level rapidly changing,” says Dr. Derebery, an associate of the House Ear Clinic and Institute and a clinical professor of otolaryngology at USC’s Keck School of Medicine.

While all House doctors treat AIED, Dr. Derebery – who also serves as director of the clinic’s allergy department – sees the largest number of cases. Diagnosis can be tricky. “You first have to rule out other causes,” she says. This requires obtaining or reviewing a patient’s history (generally, the only things that produce similar symptoms are ototoxic drugs). Doctors also order tests such as an MRI or blood work looking for evidence of markers of other known autoimmune diseases, or a test for a specific protein that is positive in about 60 percent of patients called the Western Blot (heat shock protein 70).

In the past five years, House, which probably sees a disproportionate number of cases, has made a primary diagnosis of AIED 117 times. There is no known cure. The standard treatment involves prescribing the steroid prednisone, which can stabilize or improve symptoms. “The initial thought was that if you didn’t treat aggressively, patients would go deaf,” says Dr. Derebery. “However, the thinking has changed.

Prednisone has serious side effects so, says Dr. Derebery, “the goal of therapy is to use just enough to induce serviceable hearing and long periods of remission.” Steroids are taken orally or can be injected through the eardrum, which reduces systemic impact and places a higher concentration of the drug in the inner ear. House is working with rheumatologists to find alternatives to steroids. So far, no clear winner has emerged.

The businessman in his 50s responded well to four weeks of a highdose (60 milligrams-a-day) prednisone regimen. As soon as it ended, however, his hearing worsened. The high-dose regimen was resumed and, after five months of tapering, he was able to go steroid-free. “He’s been stable for the past eight years and does well with hearing aids,” says Dr. Derebery.

The search for better ways to treat AIED continues. House recently hosted a small conference of experts in hopes of designing a clinical trial to look for alternatives to steroid therapy. “I tell patients that contrary to what they see on the Internet and in older ENT literature only a small percentage will lose their hearing,” says Dr. Derebery. “There are things that we can do.”