Review Article

The Association of Autoimmune Diseases with Tinnitus: A Narrative Review

Abstract

Background and Aim: Autoimmune Diseases (ADs), marked by immune system dysfunction and self-targeting immune responses, can damage the auditory system, leading to symptoms like tinnitus and sensorineural hearing loss. This study explores the prevalence, underlying mechanisms, diagnosis, and treatment of tinnitus within the context of ADs.
Recent Findings: This narrative review identified relevant articles through searches of Web of Science, Science Direct, PubMed, and Google Scholar, using keywords related to both tinnitus (e.g. "Tinnitus", "Ringing") and ADs (e.g. "Autoimmune Disease", "Multiple Sclerosis", "Lupus"). This review found a significant prevalence of tinnitus across various ADs. Autoimmune inner ear disease exhibited the highest tinnitus rates (50–95.7%), followed by sarcoidosis (61%) and Susac’s syndrome (54–77%). Systemic lupus erythematosus (6.7–44.4%), Sjögren's syndrome (10.1–41.7%), and multiple sclerosis (up to 30%) also showed notable tinnitus prevalence. The main mechanisms involve vasculitis affecting inner ear vessels, immune complex accumulation, demyelination of auditory pathways, and hair cell damage from ototoxic immunosuppressants (e.g. cyclosporine). Regarding tinnitus treatment, corticosteroids (e.g. prednisone) offer potential benefit, though symptom relief is often temporary. Biologics (e.g. rituximab, adalimumab) have also been explored with varying degrees of success in specific cases.
Conclusion: Due to the significant prevalence of tinnitus observed in ADs, it is recommended that relevant specialists consider incorporating hearing assessments, particularly those specific to tinnitus, into their evaluation protocols. This may facilitate earlier diagnosis and ultimately lead to more appropriate and timely management and rehabilitation strategies.

1. Pisetsky DS. Pathogenesis of autoimmune disease. Nat Rev Nephrol. 2023;19(8):509-24. [DOI:10.1038/s41581-023-00720-1]
2. Albarbar B, Aga H. A Review on Autoimmune Diseases: Recent Advances and Future Perspectives. Alq J Med App Sci. 2024:7(3):718-29. [DOI:10.54361/ajmas.247337]
3. Ralli M, D'Aguanno V, Di Stadio A, De Virgilio A, Croce A, Longo L, et al. Audiovestibular Symptoms in Systemic Autoimmune Diseases. J Immunol Res. 2018;2018:5798103. [DOI:10.1155/2018/5798103]
4. Wang M, Zhang P, Li Q, Kong C. Investigating the Process of Autoimmune Inner Ear Disease: Unveiling the Intricacies of Pathogenesis and Therapeutic Strategies. Int J Med Sci. 2025;22(1):179-87. [DOI:10.7150/ijms.97831]
5. Baradaranfar MH, Doosti A. A survey of relationship between rheumatoid arthritis and hearing disorders. Acta Med Iran. 2010;48(6):371-3.
6. Tsirves GK, Voulgari PV, Pelechas E, Asimakopoulos AD, Drosos AA. Cochlear involvement in patients with systemic autoimmune rheumatic diseases: a clinical and laboratory comparative study. Eur Arch Otorhinolaryngol. 2019;276(9):2419-26. [DOI:10.1007/s00405-019-05487-5]
7. Rahne T, Plontke S, Keyßer G. Vasculitis and the ear: a literature review. Curr Opin Rheumatol. 2020;32(1):47-52. [DOI:10.1097/BOR.0000000000000665]
8. 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. [DOI:10.1177/0300060516688600]
9. 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. [DOI:10.1007/s11845-021-02508-3]
10. Tomás CC, Oliveira E, Sousa D, Uba-Chupel M, Furtado G, Rocha C, et al. Proceedings of the 3rd IPLeiria's International Health Congress : Leiria, Portugal. 6-7 May 2016. BMC Health Serv Res. 2016;16 Suppl 3(Suppl 3):200. [DOI:10.1186/s12913-016-1423-5]
11. Han BI, Lee HW, Ryu S, Kim JS. Tinnitus Update. J Clin Neurol. 2021;17(1):1-10. [DOI:10.3988/jcn.2021.17.1.1]
12. Haider HF, Ribeiro SF, Martins C, Ribeiro D, Trigueiros N, Szczepek AJ, et al. Tinnitus, hearing loss and inflammatory processes in an older Portuguese population. Int J Audiol. 2020;59(5):323-32. [DOI:10.1080/14992027.2019.1698775]
13. Lee HY, Jung DJ. Recent Updates on Tinnitus Management. J Audiol Otol. 2023;27(4):181-92. [DOI:10.7874/jao.2023.00416]
14. Natarajan N, Batts S, Gombar S, Manickam R, Sagi V, Curhan SG, et al. Associations of Tinnitus Incidence with Use of Tumor Necrosis Factor-Alpha Inhibitors among Patients with Autoimmune Conditions. J Clin Med. 2023;12(5):1935. [DOI:10.3390/jcm12051935]
15. Matsuoka AJ, Harris JP. Autoimmune inner ear disease: a retrospective review of fortyseven patients. Audiol Neurootol. 2013;18(4):228-39. [DOI:10.1159/000351289]
16. Kühn D, Hospowsky C, Both M, Hey M, Laudien M. Manifestation of granulomatosis with polyangiitis in head and neck. Clin Exp Rheumatol. 2018;36 Suppl 111(2):78-84. Epub 2018 May 18.
17. Hardy TA, Taylor RL, Qiu J, O'Brien B, Gopinath S, Trewin B, et al. The neuro-otology of Susac syndrome. J Neurol. 2020;267(12):3711-22. [DOI:10.1007/s00415-020-10086-0]
18. Raj-Koziak D, Gos E, Swierniak W, Rajchel JJ, Karpiesz L, Niedzialek I, et al. Visual Analogue Scales as a Tool for Initial Assessment of Tinnitus Severity: Psychometric Evaluation in a Clinical Population. Audiol Neurootol. 2018;23(4):229-37. [DOI:10.1159/000494021]
19. Kim TS, Yakunina N, Ryu YJ, Chung IJ, Nam EC. Self-Administered Tinnitus Pitch Matching versus a Conventional Audiometric Procedure. Audiol Neurootol. 2017;22(1):1-8. [DOI:10.1159/000465512]
20. Karadağ A, Karadağ M, Bora A, Hayta E, Çetin AB, Doğan SC, et al. Evaluation of hearing loss and tinnitus in Behcet's disease. Eur Arch Otorhinolaryngol. 2019;276(10):2691-6. [DOI:10.1007/s00405-019-05506-5]
21. Biswas R, Genitsaridi E, Trpchevska N, Lugo A, Schlee W, Cederroth CR, et al. Low Evidence for Tinnitus Risk Factors: A Systematic Review and Meta-analysis. J Assoc Res Otolaryngol. 2023;24(1):81-94. [DOI:10.1007/s10162-022-00874-y]
22. Mijovic T, Zeitouni A, Colmegna I. Autoimmune sensorineural hearing loss: the otology-rheumatology interface. Rheumatology (Oxford). 2013;52(5):780-9. [DOI:10.1093/rheumatology/ket009]
23. Bustos-Merlo A, Ortiz-Parra A, Rosales-Castillo A, Espinosa-Sanchez JM, Navarrete-Navarrete N. Autoimmune inner ear disease associated with antiphospholipid antibodies. Clin Exp Rheumatol. 2024;42(11):2268-71. [DOI:10.55563/clinexprheumatol/gu70rz]
24. Heywood RL, Hadavi S, Donnelly S, Patel N. Infliximab for autoimmune inner ear disease: case report and literature review. J Laryngol Otol. 2013;127(11):1145-7. [DOI:10.1017/S002221511300217X]
25. Sakano H, Harris JP. Emerging options in immune-mediated hearing loss. Laryngoscope Investig Otolaryngol. 2018;4(1):102-8. [DOI:10.1002/lio2.205]
26. Fioretti A, Di Rubbo V, Peri G, Vitti E, Cisternino S, Varakliotis T, et al. Cochlear Impairment and Autoimmune Ear Disorder in a Patient with Breast Cancer. Audiol Res. 2017;7(1):165. [DOI:10.4081/audiores.2017.165]
27. 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(5):437-42. [DOI:10.1177/0961203313477223]
28. Abbasi M, Yazdi Z, Kazemifar AM, Bakhsh ZZ. Hearing loss in patients with systemic lupus erythematosus. Glob J Health Sci. 2013;5(5):102-6. [DOI:10.5539/gjhs.v5n5p102]
29. 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(1):82-6. [DOI:10.1016/j.otohns.2006.06.1255]
30. Kimyon U, Gözen ED, Karaman E, Günay EE, Esatoglu SN, Atas A, et al. Hearing loss in Takayasu's arteritis. Clin Exp Rheumatol. 2024;42(4):872-8. [DOI:10.55563/clinexprheumatol/gtd2uv]
31. Maciaszczyk K, Durko T, Waszczykowska E, Erkiert-Polguj A, Pajor A. Auditory function in patients with systemic lupus erythematosus. Auris Nasus Larynx. 2011;38(1):26-32. [DOI:10.1016/j.anl.2010.04.008]
32. Gomides AP, do Rosário EJ, Borges HM, Gomides HH, de Pádua PM, Sampaio-Barros PD. Sensorineural dysacusis in patients with systemic lupus erythematosus. Lupus. 2007;16(12):987-90. [DOI:10.1177/0961203307084160]
33. 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. [DOI:10.1055/s-0036-1586162]
34. Al Dousary S. Auditory and vestibular manifestations of Vogt-Koyanagi-Harada disease. J Laryngol Otol. 2011;125(2):138-41. [DOI:10.1017/S0022215110001817]
35. Ferreira MC, Matos IL, Mondelli MFCG. Audiological findings in the Vogt-Koyanagi-Harada Syndrome. Codas. 2020;32(5):e20190183. Portuguese, English. [DOI:10.1590/2317-1782/20202019183]
36. Ruiz-Allec LD, Peñaloza-López YR, Ocaña-Plante Ndel R, Valdez-González T, López-Star E. Hallazgos audiológicos, periféricos y centrales en pacientes con síndrome de Vogt-Koyanagi-Harada [Peripheral and central audiologic findings in patients with Vogt-Koyanagi-Harada syndrome]. Acta Otorrinolaringol Esp. 2009;60(4):253-9. Spanish. [DOI:10.1016/j.otorri.2009.01.002]
37. Sydlowski SA, Luffler C, Haberkamp T. Successful cochlear implantation in a case of Vogt-Koyanagi-Harada disease. Otol Neurotol. 2014;35(9):1522-4. [DOI:10.1097/MAO.0000000000000353]
38. Horie Y, Kitaichi N, Takemoto Y, Namba K, Yoshida K, Hirose S, et al. Polymorphism of IFN-gamma gene and Vogt-Koyanagi-Harada disease. Mol Vis. 2007;13:2334-8.
39. Ondrey FG, Moldestad E, Mastroianni MA, Pikus A, Sklare D, Vernon E, et al. Sensorineural hearing loss in Vogt-Koyanagi-Harada syndrome. Laryngoscope. 2006;116(10):1873-6. [DOI:10.1097/01.mlg.0000234946.31603.fe]
40. Pelosi S, Chandrasekhar SS. Intratympanic steroid use for hearing salvage in Vogt-Koyanagi-Harada syndrome. Ear Nose Throat J. 2011;90(12):574-7. [DOI:10.1177/014556131109001206]
41. Choung YH, Cho MJ, Park K, Choi SJ, Shin YR, Lee ES. Audio-vestibular disturbance in patients with Behçet's disease. Laryngoscope. 2006;116(11):1987-90. [DOI:10.1097/01.mlg.0000237442.80711.65]
42. Kulahli I, Balci K, Koseoglu E, Yuce I, Cagli S, Senturk M. Audio-vestibular disturbances in Behcet's patients: report of 62 cases. Hear Res. 2005;203(1-2):28-31. [DOI:10.1016/j.heares.2004.11.020]
43. Marsili M, Marzetti V, Lucantoni M, Lapergola G, Gattorno M, Chiarelli F, et al. Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: a case report. Ital J Pediatr. 2016;42(1):81. [DOI:10.1186/s13052-016-0291-2]
44. Dardennes R, Al Anbar N, Rouillon F. Propofol-interrupted tinnitus later suppressed by amantadine: a case-report. J Clin Pharmacol. 2013;53(3):356-8. [DOI:10.1002/jcph.6]
45. 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. [DOI:10.26355/eurrev_201807_15520]
46. Konieczka K, Koch S, Binggeli T, Schoetzau A, Kesselring J. Multiple sclerosis and primary vascular dysregulation (Flammer syndrome). EPMA J. 2016;7(1):13. [DOI:10.1186/s13167-016-0062-6]
47. Anagnostouli MC, Sotirchos ES, Zalonis I, Christidi F, Korres S, Rentzos M, et al. Monosymptomatic clinically isolated syndrome with sudden sensorineural hearing loss: case report and critical review of the literature. Neurologist. 2012;18(5):302-5. [DOI:10.1097/NRL.0b013e3182675479]
48. Fox RI. Sjögren's syndrome. Lancet. 2005;366(9482):321-31. [DOI:10.1016/S0140-6736(05)66990-5]
49. Yang TH, Xirasagar S, Cheng YF, Chen CS, Lin HC. Increased prevalence of hearing loss, tinnitus and sudden deafness among patients with Sjögren's syndrome. RMD Open. 2024;10(1):e003308. [DOI:10.1136/rmdopen-2023-003308]
50. Frejo L, Lopez-Escamez JA. Cytokines and Inflammation in Meniere Disease. Clin Exp Otorhinolaryngol. 2022;15(1):49-59. [DOI:10.21053/ceo.2021.00920]
51. Britto DBLA, Rocha MFB, Silva JDD, Pinheiro NDS, Silva LKD, Advíncula KP. Auditive and Vestibular Changes Related to the Use of Hydroxychlorochine and Chlorochine: An Integrative Review. Int Arch Otorhinolaryngol. 2021;25(2):e309-17. [DOI:10.1055/s-0040-1722292]
52. Arnaud L, Audemard-Verger A, Belot A, Bienvenu B, Burillon C, Chasset F, et al. French protocol for diagnosis and management of Cogan's syndrome. Rev Med Interne. 2025;46(2):74-88. [DOI:10.1016/j.revmed.2024.09.007]
53. Pagnini I, Zannin ME, Vittadello F, Sari M, Simonini G, Cimaz R, et al. Clinical features and outcome of Cogan syndrome. J Pediatr. 2012;160(2):303-7.e1. [DOI:10.1016/j.jpeds.2011.07.051]
54. Safavi Naini A, Ghorbani J, Montazer Lotfe Elahi S, Beigomi M. Otologic Manifestations and Progression in Patients with Wegener's granulomatosis: A Survey in 55 Patients. Iran J Otorhinolaryngol. 2017;29(95):327-31. [DOI:10.22038/IJORL.2017.25253.1822]
55. Maciaszczyk K, Waszczykowska E, Pajor A, Bartkowiak-Dziankowska B, Durko T. Hearing organ disorders in patients with systemic sclerosis. Rheumatol Int. 2011;31(11):1423-8. [DOI:10.1007/s00296-010-1503-5]
56. Hung SH, Xirasagar S, Dang LH, Viet-Nhi NK, Cheng YF, Chen CS, et al. Prevalence of hearing loss, tinnitus, vertigo and sudden deafness among patients with polymyositis and dermatomyositis. Sci Rep. 2024;14(1):5774. [DOI:10.1038/s41598-024-56372-z]
57. Sehara Y, Otsuka H, Sakamoto S, Ando Y, Sawada M. Neurosarcoidosis Occurring 6 Years after Onset of Vogt-Koyanagi-Harada Disease. Case Rep Ophthalmol. 2019;10(1):32-40. [DOI:10.1159/000496384]
58. Haider HF, Bojić T, Ribeiro SF, Paço J, Hall DA, Szczepek AJ. Pathophysiology of Subjective Tinnitus: Triggers and Maintenance. Front Neurosci. 2018;12:866. [DOI:10.3389/fnins.2018.00866]
59. Couette N, Gagne S, Paul J. AB1855 Susac syndrome: case series from adult & pediatric tertiary academic medical centers. Ann Rheum Dis. 2023;82(Suppl 1):2155-6. [DOI:10.1136/annrheumdis-2023-eular.3980]
60. Girasoli L, Cazzador D, Padoan R, Nardello E, Felicetti M, Zanoletti E, et al. Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment. J Immunol Res. 2018;2018:5072582. [DOI:10.1155/2018/5072582]
61. You YS, Park SW, Yun SK, Lee EJ. Bilateral sudden sensorineural hearing loss with Sweet syndrome: A case report. Medicine (Baltimore). 2020;99(36):e22127. [DOI:10.1097/MD.0000000000022127]
62. Konieczka K, Erb C. Diseases potentially related to Flammer syndrome. EPMA J. 2017;8(4):327-32. [DOI:10.1007/s13167-017-0116-4]
63. Chen Y, Hu J, Wu D, Wu T, Zhu J. Tocilizumab Successfully Treating Refractory Hearing Impairment in a Patient With Cogan Syndrome: A Case Report and Review of the Literature. Ear Nose Throat J. 2024:1455613241237079. [DOI:10.1177/01455613241237079]
64. Torere BE, Chittipolu S, Alugba G, Aiwuyo HO, Kennard JL. Sudden-Onset Sensorineural Hearing Loss and Tinnitus in a Patient With Rheumatoid Arthritis: A Case Report and Literature Review. Cureus. 2023;15(5):e38739. [DOI:10.7759/cureus.38739]
65. Franz L, Frosolini A, Parrino D, Lovato A, de Filippis C, Marioni G. Ototoxicity of Immunosuppressant Drugs: A Systematic Review. J Int Adv Otol. 2022;18(2):167-76. [DOI:10.5152/iao.2022.21416]
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Tinnitus autoimmune diseases hearing loss vasculitis hearing tests

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Doosti A, Shabani MA, Jeddi Z, Hajimohammadi A. The Association of Autoimmune Diseases with Tinnitus: A Narrative Review. Aud Vestib Res. 2026;.