Four Rare Hearing Conditions

At the Art of Hearing, we know that seeing you is the key to better hearing. Which is why we’re shedding some light on these rare hearing conditions, to help you better understand the risks and symptoms associated with different types of hearing loss.

Autoimmune inner ear disease (AIED)

Autoimmune inner ear disease, or AIED, is caused by antibodies which attack the inner ear. There are several theories as to what causes the condition. It may be the result of genetic factors. A delayed immune system response after damage or infection in the inner ear, it could even be accidental damage caused by antibodies fighting a different threat.

Symptoms of this rare condition include progressive hearing loss, accompanied by dizziness. Diagnosis requires a series of hearing tests and blood tests for general autoimmune disease. AIED accounts for less than 1% of cases of hearing loss or dizziness, but it is a chronic and incurable disease. There is no treatment, but symptoms can be managed using steroids, cell therapy and cochlear implants.

Meniere’s disease

Meniere’s disease affects one out of every 1000 people. Doctors and audiologists aren’t sure what causes the diseases, but the dominant theory is that it is the result of an abnormal amount of fluid in the inner ear.

Symptoms of Meniere’s disease include fluctuating hearing loss, tinnitus, vertigo, and a feeling of pressure in the ear. In most cases, Meniere’s disease is unilateral, affecting only one ear. It normally occurs in adults between the ages of 20 and 50.

Testing for Meniere’s includes hearing tests, balance assessments and can sometimes involve blood tests to rule out other disorders. Like AIED, Meniere’s disease is a chronic condition, but symptoms can be managed through treatment plans. Successful therapies include vestibular rehabilitation, prescription medications for motion sickness and nausea and wearing a hearing aid.

Benign Paroxysmal Positional Vertigo (BPPV)

Benign paroxysmal positional vertigo, more commonly BPPV, is caused by the collection of calcium carbonate crystals in the inner ear. The medical term for these crystals is otoconia, but they’re more commonly referred to as ear rocks. Ear rocks are usually caused by a head injury or infection.

Symptoms of BPPV include dizziness, light-headedness, nausea and trouble with balance. Symptoms can also worsen when the head moves or changes position.

While BPPV can resolve on its own, the most common form of treatment is head exercises designed to move the crystals out of the inner ear, but drug therapy can also be used and in some cases, treatment will require surgical removal of the otoconia.


Labyrinthitis is a rare condition where a viral or bacterial infection causes swelling of the inner ear and hearing nerves. The swelling disrupts the transmission of sensory information to the brain, resulting in problems with balance, hearing and vision. Symptoms can include vertigo, nausea, hearing loss or blurred or double vision.

A diagnosis is usually based on a physical examination where both ears are checked for signs of inflammation or infection. Labyrinthitis is treated using antiviral or antibacterial drugs and medication or therapies used to control symptoms like dizziness or nausea. Diagnosing Labyrinthitis quickly is important, as left untreated, it can result in permanent hearing loss.

Seeing you is the key to better hearing

Concerned about your hearing health or experiencing any of these symptoms? Reach out to our experts, here at The Art of Hearing, for a chat and find out how we can help you hear better.

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