Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome

Abstract

An autoimmune basis for sudden sensorineural hearing loss (SSNHL) has long been suspected. Immunoserological assays of patients with SSNHL have revealed the presence of different antibodies. Recently a number of case reports have highlighted the association of anticardiolipin antibodies (aCA) and SSNHL in systemic lupus erythematosus (SLE) [1]. Here we report a case with primary antiphospholipid syndrome (APS) who had SSNHL. A twenty eight years old male patient was applied to our hospital with the complaint of bilateral, sudden onset deafness. Past medical history revealed that he had Wernicke aphasia lasted one hour one year ago. Systemic and neurologic examinations were normal except bilateral sensorineural hearing loss. Complete blood count, PT, aPPT, sedimentation, biochemistry, protein C, S antithrombin III and complement levels were in normal limits. ECG and echocardiogram were normal. We confirmed bilateral sensorineural hearing loss with audiogram. Cranial and temporal bone MRI were normal. Anti HIV and VDRL were negative. Anticardiolipin antibody Ig G was positive (28 IU/mL). In repeated tests, ANA, anti-DNA and aCA IgM were found negative, but aCA Ig G was positive. Prednisone 80 mg/day was started and continued with decreasing doses for 3 months. He has completely recovered in one month. Then he was anticoagulated. No other ischemic attack, any systemic finding or audiovestibular complaint have been seen for two years. APS occurs in isolation (primary APS) or in association with connective tissue diseases (secondary APS), particularly with SLE [2]. The most common clinical manifestation of APS is thrombosis, which affects the vessels of any organ. Arterial thrombosis involves the brain in up to 50% of cases, causing transient ischemic attack or stroke [3]. Other neurological mani-