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. 1. Introduction Sudden sensorineural hearing loss (SSNHL) is defined as a loss of 30？dB or more at one or more frequencies over a period of 3 days or less; it is a frustrating and frightening condition, especially if the hearing loss is bilateral. Although vascular and viral mechanisms have been implicated in the aetiology of SSNHL, a high prevalence of autoantibody titer was reported in cases of bilateral SSNHL, possibly suggesting an underlying autoimmune process [1]. We present here a case of bilateral SSNHL associated with the involvement of autoantibodies in the presence of a certain human leukocyte antigen (HLA) haplotype, the HLA A1-B8-DR3 superhaplotype. 2. Case Report A 28-year-old female presented to our clinic complaining of bilateral hearing loss, tinnitus, intense rotatory vertigo, and nausea. She reported that, during the previous two weeks, she experienced 2 episodes of vertigo lasting several hours with associated nausea and vomiting, fullness in her right ear, and pain in both ears. A CT scan and an MRI were normal. Upon admission, otoneurological examination revealed horizontal nystagmus with fast phase to the left. Otoscopy was normal. The tonic audiogram showed bilateral sensorineural hearing loss, greater in the right ear (Figure 1 ); in the right ear the hearing loss was moderate with deterioration at high frequencies; in the left ear mild hearing loss was present also with deterioration at high frequencies. The patient was administered
References
[1]
B. L. Fetterman, W. M. Luxford, and J. E. Saunders, “Sudden bilateral sensorineural hearing loss,” Laryngoscope, vol. 106, no. 11, pp. 1347–1350, 1996.
[2]
C. E. Larsen and C. A. Alper, “The genetics of HLA-associated disease,” Current Opinion in Immunology, vol. 16, no. 5, pp. 660–667, 2004.
[3]
D. Lio, G. Candore, A. Colombo et al., “A genetically determined high setting of TNF-α influences immunologic parameters of HLA-B8,DR3 positive subjects: implications for autoimmunity,” Human Immunology, vol. 62, no. 7, pp. 705–713, 2001.
[4]
C. Caruso, G. Candore, M. A. Modica et al., “Major histocompatibility complex regulation of cytokine production,” Journal of Interferon and Cytokine Research, vol. 16, no. 12, pp. 983–988, 1996.
[5]
G. Candore, D. Lio, G. C. Romano, and C. Caruso, “Pathogenesis of autoimmune diseases associated with 8.1 ancestral haplotype: effect of multiple gene interactions,” Autoimmunity Reviews, vol. 1, no. 1-2, pp. 29–35, 2002.
[6]
J. M. Bernstein, T. C. Shanahan, and F. M. Schaffer, “Further observations on the role of the MHC genes and certain hearing disorders,” Acta Oto-Laryngologica, vol. 116, no. 5, pp. 666–671, 1996.
[7]
S. W. Yeo, K.-H. Chang, B.-D. Suh, T.-G. Kim, and H. Han, “Distribution of HLA-A, -B and -DRB1 alleles in patients with sudden sensorineural hearing loss,” Acta Oto-Laryngologica, vol. 120, no. 6, pp. 710–715, 2000.
[8]
J. C. Amor-Dorado, L. Paco, J. Martin, M. A. Lopez-Nevot, and M. A. Gonzalez-Gay, “Human leukocyte antigen-DQB1 and -DRB1 associations in patients with idiopathic sudden sensorineural hearing loss from a defined population of Northwest Spain,” Acta Oto-Laryngologica, vol. 125, no. 12, pp. 1277–1282, 2005.
[9]
S. W. Yeo, S.-N. Park, Y.-S. Park et al., “Different distribution of HLA class II alleles according to response to corticosteroid therapy in sudden sensorineural hearing loss,” Archives of Otolaryngology—Head & Neck Surgery, vol. 127, no. 8, pp. 945–949, 2001.
[10]
J. Xenellis, A. W. Morrison, D. McClowskey, and H. Festenstein, “HLA antigens in the pathogenesis of Meniere's disease,” Journal of Laryngology and Otology, vol. 100, no. 1, pp. 21–24, 1986.
[11]
L. Melchiorri, A. Martini, R. Rizzo, A. Berto, E. Adinolfi, and O. R. Baricordi, “Human leukocyte antigen-A, -B, -C and -DR alleles and soluble human leukocyte antigen class I serum level in Ménière's disease,” Acta Oto-Laryngologica, Supplement, vol. 548, pp. 26–29, 2002.
[12]
A. Greco, M. Fusconi, A. Gallo, C. Marinelli, G. F. Macri, and M. de Vincentiis, “Sudden sensorineural hearing loss: an autoimmune disease?” Autoimmunity Reviews, vol. 10, no. 12, pp. 756–761, 2011.
[13]
C. A. Solares, G. B. Hughes, and K. K. Tuohy, “Immunologic disorders of the inner ear,” in Clinical Otology, G. B. Hughes and M. L. Pensak, Eds., pp. 390–399, Thieme, New York, NY, USA, 3rd edition, 2006.
[14]
T. H. Alexander, M. H. Weisman, J. M. Derebery et al., “Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease,” Otology and Neurotology, vol. 30, no. 4, pp. 443–448, 2009.
[15]
L. S. Parnes, A.-H. Sun, and D. J. Freeman, “Corticosteroid pharmacokinetics in the inner ear fluids: an animal Study followed by clinical application,” Laryngoscope, vol. 109, no. 7, part 2, pp. 1–17, 1999.
[16]
J. R. Berrocal and R. Ramírez-Camacho, “Sudden sensorineural hearing loss: supporting the immunologic theory,” Annals of Otology, Rhinology and Laryngology, vol. 111, no. 11, pp. 989–997, 2002.
[17]
S. Ho, N. Clipstone, L. Timmermann et al., “The mechanism of action of cyclosporin A and FK506,” Clinical Immunology and Immunopathology, vol. 80, no. 3, part 2, pp. S40–S45, 1996.
