INNER EAR IN AUTOIMMUNE DISEASE

Hearing loss is often associated with human immune disorders, including
both autoimmune disease and systemic immune complex disease. At present
we have very little understanding of the cellular and molecular
mechanisms of the ear that are compromised to lead to this deficit. The
general aim of the study is to evaluate inner ear morphology,
electrophysiology, and immunopathology in mouse models of autoimmune
disease and systemic immune complex disease. C3H control mice, C3H/lpr autoimmune disease mice, and C3H mice with
induced systemic immune complex disease will be evaluated with auditory
brainstem response electrophysiology at ages of 2, 4, and 6 months which
are before, during and after onset of autoimmune disease at 3 months.
Serum analyse of antinuclear antibody, immune complexes, cryoglobulins,
and total globulins will be used to assess systemic disease. Light and
electron microscopy of the cochlea will indicate the areas of pathology
and light and electron microscopic immunocytochemistry of immunoglobulins
and complement will indicate the areas of immunopathology.
Corticosteroid therapy will be attempted to prevent the effects of
systemic autoimmune disease on the cochlea to identify possible
interventional methods. Cochlear electrophysiology, pathology, and
immunopathology will be compared in the groups to determine the extent
and similarities of cochlear damage with the two immune disease models. The study will allow us to identify the potential inner ear disease
mechanisms in human immune disorders. By understanding the sensorineural
pathology related to these diseases, we can more effectively intervene in
such cases of human inner ear dysfunction.