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The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls

Abstract

The purpose of this paper is to review the current diagnostic work-up for patients with suspected Autoimmune Inner Ear Disease (AIED). AIED is a rare disease accounting for less than 1% of all cases of hearing impairment or dizziness, characterized by a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. While specific tests for autoimmunity to the inner ear would be valuable, at the time of writing, there are none that are both commercially available and proven to be useful. Thus far, most of the identified antigens lack a clear association with localized inner ear pathology and the diagnosis of AIED is based either on clinical criteria and/or on a positive response to steroids. For clinical practice, we recommend an antigen-non-specific test battery including blood test for autoimmune disorders and for conditions that resemble autoimmune disorders. Nevertheless, if financial resources are limited, a very restricted work-up study may have a similar efficiency.

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References

  1. Hughes G, Kinney S, Barna B, Calabrese L, Hamid M (1983) Autoimmune reactivity in Menière’s disease: preliminary report. Laryngoscope 43:410–417

    Google Scholar 

  2. Gloddek B, Arnold W (2002) Clinical and experimental studies of autoimmune inner ear disease. Acta Otolaryngol Suppl 548:10–14

    PubMed  Article  Google Scholar 

  3. Soliman A (1992) Immune-mediated inner ear disease. Am J Otol 6:575–579

    Google Scholar 

  4. Hefeneider SH, McCoy SL, Hausman FA, Trune DR (2004) Autoimmune mouse antibodies recognize multiple antigens proposed in human immune-mediated hearing loss. Otol Neurotol 25:250–256

    PubMed  Article  Google Scholar 

  5. Soliman A (1989) Experimental autoimmune inner ear disease. Laryngoscope 99:188–193

    PubMed  Article  CAS  Google Scholar 

  6. Bernstein JM, Shanahan TC, Schaffer FM (1996) Further observations on the role of the MHC genes and certain hearing disorders. Acta Otolaryngol 116(5):666–671

    PubMed  Article  CAS  Google Scholar 

  7. García-Berrocal JR, Ramírez-Camacho R, González-García JA, Verdaguer JM, Trinidad A (2008) Does the serological study for viral infection in autoimmune inner ear disease make sense? ORL J Otorhinolaryngol Relat Spec 70:16–19

    PubMed  Google Scholar 

  8. Berger P, Hillman M, Tabak M, Vollrath M (1991) The lymphocyte transformation test with type II collagen as a diagnostic tool of autoimmune sensorineural hearing loss. Laryngoscope 101:895–899

    PubMed  Article  CAS  Google Scholar 

  9. Harris J, Sharp P (1990) Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss. Laryngoscope 100:516–524

    PubMed  Article  CAS  Google Scholar 

  10. Moscicki RA, San Martin JE, Quintero CH, Rauch SD, Nadol JB, Bloch KJ (1994) Serum antibody to inner ear proteins in patients with progressive hearing loss. JAMA 272:611–661

    PubMed  Article  CAS  Google Scholar 

  11. Gottschlich S, Billings PB, Keithley EM, Weisman MH, Harris JP (1995) Assessment of serum antibodies in patients with rapidly progressive sensorineural hearing loss and Meniere’s disease. Laryngoscope 105:1347–1352

    PubMed  Article  CAS  Google Scholar 

  12. Billings P, Keithley E, Harris J (1995) Evidence linking the 68 kilo Dalton antigen identified in progressive sensorineural hearing loss patient sera with heat shock protein 70. Ann Otol Rhinol Laryngol 104:181–188

    PubMed  CAS  Google Scholar 

  13. Bloch DB, San Martin JE, Rauch SD, Moscicki RA, Bloch KJ (1995) Serum antibodies to heat shock protein 70 in sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 121:1167–1171

    PubMed  CAS  Google Scholar 

  14. Yeom K, Gray J, Nair TS, Arts HA, Telian SA, Disher MJ, El-Kashlan H, Sataloff RT, Fisher SG, Carey TE (2003) Antibodies to HSP-70 in normal donors and autoimmune hearing loss patients. Laryngoscope 113:1770–1776

    PubMed  Article  CAS  Google Scholar 

  15. García Berrocal JR, Ramírez-Camacho R, Vargas JA, Millan I (2002) Does the serological testing really play a role in the diagnosis immune-mediated inner ear disease? Oto-Laryngologica 122:243–248

    Article  Google Scholar 

  16. Shin SO, Billings PB, Keithley EM, Harris JP (1997) Comparison of anti-heat shock protein 70 (anti-hsp70) and anti-68-kDa inner ear protein in the sera of patients with Meniére disease. Laryngoscope 107:222–227

    PubMed  Article  CAS  Google Scholar 

  17. Yoo TJ, Yazawa Y (2003) Immunology of cochlear and vestibular disorders. In: Luxon L., Furman JM, Martini A, Stephens D (eds) Audiological medicine clinical aspects of hearing and balance. Martin Dunitz, Taylor and Francis, London, pp 74–75

  18. Savastano M, Giacomelli L, Marioni G (2007) Non-specific immunological determinations in Meniere’s disease: any role in clinical practice? Eur Arch Otorhinolaryngol 264(1):15–19

    PubMed  Article  Google Scholar 

  19. Cao M, Gersdorff M, Deggouj N, Warny M, Tomasi J (1995) Detection of inner ear disease autoantibodies by immunoblotting. Mol Cell Biochem 24(146):157–163

    Article  Google Scholar 

  20. Passali D, Damiani V, Mora R, Passali FM, Passali GC, Bellussi L (2004) P0 antigen detection in sudden hearing loss and Meniere’s disease: a new diagnostic marker? Acta Otolaryngol 124(10):1145–1148

    PubMed  Article  Google Scholar 

  21. Pham BN, Rudic M, Bouccara D, Sterkers O, Belmatoug N, Bebear JP, Couloigner V, Fraysse B, Gentine A, Ionescu E, Robier A, Sauvage JP, Truy E, Van Den Abbeele T, Ferrary E (2007) Antibodies to myelin protein zero (P0) protein as markers of auto-immune inner ear diseases. Autoimmunity 40(3):202–207

    PubMed  Article  CAS  Google Scholar 

  22. Plester D, Zanetti F, Berg P et al (1988) Diagnostic laboratory tool for immune-mediated sensorineural hearing loss. In: Veldman JE, McCabe BF (eds) Immunobiology, histopathology and tumor immunology in otolaryngology. Kugler Publications, Amsterdam, pp 33–37

    Google Scholar 

  23. Arnold W, Weidauer H, Seelig H (1976) Experimentellerbeweiß einer gemeinsamen Anti-genizitat zwischen Innenohr und Niere. Arch Otorhinolaryngol 212:99–117

    PubMed  Article  CAS  Google Scholar 

  24. Zeitoun H, Beckman JG, Arts HA et al (2005) Arch Otolaryngol Head Neck Surg 131:665–672

    PubMed  Article  Google Scholar 

  25. Gray et al (1999) ARO abstracts #246

  26. García-Berrocal JR, Trinidad A, Ramírez-Camacho R, Lobo D, Verdaguer M, Ibáñez A (2005) Immunologic work-up study for inner ear disorders: looking for a rational strategy. Acta Otolaryngol 125(8):814–818

    PubMed  Article  Google Scholar 

  27. Agrup C, Luxon LM (2006) Immune-mediated inner-ear disorders in neuro-otology. Curr Opin Neurol 19(1):26–32

    PubMed  CAS  Article  Google Scholar 

  28. Bovo R, Aimoni C, Martini A (2006) Immune-mediated inner ear disease. Acta Otolaryngol 126(10):1012–1021

    PubMed  Article  CAS  Google Scholar 

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The authors declare that they have no conflict of interest to report.

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Correspondence to Roberto Bovo.

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Bovo, R., Ciorba, A. & Martini, A. The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. Eur Arch Otorhinolaryngol 266, 37 (2009). https://doi.org/10.1007/s00405-008-0801-y

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  • DOI: https://doi.org/10.1007/s00405-008-0801-y

Keywords

  • Immunology
  • Cochlear disorders
  • Vestibular disorders
  • Hearing loss