Hearing loss in autoimmune disorders: prevalence and therapeutic options

The objective of this study was to review our current knowledge relative to the correlation between sensorineural hearing loss (SNHL) and autoimmune diseases, focusing on the prevalence of hearing loss in different pathologies and possible therapeutic approaches. A review of the literature on hearing loss in different forms of autoimmune disease has been carried out, with emphasis on incidence and prevalence of SNHL. Therapeutic protocols have been assessed including both conservative medical and rehabilitative methods. Cochlear implant outcomes have been investigated. The prevalence of hearing loss in autoimmune and immune-mediated inner ear diseases, as referred by case reports or single-center statistics, is widely variable. More difficult is the evaluation of severe/profound SNHL, usually reported in relation to cochlear implantation. Though these patients represent ideal candidates for cochlear implantation, as they become deaf after years of hearing; the associated systemic disease, the specific damage on inner ear structures and the medication taken may influence the result of cochlear implantation. The main problem is the cochlear fibrosis or ossification that has been found to affect 50% of implanted ears in patients suffering from autoimmune and immune-mediated SNHL. Hence, in the presence of severe/profound SNHL earlier implantation may be indicated before post-inflammatory obliterative changes to the cochlea.