Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype

Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date speci...

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Main Authors: G. Psillas, M. Daniilidis, A. Gerofotis, K. Veros, A. Vasilaki, I. Vital, K. Markou
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Otolaryngology
Online Access:http://dx.doi.org/10.1155/2013/590157
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author G. Psillas
M. Daniilidis
A. Gerofotis
K. Veros
A. Vasilaki
I. Vital
K. Markou
author_facet G. Psillas
M. Daniilidis
A. Gerofotis
K. Veros
A. Vasilaki
I. Vital
K. Markou
author_sort G. Psillas
collection DOAJ
description Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.
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publishDate 2013-01-01
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series Case Reports in Otolaryngology
spelling doaj-art-357f454b15454c17bdf42cb0c2adaa352025-08-20T02:24:08ZengWileyCase Reports in Otolaryngology2090-67652090-67732013-01-01201310.1155/2013/590157590157Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 HaplotypeG. Psillas0M. Daniilidis1A. Gerofotis2K. Veros3A. Vasilaki4I. Vital5K. Markou61st Academic ENT Department, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, GreeceClinical Immunology and Immunogenetics Lab., 1st Department of Internal Medicine, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, GreeceClinical Immunology and Immunogenetics Lab., 1st Department of Internal Medicine, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, Greece1st Academic ENT Department, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, Greece1st Department of Internal Medicine, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, Greece1st Academic ENT Department, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, Greece1st Academic ENT Department, Ahepa University Hospital, Aristotle University, 54006 Thessaloniki, GreeceSudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.http://dx.doi.org/10.1155/2013/590157
spellingShingle G. Psillas
M. Daniilidis
A. Gerofotis
K. Veros
A. Vasilaki
I. Vital
K. Markou
Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
Case Reports in Otolaryngology
title Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
title_full Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
title_fullStr Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
title_full_unstemmed Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
title_short Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype
title_sort sudden bilateral sensorineural hearing loss associated with hla a1 b8 dr3 haplotype
url http://dx.doi.org/10.1155/2013/590157
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