Autoimmune Inner Ear Disease

  • Diagnosis
  • Background
  • Lab Tests
  • References
  • Related Topics

Indications for Testing

  • Appropriate clinical presentation and exclusion of other causes of hearing loss, including Ménière disease, other autoimmune diseases (with associated hearing loss), otosclerosis, retrocochlear disorders and infectious diseases such as syphilis and Lyme disease

Criteria for Diagnosis

  • Autoimmune inner ear disease (AIED) considered if 3 major or 2 major plus >2 minor criteria fulfilled (Berrocal, 2002)

Initial Testing

  • Audiology – initial testing for pure tones, speech discrimination, tympanometry, acoustic reflex testing
  • If vestibular symptoms present
    • Vestibular function testing for pursuit, saccade optokinetic nystagmus, positional and spontaneous nystagmus, caloric stimulation and rotation chair stimulation

Laboratory Testing

  • No single laboratory test recommended for evaluation of disorder; consider non-specific antibody screening to rule out autoimmune or infectious disease associated with hearing loss
  • Specific hearing loss autoimmune testing
    • HSP70 testing helpful if positive; however, negative result does not rule out autoimmune hearing loss
      • HSP70 positivity is an indicator of steroid responsiveness of the disease; however, a subset of AIED patients who are HSP70 antibody negative may respond to steroid treatment
    • Cochlin antibody testing helpful if HSP70 is negative

Differential Diagnosis

Autoimmune inner ear disease (AIED), also called autoimmune sensorineural hearing loss (ASNHL), is characterized by bilateral, rapidly progressive sensorineural hearing loss over a period of weeks to months. Ménière disease (MD), which is a recurrent and spontaneous episodic vertigo associated with hearing loss, fullness in the ear, and tinnitus, may sometimes be confused with AIED. Individuals with MD or variants of MD may have symptoms due to immune dysfunction.


  • Prevalence – rare disease, accounting for <1% of all cases of hearing impairment; true prevalence unknown
  • Age – peak onset in 20s-40s, rare in pediatric ages
  • Sex – M:F ratio unknown due to low prevalence of disease


  • Inner ear cells are delicate and have limited ability for regeneration and repair
  • Disruption of regulating mechanisms may cause substantial damage to inner ear structures, resulting in loss of hearing function
  • Both cell-mediated and antibody responses have been associated with AIED; immune response may cause substantial damage to inner ear structures
  • Possible antibodies involved in AIED
    • Heat shock protein 70 (HSP70) – also known as anti-68kDa antigen
    • Cochlin protein
    • Choline transporter-like protein 2
    • Myelin protein P0
    • Beta-tectorin

Clinical Presentation

Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies 2002068
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Anti-Nuclear Antibodies (ANA), IgG by ELISA with Reflexes to ANA, IgG by IFA and to dsDNA, RNP, Smith, SSA 52, SSA 60, and SSB Antibodies, IgG 0050317
Method: Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Rheumatoid Factors, IgA, IgG, and IgM by ELISA 0051298
Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Quantitative Enzyme-Linked Immunosorbent Assay.

Heat Shock Protein 70, IgG by Immunoblot 2013590
Method: Qualitative Immunoblot


False positives from other systemic autoimmune diseases

Treponema pallidum Antibody, IgG by IFA (FTA-ABS), Serum 0050477
Method: Semi-Quantitative Indirect Fluorescent Antibody


FTA tests for syphilis may be false positive in autoimmune disease, leprosy, febrile illnesses, advanced age, Lyme disease and endemic treponematoses

Borrelia burgdorferi Antibodies, Total by ELISA with Reflex to IgG and IgM by Western Blot (Early Disease) 0050267
Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Qualitative Western Blot


No objective tests for Lyme borreliosis are 100% sensitive and 100% specific

Diagnosis depends on clinical features combined with available laboratory tests

Borrelia burgdorferi Total Antibodies, IgG and/or IgM by ELISA with Reflex to IgG by Western Blot (Late Disease) 0050268
Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Qualitative Western Blot

Hemoglobin A1c 0070426
Method: Quantitative High Performance Liquid Chromatography/Boronate Affinity


Unstable hemoglobins or hemolytic anemia may yield falsely low results

Iron deficiency anemia may yield falsely high results

Human Immunodeficiency Virus Types 1 and 2 (HIV-1, HIV-2) Antibodies by CIA with Reflex to HIV-1 Antibody Confirmation by Western Blot 2005377
Method: Qualitative Chemiluminescent Immunoassay/Qualitative Western Blot


American Society for Clinical Pathology. Choosing Wisely - Five Things Physicians and Patients Should Question. An initiative of the ABIM Foundation. [Last revision Feb 2015; Accessed: Jan 2016]

General References

Berti E, Vannucci G, Lunardi C, Bianchi B, Bason C, Puccetti A, Giani T, Pagnini I, Cimaz R, Simonini G. Identification of autoantibodies against inner ear antigens in a cohort of children with idiopathic sensorineural hearing loss. Autoimmunity. 2013; 46(8): 525-30. PubMed

Bonaguri C, Orsoni JG, Zavota L, Monica C, Russo A, Pellistri I, Rubino P, Giovannelli L, Manzotti F, Piazza F. Anti-68 kDa antibodies in autoimmune sensorineural hearing loss: are these autoantibodies really a diagnostic tool? Autoimmunity. 2007; 40(1): 73-8. PubMed

Bovo R, Aimoni C, Martini A. Immune-mediated inner ear disease. Acta Otolaryngol. 2006; 126(10): 1012-21. PubMed

Khalidi NA, Rebello R, Robertson DD. Sensorineural hearing loss in systemic lupus erythematosus: case report and literature review. J Laryngol Otol. 2008; 122(12): 1371-6. PubMed

Yehudai D, Shoenfeld Y, Toubi E. The autoimmune characteristics of progressive or sudden sensorineural hearing loss. Autoimmunity. 2006; 39(2): 153-8. PubMed

References from the ARUP Institute for Clinical and Experimental Pathology®

Tebo AE, Szankasi P, Hillman TA, Litwin CM, Hill HR. Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss. Clin Exp Immunol. 2006; 146(3): 427-32. PubMed

Medical Reviewers

Last Update: October 2016