Unusual Causes of Hearing Loss

Most people focus on the bigger picture. Do I have enough health and life insurance coverage? What will happen if I have an accident and break a leg or arm? Does my genetic history mean I’ll have heart troubles? Will I get hit by a bus because I was texting and walking?
For the general public, hearing is an afterthought, so often taken for granted. If we had it at birth, we expect it to always be there, not really giving a lot of thought to the possibility that it could someday diminish or be gone completely.
Within the four basic parts of the ear, there is any number of things that can happen to cause reduced hearing or even deafness.

  • The outer ear includes the ear canal and the eardrum.
  • The middle ear includes the middle ear bones (also called ossicles or ossicular chain) as well as the portion of the eardrum located within the cavity of the middle ear.
  • The inner ear, which includes the cochlea used for hearing, and the vestibular system that involves our balance.
  • The auditory cortex is the central auditory system in which many highly sophisticated nerve networks connect the cochlea (the inner ear) to the auditory cortex (hearing centers within the brain).

By studying the conditions of the ear, doctors are able to determine more than just hearing health. Hearing health professionals can see a lot more than just an eardrum when they look into our ears. They can locate a lot of basic problems such as ear infections, ruptured eardrums, or a buildup of ear wax. But they can also diagnose some rare hearing disorders that can be disruptive to our daily lives.

Autoimmune Inner Ear Disease (AIED)

There has been an increase in autoimmune disorders the past decade or so, but not as many cases of Autoimmune Inner Ear Disease (AIED), which is defined as a bilateral sensorineural hearing loss (SNHL).  The condition is caused by antibodies, or uncontrolled immune cells, attacking the inner ear. Symptoms can include gradually worsening hearing loss accompanied by dizziness.
The onset of the disease could be as fast as three days or as long as ninety days. The yearly estimated occurrence of the disease is less than five cases per 100,000. It’s reported more often in women than men, with higher numbers of occurrence reported in their thirties and sixties. AIED is incredibly rare and is attributed to less than one percent of all diagnosed cases of SNHL.
The condition is diagnosed through a series of both blood and hearing tests for general autoimmune disease. This chronic disorder currently has no cure, though its effects on hearing and balance can be managed through the use of steroids and other medications as well as cell and gene therapy or surgery for cochlear implantation.


More common than AIED, Cholesteatoma has an incident rate of one in 10,000. There are two types of the disorder. Congenital, which is present at birth and involves a cyst-like growth located in the inner ear behind the eardrum. Its surmised in the medical field that it’s caused by skin cells growing in the wrong location. Acquired cholesteatoma is often found in adults, and usually in those with chronic or often occurring ear infections.
Symptoms involve hearing loss in addition to a foul-smelling discharge. While not a malignant mass, this pearl white-colored growth can cause permanent deafness if it is not taken care of promptly. If left undiagnosed, a cholesteatoma will get larger and expand, often leading to permanent damage within the inner ear.
Destruction of these delicate structures can result in balance issues or dizziness as well as problems with nerves and facial muscles, occasionally leading to paralysis. Often infected, this toxic substance can spread throughout the body and in rare instances cause severe conditions such as meningitis and abscesses of the brain. Fortunately, these masses can be surgically removed.

Meniere’s Disease

Normally affecting just one ear, Meniere’s Disease, occasionally referred to as endolymphatic hydrops, is often found in people between the ages of twenty and fifty. Symptoms include increased pressure or a feeling of fullness within the ear, vertigo, and tinnitus as well as a fluctuating hearing loss.
There is currently no known cause for the disease, but doctors presume it’s due to an unnatural amount of fluid in the tubes of the inner ear. It affects approximately one out of every one thousand people. Doctors use hearing assessments and balance testing in addition to blood tests to exclude other diseases.
Considered a chronic condition, Meniere’s discomfort can be minimized through treatments of medications used for nausea and motion sickness. There has also been a success when using vestibular rehabilitation in conjunction with hearing aids and a Meniett pulse generator to apply pulses of pressure through a ventilation tube to the ear canal.

Superior Semicircular Canal Dehiscence Syndrome (SCDS)

Affecting fewer than one percent of the population, Superior Semicircular Canal Dehiscence Syndrome can cause nausea, vertigo, and such an incredible sensitivity to noises that sufferers don’t even want to perform such simple tasks as turning their head to look the other way or walk across a room.
Caused by a hole, even as small as a pin, within the bone of the delicate inner ear, this debilitating disease can make patients complain that the sound of their own voice or pulse is too loud to tolerate. One such patient described it as being able to hear his eyes move.
In order to treat the condition, doctors will use a CT scan of the inner ear to determine if there are any open cavities in the temporal bone which covers the superior canal. Often corrective surgery is an option, though there are risks of loss of hearing as well as nerve damage. Rehabilitation while healing is taking place is required to allow the brain to adjust and the inner ear to heal.
If you or a loved one have symptoms of hearing loss, seek out a qualified hearing health professional. They will do a complete exam and make recommendations based on their findings. While these disorders are incredibly rare, the ability to hear is a precious gift that we should not take for granted. Make an appointment to get your hearing checked today.


Related posts