AISNHLAutoimmune Sensorineural Hearing Loss
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AISNHL is most likely an immune-mediated condition, although there is no direct evidence of its autoimmune etiology.
Treatment of patients in the acute stage of AISNHL usually includes the use of corticosteroids, which is the only validated treatment option.
21) Van Wijk et al conducted a preliminary study of 9 patients with AISNHL who were treated with transtympanic infliximab and found the results encouraging.
However, all of the patients in our study had been selected for infliximab treatment because their AISNHL was already refractory to all recommended treatments, including steroids, methotrexate, and/or cyclophosphamide.
Finally, in studying the association between AISNHL and HLA types, Bowman and Nelson found that AISNHL was associated with the presence of HLA-B35, -CW4, and -CW7 and the absence of HLA-DR4.
AISNHL may cause hearing loss of any pattern, tinnitus, and dizziness.
The pretreatment audiometric analysis is a crude control tool, but it helps define the natural history of AISNHL.
Thus, as McCabe has suggested, there might be a window of opportunity to treat AISNHL before irreversible inner ear damage occurs.
11) Further evidence has suggested that AISNHL is associated with the presence of an antibody to a 68 kD inner ear protein (isolated by Western blot assay).
15,16) Disher et al reported that KHRI-3 precipitates a 68-70kD antigen that binds human antibodies in the sera of suspected AISNHL patients.
8) Although the treatment of suspected AISNHL has not been well defined, steroids and cytotoxic immunosuppressants have been advocated most often.
6% of tested patients are similar to those previously reported by Bowman and Nelson, (7) and they further support the proposed association of these antigens with AISNHL.