Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed investigation given that the auditory symptoms and their responsiveness to immunosuppression are the focus of the few proposed diagnostic criteria for AIED. We present three cases of vestibulopathy occurring in the context of autoimmune inner ear conditions, including the first known report of autoimmune inner ear pathology arising with a temporal association to administration of the Pfizer-BioNTech SARS-CoV2 vaccination. We review the available literature pertinent to each case and summarise the key learning points, highlighting the variable presentation of vestibular impairment in AIED.
Keywords: AIED; COVID-19; Cogan syndrome; autoimmune; ulcerative colitis; vaccination; vestibular; vestibulopathy.