Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Ménière's disease()

OBJECTIVE: To investigate immune-related genetic background in intractable Meniere’s disease (MD) and the immediate results of a novel therapy by delivering steroids to the surface of the intact endolymphatic sac (ES) and incus in a sustainable manner. CASE REPORT AND METHODS: Candidate genes involved in immune regulation were sequenced using a next-generation sequencing method in a patient with intractable MD. Mutations were confirmed using the Sanger sequencing method. The ES was exposed, and gelatin sponge particles were immersed in high-dose methylprednisolone solution and placed onto the surface of ES. “L”-shaped gelatin sponge strips were immersed in dexamethasone solution and served as a guiding device for the steroids by touching the incus and gelatin sponge particles on the surface of the ES. Gelatin sponge particles immersed in dexamethasone solution were placed around the gelatin sponge strips and sealed using fibrin glue. RESULTS: Autoinflammation in the refractory MD case was indicated by genotype, including novel heterozygous mutations of PRF1, UNC13D, SLC29A3, ITCH, and JAK3, as well as phenotype. The vertigo was fully relieved immediately after operation. Tinnitus and aural fullness were resolved 3 weeks after operation, whereas hearing improved in 2 mon postoperation. No recurrence was noted during the 5-monfollow-up, and the final MRI supported the novel therapeutic hypothesis. CONCLUSION: Autoinflammation was involved in a refractory MD. This novel therapy, which involves the delivery of steroids to the surface of the intact ES and incus, is effective in relieving vertigo and tinnitus and improves hearing function of refractory MD.