The difficulty with this disease entity is that it is a heterogeneous group of conditions affecting the ear and a widely accepted diagnostic criteria does not exist. It is therefore difficult to conduct a well controlled trial and this systematic review reflects the issue. For otolaryngologists this “voodoo” condition is poorly understood although we love to speculate on the aetiology, and even sometimes manage to convince our patients that we know what the cause of their hearing loss is. Steroids such as prednisolone are the mainstay of treatment and if this fails, most of us would give up. Others would throw the book (and the kitchen sink) at them. This well researched paper provides a brief summary of other treatments such as cyclophosphamide, methotrexate, TNF-apha inhbitors (Golimumab, infliximab, etanercept, rituximab), enoxaparin (Clexane), azathioprine and plasmapheresis. Do any of these agents work? Well, the evidence would suggest not unfortunately, although I am inclined to believe that this is a reflection of the difficulty in conducting studies to test efficacy of treatments and to identify subsets of patients with particular types of autoimmune disease. The important points to note are that: 1) if conducting a trial of patients with autoimmune disease, the patient must have had a trial of steroids to check that the aetiology is indeed autoimmune; 2) steroids may need to be continued for years to prevent (a quicker) hearing loss; 3) cochlear implantation is a viable option for those that are rendered bilaterally profoundly deaf with outcomes as good as matched controls.