Hearing Loss in Scleroderma (Question and Answer)

By W. Leroy Griffing, M.D., Chair, Division of Rheumatology, Mayo Clinic Scottsdale, Scottsdale, Ariz. (originally published in Scleroderma Voice, 2003 #1)

Question: I have been diagnosed with scleroderma of the middle and inner ear, resulting in a progressive hearing loss. I have been unsuccessful in finding any information on this type of scleroderma. Can you provide me with any information?

Answer: Hearing loss in autoimmune illnesses, including scleroderma, is one of the least written-about topics in medical journals. This is on account of its rarity. So it is not surprising that you would have difficulty locating information about this diagnosis.

Although rare, the impact and potential disability of Immune Mediated Inner Ear Disease (IMIED) can be profound for those experience it, especially if not recognized and treated early.

IMIED is a syndrome characterized by hearing loss, vertigo (a specific form of dizziness), and tinnitus (noises or ringing in the ear). Its onset is usually abrupt, with progressive loss of hearing over a few days to weeks. It commonly affects both ears, often beginning on one side and then, a few weeks later, including the other side. The severity of hearing loss can fluctuate spontaneously, better or worse, usually in combination with the vertigo and tinnitus. The most severe cases may lead to permanent deafness.

IMIED can occur as an autoimmune illness by itself. It is known to occur also in conjunction with other illnesses, most often the autoimmune diseases called vasculitis, or inflammation of blood vessels. Examples of vasculitis are Wegener's Granulomatosis, Polyarteritis nodosa, and Cogan's Syndrome. When occurring with arthritis illnesses, the most commonly reported, and the most common in my own experience, is systemic lupus.

Little is known of the exact mechanism of this illness. Studies are made more difficult by the fact that the inner ear is located deep inside the bones of the skull where it is inaccessible.

In contrast to the skin, lung, or kidney—which are more directly accessible and if necessary, can be biopsied with relative safety—a biopsy of the inner ear is not practical. Because of the tiny size of the inner ear, a biopsy also would result in additional and permanent hearing loss and more imbalance.

From the few reports available, it is intriguing that the blood vessels of the inner ear in this illness do not show vasculitis, as might be anticipated based on its occurrence with the various types ofvasculitis mentioned. Instead, a thickening of the blood vessel walls is found, similar to the thickening seen in scleroderma blood vessels.

About 60% of IMIED patients have a serum antibody directed against an antigen called 68-kD antigen. This antibody, for which testing is available in many laboratories, also reacts with a protein called HSP-70. IMIED patients with this antibody, in comparison to those without it, appear more likely to respond to treatment.

Because of the rarity of this illness, no controlled treatment trials have been possible. Usually high doses of Prednisone are used at first. Cytoxan or methotrexate is used for longerterm treatment. It appears the sooner treatment is started, the better the outcome in preventing partial or complete permanent hearing loss.