Skip to main content
SpringerLink
Log in

Hearing loss in patients with scleroderma: associations with clinical manifestations and capillaroscopy

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Systemic sclerosis is a multi-systemic disease with widespread small-vessel vasculopathy and fibrosis. Involvement of the middle and inner ear and hearing loss has been reported as an uncommon manifestation of scleroderma in some studies. In this study, we evaluated hearing problems in scleroderma patients and determined its association with clinical manifestations and capillaroscopy. We evaluated 54 patients with scleroderma referred to Hafez Hospital clinic of scleroderma related to Shiraz University of Medical Science; they fulfilled the LeRoy and ACR/EULAR criteria for scleroderma. Control group consisted of 60 normal individuals. All clinical manifestations, nail fold capillaroscopy, pure tone audiometry, speech reception threshold, and speech audiometry were recorded during evaluation. Subjective hearing loss and objective hearing loss were seen in 10 and 36 patients of the case group (18.5%, 66.7%) and 6 and 10 of the control group (10%, 28.3%) (P values 0.03, < 0.001). Sensorineural hearing loss, abnormal pure tone audiometry, and abnormal speech reception threshold were more common in scleroderma patients compared to the control group (P values of < 0.001, < 0.001, and < 0.001). There was no correlation between objective hearing loss and type of scleroderma, duration of disease, skin score, interstitial lung disease, digital ulcer, gastrointestinal involvement, or nail fold capillaroscopy patterns (all P values > 0.05). In our study, subjective and objective hearing loss were higher in patients with scleroderma compared to the control group and also sensorineural hearing loss, abnormal pure tone audiometry, and abnormal speech reception threshold. There was no correlation between objective hearing loss and clinical manifestations or capillaroscopy findings.

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

Similar content being viewed by others

References

  1. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205

    PubMed  CAS  Google Scholar 

  2. Herrick AL (2000) Vascular function in systemic sclerosis. Curr Opin Rheumatol 12(6):527–533

    Article  PubMed  CAS  Google Scholar 

  3. Chora I, Guiducci S, Manetti M, Romano E, Mazzotta C, Bellando-Randone S, Ibba-Manneschi L, Matucci-Cerinic M, Soares R (2015) Vascular biomarkers and correlation with peripheral vasculopathy in systemic sclerosis. Autoimmun Rev 14(4):314–322

    Article  PubMed  CAS  Google Scholar 

  4. Cutolo M, Pizzorni C, Sulli A, Smith V (2013) Early diagnostic and predictive value of capillaroscopy in systemic sclerosis. Curr Rheumatol Rev 9(4):249–253

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  6. 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 87(3):131–141

    Article  PubMed  CAS  Google Scholar 

  7. Maciaszczyk K, Durko T, Waszczykowska E, Bartkowiak-Dziankowska B, Pajor A, Erkiert-Polguj A, Józefowicz-Korczyńska M (2011) [Vestibular system in patients with systemic sclerosis]. Otolaryngol Pol 65(3):202–207

    Article  PubMed  Google Scholar 

  8. Deroee AF, Huang TC, Morita N, Hojjati M (2009) Sudden hearing loss as the presenting symptom of systemic sclerosis. Otol Neurotol 30(3):277–279

    Article  PubMed  Google Scholar 

  9. Baek MJ, Park HM, Johnson JM, Altuntas CZ, Jane-Wit D, Jaini R et al (2006) Increased frequencies of cochlin-specific T cells in patients with autoimmune sensorineural hearing loss. J Immunol 177(6):4203–4210

    Article  PubMed  CAS  Google Scholar 

  10. Rossini BAA, Penido NO, Munhoz MSL, Bogaz EA, Curi RS (2017) Sudden sensorioneural hearing loss and autoimmune systemic diseases. Int Arch Otorhinolaryngol 21(3):213–223

    Article  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. Gomides AP, do Rosario EJ, Borges HM, Gomides HH, de Padua PM, Sampaio-Barros PD (2007) Sensorineural dysacusis in patients with systemic lupus erythematosus. Lupus 16(12):987–990

    Article  PubMed  CAS  Google Scholar 

  13. Monteiro TA, Christmann RB, Bonfa E, Bento RF, Novalo-Goto ES, Vasconcelos LG (2011) Hearing loss in diffuse cutaneous systemic scleroderma. Scand J Rheumatol 40(6):467–471

    Article  PubMed  CAS  Google Scholar 

  14. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747

    Article  PubMed  PubMed Central  Google Scholar 

  15. Clements PJ, Lachenbruch PA, Ng SC, Simmons M, Sterz M, Furst DE (1990) Skin score: a semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 33(8):1256–1263

    Article  PubMed  CAS  Google Scholar 

  16. Medsger TA Jr, Bombardieri S, Czirjak L, Scorza R, Della Rossa A, Bencivelli W (2003) Assessment of disease severity and prognosis. Clin Exp Rheumatol 21(3 Suppl 29):S42–S46

    PubMed  Google Scholar 

  17. Shenavandeh S, Habibi S (2017) Nailfold capillaroscopic changes in patients with systemic lupus erythematosus: correlations with disease activity, skin manifestation and nephritis. Lupus 26(9):959–966

    Article  PubMed  CAS  Google Scholar 

  18. Cutolo M, Sulli A, Smith V (2013) How to perform and interpret capillaroscopy. Best Pract Res Clin Rheumatol 27(2):237–248

    Article  PubMed  Google Scholar 

  19. Pruszewicz A, Demenko G, Richter L, Wika T (1994) [New articulation lists for speech audiometry: part I]. Otol Pol 48(1):50–55

    CAS  Google Scholar 

  20. Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandala M et al (2015) Diagnostic criteria for Meniere’s disease. J Vestib Res 25(1):1–7

    PubMed  Google Scholar 

  21. Sabbe A, Verhaert N, Joossen I, Lammens A, Debruyne F (2015) Otosclerosis: Shift in bone conduction after stapedotomy. B-ENT 11(3):183–189

    PubMed  CAS  Google Scholar 

  22. Carhart R, Porter LS (1971) Audiometric configuration and prediction of threshold for spondees. J Speech Hear Res 14(3):486–495

    Article  PubMed  CAS  Google Scholar 

  23. Jerger J (1970) Clinical experience with impedance audiometry. Arch Otolaryngol 92(4):311–324

    Article  PubMed  CAS  Google Scholar 

  24. Pohl F, Paasche G, Lenarz T, Schuon R (2017) Tympanometric measurements in conscious sheep-a diagnostic tool for pre-clinical middle ear implant studies. Int J Audiol 56(1):53–61

    Article  PubMed  Google Scholar 

  25. Hunter LL, Margolis R Middle ear measurement. Comprehensive handbook of pediatric audiology, 2010:365–88

  26. METZ O (1952) Threshold of reflex contractions of muscles of middle ear and recruitment of loudness. AMA Arch Otolaryngol 55(5):536–543

    Article  PubMed  CAS  Google Scholar 

  27. McGregor KD, Flamme GA, Tasko SM, Deiters KK, Ahroon WA, Themann CL, et al Acoustic reflexes are common but not pervasive: evidence using a diagnostic middle ear analyser. Int J Audiol 2017:1–9

  28. Verbist BM (2012) Imaging of sensorineural hearing loss: a pattern-based approach to diseases of the inner ear and cerebellopontine angle. Insights Imaging 3(2):139–153

    Article  PubMed  Google Scholar 

  29. Kastanioudakis I, Ziavra N, Politi EN, Exarchakos G, Drosos AA, Skevas A (2001) Hearing loss in progressive systemic sclerosis patients: a comparative study. Otolaryngol Head Neck Surg 124(5):522–525

    Article  PubMed  CAS  Google Scholar 

  30. Shenavandeh S, Haghighi MY, Nazarinia MA (2017) Nailfold digital capillaroscopic findings in patients with diffuse and limited cutaneous systemic sclerosis. Reumatologia 55(1):15–23

    PubMed  PubMed Central  Google Scholar 

  31. Mancini P, Atturo F, Di Mario A, Ginevra P, Ralli M, De Virgilio A, et al (2018) Hearing loss in autoimmune disorders: prevalence and therapeutic options. Autoimmun Rev

  32. Wigley FM, Flavahan NA (2016) Raynaud's phenomenon. N Engl J Med 375(6):556–565

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The present article was extracted from the thesis written by Dr. MahmoodReza Masoudi and financially supported by Shiraz University of Medical Science Grant number 94-01-01-10820. The authors would like to thank Shiraz University of Medical Sciences, Shiraz, Iran, and the audiologist Mrs. Maryam Abed; also, we thank the Center for Development of Clinical Research of Namazee Hospital and Dr. Nasrin Shokrpour for the editorial assistance.

Author information

Authors and Affiliations

  1. Departments of Internal Medicine, Division of Rheumatology, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz, 71345-1414, Iran

    S. Shenavandeh, MahmoodReza Masoudi, M. A. Nazarinia & A. Zare

  2. Department of Otolaryngology, Shiraz University of Medical Sciences, Shiraz, Iran

    S. B. Hashemi

  3. Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

    M. A. Nazarinia

Authors
  1. S. Shenavandeh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. S. B. Hashemi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. MahmoodReza Masoudi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. A. Nazarinia
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A. Zare
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to MahmoodReza Masoudi.

Ethics declarations

Disclosures

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shenavandeh, S., Hashemi, S.B., Masoudi, M. et al. Hearing loss in patients with scleroderma: associations with clinical manifestations and capillaroscopy. Clin Rheumatol 37, 2439–2446 (2018). https://doi.org/10.1007/s10067-018-4162-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-018-4162-7

Keywords

Search

Navigation