Gums a defense from disease
inner ear syndrome.
Department of Otolaryngology, University
of California-Davis, Sacramento,
OBJECTIVE: This study was initiated to
clarify the possible association between
CONCLUSIONS: These data support the
hypothesis that antiphospholipid
antibodies are involved in the pathogenesis of some forms of inner ear
dysfunction, presumably by causing microthrombus formation in the labyrinthine vasculature. Basic science
studies are required to better
understand the mechanisms by which antiphospholipid antibodies mediate
inner ear dysfunction. Clinical studies
to evaluate the efficacy of
anticoagulation in this group of
patients are also required.
PMID: 15867658 [PubMed - indexed for
Semin Arthritis Rheum. 2004
Immune-mediated inner ear disease:
Broughton SS, Meyerhoff WE, Cohen SB.
Otolaryngology, University of Texas
Southwestern Medical School, Dallas,
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.
To analyze data from 42 patients with
documented immune mediated inner ear
disease to further define this syndrome.
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
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
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.
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.
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