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Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis

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Abstract

Sjögren syndrome (SS) is a multisystem autoimmune disease, primarily targeting salivary and lacrimal glands; skin, nasal and vaginal dryness, along with musculoskeletal pain and fatigue are the most commonly reported symptoms. Hearing loss is hypothesized to be frequent as well. The purpose of this systematic review was to estimate the prevalence of Hearing loss and its different subtypes in patients with Sjögren syndrome. PRISMA guidelines were followed to ensure highest quality for our systematic review. A random effects model meta-analysis and meta-regression was conducted using I2 as heterogeneity indicator. Eleven observational studies were included in this systematic review. Ten of them were cross-sectional, while one study was case–control. Studies were assessed for risk of bias: all were rated to a moderate level, except for two rated to a low level. Pooled prevalence of any type of hearing loss was 52.2%. After excluding studies rated to moderate bias, the pooled prevalence of hearing loss was 36.7%. We also conducted a subgroup analysis depending on type of hearing loss. Pooled prevalence of sensorineural hearing loss was 42.6%., while pooled prevalence of conductive hearing loss and mixed hearing loss were 5% and 2.3%, respectively. Meta-regression was conducted in an effort to identify possible variables capable to explain high heterogeneity between studies. Sample size and year of study were separately found to account for a portion of heterogeneity between studies of sensorineural hearing loss. Year of study was also found to account for a portion of heterogeneity between studies of conductive hearing loss. In conclusion, sensorineural hearing loss, is highly prevalent in patients with Sjögren syndrome. On this basis, early screening and follow-up of patients with Sjögren syndrome by pure tone audiometry is important.

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References

  1. Qin B, Wang J, Yang Z et al (2015) Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis 74:1983–1989

    Article  CAS  Google Scholar 

  2. Mavragani CP, Schini M et al (2012) Brief report: adrenal autoimmunity in primary Sjögren’s syndrome. Arthritis Rheum 64:4066–4071. https://doi.org/10.1002/art.34679

    Article  CAS  Google Scholar 

  3. Teos LY, Alevizos I (2017) Genetics of Sjögren’s syndrome. Clin Immunol 182:41–47. https://doi.org/10.1016/j.clim.2017.04.018

    Article  CAS  Google Scholar 

  4. Liu Z, Chu A (2021) Sjögren’s syndrome and viral infections. Rheumatol Ther 8:1051–1059. https://doi.org/10.1007/s40744-021-00334-8

    Article  Google Scholar 

  5. Teruel M, Barturen G, Martínez-Bueno M et al (2021) Integrative epigenomics in Sjögren’s syndrome reveals novel pathways and a strong interaction between the HLA, autoantibodies and the interferon signature. Sci Rep 11:23292. https://doi.org/10.1038/s41598-021-01324-0

    Article  CAS  Google Scholar 

  6. Nocturne G, Mariette X (2013) Advances in understanding the pathogenesis of primary Sjögren’s syndrome. Nat Rev Rheumatol 9:544–556

    Article  CAS  Google Scholar 

  7. Brito-Zerón P, Baldini C, Bootsma H et al (2016) Sjögren syndrome. Nat Rev Dis Prim 2:1–20. https://doi.org/10.1038/nrdp.2016.47

    Article  Google Scholar 

  8. Sebastian A, Szachowicz A, Wiland P (2019) Classification criteria for secondary Sjögren’s syndrome. Current state of knowledge. Reumatologia 57:277–280

    Article  Google Scholar 

  9. van Nimwegen JF, van Ginkel MS et al (2018) Validation of the ACR-EULAR criteria for primary Sjögren’s syndrome in a Dutch prospective diagnostic cohort. Rheumatology (Oxford) 57(5):818–825. https://doi.org/10.1093/rheumatology/kex495

    Article  Google Scholar 

  10. Gündüz B, Yildirim N, Güven SC et al (2019) Evaluation of medial olivocochlear efferent system and hearing loss in patients with primary Sjögren’s syndrome. Turk J Med Sci 49:1647–1654. https://doi.org/10.3906/sag-1901-128

    Article  CAS  Google Scholar 

  11. Hatzopoulos S, Amoroso C, Aimoni C et al (2002) Hearing loss evaluation of Sjogren’s syndrome using distortion product otoacoustic emissions. Acta Otolaryngol 122:20–25. https://doi.org/10.1080/00016480260094929

    Article  Google Scholar 

  12. Seeliger T, Bönig L, Witte T et al (2020) Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study. Ann Transl Med 8:1069

    Article  Google Scholar 

  13. Thanooja CV, Augustine AM, Lepcha A et al (2018) Audiological profile in primary Sjögren’s syndrome in a tertiary care setting and its clinical implications. Indian J Otolaryngol Head Neck Surg 70:59–65. https://doi.org/10.1007/s12070-015-0956-y

    Article  CAS  Google Scholar 

  14. Gk T, Pv V, Pelechas E et al (2019) Cochlear involvement in patients with systemic autoimmune rheumatic diseases: a clinical and laboratory comparative study. Eur Arch Otorhinolaryngol 276:2419–2426

    Article  Google Scholar 

  15. Dalton DS, Cruickshanks KJ, Klein BEK et al (2003) The impact of hearing loss on quality of life in older adults. Gerontologist 43:661–668. https://doi.org/10.1093/geront/43.5.661

    Article  Google Scholar 

  16. Liu CM, Lee CTC (2019) Association of hearing loss with dementia. JAMA Netw Open 2:e198112. https://doi.org/10.1001/jamanetworkopen.2019.8112

    Article  Google Scholar 

  17. Michels TC, Duffy MT, Rogers DJ (2019) Hearing loss in adults: differential diagnosis and treatment. Am Fam Physician 100(2):98–108

    Google Scholar 

  18. Shamseer L, Moher D, Clarke M et al (2015) Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015: elaboration and explanation. BMJ. https://doi.org/10.1136/bmj.g7647

    Article  Google Scholar 

  19. Sterne JA, Hernán MA, Reeves BC et al (2016) ROBINS-I: A tool for assessing risk of bias in non-randomised studies of interventions. BMJ. https://doi.org/10.1136/bmj.i4919

    Article  Google Scholar 

  20. Higgins JPT, Thompson SG, Deeks JJ, Altman DG (2003) Measuring inconsistency in meta-analyses. BMJ 327:557–560

    Article  Google Scholar 

  21. Sterne JAC, Sutton AJ, Ioannidis JPA et al (2011) Recommendations for examining and interpreting funnel plot asymmetry in meta-analyses of randomised controlled trials. BMJ. https://doi.org/10.1136/bmj.d4002

    Article  Google Scholar 

  22. Doig JA, Whaley K, Dick WC et al (1971) Otolaryngological aspects of Sjögren’s syndrome. Br Med J 4:460–463

    Article  CAS  Google Scholar 

  23. Galarza-Delgado DA, Villegas Gonzalez MJ, Riega Torres J et al (2018) Early hearing loss detection in rheumatoid arthritis and primary Sjögren syndrome using extended high frequency audiometry. Clin Rheumatol 37:367–373. https://doi.org/10.1007/S10067-017-3959-0

    Article  Google Scholar 

  24. Gusmão Fernandesa FL, Guimarãesa AC, Scaramussaa LS et al (2014) Otorhinolaryngological findings in a group of patients with rheumatic diseases. Rev Bras Reumatol 54:172–178

    Google Scholar 

  25. Trott MS, Hughes GB, Calabrese LH et al (1996) Hearing and Sjögren’s syndrome. Ear Nose Throat J 75:666–6668

    Article  CAS  Google Scholar 

  26. Tsirves GK, Voulgari PV, Pelechas E et al (2019) Cochlear involvement in patients with systemic autoimmune rheumatic diseases: a clinical and laboratory comparative study. Eur Arch Otorhinolaryngol 276:2419–2426. https://doi.org/10.1007/s00405-019-05487-5

    Article  Google Scholar 

  27. Tumiati B, Casoli P, Parmeggiani A (1997) Hearing loss in the Sjögren syndrome. Ann Intern Med 126:450–453

    Article  CAS  Google Scholar 

  28. Ziavra N, Politi EN, Kastanioudakis I et al (2000) Hearing loss in Sjögren’s syndrome patients. A comparative study. Clin Exp Rheumatol 18:725–728

    CAS  Google Scholar 

  29. Haile LM, Kamenov K, Briant PS et al (2021) Hearing loss prevalence and years lived with disability, 1990–2019: findings from the global burden of disease study 2019. Lancet 397:996–1009. https://doi.org/10.1016/S0140-6736(21)00516-X/ATTACHMENT/A552CF62-9122-4B03-B1AF-AFCE71BE7E9E/MMC1.PDF

    Article  Google Scholar 

  30. Goman AM, Lin FR (2016) Prevalence of hearing loss by severity in the United States. Am J Public Health 106:1820–1822. https://doi.org/10.2105/AJPH.2016.303299

    Article  Google Scholar 

  31. Emamifar A, Bjoerndal K, Hansen IM (2016) Is hearing impairment associated with rheumatoid arthritis? A review. Open Rheumatol J. https://doi.org/10.2174/1874312901610010026

    Article  Google Scholar 

  32. Ahmadzadeh A, Daraei M, Jalessi M et al (2017) Hearing status in patients with rheumatoid arthritis. J Laryngol Otol 131(10):895–899. https://doi.org/10.1017/S0022215117001670

    Article  CAS  Google Scholar 

  33. Jeong J, Lim H, Lee K et al (2019) High risk of sudden sensorineural hearing loss in several autoimmune diseases according to a population-based national sample cohort study. Audiol Neurotol 24:224–230. https://doi.org/10.1159/000502677

    Article  Google Scholar 

  34. Tanaka EA, Barros H, Polanski JF et al (2019) AB0507 hearing loss in systemic lupus erythematosus. Ann Rheum Dis 78:1716–1717. https://doi.org/10.1136/ANNRHEUMDIS-2019-EULAR.5998

    Article  Google Scholar 

  35. Abbasi M, Yazdi Z, Kazemifar AM, Bakhsh ZZ (2013) Hearing loss in patients with systemic lupus erythematosus. Glob J Health Sci 5:102–106

    Article  Google Scholar 

  36. Talal N, Moutsopoulos HM, Kassan SS (eds) (1987) Sjögren’s syndrome. Springer Berlin Heidelberg, Heidelberg

    Google Scholar 

  37. Fauchais AL, Lambert M, Launay D et al (2004) Antiphospholipid antibodies in primary Sjögren’s syndrome: prevalence and clinical significance in a series of 74 patients. Lupus 13:245–248. https://doi.org/10.1191/0961203304lu1006oa

    Article  CAS  Google Scholar 

  38. Compadretti GC, Brandolini C, Ignazio T (2005) Sudden sensorineural hearing loss in lupus erythematosus associated with antiphospholipid syndrome: case report and review. Ann Otol Rhinol Laryngol 114:214–218. https://doi.org/10.1177/000348940511400308

    Article  Google Scholar 

  39. Teixeira F, Moreira I, Silva AM et al (2013) Neurological involvement in primary Sjögren syndrome. Acta Reumatol Port 38:29–36

    Google Scholar 

  40. Delalande S, de Seze J, Fauchais A-L, Hachulla E et al (2004) Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 83:280–291. https://doi.org/10.1097/01.md.0000141099.53742.16

    Article  Google Scholar 

  41. Jourde-Chiche N, Mancini J, Dagher N et al (2012) Antimalarial ototoxicity: an underdiagnosed complication? A study of spontaneous reports to the French pharmacovigilance network. Ann Rheum Dis 71:1586–1587

    Article  Google Scholar 

  42. Johansson MSK, Arlinger SD (2003) Prevalence of hearing impairment in a population in Sweden. Int Audiol 42:18–28. https://doi.org/10.3109/14992020309056081

    Article  Google Scholar 

  43. Rodríguez-Valiente A, Álvarez-Montero Ó, Górriz-Gil C, García-Berrocal JR (2020) Prevalence of presbycusis in an otologically normal population. Acta Otorrinolaringologica 71:175–180. https://doi.org/10.1016/j.otoeng.2019.05.003

    Article  Google Scholar 

  44. Nezos A, Mavragani CP (2015) Contribution of genetic factors to Sjögren’s syndrome and Sjögren’s syndrome related lymphomagenesis. J Immunol Res 2015:754825. https://doi.org/10.1155/2015/754825

    Article  CAS  Google Scholar 

  45. Psillas G, Binos P, Dimas GG, Daniilidis M, Constantinidis J (2021) Human leukocyte antigen (HLA) influence on prognosis of autoimmune hearing loss. Audiol Res 11:31–37. https://doi.org/10.3390/audiolres11010004

    Article  Google Scholar 

  46. Papadimitraki ED, Kyrmizakis DE, Kritikos I, Boumpas DT (2004) Ear-nose-throat manifestations of autoimmune rheumatic diseases. Clin Exp Rheumatol 22:485–494

    CAS  Google Scholar 

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PV and CN conceptualized, proposed and designed the study. PV and PS searched the literature, screened and selected the eligible studies. SP and PS assessed the quality of the studies included. PV, ZP and SP analyzed the data and carried out the statistical analyses. PV and NC contributed in drafting the manuscript, and all other authors reviewed it critically. All authors take full responsibility for the integrity and accuracy of all aspects of the work.

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Correspondence to Vasileios Paraschou.

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Paraschou, V., Partalidou, S., Siolos, P. et al. Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis. Rheumatol Int 43, 233–244 (2023). https://doi.org/10.1007/s00296-022-05235-9

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