Objectives: This study aimed (1) to report the long-term effects of infliximab, a murine monoclonal antibody directed against tumour necrosis factor-, on autoimmune inner ear disease, and (2) to discuss dilemmas surrounding the long-term management of autoimmune inner ear disease. Case report: A 49-year-old man presented with sudden-onset, left-sided, sensorineural hearing loss, tinnitus and vertigo. He was prescribed oral prednisolone, with benefit. Over several subsequent months, he experienced frequent relapses and progressive deterioration of high-frequency hearing bilaterally. Multiple agents failed to stabilise his condition. Following infliximab treatment, there was a documented and sustained improvement in his hearing and tinnitus. He stopped the treatment after 46 weeks, with rapid relapse of his condition. His hearing recovered quickly again after recommencing infliximab. Conclusion: The benefits of prolonged infliximab use and potential side effects must be balanced against allowing the disease to take its course, with progressive deafness. Randomised controlled trials are required to assess infliximab's optimal duration of use, long-term efficacy and safety in the treatment of autoimmune inner ear disease.