Cyclophosphamide is tapered first followed by steroids. If symptoms recur both drugs are restarted. Three month cycles are continued until patients can be weaned. No patient required more than 24 months of treatment in McCabe's study. Hughes advises plasmapheresis for those patients unresponsive to steroids and cytoxan after 6 to 8 weeks at the above stated doses.
Plasmapheresis theoretically removes unwanted humoral and cellular elements.
Treatments are given three times weekly for 2 weeks followed by once weekly for 4 additional weeks. In summary, otolaryngologists need a high index of suspicion for autoimmune etiologies in patients with sensorineural hearing loss. Ophthalmologic, neurologic and rheumatologic consultations are useful in ruling out systemic vasculitic diseases. Steroids and cyclophosphamide remain the cornerstones of treatment in autoimmune inner ear disease, with reservation of plasmapheresis for refractory cases. If caught early, and with aggressive medical management, hearing stabilization and possible improvement are feasible.