Autoimmune Inner Ear Disease

By  //  February 11, 2023

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Autoimmune inner ear disease (AIED) is an inflammatory disorder brought on by an unchecked immune response that damages the inner ear, resulting in progressive sensorineural hearing loss (SNHL), which typically begins in one ear before spreading to the other.

 The body produces antibodies and defenses, also known as antigen-antibody, complexes that damage the inner ear because it believes a portion of the inner ear shouldn’t be there. This results in hearing loss, inner ear tissue destruction, and blood vessel inflammation. Antibodies are proteins that the body uses to defend itself from these antigens. Antigens are the substances that trigger an immunological response.

When the inner ear is the sole organ impacted by AIED, it is referred to as “primary.” However, 15 to 30% of the time, AIED is classified as “secondary” when it develops alongside another autoimmune condition that affects the entire body, such as lupus, rheumatoid arthritis, scleroderma, ulcerative colitis, or Sjogren’s disease (an immune disorder marked by dry mouth and dry eyes). Middle-aged women are more likely to have AIED.

Due to the absence of specific blood or imaging criteria to measure, diagnosing AIED can be difficult. An essential diagnostic indicator for AIED is the timing of hearing loss progression over weeks to months. Early corticosteroid and immunosuppressive therapy may help avoid irreparable hearing loss if AIED is diagnosed. To manage the problem, a multidisciplinary team approach involving an ENT (ear, nose, and throat) expert, or otolaryngologist, an audiologist, and a rheumatologist is advised.


Anyone can be impacted by AIED. The causes for its emergence are unknown.

The American Academy of Otolaryngology claims that individuals who have one or more autoimmune illnesses are frequently affected by it. 

The causes of autoimmune ear disorder might be:

  • Immune complexes and antibodies are produced when the body’s unchecked immune system targets an inner ear protein, leading to bilateral progressive hearing loss.
  • The immune system attacks cochlin, a protein found in the inner ears.
  • An inner ear component called the endolymphatic sac may expand as a result of an immunological reaction.


Hearing loss that starts in one ear and progresses to both is the primary sign of AIED. It could take weeks or months for this to develop. It’s not usually the same in both ears how much hearing loss there is.

Additional signs include:

  • Tinnitus, or ringing in the ears
  • systemic autoimmune diseases symptoms, such as weariness and muscular aches 
  • hearing that fluctuates in quality
  • random dizziness or impaired balance

AIED is commonly mistaken for other inner ear disorders, such as Meniere’s disease, that can cause hearing loss. Both disorders share symptoms like tinnitus and poor balance. Meniere’s disease often only affects one ear, which is the primary distinction between the two illnesses.


You should visit an ENT doctor if your hearing loss in both ears worsens over weeks to months so they can diagnose you after examining your hearing tests and imaging studies. If you receive medical treatment early on if your doctor suspects you have AIED, such as steroid and immunosuppressive medicine, you may benefit from it.

In case you have any of the associated symptoms consult an ENT specialist for the right course of treatment. ENT specialists are health experts health professionals who are capable of diagnosing and treating a large number of diseases related to the ears, nose, and throat. The consultation with the ENT specialists can be done through both online and offline modes. ENT Specialists in Delhi can charge a minimal consultation fee of 500 INR to 3000.

The primary method of treatment for reducing swelling and inflammation in the inner ear is corticosteroids. Your SNHL may be reversed with quick AIED identification and steroid treatment. Alternative treatments include cyclophosphamide, methotrexate, azathioprine, and rituximab if you cannot tolerate steroid therapy.

You could also have a hearing test from your doctor to see whether you need any additional assistive listening gear. Your doctor might suggest a cochlear implant if your hearing loss is significant. Discuss additional particular treatment choices with your doctor.


Concerning the risk factors for AIED, little is known. However, we are aware that individuals with other autoimmune conditions are more prone to acquire AIED than those without.

Along with AIED, some individuals may also have the following autoimmune diseases:

ulcerative colitis, scleroderma, lupus, and rheumatoid arthritis.

One of the many things that increase your risk of acquiring AIED is having an autoimmune condition. According to a reliable source, women are more likely than males to experience AIED. According to a reliable source, women are more likely to get AIED, and their risk is highest in their 20s and 50s.


A diagnosis of AIED may appear slightly concerning, although it can be encouraging to understand the cause of your hearing loss and to be aware that treatment is frequently an option.

Once you have received a proper diagnosis from an ENT specialist and begun treatment, you may remain on medications designed to keep your immune system in check. Based on elements like drug resistance or side effects, it may occasionally be necessary to modify the types and dosages of pharmaceuticals.

Living with an AIED will also include routinely monitoring your hearing. An audiogram, which uses tones to demonstrate how loud sounds need to be for you to hear clearly, is one type of regular hearing test you can anticipate. AIED typically results in total hearing loss and balance issues if left untreated.

The progression of symptoms doesn’t follow a predictable pattern. You should consult an ENT specialist as soon as possible to avoid further damage to your ears.