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Autoimmune inner ear disease.
Autoimmune inner
ear disease (AIED) is an inflammatory
condition of the inner ear. It occurs when
the body's immune system attacks cells in
the inner ear that are mistaken for a virus
or bacteria. AIED is a rare disease
occurring in less than one percent of the 28
million Americans with a hearing loss.
Ruckenstein MJ.
Department of
Otorhinolaryngology, Head and Neck
Surgery, University of Pennsylvania,
REVIEW:
The role of the
immune system in inner ear disease has
received considerable attention over the
past two decades. To
summarize recent basic science
research into the pathogenesis of
autoimmune inner ear disease (AIED),
review the current diagnostic
work-up for patients with suspected
AIED, and delineate treatment
strategies.
RECENT FINDINGS:
 Basic
science and clinical studies have
been performed to delineate the
mechanisms by which autoimmune
processes may affect the ear and to
develop treatment strategies to
reverse this pathology.
AIED refers
to a rapidly progressive (over a
course of weeks to months)
sensorineural hearing loss that
responds to the administration of
corticosteroids. In addition,
systemic autoimmune diseases (eg,
vasculitis, Lupus, Wegener) can secondarily
affect the inner ear.
Although a
number of diagnostic tests for AIED
have been advocated, the diagnosis
of this entity is still predicated
on a positive therapeutic response
to corticosteroid administration.
Alternate immunosuppressive
regimens, designed to be used in
patients who cannot be weaned off
corticosteroids or whose disease
becomes refractory to treatment,
have proven to be difficult to
develop. Methotrexate, which
initially showed promise, has
recently been shown to be
ineffective in preventing
progression of hearing loss. Further
research is required to better
delineate the pathophysiologic
mechanisms involved in AIED and to
establish more effective and better
tolerated treatment regimens.
SUMMARY: The original enthusiasm
that was generated by the concept
that immune-mediated mechanisms may
mediate reversible forms of inner
ear pathology has been tempered by
the realities conveyed by scientific
research. The pathophysiology of
AIED is still not well understood.
Multiple potential mechanisms have
been identified that can result in
immune-mediated inner ear pathology.
The diagnosis of AIED is based on
clinical presentation and response
to the administration of
corticosteroids. Diagnostic testing
may support the diagnosis, and the
results of a recent multicenter
study should clarify the role of
immunologic testing in the diagnosis
of this entity. Treatment options
are limited, with corticosteroids
being the only validated treatment
option, and methotrexate offering no
significant benefit to patients. The
role of cyclophosphamide is
restricted to patients willing to
endure the attendant risks. With the
advent of cochlear implants, the
administration of toxic medications
to preserve hearing at all costs is
a less desirable
CIDPUSA has
discovered that the disease is triggered
by a virus & bacteria is very difficult
to treat and by using electronic means
the treatmentshould be limited. As the
Herx reaction tend to make the attacks
worse in the initial treatment period.
Steroids in the ear are very effective.
-
Semin Arthritis Rheum.
2004 Oct;34(2):544-8.
-
Immune-mediated inner ear
disease: 10-year experience.
Broughton SS,
Otolaryngology, University of Texas
Southwestern Medical School, Dallas,
Texas, USA.
BACKGROUND:
Autoimmune inner ear disease (AIED)
was first described in 1979 and the
disease has become more widely
recognized over the last decade.
Limited information is available
regarding clinical features of the
disease, disease course, and
response to treatment.
OBJECTIVE: To
analyze data from 42 patients with
documented immune mediated inner ear
disease to further define this
syndrome.
METHODS: A retrospective
chart review was conducted on all
patients considered to have AIED by
the Otolaryngology division
physicians and on all patients
positive for antibody testing to
inner ear antigens from 1990 to
1999. Patients who were antibody
positive with a clinical diagnosis
of AIED were included in this
review.
RESULTS: Patients with AIED
presented with rapidly progressive,
frequently bilateral (79%), often
fluctuating sensironeural hearing
loss. Mean age at presentation was
50 years (22-80) with no gender
predilection. Tinnitus (83%),
vestibular complaints (79%), and Menieres (50%) were common
concomitant symptoms along with
hearing loss. Seven of 42 (17%) of
the patients had evidence for other
systemic autoimmune disorders. In 4
of the patients the onset of
vestibuloauditory complaints
preceded the diagnosis of autoimmune
disorder. Thirty-three of 42
demonstrated antibodies to inner ear
antigens but other autoantibodies
were infrequent except in patients
with systemic autoimmune disorders.
Twenty-three of 33 (70%) of patients
treated with corticosteroids
improved clinically, often
short-term. Sixteen patients
received treatment with other
immunosuppressive drugs including methotrexate, cyclophosphamide,
azathioprine, mycophenolic mofetil,
and intravenous immunoglobulin
(IVIg) generally with a limited
modest response. Using clinical
trial criteria for response, only
5/35 (14%) demonstrated improvement
over a mean 34.4 months follow-up.
CONCLUSIONS: Immune-mediated inner
ear disease is not a uniform disease
with simple diagnosis or treatment.
The course of the disease often
results in significant long-term
disability due to hearing loss and
response to aggressive immunosuppression including
corticosteroids is poor.
PMID: 15505770 [PubMed
- indexed for MEDLINE]
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