Utility of Laboratory Testing in Autoimmune Inner Ear Disease

Hirose, Keiko; Wener, Mark H.; Duckert, Larry G.

doi:10.1097/00005537-199911000-00005

Cited by 84 publications

(56 citation statements)

References 10 publications

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“…Harris and Sharp [10] first reported that a significant proportion of sera from patients affected by idiopathic SNHL showed antibody recognition of a 68-kDa protein in bovine inner ear extract which correlated with disease activity. Subsequent studies by other investigators reported that patients who were antibody-positive for the 68 kDa antigen responded to steroid treatment more frequently than non-responders [3,4,11]. The identity of this antigen was shown by Billing et al [11] to be HSP70, which is a nonspecific response associated with several inflammatory responses.…”

Section: Discussionmentioning

confidence: 95%

“…The identity of self-antigens responsible for disease induction and maintenance remain to be defined clearly. At present, diagnosis of AIED is by the exclusion of Menière's disease, retrocochlear disorders, otosclerosis, presbycusis, infectious or other autoimmune aetiologies and response to immunosuppressive therapy [1][2][3]. Available therapies are only partially effective, as progression in hearing loss has been documented regardless of treatment [4][5][6].…”

Section: Introductionmentioning

confidence: 99%

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Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss

Tebo

Szankasi

Hillman

et al. 2006

Clinical and Experimental Immunology

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SummaryDeafness is attributable to autoimmunity in a subset of adult patients with sensorineural hearing loss (SNHL) of unknown aetiology. To determine the roles of self-antigens in the pathogenesis of idiopathic SNHL, we analysed antibody responses to the inner ear-specific proteins, cochlin and b-tectorin as well as the non-specific heat shock protein 70 (HSP70). Recombinant cochlin and b-tectorin proteins were used in a qualitative Western blot assay for the detection of antigen-specific IgG antibodies in 58 patients with idiopathic SNHL and 28 healthy blood donors. In the same study cohort, we also used a Western blot assay to assess IgG antibody responses to the recombinant human HSP70. Of the 58 patient samples analysed, 19 tested positive to the HSP70, eight to cochlin and one to b-tectorin, giving a prevalence of 33, 14 and 2%, respectively. Only one patient sample was reactive for HSP70, cochlin and b-tectorin, seven of the remaining eight cochlin IgG antibody-positive samples were monospecific. Thus, cochlin-specific antibodies were observed predominantly in HSP70 IgG-negative patients demonstrating an additive value for testing this antibody response in patients with idiopathic SNHL.

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Section: Discussionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss

Tebo

Szankasi

Hillman

et al. 2006

Clinical and Experimental Immunology

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“…Aproximadamente 60% dos pacientes relatam vertigem 5 , o que coincide com o nosso estudo, onde 66% (N=2) dos pacientes apresentaram este sintoma. Na eletronistagmografia podemos encontrar comumente redução da resposta calórica.…”

Section: Discussionunclassified

“…No caso desses pacientes apresentarem resultados à eletrococleografia compatíveis com hydrops endolinfático (relação SP/AP > 0,33) e não apresentarem resposta à terapia imunossupressora, serão tidos como portadores de doença de Ménière. Entretanto, se estes pacientes apresentarem hydrops e resposta positiva à terapia imunossupressora, deverão ser classificados como portadores de disacusia imunomediada, manifestada por hydrops endolinfático 5 . A paciente relatada no caso 2 está inserida nesta classificação.…”

Section: Discussionunclassified

“…4 Médico Pós-Graduando (Mestrado) em Otorrinolaringologia pela UNIFESP-EPM. 5 O quadro clínico da DNSI é caracterizado por uma disacusia neurossensorial, geralmente bilateral, progressiva, associada a sintomas vestibulares (vertigens e instabilidade) e resposta vestibular diminuída ao exame otoneurológico. Em alguns casos, zumbido e plenitude aural podem estar presentes.…”

Section: Disacusia Neurossensorial Imunomediadaunclassified

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Disacusia neurossensorial imunomediada

Penido¹,

Aumond²,

Leonhardt³

et al. 2002

Rev. Bras. Otorrinolaringol.

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A disacusia neurossensorial imunomediada (DNSI) é caracterizada geralmente por uma disacusia neurossensorial bilateral, progressiva e assimétrica, acompanhada ou não por outros sintomas da orelha interna. Três pacientes com DNSI cujo quadro clínico e audiométrico eram sugestivos de doença auto-imune, e apresentaram resposta positiva à terapia imunossupressora ou pesquisa positiva de anticorpo anti hsp-70 68kD, foram estudados com relação às características clínicas, testes diagnósticos, alternativas terapêuticas e evolução da doença. Dois pacientes apresentaram quadro de disacusia neurossensorial rapidamente progressiva, associado a quadro vestibular, e outro, quadro de surdez súbita unilateral. Nenhum paciente apresentou positividade às provas reumatológicas, e apenas um paciente apresentou aumento na velocidade de hemossedimentação. Nenhum paciente obteve resposta adequada sustentada à corticoterapia, mas dois deles melhoraram com outras terapias imunossupressoras. O diagnóstico da DNSI é clínico e baseado na resposta positiva ao teste terapêutico com imunossupressores. A pesquisa de anticorpo anti-hsp70 de 68 kD pelo Western Blot é o único exame laboratorial específico para seu diagnóstico, possuindo sensibilidade de 42% e especificidade de 90%. Apenas 1 paciente apresentou positividade para este teste e não respondeu à terapia imunossupressora. Os dois pacientes com teste negativo responderam satisfatoriamente ao tratamento. A baixa sensibilidade do Western Blot e seu alto custo dificultam sua difusa utilização em nosso meio. A introdução precoce do tratamento é de suma importância por auxiliar no diagnóstico e por proporcionar um melhor prognóstico auditivo.

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Corticosteroid Response and Supporting Cell Antibody in Autoimmune Hearing Loss

Zeitoun¹,

Beckman²,

Arts³

et al. 2005

Arch Otolaryngol Head Neck Surg

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To determine whether antibodies to supporting cells are associated with response to corticosteroids in patients with autoimmune sensorineural hearing loss. Design: Prospective analysis of antibody to inner-ear antigens. Setting: Collaborating otology practices in Pennsylvania, Michigan, and Indiana. Patients: Sixty-three patients with rapidly progressive unilateral or bilateral sensorineural hearing loss of unknown cause suggestive of autoimmune sensorineural hearing loss. Interventions: Pretreatment audiometry, serum analysis by Western blot (WB) and immunofluorescence (IF) tests, corticosteroid therapy, and follow-up audiometry. Main Outcome Measures: Antibody reactivity and audiogram changes were analyzed for association with response to treatment. Results: More than half of the patients (37/63) had antibodies to both a 68-to 72-kDa protein and to innerear supporting cells, 16 patients had positive results on one assay only, and 10 had negative results on both. Twenty-eight patients improved and 35 did not. The WB findings did not correlate with response. Of the WBpositive patients, 49% (21/43) improved, as did 35% (7/ 20) of the WB-negative patients (P=.30). In contrast, 53% (25/47) of IF-positive patients improved, compared with only 19% (3/16) in the IF-negative group (P = .02). Of those who improved, 89% (25/28) were IF positive. Conclusions: Antibody to an inner-ear supporting cell antigen was significantly associated with hearing improvement after corticosteroid therapy (relative rate, 2.8). Patients with IF-positive serum are nearly 3 times more likely to experience improved hearing with corticosteroid treatment than those who are IF negative. Antibodies to inner-ear supporting cell antigen may have value in diagnosis and treatment of patients with autoimmune sensorineural hearing loss.

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Utility of Laboratory Testing in Autoimmune Inner Ear Disease

Cited by 84 publications

References 10 publications

Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss

Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss

Disacusia neurossensorial imunomediada

Corticosteroid Response and Supporting Cell Antibody in Autoimmune Hearing Loss

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