How is IMIED treated?
Corticosteroids should be started at a dose of 1 mg/kg per day and continued for 2 to 4 weeks. If there is no response, they should be tapered off quickly over a period of 2 weeks. If a response in hearing is documented by repeat audiology testing after a 2 to 4-week trial of therapy, steroid tapering should be performed at a slower rate over an additional 2 to 3 months. There is no consensus regarding whether corticosteroid-sparing therapy should be given concurrently at the time of diagnosis or which agents to consider in patients who relapse during steroid taper, as data from clinical trials is limited. Methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, anti-TNF therapy (systemic and intratympanic), rituximab, and intratympanic steroids have also been used with variable success (although a placebo-controlled trial of methotrexate failed to show benefit). Interpretation of the efficacy of these agents is limited given the lack of systematic studies. Given the toxicities of these therapies, cochlear implants should be considered as an alternative in severe cases.
