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.
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References
Heyworth T, Liyanage SP (1972) A pilot survey of hearing loss in patients with rheumatoid arthritis. Scand J Rheumatol 1(2):81–83
Goodwill CJ, Lord IJ, Jones RP (1972) Hearing in rheumatoid arthritis. A clinical and audiometric survey. Ann Rheum Dis 31(3):170–173
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
Baradaranfar MH, Doosti A (2010) A survey of relationship between rheumatoid arthritis and hearing disorders. Acta Med Iran 48(6):371–373
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
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
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
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
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
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
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
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
Christian CL, Elkon KB (1986) Autoantibodies to intracellular proteins. Clinical and biologic significance. Am J Med 80(1):53–61
Van Venrooij WJ, Sillekens PT (1989) Small nuclear RNA associated proteins: autoantigens in connective tissue diseases. Clin Exp Rheumatol 7(6):635–645
Mohan C, Datta SK (1995) Lupus: key pathogenic mechanisms and contributing factors. Clin Immunol Immunopathol 77(3):209–220
McCabe BF (1979) Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 88(5 Pt 1):585–589
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
Recommandation BIAP 02/1: Structures en audiophonologie (1997). https://www.biap.org/
Carhart R (1945) An improved method for classifying audiograms. Laryngoscope 55:640–662
Metz O (1952) Threshold of reflex contractions of muscles of middle ear and recruitment of loudness. AMA Arch Otolaryngol. 55(5):536–543
Wong AC, Ryan AF (2015) Mechanisms of sensorineural cell damage, death and survival in the cochlea. Front Aging Neurosci. 21(7):58
Emamifar A, Bjoerndal K, Hansen IM (2016) Is hearing impairment associated with rheumatoid arthritis? A Review. Open Rheumatol J. 15(10):26–32
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
Tumiati B, Casoli P, Parmeggiani A (1997) Hearing loss in the Sjögren syndrome. Ann Intern Med. 126(6):450–453
Rabelo MB, Corona AP (2014) Auditory and vestibular dysfunctions in systemic sclerosis: literature review. Codas 26(5):337–342
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
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
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
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
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
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
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
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
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This study was funded by the University of Ioannina.
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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
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DOI: https://doi.org/10.1007/s00405-019-05487-5