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Autoimmune vertigo: an update on vestibular disorders associated with autoimmune mechanisms

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Abstract

The role of the immune system in mediating cochleovestibular pathologies has received increasing attention in recent years. Autoimmune vertigo may be an invalidating condition and may worsen the quality of life of affected patients, especially in the cases of delayed diagnosis. Since the etiopathogenesis is still not clear, also the treatment is not yet completely delineated. According to the clinical presentation, autoimmune vertigo can present as an isolated disorder or in association with systemic autoimmune diseases. The main feature in autoimmune vertigo is the presence of an abnormal immune response, in either absence or presence of systemic autoimmune disease, directed against delicate components of the inner ear. This may determine a functional or anatomical alteration, with an inflammatory reaction often devastating for hearing and balance. Being the exact pathogenesis unknown, the diagnosis of autoimmune vertigo is based either on clinical criteria or on a positive response to steroids. The earlier the diagnosis is made, the sooner the therapy can be installed, giving a chance to the recovery of inner ear damages. Corticosteroids represent the most effective and universally accepted treatment, even if other immunomodulatory drugs are now having a more extensive use.

Highlights

  • Vertigo is relatively frequent in autoimmune diseases; however, it is often misdiagnosed or attributed to central nervous system alterations rather to specific inner ear involvement.

  • Vertigo and other audiovestibular symptoms may be the first manifestation of an autoimmune disease and if correctly addressed could significantly contribute to early diagnosis of the underlying autoimmune disease.

  • Early diagnosis of immune-related vertigo can lead to prompt initiation of targeted therapy with elevate chances of preventing irreversible damages to the inner ear.

  • The presence of alternating phases of well-being and disabling symptoms in patients with vertigo should always been considered, as they could suggest an underlying autoimmune condition.

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References

  1. Ruckenstein MJ. Autoimmune inner ear disease. Curr Opin Otolaryngol Head Neck Surg. 2004;12:426–30.

    Article  PubMed  Google Scholar 

  2. Hughes G, Kinney S, Barna B, Calabrese L, Hamid M. Autoimmune reactivity in Menière’s disease: preliminary report. Laryngoscope. 1983;43:410–7.

    Article  Google Scholar 

  3. McCabe B. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol. 1979;88:585–9.

    Article  CAS  PubMed  Google Scholar 

  4. Tomiyama S, Harris JP. The endolymphatic sac: its importance in inner ear immune responses. Laryngoscope. 1986;96(6):685–91.

    Article  CAS  PubMed  Google Scholar 

  5. Vambutas A, Pathak S. AAO: autoimmune and autoinflammatory (disease) in otology: what is new in immune-mediated hearing loss. Laryngoscope Investig Otolaryngol. 2016;1:110–5.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Russo FY, De Seta D, Lahlou G, Borel S, Nguyen Y, Bouccara D, et al. Fluctuating hearing loss in the only hearing ear: Cochlear implantation in the contralateral deaf side. Otolaryngol Head Neck Surg. 2018;1:194599818763137.

    Google Scholar 

  7. Ding CR, Xu XD, Wang XW, Jia XH, Cheng X, Liu X, et al. Effect of endolymphatic hydrops on sound transmission in live Guinea pigs measured with a laser Doppler vibrometer. Neural Plast. 2016;2016:8648297.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Mancini P, Atturo F, Di Mario A, Portanova G, Ralli M, De Virgilio A, et al. Hearing loss in autoimmune disorders: prevalence and therapeutic options. Autoimmun Rev. 2018;17(7):644–52.

    Article  PubMed  Google Scholar 

  9. Wolfson RJ, Leiberman A. Unilateral deafness with subsequent vertigo. Laryngoscope. 1975;85:1762–6.

    Article  CAS  PubMed  Google Scholar 

  10. Nadol JB Jr, Weiss AD, Parker SW. Vertigo of delayed onset after sudden deafness. Ann Otol Rhinol Laryngol. 1975;84:841–6.

    Article  PubMed  Google Scholar 

  11. Schuknecht HF, Suzuka Y, Zimmermann C. Delayed endolymphatic hydrops and its relationship to Meniére's disease. Ann Otol Rhinol Laryngol. 1990;99:843–53.

    Article  CAS  PubMed  Google Scholar 

  12. Harris JP, Aframian D. Role of autoimmunity in contralateral delayed endolymphatic hydrops. Am J Otolaryngol. 1994;15:710–6.

    CAS  Google Scholar 

  13. Morgan AE, Ismail EI, Ashraf B. Intratympanic injections of dexamethasone in delayed endolymphatic hydrops: a prospective clinical study. ORL J Otorhinolaryngol Relat Spec. 2018;80:19–27.

    Article  CAS  PubMed  Google Scholar 

  14. Committee on Hearing and Equilibrium: guidelines for the diagnosis and evaluation of therapy inMeniere's disease. Amer Acad Otolaryngol Head Neck Surg Foundation Inc. Otolaryngol Head Neck Surg. 1995;113:176–8.

  15. Paparella MM. Pathogenesis of Meniere’s disease and Meniere’s syndrome. Acta Otolaryngol Suppl. 1984;406:10–25.

    CAS  PubMed  Google Scholar 

  16. Merchant SN, Adams JC, Nadol JB. Pathophysiology of Meniere’s syndrome: are symptoms caused by endolymphatic hydrops. Otol Neurotol. 2005;26:74–81.

    Article  PubMed  Google Scholar 

  17. Schuknecht HF. Pathology of the ear. 2nd ed. Philadelphia/Baltimore: Lea & Febiger; 1993.

    Google Scholar 

  18. Wackym PA, Schuknecht HF, Ward PH, Linthicum FH, Kerner MM, Aframian D, et al. Blinded control study of endolymphatic duct and sac fibrosis in Meniere’s disease. In: Filipo R, Barbara M, editors. Meniere’s disease: perspectives in the 90s. Amsterdam/New York: Kugler Publ; 1994. p. 209–15.

    Google Scholar 

  19. Dornhoffer JL, Arenberg IK. Immune mechanisms in Meniere’s syndrome. Otolaryngol Clin N Am. 1997;30:1017–26.

    CAS  Google Scholar 

  20. Greco A, Gallo A, Fusconi M, Marinelli C, Macri GF, de Vincentiis M. Meniere’s disease might be an autoimmune condition? Autoimmun Rev. 2012;11:731–8.

    Article  CAS  PubMed  Google Scholar 

  21. Gazquez I, Soto-Varela A, Aran I, Santos S, Batuecas A, Trinidad G, et al. High prevalence of systemic autoimmune diseases in patients with Meniere’s disease. PLoS One. 2011;6:e26759.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Wackym PA, Friberg U, Linthicum FH Jr, Bagger-Sjoback D, Bui HT, Hofman F, et al. Human endolymphatic sac: morphologic evidence of immunologic function. Ann Otol Rhinol Laryngol. 1987;96:276–81.

    Article  CAS  PubMed  Google Scholar 

  23. Yoo TJ, Yazawa Y, Tomoda K, Floyd R. Type II collagen-induced autoimmune endolymphatic hydrops in Guinea pig. Science. 1983;113:65–7.

    Article  Google Scholar 

  24. Fattori B, Nacci A, Dardano A, Dallan I, Grosso M, Traino C, et al. Possible association between thyroid autoimmunity and Meniere’s disease. Clin Exp Immunol. 2008;152:28–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Hornibrook J, George P, Spellerberg M, Gourley J. HSP70 antibodies in 80 patients with “clinically certain” Meniere’s disease. Ann Otol Rhinol Laryngol. 2011;120:651–5.

    Article  PubMed  Google Scholar 

  26. Pham BN, Rudic M, Bouccara D, Sterkers O, Belmatoug N, Bébéar JP, et al. Antibodies to myelin protein zero (P0) protein as markers of auto-immune inner ear diseases. Autoimmunity. 2007;40:202–7.

    Article  CAS  PubMed  Google Scholar 

  27. Shin SO, Billings PB, Keithley EM, Harris JP. Comparison of anti-head shock protein 70 (anti-hsp70) and anti-68-kDa inner ear protein in the sera of patients with Meniere’s disease. Laryngoscope. 1997;107:222–7.

    Article  CAS  PubMed  Google Scholar 

  28. Yoo TJ. Etiopathogenesis of Ménière’s disease: a hypothesis. Ann Otol Rhinol Laryngol. 1984;113:6–12.

    Article  CAS  Google Scholar 

  29. Suchan M, Kaliarik L, Krempaska S, Koval J. Immune-mediated cochleovestibular disease. Bratisl Lek Listy. 2016;117:87–90.

    CAS  PubMed  Google Scholar 

  30. Riente L, Bongiorni F, Nacci A, Migliorini P, Segnini G, Delle Sedie A, et al. Antibodies to inner ear antigens in Meniere’s disease. Clin Exp Immunol. 2004;135:159–63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Brookes G. Circulating immune complexes in Ménière’s disease. Arch Otolaryngol Head Neck Surg. 1986;112:536–40.

    Article  CAS  PubMed  Google Scholar 

  32. Kim SH, Kim JY, Lee HJ, Gi M, Kim BG, Choi JY. Autoimmunity as a candidate for the etiopathogenesis of Meniere’s disease: detection of autoimmune reactions and diagnostic biomarker candidate. PLoS One. 2014;9:e111039.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Wei NR, Helms J, Giebel W. Immunohistochemical findings in the vestibular ganglion from a patient with Meniere’s disease. Eur Arch Otorhinolaryngol. 1990;247:340–4.

    Article  CAS  PubMed  Google Scholar 

  34. Yoo TJ, Stuart JM, Kang AH, Townes AS, Tomoda K, Dixit S. Type II collagen autoimmunity in otosclerosis and Ménière’s disease. Science. 1982;217:1153–5.

    Article  CAS  PubMed  Google Scholar 

  35. Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, et al. Meniere’s disease. Nat Rev Dis Primers. 2016;2:16028.

    Article  PubMed  Google Scholar 

  36. Zingler VC, Cnyrim C, Jahn K, Weintz E, Fernbacher J, Frenzel C, et al. Causative factors and epidemiology of bilateral vestibulopathy in 255 patients. Ann Neurol. 2007;61:524–32.

    Article  PubMed  Google Scholar 

  37. Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, et al. Bilateral vestibulopathy: diagnostic criteria consensus document of the Classification Committee of the Bárány Society. J Vestib Res. 2017;27:177–89.

    Article  PubMed  Google Scholar 

  38. Greco A, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Turchetta R, et al. Idiopathic bilateral vestibulopathy: an autoimmune disease? Autoimmun Rev. 2014;13:1042–7.

    Article  PubMed  Google Scholar 

  39. Kattah JC. Clinical characteristics and etiology of bilateral vestibular loss in a cohort from Central Illinois. Front Neurol. 2018;9:46.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Arbusow V, Strupp M, Dieterich M, Stocker W, Naumann A, Schulz P, et al. Serum antibodies against membraneous labyrinth in patients with “idiopathic” bilateral vestibulopathy. J Neurol. 1998;245:132–6.

    Article  CAS  PubMed  Google Scholar 

  41. Agrup C, Keir G, Thompson EJ, Bronstein AM. Systemic autoantibodies against discrete inner ear compartments in bilateral vestibular loss. Neurology. 2005;65:167.

    Article  PubMed  Google Scholar 

  42. 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;45:1470–80.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Cogan D. Syndrome of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol. 1945;33:144–9.

    Article  Google Scholar 

  44. Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine. 1980;59:426–41.

    Article  CAS  PubMed  Google Scholar 

  45. Cheson BD, Bluming AZ, Alroy J. Cogan’s syndrome: a systemic vasculitis. Am J Med. 1976;60:549–55.

    Article  CAS  PubMed  Google Scholar 

  46. Greco A, Gallo A, Fusconi M, Magliulo G, Turchetta R, Marinelli C, et al. Cogan’s syndrome: an autoimmune inner ear disease. Autoimmun Rev. 2013;12:396–400.

    Article  CAS  PubMed  Google Scholar 

  47. Brinkman CJ, Broekhuyse RM. Cell-mediated immunity after retinal detachment as determined by lymphocyte stimulation. Am J Ophthalmol. 1978;86:260–5.

    Article  CAS  PubMed  Google Scholar 

  48. Schuknecht HF. Ear pathology in autoimmune diseases. Adv Otorhinolaryngol. 1991;46:50–70.

    CAS  PubMed  Google Scholar 

  49. Hughes GB, Kinney SE, Barna BP, Tomsak RL, Calabrese LH. Autoimmune reactivity in Cogan’s syndrome: a preliminary report. Arch Otolaryngol Head Neck Surg. 1983;91:24–32.

    Article  CAS  Google Scholar 

  50. Peeters GJ, Cremers CW, Pinckers AJ, Hoefnagels WHL. Atypical Cogan’s syndrome: an autoimmune disease? Ann Otol Rhinol Laryngol. 1986;95:173–5.

    Article  CAS  PubMed  Google Scholar 

  51. Lunardi C, Bason C, Leandri M, Navone R, Lestani M, Millo E, et al. Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet. 2002;360:915–21.

    Article  CAS  PubMed  Google Scholar 

  52. Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, et al. Lambiase Behçet's disease: new insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018: S1568–9972(18)30079-X;17:567–75.

    Article  CAS  PubMed  Google Scholar 

  53. Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P. Behçet’s disease physiopathology: a contemporary review. Auto Immun Highlights. 2016;7:4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Gemignani G, Berrettini S, Bruschini P, Sellari-Franceschini S, Fusari P, Piragine F, et al. Hearing and vestibular disturbances in Behçet’s syndrome. Ann Otol Rhinol Laryngol. 1991;100:459–63.

    Article  CAS  PubMed  Google Scholar 

  55. Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999;122:2171–82.

    Article  PubMed  Google Scholar 

  56. Pollak L, Luxon LM, Haskard DO. Labyrinthine involvement in Behçet’s syndrome. J Laryngol Otol. 2001;115:522–9.

    Article  CAS  PubMed  Google Scholar 

  57. Kulahli I, Balci K, Koseoglu E, Yuce I, Cagli S, Senturk M. Audio-vestibular disturbances in Behçet’s patients: report of 62 cases. Hear Res. 2005;203:28–31.

    Article  PubMed  Google Scholar 

  58. Kocer N, Islak C, Siva A, Saip S, Akman C, Kantarci O, et al. CNS involvement in neuro-Behçet syndrome: an MR study. Am J Neuroradiol. 1999;20:1015–24.

    CAS  PubMed  PubMed Central  Google Scholar 

  59. Pay S, Simsek I, Erdem H, Dinc A. Immunopathogenesis of Behçet’s disease with special emphasize to the possible role of antigen presenting cells. Rheumatol Int. 2007;27:417–24.

    Article  CAS  PubMed  Google Scholar 

  60. Kulaber A, Tugal-Tutkun I, Sibel P, Akman-Demir G, Kaneko F, Gul A, et al. Proinflammatory cellular immune response in Behçet’s disease. Rheumatol Int. 2007;27:1113–8.

    Article  CAS  PubMed  Google Scholar 

  61. Vitale A, Rigante D, Lopalco G, Selmi C, Galeazzi M, Iannone F, et al. Interleukin-1 inhibition in Behçet’s disease. Isr Med Assoc J. 2016;18(3-4):171–6. Review

    PubMed  Google Scholar 

  62. Shi T, Lv W, Zhang L, et al. Association of HLA-DR4/HLA-DRB1∗04 with Vogt-Koyanagi-Harada disease: a systematic review and meta-analysis. Sci Rep. 2014;4:6887.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Ng JY, Luk FO, Lai TY, et al. Influence of molecular genetics in Vogt-Koyanagi-Harada disease. J Ophthalmic Inflamm Infect. 2014;4:20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  64. Perry HD, Font RL. Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 1977;83:242–54.

    Article  CAS  PubMed  Google Scholar 

  65. Yang P, Ren Y, Li B, Fang W, Meng Q, Kijlstra A. Clinical characteristics of Vogt-Koyanagi-Harada syndrome in Chinese patients. Ophthalmology. 2007;114:606–14.

    Article  PubMed  Google Scholar 

  66. Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol. 1995;39:265–92.

    Article  CAS  PubMed  Google Scholar 

  67. Greco A, Fusconi M, Gallo A, Turchetta R, Marinelli C, Macri GF, et al. Vogt-Koyanagi-Harada syndrome. Autoimmun Rev. 2013;12:1033–8.

    Article  CAS  PubMed  Google Scholar 

  68. Liu B, Deng T, Zhu L, Zhong J. Association of human leukocyte antigen (HLA)-DQ and HLA-DQA1/DQB1 alleles with Vogt-Koyanagi-Harada disease: a systematic review and meta-analysis. Medicine (Baltimore). 2018;97:e9914.

    Article  CAS  Google Scholar 

  69. Du L, Yang P, Hou S, et al. Association of the CTLA-4 gene with Vogt-Koyanagi-Harada syndrome. Clin Immunol. 2008;127:43–8.

    Article  CAS  PubMed  Google Scholar 

  70. Lacomba MS, Martin CM, Chamond RR, Galera JM, Omar M, Estevez EC. Aqueous and serum interferon gamma, interleukin (IL) 2, IL-4, and IL-10 in patients with uveitis. Arch Ophthalmol. 2000;118:768–72.

    Article  CAS  PubMed  Google Scholar 

  71. Sheereen A, Gaafar A, Iqneibi A, Eldali A, Tabbara KF, Adra C, et al. A study of KIR genes and HLA-C in Vogt-Koyanagi-Harada disease in Saudi Arabia. Mol Vis. 2011;17:3523–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  72. Levinson RD, Okada AA, Ashouri E, Keino H, Rajalingam R. Killer cell immunoglobulin-like receptor gene-cluster 3DS1-2DL5-2DS1-2DS5 predisposes susceptibility to Vogt-Koyanagi-Harada syndrome in Japanese individuals. Hum Immunol. 2010;71:192–4.

    Article  CAS  PubMed  Google Scholar 

  73. Mittal KK. Immunobiology of the human major histocompatibility complex: association of HLA antigens with disease. Acta Anthropogenet. 1984;8:245–68.

    CAS  PubMed  Google Scholar 

  74. Peng Z, Jiang S, Wu M, Zhou X, Wang Q. Expression and role of interleukin-9 in Vogt-Koyanagi-Harada disease. Mol Vis. 2017;23:538–47. eCollection 2017

    CAS  PubMed  PubMed Central  Google Scholar 

  75. Pelosi S, Chandrasekhar SS. Intratympanic steroid use for hearing salvage in Vogt-Koyanagi-Harada syndrome. Ear Nose Throat J. 2011;90:574–7.

    Article  PubMed  Google Scholar 

  76. Berrih-Aknin S. Myasthenia gravis: paradox versus paradigm in autoimmunity. J Autoimmun. 2014;52:1–28.

    Article  CAS  PubMed  Google Scholar 

  77. 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;18:95–100.

    Article  PubMed  Google Scholar 

  78. Lam NC, Ghetu MV, Bieniek ML. Systemic lupus erythematosus: primary care approach to diagnosis and management. Am Fam Physician. 2016;94:284–94.

    PubMed  Google Scholar 

  79. McCarty DJ, Manzi S, Medsger TA Jr, et al. Incidence of systemic lupus erythematosus. Race and gender differences. Arthritis Rheum. 1995;38:1260–70.

    Article  CAS  PubMed  Google Scholar 

  80. Karatas E, Onat AM, Durucu C, Baglam T, Kanlikama M, Altunoren O, et al. Audiovestibular disturbance in patients with systemic lupus erythematosus. Otolaryngol Head Neck Surg. 2007;136:82–6.

    Article  PubMed  Google Scholar 

  81. Batuecas-Caletrío A, del Pino-Montes J, Cordero-Civantos C, Calle-Cabanillas MI, Lopez-Escamez JA. Hearing and vestibular disorders in patients with systemic lupus erythematosus. Lupus. 2013;22:437–42.

    Article  PubMed  Google Scholar 

  82. Weckerle CE, Franek BS, Kelly JA, Kumabe M, Mikolaitis RA, Green SL, et al. Network analysis of associations between serum interferon-α activity, autoantibodies, and clinical features in systemic lupus erythematosus. Arthritis Rheum. 2011;63:1044–53.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Riggs JM, Hanna RN, Rajan B, Zerrouki K, Karnell JL, Sagar D, et al. Characterisation of anifrolumab, a fully human anti-interferon receptor antagonist antibody for the treatment of systemic lupus erythematosus. Lupus Sci Med. 2018;5:e000261.

    Article  PubMed  PubMed Central  Google Scholar 

  84. Wu M, Yang J, Li X, et al. The role of γδ T cells in systemic lupus erythematosus. J Immunol Res. 2016;2016:2932531.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Fukushima N, Fukushima H, Cureoglu S, Schachern PA, Paparella MM. Hearing loss associated with systemic lupus erythematosus: temporal bone histo-pathology. Otol Neurotol. 2006;27:127–8.

    Article  PubMed  Google Scholar 

  86. Kariya S, Hızlı O, Kaya S, et al. Histopathologic findings in peripheral vestibular system from patients with systemic lupus erythematosus: a human temporal bone study. Otol Neurotol. 2015;36:1702–7.

    Article  PubMed  Google Scholar 

  87. McCabe BF. Autoimmune inner ear disease: results of therapy. Adv Otorhinolaryngol. 1991;46:78–81.

    CAS  PubMed  Google Scholar 

  88. Dasgupta S. Therapeutic interventions of tissue specific autoimmune onset in systemic lupus erythematosus. Mini-Rev Med Chem. 2016;16:1.

    Article  Google Scholar 

  89. Petri M. Systemic lupus erythematosus and related diseases: clinical features. In: Rose NR, Mackay IR, editors. The autoimmune dis-eases. 4th ed. St Louis: Elsevier Academic Press; 2006. p. 351–6.

    Chapter  Google Scholar 

  90. Relle M, Weinmann-Menke J, Scorletti E, Cavagna L, Schwarting A. Genetics and novel aspects of therapies in systemic lupus erythematosus. Autoimmun Rev. 2015;14:1005–18.

    Article  CAS  PubMed  Google Scholar 

  91. Greco A, De Virgilio A, Gallo A, Fusconi M, Turchetta R, Tombolini M, et al. Susac’s syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014;13:814–21.

    Article  CAS  PubMed  Google Scholar 

  92. Gross M, Banin E, Eliashar R, Ben-Hur T. Susac syndrome. Otol Neurotol. 2004;25:470–3.

    Article  PubMed  Google Scholar 

  93. Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1944;44:591–3.

    Article  Google Scholar 

  94. Martinet N, Fardeau C, Adam R, Bodaghi B, Papo T, Piette JC, et al. Fluorescein and indocyanine green angiographies in Susac syndrome. Retina. 2007;27:1238–42.

    Article  PubMed  Google Scholar 

  95. Bucciarelli S, Cervera R, Martinez M, Latorre X, Font J. Susac’s syndrome or catastrophic antiphospholipid syndrome. Lupus. 2004;3:607–8.

    Article  Google Scholar 

  96. Petty GW, Engel AG, Younge BR, Duffy J, Janagihara T, Lucchinetti CF, et al. Retinocochleocerebral vasculopathy. Medicine (Baltimore). 1998;77:12–40.

    Article  CAS  Google Scholar 

  97. Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B, et al. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp Ophthalmol. 2000;28:373–81.

    Article  CAS  PubMed  Google Scholar 

  98. Magro CM, Poe JC, Lubow M, Susac JO. Susac syndrome: an organ-specific autoim-mune endotheliopathy syndrome associated with anti-endothelial cell antibodies. Am J Clin Pathol. 2011;136:903–12.

    Article  CAS  PubMed  Google Scholar 

  99. Jarius S, Neumayer B, Wandinger KP, Hartmann M, Wildemann B. Anti-endothelial serum antibodies in a patient with Susac’s syndrome. J Neurol Sci. 2009;285:259–61.

    Article  CAS  PubMed  Google Scholar 

  100. Magro CM, Ross P, Marsh CB, Marsh JN, Allen JN, Liff D, et al. The role of anti-endothelial cell antibody-mediated microvascular injury in the evolution of pulmonary fibrosis in the setting of collagen vascular disease. Am J Clin Pathol. 2007;127:237–47.

    Article  CAS  PubMed  Google Scholar 

  101. Servettaz A, Guilpain A, Camoin L, Mayeux P, Broussard C, Tamby MC, et al. Identification of target antigens of antiendothelial cell antibodies in healthy individuals: a proteomic approach. Proteomics. 2008;8:1000–8.

    Article  CAS  PubMed  Google Scholar 

  102. Ayache D, Plouin-Gaudon I, Bakouche P, Elbaz P, Gout O. Microangiopathy of the inner ear, retina, and brain (Susac syndrome): report of a case. Arch Otolaryngol Head Neck Surg. 2000;126:82–4.

    Article  CAS  PubMed  Google Scholar 

  103. Petty GW, Matteson EL, Younge BR, McDonald TJ, Wood CP. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc. 2001;76:958–60.

    Article  CAS  PubMed  Google Scholar 

  104. García-Carrasco M, Jiménez-Hernández C, Jiménez-Hernández M, Voorduin-Ramos S, Mendoza-Pinto C, Ramos-Alvarez G, et al. Susac’s syndrome: an update. Autoimmun Rev. 2011;10:548–52.

    Article  PubMed  Google Scholar 

  105. García-Carrasco M, Mendoza-Pinto C, Cervera R. Diagnosis and classification of Susac syndrome. Autoimmun Rev. 2014;13:347–50.

    Article  PubMed  Google Scholar 

  106. Fox RJ, Costello F, Judkins AR, Galetta SL, Maguire ML, Leonard B, et al. Treatment of Susac syndrome with gamma globulin and corticosteroids. J Neurol Sci. 2006;251:17–22.

    Article  CAS  PubMed  Google Scholar 

  107. Milo R, Miller A. Revised diagnostic criteria of multiple sclerosis. Autoimmun Rev. 2014;13:518–24.

    Article  PubMed  Google Scholar 

  108. Burina A, Sinanović O, Smajlović D, Vidović M, Brkić F. Some aspects of balance disorder in patients with multiple sclerosis. Bosn J Basic Med Sci. 2008;8:80–5.

    Article  PubMed  PubMed Central  Google Scholar 

  109. Pula JH, Newman-Toker DE, Kattah JC. Multiple sclerosis as a cause of the acute vestibular syndrome. J Neurol. 2013;260:1649–54.

    Article  CAS  PubMed  Google Scholar 

  110. Tarnutzer AA, Berkowitz AL, Robinson KA, Hsieh YH, Newman-Toker DE. Does my dizzy patient have a stroke? A systematic review of bedside diagnosis in acute vestibular syndrome. CMAJ. 2011;183:E571–92.

    Article  PubMed  PubMed Central  Google Scholar 

  111. Commins DJ, Chen JM. Multiple sclerosis: a consideration in acute cranial nerve palsies. Am J Otol. 1997;18:590–5.

    CAS  PubMed  Google Scholar 

  112. Frohman EM, Zhang H, Dewey RB, Hawker KS, Racke MK, Frohman TC. Vertigo in MS: utility of positional and particle repositioning maneuvers. Neurology. 2000;55:1566–9.

    Article  CAS  PubMed  Google Scholar 

  113. Anagnostou E, Mandellos D, Limbitaki G, Papadimitriou A, Anastasopoulos D. Positional nystagmus and vertigo due to a solitary brachium conjunctivum plaque. J Neurol Neurosurg Psychiatry. 2006;77:790–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  114. Anagnostou E, Varaki K, Anastasopoulos D. A minute demyelinating lesion causing acute positional vertigo. J Neurol Sci. 2008;266:187–9.

    Article  CAS  PubMed  Google Scholar 

  115. Degirmenci E, Bir LS, Ardic FN. Clinical and electronystagmographical evaluation of vestibular symptoms in relapsing remitting multiple sclerosis. Neurol Res. 2010;32:986–91.

    Article  PubMed  Google Scholar 

  116. Hellmann MA, Steiner I, Mosberg-Galili R. Sudden sensorineural hearing loss in multiple sclerosis: clinical course and possible pathogenesis. Acta Neurol Scand. 2011;124:245–59.

    Article  CAS  PubMed  Google Scholar 

  117. Di Stadio A, Dipietro L, Ralli M, Meneghello F, Minni A, Greco A, et al. Sudden hearing loss as an early detector of multiple sclerosis: a systematic review. Eur Rev Med Pharmacol Sci. 2018;22(14):4611–24.

    PubMed  Google Scholar 

  118. Di Stadio A, Ralli M. Inner ear involvement in multiple sclerosis: an underestimated condition? Mult Scler. 2018;1:1352458518750010.

    Google Scholar 

  119. Merkel B, Butzkueven H, Traboulsee AL, Havrdova E, Kalincik T. Timing of high-efficacy therapy in relapsing-remitting multiple sclerosis: a systematic review. Autoimmun Rev. 2017;16:658–65.

    Article  PubMed  Google Scholar 

  120. McLeod DS, Cooper DS. The incidence and prevalence of thyroid autoimmunity. Endocrine. 2012;42:252–65.

    Article  CAS  PubMed  Google Scholar 

  121. Weetman AP. Determinants of autoimmune thyroid disease. Nat Immunol. 2001;2:76970. https://doi.org/10.1038/ni0901-769.

    Article  CAS  Google Scholar 

  122. De Leo S, Pearce EN. Autoimmune thyroid disease during pregnancy. Lancet Diabetes Endocrinol. 2017;12:S2213–8587(17)30402-3.

    Google Scholar 

  123. Yoo TJ, Yazawa Y. Immunology of cochlear and vestibular disorders. In: Luxon L, Furman JM, Martini A, Stephens D, editors. Audiological medicine: clinical aspect of hearing and balance. London: Martin Dunitz-Taylor and Francis Group; 2003. p. 61–87.

    Google Scholar 

  124. Tamura T. Studies on the correlation between thyroid function and Meniere’s syndrome. Nippon Jibiinkoka Gakkai Kaiho. 1964;67:1434–68.

    Article  CAS  PubMed  Google Scholar 

  125. Brenner M, Hoistad DL, Hain TC. Prevalence of thyroid dysfunction in patients with Meniere’s disease. Arch Otolaryngol Head Neck Surg. 2004;130:226–8.

    Article  PubMed  Google Scholar 

  126. Fattori B, Nacci A, Dardano A, Dallan I, Grosso M, Traino C, et al. Possible association between thyroid autoimmunity and Ménière’s disease. Clin Exp Immunol. 2008;152:28–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  127. Chiarella G, Tognini S, Nacci A, Sieli R, Costante G, Petrolo C, et al. Vestibular disorders in euthyroid patients with Hashimoto’s thyroiditis: role of thyroid autoimmunity. Clin Endocrinol. 2014;81:600–5.

    Article  CAS  Google Scholar 

  128. Modugno GC, Pirodda A, Ferri GG, Montana R, Rasciti L, Ceroni AR. A relationship between autoimmune thyroiditis and benign paroxysmal positional vertigo? Med Hypotheses. 2000;54:614–5.

    Article  CAS  PubMed  Google Scholar 

  129. Papi G, Corsello SM, Milite MT, Zanni M, Ciardullo AV, Donato CDI, et al. Association between benign paroxysmal positional vertigo and auto-immune chronic thyroiditis. Clin Endocrinol. 2009;70:169–70.

    Article  Google Scholar 

  130. Sari K, Yildirim T, Borekci H, Akin I, Aydin R, Ozkiris M. The relationship between benign paroxysmal positional vertigo and thyroid autoimmunity. Acta Otolaryngol. 2015;135:754–7.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00100, Rome, Italy

    Francesca Yoshie Russo, Massimo Ralli, Patrizia Mancini, Alessandro Lambiase, Marco Artico & Antonio Greco

  2. Department of Oral and Maxillo-facial Surgery, Sapienza University of Rome, Viale del Policlinico 155, 00100, Rome, Italy

    Daniele De Seta & Marco de Vincentiis

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  1. Francesca Yoshie Russo
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  2. Massimo Ralli
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  3. Daniele De Seta
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Correspondence to Daniele De Seta.

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Russo, F.Y., Ralli, M., De Seta, D. et al. Autoimmune vertigo: an update on vestibular disorders associated with autoimmune mechanisms. Immunol Res 66, 675–685 (2018). https://doi.org/10.1007/s12026-018-9023-6

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