Autoimmune Inner Ear Disease: The Current State of Knowledge

Background/Objectives: An increasing number of cases of sensorineural hearing loss are caused by autoimmune inflammatory reactions within the cochlea, which can either be limited to the inner ear or associated with other systemic autoimmune reactions. The objective of this work was to conduct a narrative review of the worldwide literature from the past five years, to summarize the current state of knowledge on autoimmune hearing loss, and to understand its pathology and its treatment strategies. As a narrative review, our publication aimed to describe the most interesting and up-to-date case reports that add to our knowledge of autoimmune inner ear conditions. Methods: We searched the Scopus, Web of Science, and PubMed databases using the key phrases “autoimmune inner ear disease”, “autoimmune sensorineural hearing loss”, and “sudden sensorineural hearing loss”. The selection covered papers published within the past five years. Results: Based on the selection criteria, we identified 122 relevant articles. Further analysis and limitation to the main scope of the review led to the final selection of 48 papers, which formed the basis for this review. Conclusions: Autoimmune hearing loss appears to be an increasing clinical problem. The underlying pathology can be limited to the ear; however, it may also occur during the course of other autoimmune processes with multisymptomatic manifestations in different systems and organs. The underlying pathology is an immune-mediated inflammatory response to antigens, including self-antigens located in a patient’s body tissues. This pathology leads to various dysfunctions. Diagnosis is challenging, as there are no specific diagnostic criteria. Inflammatory markers are usually elevated; however, they are not specific enough to determine a final diagnosis. Treatment strategies differ, depending on the symptoms; however, immunosuppressive drugs, mainly corticosteroids (systemic or trans tympanic), appear to be a treatment of choice.