Skip to main content

Advertisement

SpringerLink
Log in

Secondary autoimmune immune ear disease (AIED): a systematic review and meta-analysis on vestibular manifestations of systemic autoimmune and inflammatory disorders

  • REVIEW ARTICLE
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Secondary autoimmune inner ear disease (AIED) is often bilateral and asymmetric in patients presenting with audiovestibular symptoms due to a systemic autoimmune disease. This systematic review and meta-analysis are aimed at identifying and highlighting patterns in prevalence of vestibular dysfunction, symptom presentation, and diagnostic methods in extant literature by combining clinical context from case reports with quantitative analyses from cohort studies. Screening of articles by title, abstract, and full text was completed by four reviewers (K.Z., A.L., S.C., and S.J.). In this study, we grouped secondary AIED and systemic autoimmune diseases by pathophysiologic mechanism: (1) connective tissue disease (CTD), (2) vasculitides (VAS), (3) systemic inflammatory disorders (SID), and (4) other immune-mediated disorders (OIMD). The search for AIED disease identified 120 articles (cohorts and case reports) that met the final inclusion criteria. All 120 were included in the qualitative review, and 54 articles were included for meta-analysis. Of these 54 articles, 22 included a control group (CwC). Ninety individual cases or patient presentations from 66 articles were included for analysis in addition to the 54 cohort articles. Secondary AIED does not have a diagnostic algorithm for managing vestibular symptoms. The management of audiovestibular symptoms requires close collaboration between otolaryngologists and rheumatologists to preserve end-organ function of the ear. To improve our ability to understand the impact on the vestibular system, vestibular clinicians need to develop a standardized reporting method. Clinical presentation should frequently be paired with vestibular testing to contextually investigate symptom severity and provide higher quality care.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Data availability

Data will be made available per individual request.

References

  1. Moscicki RA (1994) Immune-mediated inner ear disorders. Baillieres Clin Neurol 3(3):547–563

    CAS  PubMed  Google Scholar 

  2. Harris JP, Gopen Q, Keithley E (2009) Autoimmune inner ear disease and other autoimmune diseases with inner ear involvement. James B Snow Jr and P Ashley Wackym, editors. Ballengers Otolaryngol Head Neck Surg 17:305–12

    Google Scholar 

  3. Berrettini S, Ravecca F, Bruschini L, Ursino F, Sellari-Franceschini S (1998) Progressive sensorineural hearing loss: immunologic etiology. Acta Otorhinolaryngol Ital 18(4 Suppl 59):33–41

    CAS  PubMed  Google Scholar 

  4. Vambutas A, Pathak S (2016) AAO: Autoimmune and autoinflammatory (disease) in otology: what is new in immune-mediated hearing loss. Laryngoscope Investig Otolaryngol 1(5):110–115

    PubMed  PubMed Central  Google Scholar 

  5. Ciorba A, Corazzi V, Bianchini C, Aimoni C, Pelucchi S, Skarzynski PH et al (2018) Autoimmune inner ear disease (AIED): a diagnostic challenge. Int J Immunopathol Pharmacol 32:2058738418808680

    PubMed  PubMed Central  Google Scholar 

  6. McCabe BF (1979) Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 113(7):526–30

  7. Welling DB (1996) Clinical evaluation and treatment of immune-mediated inner ear disease. Ear Nose Throat J 75(5):301–305

    CAS  PubMed  Google Scholar 

  8. Ruckenstein MJ (2004) Autoimmune inner ear disease. Curr Opin Otolaryngol Head Neck Surg 12(5):426–430

    PubMed  Google Scholar 

  9. Bovo R, Aimoni C, Martini A (2006) Immune-mediated inner ear disease. Acta Otolaryngol 126(10):1012–1021

    CAS  PubMed  Google Scholar 

  10. Agrawal Y, Ward BK, Minor LB (2013) Vestibular dysfunction: prevalence, impact and need for targeted treatment. J Vestib Res 23(3):113–117

    PubMed  PubMed Central  Google Scholar 

  11. Ralli M, D’Aguanno V, Di Stadio A, De Virgilio A, Croce A, Longo L et al (2018) Audiovestibular symptoms in systemic autoimmune diseases. J Immunol Res 2018:5798103

    PubMed  PubMed Central  Google Scholar 

  12. SafaviNaini A, Ghorbani J, MontazerLotfeElahi S, Beigomi M (2017) Otologic manifestations and progression in patients with Wegener’s granulomatosis: a survey in 55 Patients. Iran J Otorhinolaryngol 29(95):327–31

    Google Scholar 

  13. Adler YD, Jovanovic S, Jivanjee A, Krause L, Zouboulis CC (2002) Adamantiades-Behçet’s disease with inner ear involvement. Clin Exp Rheumatol 20(4 Suppl 26):S40–S42

    CAS  PubMed  Google Scholar 

  14. Agari D, Koide R, Kashiyama T, Yoshida H, Naito R, Bandoh M (2007) Neurosarcoidosis: a treatable cause of vestibular dysfunction. Lancet 369(9564):878

    PubMed  Google Scholar 

  15. Akdoğan Ö, Exilus S, Ward BK, McArthur JC, Della Santina CC, Carey JP (2021) Sudden sensorineural hearing and vestibular loss in a case of relapsing polychondritis. Ann Otol Rhinol Laryngol 130(12):1412–1416

    PubMed  Google Scholar 

  16. Albrite LCJP, Resnick DM (1961) Cogan's syndrome: case presentations. Arch Otolaryngol 74(5):501–6

  17. Almorza Hidalgo T, García González AJ, Castañeda S, Tomero EG, Pablos Álvarez JL (2021) Cogan syndrome: descriptive analysis and clinical experience of 7 cases diagnosed and treated in two third level hospitals. Reumatol Clin (Engl Ed) 17(6):318–321

    PubMed  Google Scholar 

  18. Amor-Dorado JC, Llorca J, Garcia-Porrua C, Costa C, Perez-Fernandez N, Gonzalez-Gay MA (2003) Audiovestibular manifestations in giant cell arteritis: a prospective study. Medicine 82(1):13–26

    PubMed  Google Scholar 

  19. Aydin T, Taspinar O, Guneser M, Keskin Y (2016) Association of Vogt Koyanagi Harada syndrome and seronegative rheumatoid arthritis. Ethiop J Health Sci 26(2):193–196

    PubMed  PubMed Central  Google Scholar 

  20. Bassyouni IH, Emad Y, Rafaat HA, Dabbous AO (2011) Relationship between nailfold capillary abnormalities and vestibular dysfunction in systemic sclerosis. Joint Bone Spine 78(3):266–269

    PubMed  Google Scholar 

  21. Batuecas-Caletrío A, del Pino-Montes J, Cordero-Civantos C, Calle-Cabanillas MI, Lopez-Escamez JA (2013) Hearing and vestibular disorders in patients with systemic lupus erythematosus. Lupus 22(5):437–442

    PubMed  Google Scholar 

  22. Bayir O, Çomoğlu S, Ozdek A, Aytaç E, Güven H, Ozdal P et al (2012) Vestibular evoked myogenic potential responses in Behçet’s disease. J Int Adv Otol 8:113–117

    Google Scholar 

  23. Benitez JT, Arsenault MD, Licht JM, Cohen SD, Greenberg RV (1990) Evidence of central vestibulo-auditory dysfunction in atypical Cogan’s syndrome: a case report. Am J Otol 11(2):131–134

    CAS  PubMed  Google Scholar 

  24. Benitez JT, Bojrab DI, Lubbers DE, Arsenault MD (1999) Investigation of endolymphatic hydrops by electrocochleography in patients with Cogan’s syndrome. Ear Nose Throat J 78(12):929–933

    CAS  PubMed  Google Scholar 

  25. Bennett RW, Staker LV (1987) Wegener’s granulomatosis presenting as vertigo. West J Med 146(3):359–361

    CAS  PubMed  PubMed Central  Google Scholar 

  26. Berrettini S, Ferri C, Pitaro N, Bruschini P, Latorraca A, Sellari-Franceschini S et al (1994) Audiovestibular involvement in systemic sclerosis. ORL J Otorhinolaryngol Relat Spec 56(4):195–198

    CAS  PubMed  Google Scholar 

  27. Bomholt A, Knudsen JB, Permin H, Tommerup B, Gormsen J (1982) Profound sensorineural hearing loss in polyarteritis nodosa. An atypical case of Cogan's syndrome. Arch Otorhinolaryngol 236(1):53–8

  28. Bowman CA, Linthicum FH Jr, Nelson RA, Mikami K, Quismorio F (1986) Sensorineural hearing loss associated with systemic lupus erythematosus. Otolaryngol Head Neck Surg 94(2):197–204

    CAS  PubMed  Google Scholar 

  29. Bulman CH, Kane RJ, Srivastva VN (1985) The ENT manifestations of systemic vasculitis. J Laryngol Otol 99(9):941–945

    CAS  PubMed  Google Scholar 

  30. Caldarelli DD, Rejowski JE, Corey JP (1986) Sensorineural hearing loss in lupus erythematosus. Am J Otol 7(3):210–213

    CAS  PubMed  Google Scholar 

  31. Casella G, Corbetta D, Zolezzi M, Di Bella C, Villanacci V, Salemme M et al (2015) Symptomatic sensorineural hearing loss in patients with ulcerative colitis. Tech Coloproctol 19(12):729–731

    CAS  PubMed  Google Scholar 

  32. Casoli P, Tumiati B (1995) Cogan’s syndrome: a new possible complication of antiphospholipid antibodies? Clin Rheumatol 14(2):197–198

    CAS  PubMed  Google Scholar 

  33. Casselman JW, Majoor MH, Albers FW (1994) MR of the inner ear in patients with Cogan syndrome. AJNR Am J Neuroradiol 15(1):131–138

    CAS  PubMed  PubMed Central  Google Scholar 

  34. Chiarella G, Tognini S, Nacci A, Sieli R, Costante G, Petrolo C et al (2014) Vestibular disorders in euthyroid patients with Hashimoto’s thyroiditis: role of thyroid autoimmunity. Clin Endocrinol (Oxf) 81(4):600–605

    CAS  PubMed  Google Scholar 

  35. Cinamon U, Kronenberg J, Hildesheimer M, Taitelbaum R (1997) Cochlear implantation in patients suffering from Cogan’s syndrome. J Laryngol Otol 111(10):928–930

    CAS  PubMed  Google Scholar 

  36. Cody DT, Sones DA (1971) Relapsing polychondritis: audiovestibular manifestations. Laryngoscope 81(8):1208–1222

    CAS  PubMed  Google Scholar 

  37. Cody DTR, Williams HL (1960) Cogan’s syndrome. Laryngoscope 70(4):447–478

    CAS  PubMed  Google Scholar 

  38. Das S, Bakshi SS, Seepana R (2019) Demystifying autoimmune inner ear disease. Eur Arch Otorhinolaryngol 276(12):3267–3274

    PubMed  Google Scholar 

  39. Dion J, Costedoat-Chalumeau N, Sène D, Cohen-Bittan J, Leroux G, Dion C et al (2016) Relapsing polychondritis can be characterized by three different clinical phenotypes: analysis of a recent series of 142 patients. Arthritis Rheumatol 68(12):2992–3001

    PubMed  Google Scholar 

  40. Engberg J, Jepsen O (1962) Hearing impairment and dizziness in sarcoidosis: report of four cases. Dan Med Bull 9:28–32

    CAS  PubMed  Google Scholar 

  41. Erbek S, Erbek SS, Yilmaz S, Yucel E, Ozluoglu LN (2008) Vestibular evoked myogenic potentials in Behcet’s disease. Eur Arch Otorhinolaryngol 265(11):1315–1320

    PubMed  Google Scholar 

  42. Ertugrul O, Mutlu A, Zindanci I, Cam OH, Ozluoglu L (2019) Audiological and vestibular measurements in Behçet’s disease. Eur Arch Otorhinolaryngol 276(6):1625–1632

    PubMed  Google Scholar 

  43. Evereklioglu C, Cokkeser Y, Doganay S, Er H, Kizilay A (2001) Audio-vestibular evaluation in patients with Behçet’s syndrome. J Laryngol Otol 115(9):704–708

    CAS  PubMed  Google Scholar 

  44. Aiello FV, U (1973) Atypical Cogan’s syndrome. Acta Med Romana 11:67–70

  45. Feld J, Shupak A, Zisman D (2015) Diffuse systemic sclerosis presenting as Meniere’s disease-like symptoms as part of autoimmune inner ear disease. Isr Med Assoc J 17(4):263–264

    PubMed  Google Scholar 

  46. Fidler H, Jones NS (1989) Late onset Cogan’s syndrome. J Laryngol Otol 103(5):512–514

    CAS  PubMed  Google Scholar 

  47. Fujimoto S, Kim CH, Green T, Xu H (2019) Otologic manifestations of immunoglobulin G4-related disease. Ear Nose Throat J 98(10):630–631

    PubMed  Google Scholar 

  48. Fujiwara K, Morita S, Fukuda A, Yanagi H, Hoshino K, Nakamaru Y et al (2021) Usefulness of the video head impulse test for the evaluation of vestibular function in patients with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis. Otol Neurotol 42(4):e483–e488

    PubMed  Google Scholar 

  49. Galarza-Delgado DA, Villegas Gonzalez MJ, Riega Torres J, Soto-Galindo GA, Mendoza Flores L, Treviño González JL (2018) Early hearing loss detection in rheumatoid arthritis and primary Sjögren syndrome using extended high frequency audiometry. Clin Rheumatol 37(2):367–373

    PubMed  Google Scholar 

  50. Gaudreau P, Moy J, Lindsay F (2012) An unusual cause of vertigo, tinnitus, and hyperacusis: Vogt-Koyanagi-Harada syndrome. Ear Nose Throat J 91(12):E7-9

    PubMed  Google Scholar 

  51. Gelfand ML, Kantor T, Gorstein F (1972) Cogan’s syndrome with cardiovascular involvement: aortic insufficiency. Bull N Y Acad Med 48(4):647–660

    CAS  PubMed  PubMed Central  Google Scholar 

  52. Gemignani G, Berrettini S, Bruschini P, Sellari-Franceschini S, Fusari P, Piragine F et al (1991) Hearing and vestibular disturbances in Behçet’s syndrome. Ann Otol Rhinol Laryngol 100(6):459–463

    CAS  PubMed  Google Scholar 

  53. Glišić B, Stevic-Carevic S, Ristić G, Dedović J (2018) Cogan’s syndrome – a case series. Vojnosanitetski Pregled 75:1128–33

    Google Scholar 

  54. Gopen Q, Keithley EM, Harris JP (2006) Mechanisms underlying autoimmune inner ear disease. Drug Discov Today Dis Mech 3(1):137–142

    Google Scholar 

  55. Heydari N, Hajiabolhassani F, Fatahi J, Movaseghi S, Jalaie S (2015) Vestibular evoked myogenic potentials in patients with rheumatoid arthritis. Med J Islam Repub Iran 29:216

    PubMed  PubMed Central  Google Scholar 

  56. Hirvonen TP, Aalto H (2013) Recovery of bilateral vestibular loss in Cogan’s syndrome–a case report. Otol Neurotol 34(9):1736–1738

    PubMed  Google Scholar 

  57. Hooper R, Holden H (1970) Acoustic and vestibular problems in sarcoidosis. Arch Otolaryngol 92(4):386–391

    CAS  PubMed  Google Scholar 

  58. Igarashi Y, Watanabe Y, Aso S (1994) A case of Behçet’s disease with otologic symptoms. ORL J Otorhinolaryngol Relat Spec 56(5):295–298

    CAS  PubMed  Google Scholar 

  59. Ishii T, Watanabe I, Suzuki J (1995) Temporal bone findings in Cogan’s syndrome. Acta Otolaryngol Suppl 519:118–123

    CAS  PubMed  Google Scholar 

  60. Issing WJ, Selover D, Schulz P (1999) Anti-labyrinthine antibodies in a patient with relapsing polychondritis. Eur Arch Otorhinolaryngol 256(4):163–166

    CAS  PubMed  Google Scholar 

  61. Wysocki J, Jurczak P (1997) A case of relapsing polychondritis with E.N.T. symptoms. Med Sci Monit 3(2):225–8

    Google Scholar 

  62. Jenkins HA, Pollak AM, Fisch U (1981) Polyarteritis nodosa as a cause of sudden deafness A human temporal bone study. Am J Otolaryngol 2(2):99–107

    CAS  PubMed  Google Scholar 

  63. Kang KT, Young YH (2008) Sudden sensorineural hearing loss in a patient with primary antiphospholipid syndrome. J Laryngol Otol 122(2):204–206

    PubMed  Google Scholar 

  64. Karatas E, Onat AM, Durucu C, Baglam T, Kanlikama M, Altunoren O et al (2007) Audiovestibular disturbance in patients with systemic lupus erythematosus. Otolaryngol Head Neck Surg 136(1):82–86

    PubMed  Google Scholar 

  65. Kato M, Katayama N, Naganawa S, Nakashima T (2014) Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings in a patient with relapsing polychondritis. J Laryngol Otol 128(2):192–194

    CAS  PubMed  Google Scholar 

  66. Kawamura S, Sakamoto T, Kashio A, Kakigi A, Ito K, Suzuki M et al (2010) Cochlear implantation in a patient with atypical Cogan’s syndrome complicated with hypertrophic cranial pachymeningitis. Auris Nasus Larynx 37(6):737–741

    PubMed  Google Scholar 

  67. Klement V, Hahn A, Hojdarova A, Sejna I (2007) Cogan’s syndrome: a case report. Acta Oto-Laryngol 127(10):1115–7

    Google Scholar 

  68. Kondo Y, Ito S, Ohi Y, Satou H, Hiraoka T, Tsuboi H et al (2009) Atypical Cogan’s syndrome with aortitis. Intern Med 48(12):1093–1097

    PubMed  Google Scholar 

  69. Kühn D, Hospowsky C, Both M, Hey M, Laudien M (2018) Manifestation of granulomatosis with polyangiitis in head and neck. Clin Exp Rheumatol 36 Suppl 111(2):78–84

  70. Lee SU, Kim JS, Hyon JY, Ha YJ, Kim HJ, Song JJ et al (2019) Pearls & Oy-sters: Cogan syndrome: a potentially grave disorder of audiovestibulopathy with many faces. Neurology 93(1):39–41

    PubMed  Google Scholar 

  71. Li H, Zhang M, Wang M, Zhang S, Ma S, Wang X (2021) Clinical feature and prognosis of sudden sensorineural hearing loss with rheumatoid arthritis. Otol Neurotol 42(3):e267–e271

    PubMed  Google Scholar 

  72. Maciaszczyk K, Waszczykowska E, Pajor A, Bartkowiak-Dziankowska B, Durko T (2011) Hearing organ disorders in patients with systemic sclerosis. Rheumatol Int 31(11):1423–1428

    PubMed  Google Scholar 

  73. Maciaszczyk K, Durko T, Waszczykowska E, Erkiert-Polguj A, Pajor A (2011) Auditory function in patients with systemic lupus erythematosus. Auris Nasus Larynx 38(1):26–32

    PubMed  Google Scholar 

  74. Maciążek-Chyra B, Szmyrka M, Skoczyńska M, Sokolik R, Lasocka J, Wiland P (2019) Relapsing polychondritis - analysis of symptoms and criteria. Reumatologia 57(1):8–18

    PubMed  PubMed Central  Google Scholar 

  75. McKennan KX, Nielsen SL, Watson C, Wiesner K (1993) Menière’s syndrome: an atypical presentation of giant cell arteritis (temporal arteritis). Laryngoscope 103(10):1103–1107

    CAS  PubMed  Google Scholar 

  76. Minet M, Deggouj N, Gersdorff M (1997) Cochlear implantation in patients with Cogan’s syndrome: a review of four cases. Eur Arch Otorhinolaryngol 254(9–10):459–462

    CAS  PubMed  Google Scholar 

  77. Munakomi S, Lui F. Caloric Reflex Test (2023) In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557481/

  78. Murata J, Horii A, Tamura M, Mitani K, Mizuki M, Kubo T (2006) Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis. Acta Otolaryngol 126(5):548–552

    PubMed  Google Scholar 

  79. O’Reilly BJ, Burrows EH (1995) VIIIth cranial nerve involvement in sarcoidosis. J Laryngol Otol 109(11):1089–1093

    CAS  PubMed  Google Scholar 

  80. Orsoni JG, Zavota L, Pellistri I, Piazza F, Cimino L (2002) Cogan syndrome. Cornea 21(4):356–359

    PubMed  Google Scholar 

  81. Orsoni JG, Laganà B, Rubino P, Zavota L, Bacciu S, Mora P (2010) Rituximab ameliorated severe hearing loss in Cogan’s syndrome: a case report. Orphanet J Rare Dis 5(1):18

    PubMed  PubMed Central  Google Scholar 

  82. Pelosi S, Chandrasekhar SS (2011) Intratympanic steroid use for hearing salvage in Vogt-Koyanagi-Harada syndrome. Ear Nose Throat J 90(12):574–577

    PubMed  Google Scholar 

  83. Rarey KE, Bicknell JM, Davis LE (1986) Intralabyrinthine osteogenesis in Cogan’s syndrome. Am J Otolaryngol 7(6):387–390

    CAS  PubMed  Google Scholar 

  84. Roberts RA (2018) Management of recurrent vestibular neuritis in a patient treated for rheumatoid arthritis. Am J Audiol 27(1):19–24

    PubMed  Google Scholar 

  85. Robinson PK (1954) A case of uveitis with deafness; the Vogt-Koyanagi syndrome. Br Med J 2(4891):792–793

    CAS  PubMed  PubMed Central  Google Scholar 

  86. Rowe-Jones JM, Macallan DC, Sorooshian M (1990) Polyarteritis nodosa presenting as bilateral sudden onset cochleo-vestibular failure in a young woman. J Laryngol Otol 104(7):562–564

    CAS  PubMed  Google Scholar 

  87. Ruckenstein MJ, McKown KM, Jacewicz M (2000) Unusual and instructive case of immune-mediated inner ear disease associated with central nervous system vasculitis. Otolaryngol Head Neck Surg 122(1):109–111

    CAS  PubMed  Google Scholar 

  88. Russo FY, Ralli M, De Seta D, Mancini P, Lambiase A, Artico M et al (2018) Autoimmune vertigo: an update on vestibular disorders associated with autoimmune mechanisms. Immunol Res 66(6):675–685

    CAS  PubMed  Google Scholar 

  89. Santos F, Salviz M, Domond H, Nadol JB (2015) Otopathology of vasculitis in granulomatosis with polyangitis. Otol Neurotol 36(10):1657–1662

    PubMed  PubMed Central  Google Scholar 

  90. Saravanan V, Pugmire S, Smith M, Kelly C (2019) Patient-reported involvement of the eighth cranial nerve in giant cell arteritis. Clin Rheumatol 38(12):3655–3660

    PubMed  Google Scholar 

  91. Scharl M, Frei P, Fried M, Rogler G, Vavricka SR (2011) Association between Cogan’s syndrome and inflammatory bowel disease: a case series. J Crohns Colitis 5(1):64–68

    PubMed  Google Scholar 

  92. Scherg F, Haag F, Krieger T (2019) Off-label application of intravenous immunoglobulin (IVIG) for treatment of Cogan's syndrome during pregnancy. BMJ Case Rep 12(10):e227917. https://doi.org/10.1136/bcr-2018-227917

  93. Schuknecht HF, Nadol JB Jr (1994) Temporal bone pathology in a case of Cogan’s syndrome. Laryngoscope 104(9):1135–1142

    CAS  PubMed  Google Scholar 

  94. Seccia V, Fortunato S, Cristofani-Mencacci L, Dallan I, Casani AP, Latorre M et al (2016) Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis. Laryngoscope 126(12):2792–2797

    CAS  PubMed  Google Scholar 

  95. Sekhon RK, Rocha Cabrero F, Deibel JP (2023) Nystagmus Types. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. Available from: https://www.ncbi.nlm.nih.gov/books/NBK539711/

  96. Seo YJ, Choi JY, Kim SH, Kim TJ (2012) Cochlear implantation in a bilateral sensorineural hearing loss patient with relapsing polychondritis. Rheumatol Int 32(2):479–482

    PubMed  Google Scholar 

  97. Shah UK, White JA, Gooey JE, Hybels RL (1997) Otolaryngologic manifestations of sarcoidosis: presentation and diagnosis. Laryngoscope 107(1):67–75

    CAS  PubMed  Google Scholar 

  98. Sil A, Chatrath P, Gatland DJ (2006) Deafness in Vogt-Koyanagi-Harada syndrome. J Laryngol Otol 120(5):416–418

    CAS  PubMed  Google Scholar 

  99. Smith JL (1970) Cogan’s syndrome. Laryngoscope 80(1):121–132

    CAS  PubMed  Google Scholar 

  100. Sugimoto K, Miyazawa T, Nishi H, Izu A, Enya T, Okada M et al (2014) Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. Pediatr Rheumatol Online J 12:15

    PubMed  PubMed Central  Google Scholar 

  101. Sugiura M, Naganawa S, Teranishi M, Sato E, Kojima S, Nakashima T (2006) Inner ear hemorrhage in systemic lupus erythematosus. Laryngoscope 116(5):826–828

    PubMed  Google Scholar 

  102. Szmulewicz DJ, Waterston JA (2012) Two patients with audiovestibular sarcoidosis. J Clin Neurosci 19(1):158–161

    PubMed  Google Scholar 

  103. Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S (2002) Otologic manifestations of Wegener’s granulomatosis. Laryngoscope 112(9):1684–1690

    PubMed  Google Scholar 

  104. Ten Dam L, van de Beek D, Brouwer MC (2022) Clinical characteristics and outcome of hydrocephalus in neurosarcoidosis: a retrospective cohort study and review of the literature. J Neurol 269(5):2727–2733

    PubMed  Google Scholar 

  105. Tharwat S, Mohamed SZ, Nassar MK (2021) Challenges of Egyptian patients with systemic lupus erythematosus during the COVID-19 pandemic. Reumatologia 59(4):237–243

    PubMed  PubMed Central  Google Scholar 

  106. Tirelli G, Tomietto P, Quatela E, Perrino F, Nicastro L, Cattin L et al (2015) Sudden hearing loss and Crohn disease: when Cogan syndrome must be suspected. Am J Otolaryngol 36(4):590–597

    PubMed  Google Scholar 

  107. Ulusoy B, Limon M, Yılmaz S, Çolpan B, Aygün AA, Körez MK et al (2022) Effects of primary Sjögren’s syndrome on hearing and vestibular systems. J Laryngol Otol 136(12):1254–1258

    CAS  PubMed  Google Scholar 

  108. Van Doornum S, McColl G, Walter M, Jennens I, Bhathal P, Wicks IP (2001) Prolonged prodrome, systemic vasculitis, and deafness in Cogan’s syndrome. Ann Rheum Dis 60(1):69

    PubMed  PubMed Central  Google Scholar 

  109. von Brevern M, Lempert T, Bronstein AM, Kocen R (1997) Selective vestibular damage in neurosarcoidosis. Ann Neurol 42(1):117–120

    Google Scholar 

  110. Vos FI, Merkus P, van Nieuwkerk EB, Hensen EF (2016) Rare cause of bilateral sudden deafness. BMJ Case Rep 2016:bcr2016216004. https://doi.org/10.1136/bcr-2016-216004

  111. Vyse T, Luxon LM, Walport MJ (1994) Audiovestibular manifestations of the antiphospholipid syndrome. J Laryngol Otol 108(1):57–59

    CAS  PubMed  Google Scholar 

  112. Wagner W, Fehrmann A (2006) Association of retinal vasculitis (Eales’ disease) and Meniere-like vestibulocochlear symptoms. Eur Arch Otorhinolaryngol 263(2):100–104

    PubMed  Google Scholar 

  113. Weber RS, Jenkins HA, Coker NJ (1984) Sensorineural hearing loss associated with ulcerative colitis. A case report. Arch Otolaryngol 110(12):810–812

    CAS  PubMed  Google Scholar 

  114. White AS, Taylor RL, McNeill C, Garsia R, Welgampola MS (2014) Behçet’s disease presenting as a peripheral vestibulopathy. J Clin Neurosci 21(6):1060–1063

    PubMed  Google Scholar 

  115. Yamazaki H, Fujiwara K, Shinohara S, Kikuchi M, Kanazawa Y, Kurihara R et al (2012) Reversible cochlear disorders with normal vestibular functions in three cases with Wegener’s granulomatosis. Auris Nasus Larynx 39(2):236–240

    PubMed  Google Scholar 

  116. Yildirim N, Arslanoglu A, Aygun N (2008) Otologic and leptomeningeal involvements as presenting features in seronegative Wegener granulomatosis. Am J Otolaryngol 29(2):147–149

    PubMed  Google Scholar 

  117. Yilmaz S, Erbek S, Erbek SS, Ozgirgin N, Yucel E (2007) Abnormal electronystagmography in rheumatoid arthritis. Auris Nasus Larynx 34(3):307–311

    PubMed  Google Scholar 

  118. Ying YL, Hirsch BE (2010) Atypical Cogan’s syndrome: a case report. Am J Otolaryngol 31(4):279–282

    PubMed  Google Scholar 

  119. Howick J, Chalmers I, (James Lind Library), Glasziou P, Greenhalgh T, Heneghan C, Liberati A et al (2011) OCEBM levels of evidence working group. The Oxford 2011 Levels of Evidence. Oxford Centre for Evidence-Based Medicine

  120. Higgins JPT, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, Welch VA (2022) Cochrane handbook for systematic reviews of interventions version 6.3. cochrane. Available from https://www.training.cochrane.org/handbook.

  121. Macdonald NK, Kaski D, Saman Y, Al-Shaikh Sulaiman A, Anwer A, Bamiou DE (2017) Central Positional Nystagmus: A Systematic Literature Review. Front Neurol 8:141.https://doi.org/10.3389/fneur.2017.00141

  122. Strupp M, Thurtell MJ, Shaikh AG, Brandt T, Zee DS, Leigh RJ (2011) Pharmacotherapy of vestibular and ocular motor disorders, including nystagmus. J Neurol 258(7):1207–1222

    CAS  PubMed  PubMed Central  Google Scholar 

  123. Özkırış M, Kapusuz Z, Günaydın İ, Kubilay U, Pırtı İ, Saydam L (2014) Does rheumatoid arthritis have an effect on audiovestibular tests? Eur Arch Otorhinolaryngol 271(6):1383–1387

    PubMed  Google Scholar 

  124. Kimura H, Ohashi N, Aso S, Watanabe Y (1996) Clinical study of the role of melanocytes in the inner ear of patients with Harada’s disease. ORL J Otorhinolaryngol Relat Spec 58(4):233–237

    CAS  PubMed  Google Scholar 

  125. Morita Y, Takahashi K, Izumi S, Kubota Y, Ohshima S, Horii A (2017) Vestibular involvement in patients with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis. Otol Neurotol 38(1):97–101

    PubMed  Google Scholar 

  126. Noguchi Y, Nishio A, Takase H, Miyanaga M, Takahashi H, Mochizuki M et al (2014) Audiovestibular findings in patients with Vogt-Koyanagi-Harada disease. Acta Otolaryngol 134(4):339–344

    PubMed  Google Scholar 

  127. Tarnutzer AA, Straumann D, Salman MS (2018) Neuro-ophthalmologic assessment and investigations in children and adults with cerebellar diseases. Handb Clin Neurol 154:305–327

    PubMed  Google Scholar 

  128. Purves D, Augustine GJ, Fitzpatrick D, et al (2001) Editors. Neuroscience. 2nd edition. Sunderland (MA): Sinauer Associates. Available from: https://www.ncbi.nlm.nih.gov/books/NBK10799/

  129. McGrath S, Sohn H, Steele R, Benedetti A (2020) Meta-analysis of the difference of medians. Biom J 62(1):69–98

    PubMed  Google Scholar 

  130. Egger M, Davey Smith G, Schneider M, Minder C (1997) Bias in meta-analysis detected by a simple, graphical test. BMJ 315(7109):629–634

    CAS  PubMed  PubMed Central  Google Scholar 

  131. Sterne JA, Egger M (2001) Funnel plots for detecting bias in meta-analysis: guidelines on choice of axis. J Clin Epidemiol 54(10):1046–1055

    CAS  PubMed  Google Scholar 

  132. El-Wakd MM, El-Gazzar II, Hosni NA, El-Din Teleb DAG (2015) Otolith function assessment in patients with systemic sclerosis. Egypt Rheumatol 37(3):105–112

    Google Scholar 

  133. Cadoni G, Agostino S, Manganelli C, Scipione S, Turco S, Focosi F et al (2004) Vestibular evaluation in Behcet’s disease. Personal experience. Acta Otorhinolaryngol Ital 24(5):262–266

    CAS  PubMed  Google Scholar 

  134. Choung YH, Cho MJ, Park K, Choi SJ, Shin YR, Lee ES (2006) Audio-vestibular disturbance in patients with Behcet’s disease. Laryngoscope 116(11):1987–1990

    PubMed  Google Scholar 

  135. Helmchen C, Arbusow V, Jager L, Strupp M, Stocker W, Schulz P (1999) Cogan’s syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol 119(5):528–536

    CAS  PubMed  Google Scholar 

  136. Sugasawa J, Ishikawa S (1986) Vestibulo-ocular reflex abnormality in Behçet’s disease. Jpn J Ophthalmol 30(1):91–99

    CAS  PubMed  Google Scholar 

  137. Amor-Dorado JC, Arias-Nunez MC, Miranda-Filloy JA, Gonzalez-Juanatey C, Llorca J, Gonzalez-Gay MA (2008) Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies. Medicine (Baltimore) 87(3):131–141

    CAS  PubMed  Google Scholar 

  138. Bayram A, Dogan M, Koc A, Kalkan M, Akcadag A, Ozcan I (2015) Cervical and ocular vestibular evoked myogenic potentials in Behcet’s disease. Am J Otolaryngol 36(4):503–508

    PubMed  Google Scholar 

  139. Gluth MB, Baratz KH, Matteson EL, Driscoll CL (2006) Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 81(4):483–488

    PubMed  Google Scholar 

  140. Salman EJ, Tripathy K (2023) Cogans Syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. Available from: https://www.ncbi.nlm.nih.gov/books/NBK580546/

  141. Agrawal Y, Pineault KG, Semenov YR (2018) Health-related quality of life and economic burden of vestibular loss in older adults. Laryngoscope Investig Otolaryngol 3(1):8–15

    PubMed  Google Scholar 

  142. Koike H, Nishi R, Ohyama K, Morozumi S, Kawagashira Y, Furukawa S et al (2022) ANCA-associated vasculitic neuropathies: a review. Neurol Ther 11(1):21–38

    PubMed  PubMed Central  Google Scholar 

  143. Girasoli L, Cazzador D, Padoan R, Nardello E, Felicetti M, Zanoletti E et al (2018) Update on vertigo in autoimmune disorders, from diagnosis to treatment. J Immunol Res 2018:5072582

    PubMed  PubMed Central  Google Scholar 

  144. Gonçalves DU, Felipe L, Lima TM (2008) Interpretation and use of caloric testing. Braz J Otorhinolaryngol 74(3):440–446

    PubMed  Google Scholar 

  145. Hedges TR, Taylor GW (1952) Uveal and vestibuloauditory disease with sarcoid. AMA Arch Ophthalmol 48(1):88–89

    CAS  PubMed  Google Scholar 

  146. Brama I, Fainaru M (1980) Inner ear involvement in Behcet’s disease. Arch Otolaryngol 106(4):215–217

    CAS  PubMed  Google Scholar 

  147. Halmagyi GM, Chen L, MacDougall HG, Weber KP, McGarvie LA, Curthoys IS (2017) The video head impulse test. Front Neurol 8:258

    CAS  PubMed  PubMed Central  Google Scholar 

  148. Yuen E, Fried J, Nguyen SA, Rizk HG, Ward C, Meyer TA (2021) Hearing loss in patients with systemic lupus erythematosus: a systematic review and meta-analysis. Lupus 30(6):937–945

    PubMed  Google Scholar 

  149. Rahne T, Plontke S, Keyßer G (2020) Vasculitis and the ear: a literature review. Curr Opin Rheumatol 32(1):47–52

    PubMed  Google Scholar 

  150. Fousekis FS, Saridi M, Albani E, Daniel F, Katsanos KH, Kastanioudakis IG et al (2018) Ear involvement in inflammatory bowel disease: a review of the literature. J Clin Med Res 10(8):609–614

    PubMed  PubMed Central  Google Scholar 

  151. Sakano H, Harris JP (2018) Emerging options in immune-mediated hearing loss. Laryngoscope Investig Otolaryngol 4(1):102–108

    PubMed  PubMed Central  Google Scholar 

  152. Hall CD, Herdman SJ, Whitney SL, Cass SP, Clendaniel RA, Fife TD et al (2016) Vestibular rehabilitation for peripheral vestibular hypofunction: an evidence-based clinical practice guideline: from the American Physical Therapy Association Neurology Section. J Neurol Phys Ther 40(2):124–155

    PubMed  PubMed Central  Google Scholar 

  153. Hall CD, Herdman SJ, Whitney SL, Anson ER, Carender WJ, Hoppes CW et al (2022) Vestibular rehabilitation for peripheral vestibular hypofunction: an updated clinical practice guideline from the Academy of Neurologic Physical Therapy of the American Physical Therapy Association. J Neurol Phys Ther 46(2):118–177

    PubMed  Google Scholar 

  154. Mira E (2008) Improving the quality of life in patients with vestibular disorders: the role of medical treatments and physical rehabilitation. Int J Clin Pract 62(1):109–114

    CAS  PubMed  Google Scholar 

  155. Gurgel RK, Jackler RK, Dobie RA, Popelka GR (2012) A new standardized format for reporting hearing outcome in clinical trials. Otolaryngol Head Neck Surg 147(5):803–807

    PubMed  Google Scholar 

Download references

Acknowledgements

The authors would like to acknowledge Emily Brennen, Andrea Li, MD, and Neil Mehta, MD.

Author information

Author notes

  1. Sunny Shah and Shreya Chidarala contributed equally to this study, and they are co-first authors.

Authors and Affiliations

  1. Department of Otolaryngology–Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA

    Sunny Shah, Shreya Chidarala, Seth Jeong, Kathy Zhang, Shaun A. Nguyen, Rachel Wilkinson & Habib Rizk

  2. Department of Rheumatology, Medical University of South Carolina, Charleston, SC, USA

    Celine Ward

Authors
  1. Sunny Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shreya Chidarala
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Seth Jeong
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kathy Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shaun A. Nguyen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rachel Wilkinson
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Celine Ward
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Habib Rizk
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Sunny Shah: conception/design of study, acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Shreya Chidarala: conception/design of study, acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Seth Jeong: conception/design of study, acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Kathy Zhang: conception/design of study, acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Shaun A. Nguyen: acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Rachel Wilkinson: analysis, intellectual contribution, and drafting of the manuscript.

Celine Ward: interpretation of data, intellectual contribution, and drafting of the manuscript.

Habib Rizk: conception/design of study, acquisition, analysis, and interpretation of data, intellectual contribution, and drafting of the manuscript.

Corresponding author

Correspondence to Habib Rizk.

Ethics declarations

Disclosures

None.

Disclaimer

All co-authors take full responsibility for the integrity and accuracy of all aspects of the work.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 2269 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shah, S., Chidarala, S., Jeong, S. et al. Secondary autoimmune immune ear disease (AIED): a systematic review and meta-analysis on vestibular manifestations of systemic autoimmune and inflammatory disorders. Clin Rheumatol 42, 2747–2759 (2023). https://doi.org/10.1007/s10067-023-06674-w

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-023-06674-w

Keywords

Search

Navigation