- Department of Neurology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, P. R. China;
Vestibular dysfunction is a clinical syndrome characterized by symptoms such as dizziness or vertigo, abnormal control of eye movements, balance disorders, and autonomic nervous system symptoms. Immune-related vestibular dysfunction is caused by vestibular abnormalities triggered by autoimmune reactions or dysfunctions in the immune system. Some autoimmune diseases can present with vestibular dysfunction as the initial symptom or a typical manifestation. There is still a lack of understanding regarding the immune pathogenic factors of vestibular abnormalities both domestically and internationally. This paper provides a detailed summary of the types, onset, pathological mechanisms, symptoms, signs, and auxiliary examinations of immune-related vestibular dysfunction, aiming to offer new insights for the identification of such diseases.
Copyright © the editorial department of West China Medical Journal of West China Medical Publisher. All rights reserved
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- 1. Conrad N, Misra S, Verbakel JY, et al. Incidence, prevalence, and co-occurrence of autoimmune disorders over time and by age, sex, and socioeconomic status: a population-based cohort study of 22 million individuals in the UK. Lancet, 2023, 401(10391): 1878-1890.
- 2. Bovo R, Ciorba A, Martini A. Vertigo and autoimmunity. Eur Arch Otorhinolaryngol, 2010, 267(1): 13-19.
- 3. Walton C, King R, Rechtman L, et al. Rising prevalence of multiple sclerosis worldwide: insights from the Atlas of MS, third edition. Mult Scler, 2020, 26(14): 1816-1821.
- 4. Pula JH, Newman-Toker DE, Kattah JC. Multiple sclerosis as a cause of the acute vestibular syndrome. J Neurol, 2013, 260(6): 1649-1654.
- 5. Marrie RA, Cutter GR, Tyry T. Substantial burden of dizziness in multiple sclerosis. Mult Scler Relat Disord, 2013, 2(1): 21-28.
- 6. Degirmenci E, Bir LS, Ardic FN. Clinical and electronystagmographical evaluation of vestibular symptoms in relapsing remitting multiple sclerosis. Neurol Res, 2010, 32(9): 986-991.
- 7. Gür E, Binkhamis G, Kluk K. Effects of multiple sclerosis on the audio-vestibular system: a systematic review. BMJ Open, 2022, 12(8): e060540.
- 8. Tramontano M, Casagrande Conti L, Orejel Bustos AS, et al. Abnormal vestibulo-ocular reflex function correlates with balance and gait impairment in people with multiple sclerosis. Audiol Res, 2024, 14(5): 799-808.
- 9. Prosperini L, Sbardella E, Raz E, et al. Multiple sclerosis: white and gray matter damage associated with balance deficit detected at static posturography. Radiology, 2013, 268(1): 181-189.
- 10. Di Stadio A, Ralli M, Altieri M, et al. Audiovestibular symptoms in patients with multiple sclerosis: a correlation between self-reported symptomatology and MRI findings to monitor disease progression. Mult Scler Relat Disord, 2020, 45: 102431.
- 11. Doty RL, MacGillivray MR, Talab H, et al. Balance in multiple sclerosis: relationship to central brain regions. Exp Brain Res, 2018, 236(10): 2739-2750.
- 12. MacMahon H, El Refaie A. The audiovestibular manifestations as early symptoms of multiple sclerosis: a scoping review of the literature. Ir J Med Sci, 2022, 191(1): 391-400.
- 13. Cochrane GD, Christy JB, Sandroff BM, et al. Cognitive and central vestibular functions correlate in people with multiple sclerosis. Neurorehabil Neural Repair, 2021, 35(11): 1030-1038.
- 14. Nagaishi A, Takagi M, Umemura A, et al. Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes. J Neurol Neurosurg Psychiatry, 2011, 82(12): 1360-1364.
- 15. Kremer L, Mealy M, Jacob A, et al. Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients. Mult Scler, 2014, 20(7): 843-847.
- 16. Li Z, Yang M, Pan Y, et al. Neuromyelitis optica spectrum disorder with acute brainstem manifestations as initial symptoms. Heliyon, 2024, 10(12): e32539.
- 17. Jarius S, Kleiter I, Ruprecht K, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: brainstem involvement - frequency, presentation and outcome. J Neuroinflammation, 2016, 13(1): 281.
- 18. Gilligan M, McGuigan C, McKeon A. Paraneoplastic neurologic disorders. Curr Neurol Neurosci Rep, 2023, 23(3): 67-82.
- 19. Abbatemarco JR, Vedeler CA, Greenlee JE. Paraneoplastic cerebellar and brainstem disorders. Handb Clin Neurol, 2024, 200: 173-191.
- 20. Ko MW, Dalmau J, Galetta SL. Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol, 2008, 28(1): 58-68.
- 21. Peterson K, Rosenblum MK, Kotanides H, et al. Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients. Neurology, 1992, 42(10): 1931-1937.
- 22. Alfugham N, Gadoth A, Lennon VA, et al. ITPR1 autoimmunity: frequency, neurologic phenotype, and cancer association. Neurol Neuroimmunol Neuroinflamm, 2017, 5(1): e418.
- 23. Do LD, Gupton SL, Tanji K, et al. TRIM9 and TRIM67 are new targets in paraneoplastic cerebellar degeneration. Cerebellum, 2019, 18(2): 245-254.
- 24. Murphy OC, Hac NEF, Gold DR. Updates in neuro-otology. Curr Opin Neurol, 2023, 36(1): 36-42.
- 25. Graus F. Towards a better recognition of paraneoplastic brainstem encephalitis. J Neurol Neurosurg Psychiatry, 2021, 92(11): 1141.
- 26. 中华医学会神经病学分会感染性疾病与脑脊液细胞学学组, 中国罕见病联盟自身免疫性脑炎专业委员会. 自身免疫性小脑共济失调诊断专家共识. 中华神经科杂志, 2024, 57(8): 830-839.
- 27. Narayan RN, McKeon A, Fife TD. Autoimmune vestibulocerebellar syndromes. Semin Neurol, 2020, 40(1): 97-115.
- 28. London F, Hadhoum N, Zéphir H, et al. Autoimmune cerebellar ataxia with glutamic acid decarboxylase 65 antibodies associated with central vestibular symptoms. Acta Neurol Belg, 2017, 117(3): 775-776.
- 29. Uygunoğlu U, Siva A. Behçet’s syndrome and nervous system involvement. Curr Neurol Neurosci Rep, 2018, 18(7): 35.
- 30. Gemignani G, Berrettini S, Bruschini P, et al. Hearing and vestibular disturbances in Behçet’s syndrome. Ann Otol Rhinol Laryngol, 1991, 100(6): 459-463.
- 31. Kulahli I, Balci K, Koseoglu E, et al. Audio-vestibular disturbances in Behcet’s patients: report of 62 cases. Hear Res, 2005, 203(1/2): 28-31.
- 32. Choung YH, Cho MJ, Park K, et al. Audio-vestibular disturbance in patients with Behçet’s disease. Laryngoscope, 2006, 116(11): 1987-1990.
- 33. Ertugrul O, Mutlu A, Zindanci I, et al. Audiological and vestibular measurements in Behçet’s disease. Eur Arch Otorhinolaryngol, 2019, 276(6): 1625-1632.
- 34. Bayram A, Doğan M, Koç A, et al. Cervical and ocular vestibular evoked myogenic potentials in Behcet’s disease. Am J Otolaryngol, 2015, 36(4): 503-508.
- 35. Erbek S, Erbek SS, Yilmaz S, et al. Vestibular evoked myogenic potentials in Behcet’s disease. Eur Arch Otorhinolaryngol, 2008, 265(11): 1315-1320.
- 36. Park E, Lee SU, Kim HJ, et al. Neuro-Behçet’s disease presenting as isolated vestibular syndrome. J Clin Neurol, 2020, 16(3): 499-501.
- 37. Kessel A, Vadasz Z, Toubi E. Cogan syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev, 2014, 13(4/5): 351-354.
- 38. Lunardi C, Bason C, Leandri M, et al. Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet, 2002, 360(9337): 915-921.
- 39. Helmchen C, Arbusow V, Jäger L, et al. Cogan’s syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol, 1999, 119(5): 528-536.
- 40. Dekker JJ, Dinant HJ, Van Soesbergen RM. Cogan’s syndrome as an extra-articular manifestation of rheumatoid arthritis. Clin Rheumatol, 1996, 15(4): 374-377.
- 41. Jung DH, Nadol JB Jr, Folkerth RD, et al. Histopathology of the Inner ear in a case with recent onset of Cogan’s syndrome: evidence for vasculitis. Ann Otol Rhinol Laryngol, 2016, 125(1): 20-24.
- 42. Vollertsen RS, McDonald TJ, Younge BR, et al. Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc, 1986, 61(5): 344-361.
- 43. Haynes BF, Kaiser-Kupfer MI, Mason P, et al. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore), 1980, 59(6): 426-441.
- 44. Gluth MB, Baratz KH, Matteson EL, et al. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc, 2006, 81(4): 483-488.
- 45. Marrero-Gonzalez AR, Ward C, Nguyen SA, et al. Audiovestibular outcomes in adult patients with cogan syndrome: a systematic review. Eur Arch Otorhinolaryngol, 2025, 282(1): 23-35.
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