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)
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W.
Leroy Griffing, M.D. |
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. |
