Rare hearing and ear disorders


Contributed by Debbie Clason, staff writer, Healthy Hearing

If the eyes are windows to the soul as the proverbial saying goes, the ears may easily be a gateway to physical health. And while the former is a commentary on how transparent a person is when gazes meet, the latter is a medical fact. Doctors can sometimes tell a lot about what’s going on in the rest of your body from looking at your hearing health. From nausea and dizziness to paralysis and even death, our ears can hold the answer to what ails us.

woman's ear with hand held over it, ear pain
Ear pain can indicate other serious 

health concerns.

While most problems are easily discoverable during regular visits to your hearing healthcare professional, here are five rare hearing and ear disorders which can disrupt daily activities until they are diagnosed and treatment has begun.

Superior semicircular canal dehiscence syndrome (SCDS)

Structures in the inner ear are extremely delicate. Even a pin-size hole in a bone in the inner ear can create balance disorders so debilitating, sufferers dread performing daily tasks as simple as walking across the room or turning their head to look behind them. This rare balance disorder, which medical professionals estimate affects less than 1 percent of the population, can cause nausea, vertigo and incredible sensitivity to noise. Patients complain their own voices or the sound of their pulse are too loud. One sufferer told Dr. Lloyd Minor, an ear, nose and throat specialist (ENT) at The Johns Hopkins University School of Medicine in Baltimore, he could hear the sound of his own eyes moving.

Diagnosis and treatment

Physicians suspecting SCDS will most likely order a CT scan of the inner ear in an effort to detect any opening in the temporal bone covering the superior canal.

In most cases, once the condition has been diagnosed by an ENT or other hearing health professional, corrective surgery can be performed. Surgical risks include hearing loss and nerve damage; rehabilitation is required as the inner ear heals and the brain adjusts.


Another uncommon condition called Cholesteatoma affects one in every 10,000 people. This cyst-like growth develops in the inner ear. Although it isn’t malignant, it can lead to permanent deafness and other serious diseases such as meningitis if left untreated. Symptoms can include a smelly discharge and hearing loss.

The two types of cholesteatoma are:

  • Congenital cholesteatoma, which grows behind the eardrum and is present at birth. Medical professionals believe this condition occurs when skin cells grow in the wrong place.
  • Acquired cholesteatoma, which usually occurs in adults, often those who have chronic or recurring ear infections.

Diagnosis and treatment

Fortunately, physicians can see this pearly-white mass when they examine the ear. Most cholesteatomas can be surgically removed. Untreated, the cholesteatoma can grow and expand, causing permanent damage to structures of the inner ear resulting in dizziness and balance problems or interfere with facial nerves and muscles, causing paralysis. Since cholesteatoma is often infected, the toxicity can spread to other parts of the body, causing meningitis and brain abscesses in rare cases.

Autoimmune inner ear disease (AIED)

Symptoms of this rare syndrome, which is caused by antibodies or immune cells which attack the inner ear, include progressive hearing loss with accompanying dizziness. Theories as to its causes range from a delayed response by the immune system after damage to the inner ear, to accidental damage caused by antibodies fighting a threat, to genetic factors.

Diagnosis and treatment

AIED accounts for less than one percent of all cases of hearing loss or dizziness and is diagnosed after a series of hearing tests and blood tests for general autoimmune disease. Although AIED is currently considered a chronic, incurable disorder which causes permanent damage to hearing and balance, its symptoms can be managed. Treatments include the use of steroids and other medications, cell and gene therapy and cochlear implant surgery.

Meniere’s disease

Symptoms of Meniere’s disease, also sometimes called endolymphatic hydrops, include vertigo, tinnitus, a feeling of fullness or pressure in the ear and fluctuating hearing loss. Meniere’s disease is usually unilateral, meaning it typically affects just one ear, and normally occurs between the ages of 20 and 50. Medical professionals aren’t sure exactly what causes the disease, but many believe it’s the result of an abnormal amount of fluid in the inner ear. The disease is estimated to affect one out of every 1,000 people.

Diagnosis and treatment

Testing for the disease typically includes hearing and balance assessments and occasionally blood tests to rule out other disorders. Although Meniere’s disease is considered a chronic condition, there are treatments which can minimize the discomfort and disruption of daily activities. Commonly prescribed medications include those for motion sickness and nausea. Other therapies which have proven successful include vestibular rehabilitation to improve balance, wearing a hearing aid and the use of a Meniett pulse generator which applies pulses of pressure to the ear canal through a ventilation tube.

If you suspect hearing loss

These hearing and ear disorders are very rare and your chances of developing them are slim. Even so, you are always the best advocate for your personal health. Find a hearing health professional you can trust and schedule hearing evaluations on a regular basis. Always be honest with your professional and inform them of any family history involving ear disorders or other medical information. Becoming an educated health consumer and partnering with your hearing healthcare professional goes a long way toward identifying potential health problems as they occur so you can continue to lead an active, healthy life.


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