Diagram of the anatomy of the human ear

Hearing loss is growing problem in the U.S., one that affects 48 million people – or about 20 percent of the entire population. The odds you know someone who has hearing loss are around 1 in 5.

Usually, hearing loss is due to prolonged exposure to loud sounds or simply the consequences of aging. For some individuals, though, hearing loss is a symptom of a less common condition.

Consider six little-known hearing disorders you should know more about.

Benign Paroxysmal Positional Vertigo (BPPV)

BPPV refers to dizziness related to a collection of calcium carbonate crystals, referred to as “ear rocks,” that form inside the inner ear. The medical name for these crystals is otoconia.

These ear rocks typically appear after a head injury, an infection, or some similar disorders. Symptoms of BVVP include dizziness, poor balance, lightheadedness, and nausea. They may worsen with changes in head position.

BPPV can heal by itself, but there are also treatments available such as head exercises that help move crystals out of the inner ear structures. Drug therapy is sometimes used as is surgery.


Labyrinthitis is swelling of the inner ear and hearing nerves that occur usually due to a viral, or less commonly, a bacterial, infection. This inflammation interrupts the transmission of sensory information to the brain, causing difficulties with balance, hearing, and even vision.

Treatments option will include antiviral or antibacterial drugs and therapies used to control the symptoms of dizziness or nausea. If diagnosed and treated right away, labyrinthitis usually causes no permanent damage. However, if left alone, permanent hearing loss can result.  

Ménière’s disease

Ménière’s disease is illness affecting the inner ear that may cause dizziness, progressive hearing loss, ringing in the ears, and a feeling of fullness or pressure in the ear.

The cause of Ménière’s disease is poorly understood but is may be a combination of several factors, including poor fluid drainage of the inner ear, unusual immune response, viral infection, genetic predisposition, and head injury.

Treatment options include medications for vertigo, hearing aids for hearing loss and tinnitus relief, antibiotics to treat infections, and surgery for severe cases.

Acoustic neuroma

The acoustic neuroma is rare and benign tumor of the hearing and balance nerves that disrupts the transmission of hearing and balance data to the brain. Although the tumor does not spread to other organs, it can become large enough to cause hearing loss and balance problems.

There are over 5,000 cases of acoustic neuroma annually in the US. Treatment generally includes surgical removal of the tumor or radiation. Without treatment, the acoustic neuroma can push into the brain, threatening neurological function and even life.  

Autoimmune inner ear disease (AIED)

AIED is a rare, progressive condition that causes the immune cells to attack the inner ear. As with most autoimmune disorders, the cause is not well understood.  

Symptoms will include hearing loss, balance problems, and tinnitus. Diagnosis is done via hearing tests and also blood tests looking for general autoimmune diseases.

Treatment options are limited but growing; currently, they include a combination of steroids, other medications, plasmapheresis, and hearing aids or cochlear implants for hearing loss.


Otosclerosis is a disease that involves hardening of bones in the inner ear bones. These bones are critical elements in the transmission of sound vibrations to the inner ear and then to the brain. The hardening of these bones interferes will eventually lead to slowly progressing hearing loss.

Otosclerosis tends to run in families. If one parent has the disorder, there is a 25 percent chance of the child developing it, as well; two parents have the condition, the likelihood increases to 50 percent.

Treatment options for mild forms of the disease include long-term observation or hearing aids. Sodium fluoride is sometimes offered to slow the progression of the disorder. In certain situations, a surgical procedure called stapedectomy is done to remove the hardened bones and replaced them with a prosthetic device.


It’s important to pay attention to symptoms of hearing loss or changes in balance. Although these conditions are not common, they are all best treated early.





The site information is for educational and informational purposes only and does not constitute medical advice. To receive personalized advice or treatment, schedule an appointment.
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