J Thromb Thrombolysis 21(3), 277, 2006. C 2006 Springer Science + Business Media, Inc. Manufactured in The Netherlands.
Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome ¨ Levent Sinan Bir1 , Fatma Ozdemir Es¸meli1 , and Fazıl Necdet Ardıc¸2 1 Pamukkale
University Faculty of Medicine, Departments of Neurology; 2 Audiology, Denizli-Turkey
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-
Address for correspondence: Levent Sinan Bir, Pamukkale ¨ ¨ ¨ u¨ Universitesi Tıp Fakultesi Hastanesi Kınıklı Kampus N¨oroloji Anabilim Dalı B105Kınıklı-20070 Denizli, Turkey. Fax: +90 (258) 2134922; Tel.: 90 (258) 2118585/2128; E-mail:
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festations that can be seen in APS are migraine-like headaches, dementia, chorea, depression, psychosis, seizure, transverse myelopaty, amaurosis fugax [2,4]. Recent reports attracted attention to the SSNHL in patients with SLE or a lupus-like syndrome who had elevated levels of aCA [1,5,6]. Positive antiphospholipid antibodies were detected in 49% of the patients with sudden hearing loss and 50% of the patients with progressive hearing loss in a study [7]. To the best of our knowledge, there is only one report related with audiovestibular symptoms in a primary APS patient [6]. In conclusion, we want to emphasize that SSSHL can be also seen in primary APS and we recommend to test antiphospolipid antibodies in all patients with sudden hearing loss even there is no history and/or sign of a connective tissue disease. Our patient supports direct association between aCA and SSNHL.
References 1. Green L, Miller EB. Sudden sensorineural hearing loss as a first manifestation of systemic lupus erythematosus: Association with anticardiolipin antibodies. Clin Rheumatol 2001;20:220–222. 2. Brey RL, Escalante A. Neurological manifestations of antiphospholipid antibody syndrome. Lupus 1998;7(Suppl. 2):67–74. 3. Hanly JG. Antiphospholid syndrome: An overview. CMAJ 2003;168:1675–1682. 4. Asherson RA, Cervera R. Unusual manifestation of the antiphospholipid syndrome. Clin Rev Allergy Immunol 2003;25:61–78. 5. Naarendorp M, Spiera H. Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupuslike syndromes and antiphospholipid antibodies. J Rheumatol 1998;25:589–592. 6. Vyse T, Luxon LM,Walport MJ. Audivestibular manifestations of the antiphospholipid syndrome. J Laryngol Otol 1994;108:57–59. 7. Heller U, Becker EW, Zenner HP, Berg PA. Incidence and clinical relevance of antibodies to phospholipids, serotonin and ganglioside in patients with sudden deafness and progressive inner ear hearing loss. HNO 1998;46:583–586.
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