Serum antibodies against membranous labyrinth in patients with „idiopathic“ bilateral vestibulopathy

Abstract

To investigate the possibility of an autoimmune mechanism in idiopathic bilateral vestibulopathy (IBV), we screened patients' sera for antibodies against inner ear structures. IgG antibodies against membranous labyrinth (ampulla, semicircular canals, saccule and utricle) were detected in 8 of 12 patients by immunofluorescence on rat inner ear cryosections. All but one serum of 22 healthy controls and the sera of 6 patients with known autoimmune disorders showed only background staining. Low-titre anti-nuclear IgM antibodies were present in three control sera and one IBV serum. High-titre anti-nuclear IgM was found in a patient with lupus erythematosus and in one with scleroderma. Anti-nuclear IgM was not organ-specific. No human serum used contained detectable anti-vascular preformed antibodies. Cross-reactivity to sections of liver, kidney, cornea, brain and skeletal muscle was absent. Double-staining for IgG and F-actin, the primary constituent of hair cell cilia, did not show predominant Ig-coating of sensory hair cells. Immunosuppressive therapy in 3 IBV patients did not improve the disorder, probably owing to irreversible loss of sensory and neural structures. These data suggest that the bulk of anti-labyrinthine autoantibodies may be an epiphenomenon, yet a small subgroup of organ-specific autoantibodies may synergize with a cellular response in the development of vestibular lesions.

Vestibular Evoked Myogenic Potentials in Patients with Idiopathic Bilateral Vestibulopathy

“…Previous reports suggested that the progressive type could be related to autoimmune disease. Arbusow et al [6] reported that antibodies against the membranous labyrinth (including the cochlea) were detected in some IBV patients. They concluded that Matsuzaki/Murofushi only a small subgroup of organ-specific autoantibodies would play a role in the pathogenesis of IBV.…”

“…Responses to both caloric and rotational tests are markedly decreased bilaterally, but the findings of other neurological examinations are unremarkable. Various etiologies such as viral infection, autoimmune or degenerative disease, and ischemic attack have been proposed for this disorder [1][2][3][4][5][6]. However, 'idiopathic' cases still exist.…”

“…However, 'idiopathic' cases still exist. In previous cases, caloric and rotational tests were used to diagnose bilateral vestibular dysfunction [1][2][3][4][5][6]. However, they could only evaluate functions of the lateral semicircular canal (SCC) and its afferents, in other words, the superior vestibular nerve.…”

Novel subtype of idiopathic bilateral vestibulopathy: bilateral absence of vestibular evoked myogenic potentials in the presence of normal caloric responses

“…Absent or markedly decreased response of the LSCC is revealed in the caloric test, the rotation test [3,6,26,29,31] and the head thrust test [35,36]. Originally, IBV is not accompanied by cochlear or any other neurological symptoms [6].…”

Anti-labyrinthine antibodies in a patient with relapsing polychondritis

“…We presume that anti-labyrinthine antibodies represented an epiphenomenon of a cellular autoimmune process [2]. The sudden onset of the disease in our patient and its amelioration under anti-inflammatory and immunosuppressive treatment as well as the appearance of anti-labyrinthine antibodies were indications to us of an autoimmune origin.…”