Autoimmune Sensorineural Hearing Loss

  • John Martin Hempel
  • Karin Schorn


The adaptive immunological system of our body consists of both a cellular and a humoral immune reaction. Both reactions are closely connected, comprising of a big number of factors. The T-cells (T-helper-cells, cytotoxic T-cells) (cellular immunoreactivity) have to rely upon antigen-presenting cells with their major histocompatibility complex II; they attack intracellular procreating pathogens, form lymphokine, they can opsonate viruses, and stimulate B-cells and other T- cells. Furthermore, they regulate other components of the immunological system. B-cells react to cellularly procreating bacteria by synthesizing all kinds of immunoglobulines (IgA, IgG, IgM, IgD, IgE) (humoral immunreactivity). Other important functions are performed by the inborn immunologic system, the complement system, and the phagocytizing cells. The principle of the immunological system is among others the detection and elimination of a foreign antigen, which has entered the body (bacterium, virus, parasite). The outstanding feature of our immunological system is its ability to distinguish between harmful foreign structures and the tissues and molecules of its own organism that need to be protected. In case of loss of this self-detection respectively in case of a dysfunction, namely a disorder of the regulating system within the auto-reactive immunocomponents, the development of an autoimmune disease is possible. This means that specific autoantigens formed by B-cells and/or sensitized lymphocytes, T-cells, but also natural killer-cells react against endogenous substances. Antibodies can be found in patients sera. This specific immunoreactivity can lead to cellysis respectively loss of tissue. Autoimmune diseases present a multifactorial occurrence.


Hearing Loss Sensorineural Hearing Loss Immunological System Sudden Hearing Loss Progressive Hearing Loss 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Berrettini S, Ravecca F, Bruschini L, Ursino F, Sellari-Franceschini S (1999) Progressive sensorineural hearing loss: immunologic etiology. Acta otorhinolaryngol Ital 18: 33–41Google Scholar
  2. 2.
    Billings PB, Keithley EM, Harris JP (1995) Evidence linking the 68 kilodalton antigen identified in progressive sensorineural hearing loss patient sera with heat shock protein 70. Ann Otol Rhinol Laryngol 104 (3): 181–188PubMedGoogle Scholar
  3. 3.
    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 (10): 1167–1171PubMedCrossRefGoogle Scholar
  4. 4.
    Hirose K, Wener MH, Duckert LG (1999) Utility of laboratory testing in autoimmune inner ear disease. Laryngoscope 109 (11): 1749–1754PubMedCrossRefGoogle Scholar
  5. 5.
    Hoistad DL, Schachern PA, Paparella MM (1998) Autoimmune Sensorineural Hearing Loss: A Human Temporal Bone Study. Am J Otolaryngol 19 (l): 33–39PubMedCrossRefGoogle Scholar
  6. 6.
    Lehnhardt E (1958) Plötzliche Hörstörungen auf beiden Seiten gleichzeitig oder nacheinander aufgetretend. Z Laryngol Rhinol Otol 37: 1–17PubMedGoogle Scholar
  7. 7.
    McCabe BF (1979) Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 88 (5 Ptl): 585–589PubMedGoogle Scholar
  8. 8.
    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. Correlation with disease activity and response to corticosteroid treatment. Jama 272 (8): 611–616PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Tokyo 2004

Authors and Affiliations

  • John Martin Hempel
    • 1
  • Karin Schorn
    • 1
  1. 1.Department of OtorhinolaryngologyLudwig-Maximilians-University of MunichMunichGermany

Personalised recommendations