Immune-mediated inner ear disease

摘要: Immune-mediated inner ear disease (IMIED) is the preferred term for syndrome involving cochlear dysfunction, leading to degrees of sensorineural hearing loss and vertigo. Although vasculitis has been inferred be cause IMIED in some cases, histopathological proof vascu-litis this condition lacking. The does appear mediated fashion by immune system; hence, name IMIED. can occur association with a recognized autoimmune such as Sjogren's syndrome, Wegener's granulomatosis, or giant cell arteritis, an isolated entity which no other organ dysfunction evident. onset rapid compared entities that lead auditory dysfunction. Hearing occurs over course weeks months. Patients IMIED-associated have problems both acuity word discrimination. Following conversations noisy environments (e.g., crowded restaurants) may particularly challenging. For patients IMIED, vestibular result from damage challenging if not more so. Up 70% diagnosed demonstrate clinical improvement following glucocorticoid treatment. Prednisone (1 mg/kg/day) reasonable starting dose treatment rapidly progressive secondary If patient regains significant function within 2 weeks, tapering prednisone begin taper designed discontinue medication severe recurrent audiovestibular it clear whether long-term courses high-dose glucocorticoids additional immunosuppressive agents are effective restoring function. Such approaches should used cautiously.