Autoimmune Inner Ear Disease (AIED)

Overview of Autoimmune Inner Ear Disease (AIED)

Autoimmune Inner Ear Disease (AIED) is a rare condition that results from the immune system mistakenly attacking healthy cells in the inner ear. This autoimmune response can cause inflammation that in turn leads to a range of symptoms. Symptoms can include progressive sensorineural hearing loss (SNHL), tinnitus (ringing in the ears), vertigo, and a feeling of fullness in the ear. AIED accounts for less than 1% of cases of hearing loss and is often associated with other autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, or Cogan’s syndrome.

The inner ear is a complex system responsible for both hearing and balance. It contains delicate structures, including the cochlea (hearing) and vestibular apparatus (balance). In a healthy ear, sound waves are transmitted through these structures to the brain, where they are interpreted as sound. However, in AIED, immune cells mistakenly identify inner ear cells as harmful, triggering an inflammatory response. This response can damage critical components of the auditory and vestibular systems, leading to symptoms that typically progress over a period of days to weeks.

AIED can be challenging to diagnose due to its similarity to other inner ear conditions like Meniere’s disease (MD). Both conditions share symptoms such as hearing loss, vertigo, and tinnitus, but the underlying causes differ. AIED is an immune-mediated disorder, while MD is typically linked to fluid imbalance in the inner ear, known as endolymphatic hydrops (EH). However, recent research suggests that immune dysfunction may also play a role in MD, creating overlap between the two conditions.

Symptoms and Diagnosis of AIED

AIED typically presents with bilateral SNHL, which often progresses rapidly. Patients may experience an initial hearing loss in one ear, but the disease typically affects both ears over time. Vestibular symptoms such as vertigo, balance disturbances, and dizziness are also common, and tinnitus and ear fullness may accompany these vestibular symptoms.

Diagnosing AIED can be difficult due to the lack of specific diagnostic tests. Physicians often use a combination of patient history, audiometric testing, and laboratory evaluations to rule out other conditions. Blood tests may reveal the presence of antibodies associated with autoimmune diseases, such as antinuclear antibodies (ANA), rheumatoid factor (RF), or antibodies against inner ear proteins like cochlin. However, a positive response to steroid therapy is often used as a key diagnostic criterion. Steroids are effective in reducing inflammation, and improvement in hearing after steroid treatment can confirm an autoimmune origin of the hearing loss.

Treatment Options for AIED

The treatment of AIED typically begins with high-dose corticosteroids like prednisone, which reduce inflammation and suppress the immune response. While steroids are effective in many patients, long-term use can lead to significant side effects, including weight gain, mood changes, and an increased risk of infections. For patients who do not respond well to steroids or cannot tolerate them, immunosuppressive agents such as methotrexate and cyclophosphamide or biological therapies like Rituxan may be used.

In cases where hearing loss becomes irreversible, hearing aids or cochlear implants may be considered to restore auditory function. Early treatment is critical in AIED, as the condition can cause permanent damage to the inner ear structures if left untreated. The fluctuating nature of the disease also requires regular monitoring through audiograms to assess hearing thresholds and adjust treatment as needed.

MRI and Imaging in AIED Diagnosis

Advances in imaging, particularly MRI, have provided valuable insights into the mechanisms of AIED. Although MRI cannot directly detect autoimmune activity, it can reveal inflammation, abnormal fluid buildup, and other structural changes in the inner ear that may indicate an underlying autoimmune process. These imaging techniques can help differentiate AIED from other inner ear disorders, such as acoustic neuroma or Meniere’s disease.

MRI plays a critical role in the early detection of EH, which is commonly associated with Meniere’s disease. By identifying fluid buildup in the inner ear, MRI helps clinicians monitor the progression of the disease and tailor treatment strategies accordingly. The ability to visualize changes in the inner ear over time provides a clearer understanding of how autoimmune dysfunction can lead to hearing and balance disorders.

As our understanding of AIED and related disorders evolves, the role of imaging and immunotherapy will likely become increasingly important in managing these conditions and improving the quality of life for affected individuals. And Audiology will remain crucial in monitoring hearing thresholds and subsequent interventions that may be needed over time.

References

Das, S., Bakshi, S.S. & Seepana, R. (2019) Demystifying autoimmune inner ear disease. Eur Arch Otorhinolaryngol 276, 3267–3274. https://doi.org/10.1007/s00405-019-05681-5

Greco, A. Gallo, A. Fusconi, M., Magliulo, G., Turchetta, R., Marinelli, C. Macri, G., De Virgilio, A., de Vincentiis, M. (2013). Cogan’s syndrome: An autoimmune inner ear disease, Autoimmunity Reviews, 12;3, 396-400, https://doi.org/10.1016/j.autrev.2012.07.012.

Greco, A. Gallo, A., Fusconi, M., Marinelli, C., Macri, G., de Vincentiis, M. (2012) Meniere’s disease might be an autoimmune condition? Autoimmunity Reviews, 11;10, 731-738, https://doi.org/10.1016/j.autrev.2012.01.004.

Lee, B., Bae, Y.J., Choi, B.Y. et al. (2021). Construction of an MRI-based decision tree to differentiate autoimmune and autoinflammatory inner ear disease from chronic otitis media with sensorineural hearing loss. Sci Rep 11, 19171 https://doi.org/10.1038/s41598-021-98557-w

Lobo D, Tuñón M, Villarreal I, Brea B, García-Berrocal JR. (2018). Intratympanic gadolinium magnetic resonance imaging supports the role of endolymphatic hydrops in the pathogenesis of immune-mediated inner-ear disease. The Journal of Laryngology and Otology;132(6):554-559. doi:10.1017/S0022215118000749

Nathaniel K. Breslin BA, Varun V. Varadarajan MD, Eric S. Sobel MD, Rex S. Haberman MD (2020) Autoimmune inner ear disease: A systematic review of management. https://doi.org/10.1002/lio2.508

Dr Signe Steers

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