Autoimmune Inner Ear Disease

ABSTRACT

Autoimmune inner ear disease (AIED), first reported by McCabe in 1979, describes a disease process in which cochleovestibular is compromised by one's own immune system. Only indirect laboratory evidence exists regarding the underlying immune reaction, which can only be confirmed histopathologically in postmortem studies. Diagnosis involves a thorough history and complete head and neck, otomicroscopic, and audiometric evaluation. AIED classically presents with bilateral, fluctuating, or rapidly progressive asymmetric sensorineural hearing loss (SNHL), which typically occurs over weeks to months but can occur suddenly over a period of a few hours or days. Fifty percent of patients have vestibular symptoms as well, which can be unilateral or bilateral. A lengthy serological workup is unwarranted given the absence of a reliable diagnostic laboratory test. Antibodies to the 68-kDa protein (heat shock protein-70) is marker-specific but not sensitive for AIED and may correlate with steroid responsiveness. In cases where the diagnosis remains unclear, a prolonged course of steroids, with repeat audiometric testing at 1 month, can be undertaken. Treatment aims to inhibit the detrimental immune response using immunosuppressant drugs; however, no standardized treatment regimen has been found. Cochlear implantation remains a viable last resort for patients with progressive SNHL.

KEYWORDS

Sensorineural hearing loss - autoimmune inner ear disease - endolymphatic hydrops - dizziness

REFERENCES

• 1 Tan C Q, Dong W D, Guo L, Huang H, Wang D Y. Auditory function in women with autoimmune inner ear diseases and their offspring.  Int J Pediatr Otorhinolaryngol. 2009;  73 1702-1711
• 2 Rauch S D. Autoimmune inner ear disease. In: Jackler R K, Brackmann D E, eds. Neurotology. 2nd ed. Philadelphia, PA: Mosby; 2005: 639-643
• 3 Roland J T. Autoimmune inner ear disease.  Curr Rheumatol Rep. 2000;  2 171-174
• 4 McCabe B F. Autoimmune sensorineural hearing loss.  Ann Otol Rhinol Laryngol. 1979;  88 (5 Pt 1) 585-589
• 5 Lehnhardt E. [Sudden hearing disorders occurring simultaneously or successively on both sides]. [in German].  Z Laryngol Rhinol Otol. 1958;  37 1-16
• 6 Buniel M C, Geelan-Hansen K, Weber P C, Tuohy V K. Immunosuppressive therapy for autoimmune inner ear disease.  Immunotherapy. 2009;  1 425-434
• 7 Gloddek B, Arnold W. Clinical and experimental studies of autoimmune inner ear disease.  Acta Otolaryngol Suppl. 2002;  548 10-14
• 8 Agrup C, Luxon L M. Immune-mediated inner-ear disorders in neuro-otology.  Curr Opin Neurol. 2006;  19 26-32
• 9 García-Berrocal J R, Ramírez-Camacho R, Millán I et al.. Sudden presentation of immune-mediated inner ear disease: characterization and acceptance of a cochleovestibular dysfunction.  J Laryngol Otol. 2003;  117 775-779
• 10 Boulassel M R, Deggouj N, Tomasi J P, Gersdorff M. Inner ear autoantibodies and their targets in patients with autoimmune inner ear diseases.  Acta Otolaryngol. 2001;  121 28-34
• 11 Mouadeb D A, Ruckenstein M J. Antiphospholipid inner ear syndrome.  Laryngoscope. 2005;  115 879-883
• 12 Cadoni G, Fetoni A R, Agostino S et al.. Autoimmunity in sudden sensorineural hearing loss: possible role of anti-endothelial cell autoantibodies.  Acta Otolaryngol Suppl. 2002;  (548) 30-33
• 13 Passali D, Damiani V, Mora R, Passali F M, Passali G C, Bellussi L. P0 antigen detection in sudden hearing loss and Ménière's disease: a new diagnostic marker?.  Acta Otolaryngol. 2004;  124 1145-1148
• 14 Loveman D M, de Comarmond C, Cepero R, Baldwin D M. Autoimmune sensorineural hearing loss: clinical course and treatment outcome.  Semin Arthritis Rheum. 2004;  34 538-543
• 15 Zingler V C, Cnyrim C, Jahn K et al.. Causative factors and epidemiology of bilateral vestibulopathy in 255 patients.  Ann Neurol. 2007;  61 524-532
• 16 Cohen S, Shoup A, Weisman M H, Harris J. Etanercept treatment for autoimmune inner ear disease: results of a pilot placebo-controlled study.  Otol Neurotol. 2005;  26 903-907
• 17 Rawal S G, Thakkar K H, Ziai K, Santi P A, Djalilian H R. HLA-B27-associated bilateral Ménière disease.  Ear Nose Throat J. 2010;  89 122-127
• 18 Fuse T, Hayashi T, Oota N et al.. Immunological responses in acute low-tone sensorineural hearing loss and Ménière's disease.  Acta Otolaryngol. 2003;  123 26-31
• 19 Quaranta A, Scaringi A, Sallustio V, Quaranta N. Cochlear function in ears with immunomediated inner ear disorder.  Acta Otolaryngol Suppl. 2002;  (548) 15-19
• 20 Hervier B, Bordure P, Audrain M, Calais C, Masseau A, Hamidou M. Systematic screening for nonspecific autoantibodies in idiopathic sensorineural hearing loss: no association with steroid response.  Otol Neurotol. 2010;  31 687-690
• 21 García-Berrocal J R, Trinidad A, Ramírez-Camacho R, Lobo D, Verdaguer M, Ibáñez A. Immunologic work-up study for inner ear disorders: looking for a rational strategy.  Acta Otolaryngol. 2005;  125 814-818
• 22 Suzuki M, Hanamitsu M, Kitanishi T, Kohzaki H, Kitano H. Autoantibodies against inner ear proteins in patients with delayed endolymphatic hydrops and unilateral juvenile deafness.  Acta Otolaryngol. 2006;  126 117-121
• 23 Alexander T H, Weisman M H, Derebery J M et al.. Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease.  Otol Neurotol. 2009;  30 443-448
• 24 Hamid M H, Trune D. Issues, indications, and controversies regarding intratympanic steroid perfusion.  Curr Opin Otolaryngol Head Neck Surg. 2008;  16 434-440
• 25 Light J P, Silverstein H. Transtympanic perfusion: indications and limitations.  Curr Opin Otolaryngol Head Neck Surg. 2004;  12 378-383
• 26 Yang G SY, Song H T, Keithley E M, Harris J P. Intratympanic immunosuppressives for prevention of immune-mediated sensorineural hearing loss.  Am J Otol. 2000;  21 499-504
• 27 Harris J P, Weisman M H, Derebery J M et al.. Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial.  JAMA. 2003;  290 1875-1883