Refractory Bilateral Sudden Sensorineural Hearing Loss Associated with Systemic Lupus Erythematosus : Clinical Implication of Plasmapheresis

Sudden sensorineural hearing loss (SNHL) is a rare manifestation of systemic lupus erythematosus (SLE). A 38-year-old female presented headache followed by bilateral SNHL. The hearing threshold level was 75dB in the right and 45dB in the left ear. In spite of high dose steroid treatment, the hearing of both ears became profound hearing loss in several days. Serologic tests showed positive autoimmune markers corresponding to SLE with suspicious antiphospholipid (antibody) syndrome (APS). Follow up hearing tests showed no improvement in spite of anticoagulant, intravenous immunoglobulin and high dose steroid treatment. The patient underwent two cycles of plasmapheresis followed by immunosuppressant, and the hearing level in left side improved to 60dB after plasmapheresis. We presume that elevated blood viscosity and vasculitis due to immune deposits can cause cochlear vessel occlusion, which is responsible for the hearing loss. Prompt immunosuppressive treatment and, in refractory case, plasmapheresis can possibly reverse the hearing loss. (J Clinical Otolaryngol 2015;26:257-262)