In various patterns of sensorineural hearing loss including Meniere's disea
se, which may show improvement in auditory function following immunosuppres
sive therapy, an isolated autoimmune disease of the inner ear has been post
ulated. Because of the lack of well-defined diagnostic criteria to identify
autoimmune processes within the inner ear and the fact that the human inne
r ear is one of the few organs of the body not amenable to diagnostic biops
y, there has been great interest in developing animal models that mimic the
se clinical entities. Previous studies have found evidence that this proces
s might be cell mediated and that the endolymphatic sac functions as an imm
unodefensive organ for the inner ear. By heterologous immunization of inbre
d Lewis rats with inner ear tissue, an autoreactive inner-ear-specific T he
lper cell line was established. After passive transfer of these cells a lab
yrinthitis was induced in recipient animals. Immunohistochemically, T helpe
r cells were first identified in the cochlea suggesting that this cell type
might carry the autoantigenic epitope. Autoantibodies against inner ear ti
ssue were demonstrated in animals with histologically evident labyrinthitis
. We conclude that this experimental design can serve as an animal model fo
r cell-mediated autoimmune disease of the inner ear and could be used to ex
plain the etiology of certain types of sensorineural hearing loss such as M
eniere's disease. With this approach the identification of the causative au
toantigen should be possible and will lead to the development of appropriat
e clinical tests to diagnose autoimmune diseases of the inner ear in humans
.