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Current Rheumatology Reviews


ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Review Article

Sudden Sensorineural Hearing Loss in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Clinical Review

Author(s): Julia L. Riera , María del R. Maliandi, Jorge L. Musuruana and Javier A. Cavallasca *

Volume 16, Issue 2, 2020

Page: [84 - 91] Pages: 8

DOI: 10.2174/1573397115666191016101741

Price: $65


Background: Sudden sensorineural hearing loss (SSNHL) is defined as a sudden loss of hearing, usually unilateral, of more than 30 dB in 3 contiguous frequencies of the tonal audiometry. SSNHL estimates an incidence ranging from 5 to 20 per 100.000 people per year. In approximately 75% of cases, a cause cannot be identified. However, it could be a clinical manifestation of Systemic lupus erythematosus (SLE) and Antiphospholipid Syndrome (APS).

Objective: This review will focus on the clinical presentation, diagnosis, and management of the SLE and APS associated SSNHL.

Methods: We searched in PubMed, Scopus, Lilacs, and Cochrane reviewing reports of Sudden sensorineural hearing loss in SLE and/or APS. Articles written in English and Spanish, and were available in full text, were included.

Results: In patients with SLE, bilateral involvement was frequent. Antiphospholipid antibodies were positive in the majority of the patients. Corticosteroids were the mainstay of the treatment. The auditory prognosis was poor with total hearing loss recovery reached in only 22% of patients.

On the other hand, most of the patients with SSNHL and APS were males and presented associated symptoms such as vertigo, tinnitus and/or headache, 75% had bilateral disease. Lupus anticoagulant and aCL were found in equal proportions, all patients were anticoagulated, and aspirin was associated in 25% of the cases. Complete resolution or improvement of symptoms was observed in 25% of the patients.

Conclusion: Sudden sensorineural hearing loss, can be a clinical feature of SLE and APS. Treating physicians should be aware of this devastating complication, especially when bilateral involvement occurs.

Keywords: Systemic lupus erythematosus, antiphospholipid syndrome, sudden sensorineural, hearing loss, corticosteroids, anticoagulation.

George DL, Pradhan S. Idiopathic sensorineural hearing disorders in adults--a pragmatic approach. Nat Rev Rheumatol 2009; 5(9): 505-12.
[] [PMID: 19652649]
Plaza G, Durio E, Herráiz C, Rivera T, García-Berrocal JR. Asociación Madrileña de ORL. Consensus on diagnosis and treatment of sudden hearing loss. Asociación Madrileña de ORL. Acta Otorrinolaringol Esp 2011; 62(2): 144-57.
[] [PMID: 21112580]
Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC. Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis. Trends Amplif 2011; 15(3): 91-105.
[] [PMID: 21606048]
Marcucci R, Alessandrello Liotta A, Cellai AP, et al. Cardiovascular and thrombophilic risk factors for idiopathic sudden sensorineural hearing loss. J Thromb Haemost 2005; 3(5): 929-34.
[] [PMID: 15869586]
Schreiber BE, Agrup C, Haskard DO, Luxon LM. Sudden sensorineural hearing loss. Lancet 2010; 375(9721): 1203-11.
[] [PMID: 20362815]
Sara SA, Teh BM, Friedland P. Bilateral sudden sensorineural hearing loss: review. J Laryngol Otol 2014; 128(51): S8-S15.
[] [PMID: 24331736]
Suckfüll M. Perspectives on the pathophysiology and treatment of sudden idiopathic sensorineural hearing loss. Dtsch Arztebl Int 2009; 106(41): 669-75.
[] [PMID: 19946432]
Li G, You D, Ma J, Li W, Li H, Sun S. The role of autoimmunity in the pathogenesis of sudden sensorineural hearing loss. Neural Plast 2018; 137691473
Di Stadio A, Ralli M. Systemic Lupus Erythematosus and hearing disorders: Literature review and meta-analysis of clinical and temporal bone findings. J Int Med Res 2017; 45(5): 1470-80.
[] [PMID: 28345423]
Chawki S, Aouizerate J, Trad S, Prinseau J, Hanslik T. Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus: Case report and brief review of other published cases. Medicine (Baltimore) 2016; 95(36)e4345
[] [PMID: 27603334]
Yehudai D, Shoenfeld Y, Toubi E. The autoimmune characteristics of progressive or sudden sensorineural hearing loss. Autoimmunity 2006; 39(2): 153-8.
[] [PMID: 16698672]
Compadretti GC, Brandolini C, Tasca I. Sudden sensorineural hearing loss in lupus erythematosus associated with antiphospholipid syndrome: case report and review. Ann Otol Rhinol Laryngol 2005; 114(3): 214-8.
[] [PMID: 15825571]
Rossini BAA, Penido NO, Munhoz MSL, Bogaz EA, Curi RS. Sudden sensorioneural hearing loss and autoimmune systemic diseases. Int Arch Otorhinolaryngol 2017; 21(3): 213-23.
[] [PMID: 28680488]
Lin C, Lin SW, Weng SF, Lin YS. Risk of sudden sensorineural hearing loss in patients with systemic lupus erythematosus: a population-based cohort study. Audiol Neurotol 2013; 18(2): 95-100.
[] [PMID: 23257626]
Hamblin TJ, Mufti GJ, Bracewell A. Severe deafness in systemic lupus erythematosus: its immediate relief by plasma exchange. Br Med J (Clin Res Ed) 1982; 284(6326): 1374.
[] [PMID: 6803983]
Caldarelli DD, Rejowski JE, Corey JP. Sensorineural hearing loss in lupus erythematosus. Am J Otol 1986; 7(3): 210-3.
[PMID: 3717314]
Kobayashi S, Fujishiro N, Sugiyama K. Systemic lupus erythematosus with sensorineural hearing loss and improvement after plasmapheresis using the double filtration method. Intern Med 1992; 31(6): 778-81.
[] [PMID: 1392181]
Hisashi K, Komune S, Taira T, Uemura T, Sadoshima S, Tsuda H. Anticardiolipin antibody-induced sudden profound sensorineural hearing loss. Am J Otolaryngol 1993; 14(4): 275-7.
[] [PMID: 8214323]
Vyse T, Luxon LM, Walport MJ. Audiovestibular manifestations of the antiphospholipid syndrome. J Laryngol Otol 1994; 108(1): 57-9.
[] [PMID: 8133171]
Kataoka H, Takeda T, Nakatani H, Saito H. Sensorineural hearing loss of suspected autoimmune etiology: a report of three cases. Auris Nasus Larynx 1995; 22(1): 53-8.
[] [PMID: 7677637]
Naarendorp M, Spiera H. Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupus-like syndromes and antiphospholipid antibodies. J Rheumatol 1998; 25(3): 589-92.
[PMID: 9517786]
Paira SO. Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupus-like syndrome and antiphospholipid antibodies. J Rheumatol 1998; 25(12): 2476-7.
[PMID: 9858454]
Schachern PA, Paparella MM, Morizono N. Study of systemic lupus erythematosus in temporal bones. Ann Otol Rhinol Laryngol 1999; 108: 338-44.
Peeva E, Barland P. Sensorineural hearing loss in conjunction with aortic insufficiency in systemic lupus erythematosus. Scand J Rheumatol 2001; 30(1): 45-7.
[] [PMID: 11252692]
Green L, Miller EB. Sudden sensorineural hearing loss as a first manifestation of systemic lupus erythematosus: association with anticardiolipin antibodies. Clin Rheumatol 2001; 20(3): 220-2.
[] [PMID: 11434477]
Mora R, Mora F, Passali FM, Cordone MP, Crippa B, Barbieri M. Restoration of immune-mediated sensorineural hearing loss with sodium enoxaparin: a case report. Acta Otolaryngol Suppl 2004; (552): 25-8.
[] [PMID: 15219043]
Fukushima N, Fukushima H, Cureoglu S, Schachern PA, Paparella MM. Hearing loss associated with systemic lupus erythematosus: temporal bone histopathology. Otol Neurotol 2006; 27(1): 127-8.
[] [PMID: 16371860]
Sugiura M, Naganawa S, Teranishi M, Sato E, Kojima S, Nakashima T. Inner ear hemorrhage in systemic lupus erythematosus. Laryngoscope 2006; 116(5): 826-8.
[] [PMID: 16652096]
Khalidi NA, Rebello R, Robertson DD. Sensorineural hearing loss in systemic lupus erythematosus: case report and literature review. J Laryngol Otol 2008; 122(12): 1371-6.
[] [PMID: 18282337]
Ricarte IF, Dutra LA, Abrantes FF, et al. Neurologic manifestations of antiphospholipid syndrome. Lupus 2018; 27(9): 1404-14.
[] [PMID: 29768970]
Yelnik CM, Kozora E, Appenzeller S. Non-stroke central neurologic manifestations in antiphospholipid syndrome. Curr Rheumatol Rep 2016; 18(2): 11.
[] [PMID: 26923254]
Toubi E, Ben-David J, Kessel A, Podoshin L, Golan TD. Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies. Lupus 1997; 6(6): 540-2.
[] [PMID: 9256313]
Toubi E, Ben-David J, Kessel A, Halas K, Sabo E, Luntz M. Immune-mediated disorders associated with idiopathic sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol 2004; 113(6): 445-9.
[] [PMID: 15224826]
Mouadeb DA, Ruckenstein MJ. Antiphospholipid inner ear syndrome. Laryngoscope 2005; 115(5): 879-83.
[] [PMID: 15867658]
Cho CH, Jung BS, Jung JH, Lee JH, Lee JH. Expression of autoantibodies in patients with sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol 2013; 122(2): 131-4.
[] [PMID: 23534128]
Bachor E, Kremmer S, Kreuzfelder E, Jahnke K, Seidahmadi S. Antiphospholipid antibodies in patients with sensorineural hearing loss. Eur Arch Otorhinolaryngol 2005; 262(8): 622-6.
[] [PMID: 15731904]
Gross M, Eliashar R, Ben-Yaakov A, Ulmansky R, Elidan J. Prevalence and clinical significance of anticardiolipin, anti-beta2-glycoprotein-1, and anti-heat shock protein-70 autoantibodies in sudden sensorineural hearing loss. Audiol Neurotol 2008; 13(4): 231-8.
[] [PMID: 18259075]
Süslü N, Yilmaz T, Gürsel B. Utility of anti-HSP 70, TNF-alpha, ESR, antinuclear antibody, and antiphospholipid antibodies in the diagnosis and treatment of sudden sensorineural hearing loss. Laryngoscope 2009; 119(2): 341-6.
[] [PMID: 19160386]
Passamonti SM, Di Berardino F, Bucciarelli P, et al. Risk factors for idiopathic sudden sensorineural hearing loss and their association with clinical outcome. Thromb Res 2015; 135(3): 508-12.
[] [PMID: 25619439]
Sène D, Piette JC, Cacoub P. Antiphospholipid antibodies, antiphospholipid syndrome and infections. Autoimmun Rev 2008; 7(4): 272-7.
[] [PMID: 18295729]
Bir LS, Eşmeli FO, Ardiç FN. Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Thromb Thrombolysis 2006; 21(3): 277.
[] [PMID: 16683221]
Wiles NM, Hunt BJ, Callanan V, Chevretton EB. Sudden sensorineural hearing loss and antiphospholipid syndrome. Haematologica 2006; 91(12)(Suppl.).ECR46
[PMID: 17194652]
Cavallasca JA, Nasswetter GG, Gutierrez DM, Bercellini EA, Brodsky AL. Sudden sensorineural hearing loss as a manifestation of primary antiphospholipid syndrome. Joint Bone Spine 2007; 74(4): 403-4.
[] [PMID: 17587629]
Kang KT, Young YH. Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol 2008; 122(2): 204-6.
[] [PMID: 17419896]
Yin T, Huang F, Ren J, et al. Bilateral sudden hearing loss following habitual abortion: a case report and review of literature. Int J Clin Exp Med 2013; 6(8): 720-3.
[PMID: 24040484]
Wang JG, Xie QB, Yang NP, Yin G. Primary antiphospholipid antibody syndrome: a case with bilateral sudden sensorineural hearing loss. Rheumatol Int 2009; 29(4): 467-8.
[] [PMID: 18810450]
Galicia-López A, Anda-Garay JC, García de la Peña M. Bilateral sudden sensorineural hearing loss in a patient with microangiopathic antiphospholipid syndrome. Reumatol Clin 2016; 12(3): 175-7.
[] [PMID: 26184552]

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