Autoimmune Inner Ear Disease: Up-to-Date State of Knowledge

Background/Objectives: Hearing loss is a serious problem affecting individuals and societies and becoming a reason for a serious discomfort of living. It is also an increasing cost for healthcare systems worldwide. The reasons include noise exposure, elderly, ototoxic drugs, infections and others. An increasing number of cases of sensorineural hearing loss is caused by autoimmune inflammatory reactions within cochlea, that may be limited to inner ear or associated with other systemic autoimmune reactions. The objective of this paper was a review of the worldwide literature covering recent five years, to summarize the up-to-date status of knowledge on autoimmune hearing loss, both from the perspective of understanding its pathology and treatment strategies. Methods: We searched the databases Scopus, Web of Science and Pubmed using the key phrases ”autoimmune inner ear disease”, “autoimmune sensorineural hearing loss”, “sudden sensorineural hearing loss”. The selection covered the papers published within the recent 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 became a base for this review Conclusions: autoimmune hearing loss seems to be an increasing clinical problem. The underlying pathology can be limited to ear, however it may also occur in the course of other autoimmune processes with multisymptomatic manifestations in different systems and organs. The underlying pathology is a immune-mediated inflammatory response to antigens, including self-antigens located in own tissues of the body. This leads to various dysfunctions. Diagnosis is usually challenging as there are no specific diagnostic criteria. Inflammatory markers are usually elevated, however they are not specific enough to be decisive for the final diagnosis. Treatment strategies are different, depending on the symptoms, however immunosuppressive drugs, mainly corticosteroids (systemic or trans tympanic), appear to be a treatment of choice.