Skip to main content

Advertisement

Log in

Cochlear involvement in patients with systemic autoimmune rheumatic diseases: a clinical and laboratory comparative study

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

Inner ear involvement has been reported in systemic rheumatic disease while detection of cochlin-specific antibodies has been reported in patients with idiopatic sensorineural hearing loss, suggesting cochlin’s strong link to autoimmune hearing loss. The aim of this cross-sectional study was to calculate the prevalence of sensorineural hearing loss (SNHL) in patients with systemic rheumatic diseases, and to investigate any potential correlation with human antibodies to cochlin.

Methods

Patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren’s syndrome (SS) and systemic sclerosis (SSc) according to the criteria of American College of Rheumatology were included in the study. All patients underwent a complete ear-nose-throat physical examination and audiological evaluation with pure tone audiometry and impedance audiometry. Pure tone average was calculated, taking as a starting point the hearing loss in dB according to the recommendation 02/1 of “Bureau International d' Audiophonologie” (BIAP) so as an average hearing threshold value. Sera of all patients were tested for the presence of IgG antibodies to human cochline (COCH-IgG). Sex and age-matched healthy subjects were included as controls to each group.

Results

A total of 133 patients were studied; 60 with RA, 41 with SLE, 24 with SS and 8 with SSc. 61.4% of patients reported vertigo, 41% hyperacousis, 39% hearing loss, 38% tinnitus, 37.9% headache and 2.1% sensation of ear pressure with unremarkable otoscopy. The prevalence of SNHL calculated for patients affected by RA, SLE, SS and SSc was 66.6%, 31.71%, 54.17%, and 75% respectively. The calculated average hearing thresholds value in RA was increased in comparison to SLE (p < 0.05). In addition it was also higher in patients with RA and secondary SS, in comparison to RA patients (p > 0.05). There was statistically significant correlation of average hearing threshold with disease activity score 28 (DAS28) in RA, but no correlation observed with disease activity index (SLEDAI) in SLE. COCH-IgG antibodies were detected in only two samples. The results were compared with those of their respective sex and age-matched healthy subjects.

Conclusion

Our study revealed increased prevalence of SNHL in patients with systemic autoimmune rheumatic disease but no correlation of hearing loss with COCHIgG antibodies. The mechanism of inner ear damage remains unknown; thus, additional prospective studies will be needed to elucidate its pathogenesis.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Heyworth T, Liyanage SP (1972) A pilot survey of hearing loss in patients with rheumatoid arthritis. Scand J Rheumatol 1(2):81–83

    Article  CAS  PubMed  Google Scholar 

  2. Goodwill CJ, Lord IJ, Jones RP (1972) Hearing in rheumatoid arthritis. A clinical and audiometric survey. Ann Rheum Dis 31(3):170–173

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Pascual-Ramos V, Contreras-Yáñez I, Enríquez L, Valdés S, Ramírez-Anguiano J (2012) Hearing impairment in a tertiary-care-level population of Mexican rheumatoid arthritis patients. J Clin Rheumatol 18(8):393–398

    Article  PubMed  Google Scholar 

  4. Baradaranfar MH, Doosti A (2010) A survey of relationship between rheumatoid arthritis and hearing disorders. Acta Med Iran 48(6):371–373

    PubMed  Google Scholar 

  5. de la Lasso VM, Villarreal IM, López Moya J, García-Berrocal JR (2016) Extended high frequency audiometry can diagnose sub-clinic involvement in a seemingly normal hearing systemic lupus erythematosus population. Acta Otolaryngol 9:1–6

    Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  7. 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

    Article  PubMed  Google Scholar 

  8. Freeman SR, Sheehan PZ, Thorpe MA, Rutka JA (2005) Ear, nose, and throat manifestations of Sjögren’s syndrome: retrospective review of a multidisciplinary clinic. J Otolaryngol 34(1):20–24

    Article  PubMed  Google Scholar 

  9. Hatzopoulos S, Amoroso C, Aimoni C, Lo Monaco A, Govoni M, Martini A (2002) Hearing loss evaluation of Sjögren’s syndrome using distortion product otoacoustic emissions. Acta Otolaryngol Suppl 548:20–25

    Article  Google Scholar 

  10. Boki KA, Ioannidis JP, Segas JV, Maragkoudakis PV, Petrou D, Adamopoulos GK, Moutsopoulos HM (2001) How significant is sensorineural hearing loss in primary Sjögren’s syndrome? An individually matched case-control study. J Rheumatol 28(4):798–801

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  12. 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 

  13. Christian CL, Elkon KB (1986) Autoantibodies to intracellular proteins. Clinical and biologic significance. Am J Med 80(1):53–61

    Article  CAS  PubMed  Google Scholar 

  14. Van Venrooij WJ, Sillekens PT (1989) Small nuclear RNA associated proteins: autoantigens in connective tissue diseases. Clin Exp Rheumatol 7(6):635–645

    PubMed  Google Scholar 

  15. Mohan C, Datta SK (1995) Lupus: key pathogenic mechanisms and contributing factors. Clin Immunol Immunopathol 77(3):209–220

    Article  CAS  PubMed  Google Scholar 

  16. McCabe BF (1979) Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 88(5 Pt 1):585–589

    Article  CAS  PubMed  Google Scholar 

  17. Baek MJ, Park HM, Johnson JM, Altuntas CZ, Jane-Wit D, Jaini R, Solares CA, Thomas DM, Ball EJ, Robertson NG, Morton CC, Hughes GB, Tuohy VK (2006) Increased frequencies of cochlin-specific T cells in patients with autoimmune sensorineural hearing loss. J Immunol 177(6):4203–4210

    Article  CAS  PubMed  Google Scholar 

  18. Recommandation BIAP 02/1: Structures en audiophonologie (1997). https://www.biap.org/

  19. Carhart R (1945) An improved method for classifying audiograms. Laryngoscope 55:640–662

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  21. Wong AC, Ryan AF (2015) Mechanisms of sensorineural cell damage, death and survival in the cochlea. Front Aging Neurosci. 21(7):58

    Google Scholar 

  22. Emamifar A, Bjoerndal K, Hansen IM (2016) Is hearing impairment associated with rheumatoid arthritis? A Review. Open Rheumatol J. 15(10):26–32

    Article  CAS  Google Scholar 

  23. Montoya-Aranda IM, Peñaloza-López YR, Gutiérrez-Tinajero DJ (2010) Sjögren’s syndrome: Audiological and clinical behaviour in terms of age. Acta Otorrinolaringol Esp 61(5):332–337

    Article  PubMed  Google Scholar 

  24. Tumiati B, Casoli P, Parmeggiani A (1997) Hearing loss in the Sjögren syndrome. Ann Intern Med. 126(6):450–453

    Article  CAS  PubMed  Google Scholar 

  25. Rabelo MB, Corona AP (2014) Auditory and vestibular dysfunctions in systemic sclerosis: literature review. Codas 26(5):337–342

    Article  PubMed  Google Scholar 

  26. Tenório JP, Guimarães JA, Flores NG, Iório MC (2011) Comparison between classification criteria of audiometric findings in elderly. J Soc Bras Fonoaudiol 23(2):114–118

    Article  PubMed  Google Scholar 

  27. Robertson NG, Hamaker SA, Patriub V, Aster JC, Morton CC (2003) Subcellular localisation, secretion, and post-translational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9. J Med Genet. 40(7):479–486

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Robertson NG, Khetarpal U, Gutiérrez-Espeleta GA, Bieber FR, Morton CC (1994) Isolation of novel and known genes from a human fetal cochlear cDNA library using subtractive hybridization and differential screening. Genomics 23(1):42–50

    Article  CAS  PubMed  Google Scholar 

  29. de Kok YJ, Bom SJ, Brunt TM, Kemperman MH, van Beusekom E, van der Velde-Visser SD, Robertson NG, Morton CC, Huygen PL, Verhagen WI, Brunner HG, Cremers CW, Cremers FP (1999) A Pro51Ser mutation in the COCH gene is associated with late onset autosomal dominant progressive sensorineural hearing loss with vestibular defects. Hum Mol Genet 8(2):361–366

    Article  PubMed  Google Scholar 

  30. Usami S, Takahashi K, Yuge I, Ohtsuka A, Namba A, Abe S, Fransen E, Patthy L, Otting G, Van Camp G (2003) Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere’s disease. Eur J Hum Genet 11(10):744–748

    Article  CAS  PubMed  Google Scholar 

  31. Kamarinos M, McGill J, Lynch M, Dahl H (2001) Identification of a novel COCH mutation, I109N, highlights the similar clinical features observed in DFNA9 families. Hum Mutat 17(4):351

    Article  CAS  PubMed  Google Scholar 

  32. Tebo AE, Szankasi P, Hillman TA, Litwin CM, Hill HR (2006) Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss. Clin Exp Immunol 146(3):427–432

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Boulassel M, Deggouj N, Tomasi J et al (2001) Inner ear autoantibodies and their targets in patients with autoimmune inner ear diseases. Acta Otolaryngol 121:28–34

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

This study was funded by the University of Ioannina.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Georgios K. Tsirves.

Ethics declarations

Research involving human and animal rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00405-019-05487-5

Keywords

Navigation