Autoimmune inner ear disease (AIED) is characterized by bilateral, asymmetric, and fluctuating sensorineural hearing loss (SNHL) with no identifiable cause that responds to immunosuppressive therapy. Diagnosis can be challenging due to a lack of standardized diagnostic criteria and pathognomonic tests. The mainstay of treatment is corticosteroids
however, only a small number of patients remain responsive after prolonged use. There are no agreed treatment protocols for AIED following corticosteroids, as there is limited data from randomized controlled trials. We report a case of a 27-year-old man with secondary AIED on a background of ulcerative colitis (UC) who experienced frequent relapses and deterioration in his hearing despite multiple courses of high-dose corticosteroids. He received methotrexate and azathioprine but did not show clinical or audiometric improvement. After the commencement of infliximab infusions, his symptoms of AIED and UC improved, and his hearing remained stable without further use of oral corticosteroid therapy. The available studies on the efficacy of biologic therapy are limited and have produced variable results, with the majority of the data relying mainly on case reports and case series. Large, multicenter randomized controlled trials are required to confirm its efficacy in the management of AIED.