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CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2017; 21(03): 213-223
DOI: 10.1055/s-0036-1586162
Original Research
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Sudden Sensorioneural Hearing Loss and Autoimmune Systemic Diseases

Bruno Almeida Antunes Rossini
1   Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
,
Norma de Oliveira Penido
1   Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
,
Mario Sergio Lei Munhoz
1   Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
,
Eduardo Amaro Bogaz
1   Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
,
Renata Souza Curi
1   Department of ENT, Universidade Federal de São Paulo, São Paulo, Brazil
› Author Affiliations
Further Information

Publication History

15 June 2015

03 June 2016

Publication Date:
26 July 2016 (online)

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Abstract

Introduction Several authors have demonstrated the relationship between sudden sensorineural hearing loss (SNHL) and systemic autoimmune diseases (SAD). Immune-mediated SNHL can rarely present as unilateral sudden SNHL and manifests itself in the contralateral ear only after years. It presents clinical relevance for being one of the few SNHL that may be reversible given that early and appropriate treatment is applied.

Objective The objective of this study is to describe the clinical presentations and audiological findings from patients with idiopathic sudden SNHL and SAD associated with a probable diagnosis of immune-mediated SNHL. Furthermore, we strive to estimate the prevalence of SAD in patients with sudden SNHL.

Methods This is an observational retrospective cohort. We have selected and studied patients with SAD. Revision of available literature on scientific repositories.

Results We evaluated 339 patients with sudden SNHL. Among them, 13 (3.83%) patients suffered from SAD. Three patients had bilateral involvement, a total of 16 ears. We evaluate and describe various clinical, epidemiological, and audiological aspects of this sample.

Conclusion In our sample of patients with sudden SNHL, the prevalence of SAD was found relevant. The majority had tinnitus and dizziness concomitant hearing loss, unilateral involvement and had experienced profound hearing loss at the time of the installation. In spite of instituted treatment, most cases showed no improvement in audiometric thresholds. Apparently, patients with sudden SNHL and SAD have a more severe initial impairment, higher percentage of bilateral, lower response to treatment, and worse prognosis than patients with sudden SNHL of unknown etiology.

Keywords

hearing loss - sensorineural - hearing loss - sudden - autoimmune diseases - humans
 

  • References

  • 1 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 (06) 445-449
    CrossrefPubMedGoogle Scholar
  • 2 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 (03) 214-218
    CrossrefPubMedGoogle Scholar
  • 3 Kang KT, Young YH. Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol 2008; 122 (02) 204-206
    PubMedGoogle Scholar
  • 4 Deroee AF, Huang TC, Morita N, Hojjati M. Sudden hearing loss as the presenting symptom of systemic sclerosis. Otol Neurotol 2009; 30 (03) 277-279
    CrossrefPubMedGoogle Scholar
  • 5 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 Neurootol 2013; 18 (02) 95-100
    CrossrefPubMedGoogle Scholar
  • 6 Wilson WR, Byl FM, Laird N. The efficacy of steroids in the treatment of idiopathic sudden hearing loss. A double-blind clinical study. Arch Otolaryngol 1980; 106 (12) 772-776
    CrossrefPubMedGoogle Scholar
  • 7 Byl Jr FM. Sudden hearing loss: eight years' experience and suggested prognostic table. Laryngoscope 1984; 94 (5 Pt 1): 647-661
    PubMedGoogle Scholar
  • 8 Simmons FB. Theory of membrane breaks in sudden hearing loss. Arch Otolaryngol 1968; 88 (01) 41-48
    CrossrefPubMedGoogle Scholar
  • 9 Schuknecht HF, Kimura RS, Naufal PM. The pathology of sudden deafness. Acta Otolaryngol 1973; 76 (02) 75-97
    CrossrefPubMedGoogle Scholar
  • 10 Wilson WR, Veltri RW, Laird N, Sprinkle PM. Viral and epidemiologic studies of idiopathic sudden hearing loss. Otolaryngol Head Neck Surg 1983; 91 (06) 653-658
    CrossrefPubMedGoogle Scholar
  • 11 Berrocal JR, Ramírez-Camacho R. Sudden sensorineural hearing loss: supporting the immunologic theory. Ann Otol Rhinol Laryngol 2002; 111 (11) 989-997
    CrossrefPubMedGoogle Scholar
  • 12 Chau JK, Lin JR, Atashband S, Irvine RA, Westerberg BD. Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. Laryngoscope 2010; 120 (05) 1011-1021
    PubMedGoogle Scholar
  • 13 Penido NO, Aumond MD, Leonhardt FD, Abreu CEC, Toledo RN. Disacusia neurossensorial imunomediada. Rev Bras Otorrinolaringol (Engl Ed) 2002; 68: 730-734
    CrossrefGoogle Scholar
  • 14 Lehnhardt E. [Sudden hearing disorders occurring simultaneously or successively on both sides]. Z Laryngol Rhinol Otol 1958; 37 (01) 1-16
    PubMedGoogle Scholar
  • 15 Schiff M, Brown M. Hormones and sudden deafness. Laryngoscope 1974; 84 (11) 1959-1981
    CrossrefPubMedGoogle Scholar
  • 16 McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 1979; 88 (5 Pt 1): 585-589
    CrossrefPubMedGoogle Scholar
  • 17 Alvarenga EHL, Costa SS, Cruz OLM. Disacusia Neurossensorial Imunomediada. In: Cruz OLM, Costa SS. eds. Otologia Clínica e Cirúrgica. Rio de Janeiro, Brazil: Revinter; 2000: 307-13
    Google Scholar
  • 18 Bovo R, Ciorba A, Martini A. The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. Eur Arch Otorhinolaryngol 2009; 266 (01) 37-40
    CrossrefPubMedGoogle Scholar
  • 19 Hughes GB, Barna BP, Kinney SE, Calabrese LH, Nalepa NJ. Clinical diagnosis of immune inner-ear disease. Laryngoscope 1988; 98 (03) 251-253
    PubMedGoogle Scholar
  • 20 Agrup C, Luxon LM. Immune-mediated inner-ear disorders in neuro-otology. Curr Opin Neurol 2006; 19 (01) 26-32
    CrossrefPubMedGoogle Scholar
  • 21 Mazlumzadeh M, Lowe VJ, Mullan BP, Fabry DA, McDonald TJ, Matteson EL. The utility of positron emission tomography in the evaluation of autoimmune hearing loss. Otol Neurotol 2003; 24 (02) 201-204
    CrossrefPubMedGoogle Scholar
  • 22 Teszler CB, Williams MT, Belange G, Ayache D. Labyrinthitis related to Wegener granulomatosis: magnetic resonance imaging findings. Otol Neurotol 2008; 29 (05) 721-722
    PubMedGoogle Scholar
  • 23 Ianuale C, Cadoni G, De Feo E. , et al. A systematic review and meta-analysis of the diagnostic accuracy of anti-heat shock protein 70 antibodies for the detection of autoimmune hearing loss. Otol Neurotol 2013; 34 (02) 214-219
    CrossrefPubMedGoogle Scholar
  • 24 Penido NO, Cruz OL, Zanoni A, Inoue DP. Classification and hearing evolution of patients with sudden sensorineural hearing loss. Braz J Med Biol Res 2009; 42 (08) 712-716
    CrossrefPubMedGoogle Scholar
  • 25 Cooper GS, Stroehla BC. The epidemiology of autoimmune diseases. Autoimmun Rev 2003; 2 (03) 119-125
    CrossrefPubMedGoogle Scholar
  • 26 Fetterman BL, Luxford WM, Saunders JE. Sudden bilateral sensorineural hearing loss. Laryngoscope 1996; 106 (11) 1347-1350
    CrossrefPubMedGoogle Scholar
  • 27 Cecatto SB, Garcia RID, Costa KS, Anti SMA, Longone E, Rapoport PB. Perda auditiva sensorioneural no lúpus eritematoso sistêmico: relato de três casos. Rev Bras Otorrinolaringol (Engl Ed) 2004; 70: 398-403
    CrossrefGoogle Scholar
  • 28 Green L, Miller EB. Sudden sensorineural hearing loss as a first manifestation of systemic lupus erythematosus: association with anticardiolipin antibodies. Clin Rheumatol 2001; 20 (03) 220-222
    CrossrefPubMedGoogle Scholar
  • 29 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 (04) 275-277
    CrossrefPubMedGoogle Scholar
  • 30 Takatsu M, Higaki M, Kinoshita H, Mizushima Y, Koizuka I. Ear involvement in patients with rheumatoid arthritis. Otol Neurotol 2005; 26 (04) 755-761
    CrossrefPubMedGoogle Scholar
  • 31 Kastanioudakis I, Ziavra N, Politi EN, Exarchakos G, Drosos AA, Skevas A. Hearing loss in progressive systemic sclerosis patients: a comparative study. Otolaryngol Head Neck Surg 2001; 124 (05) 522-525
    CrossrefPubMedGoogle Scholar
  • 32 Montoya-Aranda IM, Peñaloza-López YR, Gutiérrez-Tinajero DJ. [Sjögren's syndrome: Audiological and clinical behaviour in terms of age]. Acta Otorrinolaringol Esp 2010; 61 (05) 332-337
    CrossrefPubMedGoogle Scholar
  • 33 Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006; 81 (04) 483-488
    CrossrefPubMedGoogle Scholar
  • 34 Orsoni JG, Zavota L, Pellistri I, Piazza F, Cimino L. Cogan syndrome. Cornea 2002; 21 (04) 356-359
    CrossrefPubMedGoogle Scholar
  • 35 Cote DN, Molony TB, Waxman J, Parsa D. Cogan's syndrome manifesting as sudden bilateral deafness: diagnosis and management. South Med J 1993; 86 (09) 1056-1060
    CrossrefPubMedGoogle Scholar
  • 36 Kleffner I, Duning T, Lohmann H. , et al. A brief review of Susac syndrome. J Neurol Sci 2012; 322 (1–2): 35-40
    CrossrefPubMedGoogle Scholar
  • 37 Gross M, Eliashar R. Update on Susac's syndrome. Curr Opin Neurol 2005; 18 (03) 311-314
    CrossrefPubMedGoogle Scholar
  • 38 Bitra RK, Eggenberger E. Review of Susac syndrome. Curr Opin Ophthalmol 2011; 22 (06) 472-476
    CrossrefPubMedGoogle Scholar
  • 39 Ozturk A, Degirmenci Y, Tunc M, Kececi H. Susac's syndrome: a case of simultaneous development of all three components of the triad. J Neurol Sci 2013; 324 (1–2): 187-189
    CrossrefPubMedGoogle Scholar
  • 40 Srikumar S, Deepak MK, Basu S, Kumar BN. Sensorineural hearing loss associated with psoriatic arthritis. J Laryngol Otol 2004; 118 (11) 909-911
    PubMedGoogle Scholar
  • 41 Akay BN, Bozkir M, Anadolu Y, Gullu S. Epidemiology of vitiligo, associated autoimmune diseases and audiological abnormalities: Ankara study of 80 patients in Turkey. J Eur Acad Dermatol Venereol 2010; 24 (10) 1144-1150
    CrossrefPubMedGoogle Scholar
  • 42 Kahveci OK, Demirdal US, Duran A, Altuntas A, Kavuncu V, Okur E. Hearing and cochlear function of patients with ankylosing spondylitis. Clin Rheumatol 2012; 31 (07) 1103-1108
    CrossrefPubMedGoogle Scholar
  • 43 Aimoni C, Bianchini C, Borin M. , et al. Diabetes, cardiovascular risk factors and idiopathic sudden sensorineural hearing loss: a case-control study. Audiol Neurootol 2010; 15 (02) 111-115
    CrossrefPubMedGoogle Scholar
  • 44 Penido NdeO, Ramos HVL, Barros FA, Cruz OLM, Toledo RN. Clinical and etiological factors and evolution of hearing in sudden deafness. Braz J Otorhinolaryngol 2005; 71 (05) 633-638
    CrossrefPubMedGoogle Scholar
  • 45 Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum 2004; 34 (02) 544-548
    CrossrefPubMedGoogle Scholar
  • 46 Ruckenstein MJ. Autoimmune inner ear disease. Curr Opin Otolaryngol Head Neck Surg 2004; 12 (05) 426-430
    CrossrefPubMedGoogle Scholar
  • 47 Zavod MB, Sataloff RT, Rao VM. Frequency of cochlear enhancement on magnetic resonance imaging in patients with autoimmune sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2000; 126 (08) 969-971
    CrossrefPubMedGoogle Scholar
  • 48 Stachler RJ, Chandrasekhar SS, Archer SM. , et al; American Academy of Otolaryngology-Head and Neck Surgery. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg 2012; 146 (3, Suppl) S1-S35
    CrossrefPubMedGoogle Scholar
  • 49 Buniel MC, Geelan-Hansen K, Weber PC, Tuohy VK. Immunosuppressive therapy for autoimmune inner ear disease. Immunotherapy 2009; 1 (03) 425-434
    CrossrefPubMedGoogle Scholar
  • 50 García-Berrocal JR, Ibáñez A, Rodríguez A. , et al. Alternatives to systemic steroid therapy for refractory immune-mediated inner ear disease: A physiopathologic approach. Eur Arch Otorhinolaryngol 2006; 263 (11) 977-982
    CrossrefPubMedGoogle Scholar
  • 51 Liu YC, Rubin R, Sataloff RT. Treatment-refractory autoimmune sensorineural hearing loss: response to infliximab. Ear Nose Throat J 2011; 90 (01) 23-28
    PubMedGoogle Scholar
  • 52 Chang NC, Ho KY, Kuo WR. Audiometric patterns and prognosis in sudden sensorineural hearing loss in southern Taiwan. Otolaryngol Head Neck Surg 2005; 133 (06) 916-922
    CrossrefPubMedGoogle Scholar
  • 53 Zeitoun H, Beckman JG, Arts HA. , et al. Corticosteroid response and supporting cell antibody in autoimmune hearing loss. Arch Otolaryngol Head Neck Surg 2005; 131 (08) 665-672
    CrossrefPubMedGoogle Scholar