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Did you know that there are autoimmune diseases that cause hearing loss?

Autoimmune diseases that cause hearing loss may be due to a dysfunction of the inner ear, the vestibulocochlear nerve, or the central processing areas of the brain. Known autoimmune conditions that may involve hearing loss are: 

  • Vogt Koyanagi Harada syndrome, Cogan’s syndrome, Susac’s syndrome, SLE, Antiphospholipid syndrome, RA, Systemic vasculitis, Panarteritis nodosa, Granulomatosis with polyangiitis, SSc, Goodpasture syndrome, Behcet’s disease, Sarcoidosis, SS, Hashimoto thyroiditis, Relapsing polychondritis, Myasthenia gravis, Polymyositis Dermatomyositis, Crohn’s disease, Ulcerative colitis, AS, GuillainBarre´ syndrome, Multiple sclerosis, ( Adapted from Acta Otolaryngol 2006;126:101221)

Autoimmune Inner Ear Disease (AIED) is the most common autoimmune disease that causes hearing loss. In this rare condition, the body’s immune system mistakenly attacks the inner ear.

Seek help from an audiologist immediately if you notice any of the following symptoms as they may be signs of these rare disorders.

  • A feeling of pressure, or fullness in either or both ears
  • dizziness or vertigo
  • tinnitus, also called ringing in the ears
  • a change in your perception of sound. This change in perception is often described as speech sounding less clear, muddled, or not as distinct.    

Autoimmune inner ear disease is difficult to diagnose because there are many other types of hearing loss with these same or similar symptoms. It will often start with hearing loss in one ear and then spreads to the other.

Autoimmune disease-causing sensorineural hearing loss is rare, estimated to be at 1% of all hearing loss. It is a loss of hearing sensitivity that occurs over several days to several months. In 50% of all cases, it is accompanied by the sensation of fullness in one or both ears, tinnitus, and feelings of unsteadiness or dizziness. These symptoms may be the first signs of this condition. 


A hearing loss caused by an autoimmune condition is one of the rare forms of hearing loss that can be treated. Steroids are a common treatment. Another is medications to calm down your immune system. If you end up with a permanent hearing impairment, hearing aids can help improve your hearing.


If you are experiencing any of these symptoms or have been diagnosed with one of the conditions above it is important to be seen by an audiologist.  The audiologist will perform a diagnostic battery of tests such as an audiometric evaluation, testing of the reflexes in your middle ear, and otoacoustic emission testing.  The audiologist will make the appropriate referral to ensure that you receive the treatment that you need and will monitor your progress.  

Need to see an audiologist. Please call our office at 415-346-6886 to schedule an appointment or use our online appointment scheduling option to make an appointment.