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
Hughes G, Kinney S, Barna B, Calabrese L, Hamid M (1983) Autoimmune reactivity in Menière’s disease: preliminary report. Laryngoscope 43:410–417
Gloddek B, Arnold W (2002) Clinical and experimental studies of autoimmune inner ear disease. Acta Otolaryngol Suppl 548:10–14
Soliman A (1992) Immune-mediated inner ear disease. Am J Otol 6:575–579
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
Soliman A (1989) Experimental autoimmune inner ear disease. Laryngoscope 99:188–193
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
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
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
Harris J, Sharp P (1990) Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss. Laryngoscope 100:516–524
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
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
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
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
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
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
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
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
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
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
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
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
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
Arnold W, Weidauer H, Seelig H (1976) Experimentellerbeweiß einer gemeinsamen Anti-genizitat zwischen Innenohr und Niere. Arch Otorhinolaryngol 212:99–117
Zeitoun H, Beckman JG, Arts HA et al (2005) Arch Otolaryngol Head Neck Surg 131:665–672
Gray et al (1999) ARO abstracts #246
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
Agrup C, Luxon LM (2006) Immune-mediated inner-ear disorders in neuro-otology. Curr Opin Neurol 19(1):26–32
Bovo R, Aimoni C, Martini A (2006) Immune-mediated inner ear disease. Acta Otolaryngol 126(10):1012–1021
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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, 37–40 (2009). https://doi.org/10.1007/s00405-008-0801-y
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DOI: https://doi.org/10.1007/s00405-008-0801-y