Sensorineural Hearing Loss Suspected of Having an Autoimmune Etiology; a Report of Three Cases

Abstract

Case 1 : Bilateral sensorineural deafness progressed rapidly to nearly total deafness. CT revealed soft granulation in the tympanic and mastoid cavities. Several open biopsies failed to show any specific inflammation. Laboratory studies revealed a highly probable autoimmune origin : positive RA and ANA, elevated ESR, complement, and immunoglobulins. Her deafness responded to steroid therapy, and her hearing returned to a usable conversational level. Case 2 : This case represents so-called steroid-responsive sensorineural hearing loss. Her bilateral inner ear deafness responded several times to steroid therapy when aggravations of hearing loss occured. Her deafness also improved slowly and slightly with the administration of isosorbide.Case 3 : Bilaeral fluctuant sensorineural deafness appeared in this patient with systemic lupus erythematosus. The glycerol test was markedly positive bilateraly.All three patients maintain usable hearing for daily life, in addition the first and the second have had no steroid therapy for 3 months.The pathogenesis of autoimmune sensorineural deafness is not yet fully understood. Although vasculitis is the most common pathologic change in autoimmune diseases, the present cases suggest that endolymphatic hydrops may also play some role in sensorineural deafness of autoimmune origin.