Otosclerosis

What is otosclerosis?

Otosclerosis (oh-tuh-skli-ROH-sis) is a condition that causes hearing loss. The term “oto” means “of the ear” and “sclerosis” means “abnormal hardening of body tissue.”

Otosclerosis happens when irregular bone remodeling/growth occurs in your middle ear or, more rarely, your inner ear. Bone remodeling is a lifelong process in which existing bone tissue repeatedly restores itself. In otosclerosis, irregular bone remodeling interferes with sound’s ability to travel through your ear.

People with otosclerosis can develop mild to severe hearing impairment. The condition rarely results in total deafness. It typically affects both ears, but one ear is usually worse than the other.

How common is otosclerosis?

Over 3 million Americans have otosclerosis. It’s the most common cause of middle ear mechanical hearing loss among young adults.

Otosclerosis can affect anyone, but it occurs most often in white people AFAB (assigned female at birth) between the ages of 20 and 45. Sometimes, otosclerosis runs in families.

What are symptoms of otosclerosis?

The most common symptom of otosclerosis is hearing loss which happens gradually. People with otosclerosis may notice that they can no longer hear whispering or low-pitched tones/sounds. In most cases, people with otosclerosis have hearing loss in both ears. Approximately 10% to 15% of individuals with otosclerosis have hearing loss in one ear.

Other possible otosclerosis symptoms include:

• Balance issues.
• Vertigo.
• Ringing in your ears (tinnitus).
• Dizziness.

If you have otosclerosis, you may speak quietly because your voice sounds loud to you.

What causes otosclerosis?

Deep inside your ear, there are three tiny bones (ossicles) that vibrate to amplify the sound waves that pass through them. These sound waves travel to the cochlea in your inner ear, where they convert into signals before moving on to your brain. Otosclerosis most often develops when the stapes bone (a small, triangular bone in your middle ear) fuses with the surrounding bone tissue. As a result, sound can’t travel effectively.

Think about the musical instrument, the triangle. When held properly by the loop at the top, the triangle hangs free and creates a rich sound through vibration when struck. But if you place your hand around the triangle itself, the sound gets muffled. The stapes bone reacts in a similar way when excess bone grows around it.

Otosclerosis risk factors

A risk factor is something that increases your chance of developing a certain health condition. Possible risk factors for otosclerosis include:

• Family history. You’re more likely to develop otosclerosis if a parent, sibling or grandparent has it.
• Gender. People AFAB are more prone to developing otosclerosis.
• Race. Overall, white people have a higher chance of getting otosclerosis.
• Pregnancy. People who are already prone to otosclerosis may develop the condition while they’re pregnant.
• Osteogenesis imperfecta (OI). Also known as brittle bone disease, OI increases your risk of otosclerosis.

How do healthcare providers diagnose otosclerosis?

If your primary care physician thinks you might have otosclerosis, they’ll refer you to an otolaryngologist (ear, nose and throat specialist). First, they’ll rule out other health conditions that share similar symptoms. Next, they’ll run hearing tests to determine the extent of hearing loss.

These tests may include a/an:

• Audiogram, which measures your hearing across a range of frequencies.
• Tympanogram, which tells your provider how well your eardrum works.

Your healthcare provider may also request a CT (computed tomography) scan. This imaging test helps your provider see the bones and tissues inside your ear in more detail.

How is otosclerosis treated?

Otosclerosis treatment depends on the location of irregular bone and the severity of your condition. Many people successfully manage otosclerosis-related hearing loss with hearing aids. In select cases, stapedectomy (a type of surgery) can help improve your hearing. If you have cochlear otosclerosis (in your inner ear), your provider may recommend a cochlear implant.

Hearing aids

Hearing aids amplify the sounds around you to help you hear better. An audiologist can customize the settings on your hearing aid according to your specific needs.

While hearing aids can improve your hearing, they can’t keep otosclerosis from getting worse. Ask your healthcare provider whether hearing aids are right for your situation.

Stapedectomy

“Stapedectomy” (stay-puh-DEK-tuh-mee) is the medical term for otosclerosis surgery. During this procedure, an otolaryngologist places a prosthesis (replacement hearing bone) in your middle ear. This prosthesis bypasses the stapes bone, allowing sound waves to travel to your inner ear. As a result, your hearing improves.

If otosclerosis affects both of your ears, your surgeon will operate on one ear at a time so each has time to heal. Once the first surgery is complete, you’ll probably have to wait at least six months to schedule your next procedure.

Cochlear implants

Cochlear implants can improve hearing in people with cochlear otosclerosis. (Your cochlea is a spiral, fluid-filled structure in your inner ear that helps with hearing.) A cochlear implant bypasses your inner ear structures and creates a new pathway on which sounds can travel to your brain.

Can I prevent otosclerosis?

Unlike some other hearing conditions, there are no preventable risk factors for otosclerosis (like exposure to loud noises). Some people are more prone to it from a genetic standpoint. As a result, there’s no way to prevent otosclerosis from developing.

How fast does otosclerosis progress?

Otosclerosis usually gets worse slowly, over the course of many years. But the timeframe can vary from person to person. It often begins in one ear and spreads to the other ear over time. In rare cases, otosclerosis can progress quickly.

If you notice sudden changes in your hearing, schedule an appointment with your healthcare provider.

How do you fix otosclerosis?

You can’t cure otosclerosis, but you can manage it with treatment. You’ll likely need a hearing aid or surgery, depending on your specific situation. Or your provider may monitor your condition and recommend treatment only if symptoms worsen.

What happens if otosclerosis is left untreated?

Without treatment, otosclerosis may get worse over time. In some cases, it can spread to your inner ear and cause cochlear otosclerosis. Your healthcare provider can discuss your options with you and tell you whether they recommend monitoring your condition or moving forward with treatment.

When should I see my healthcare provider?

Any time you notice a change or decrease in your hearing ability, you should schedule an appointment with your healthcare provider. Prompt diagnosis and treatment can help ease troublesome symptoms and improve your quality of life.

What questions should I ask my healthcare provider?

If you received an otosclerosis diagnosis, you may want to ask your provider the following questions:

• How serious is my condition?
• Would hearing aids help or do I need surgery?
• How long can I wait before scheduling surgery?
• How long will it take for my hearing to worsen without treatment?

Otospongiosis vs. otosclerosis: What’s the difference?

“Otospongiosis” refers to the early stages of otosclerosis. During this time, soft bone begins to form. Over time, these areas of bone scar and become hardened. Once this occurs, providers call it “otosclerosis.”

A note from Cleveland Clinic

It can feel scary any time you notice changes in your hearing. Otosclerosis can develop slowly, so it might take time to realize that something’s not quite right. The best thing you can do is call your healthcare provider at the first sign of trouble. They can run tests to confirm your diagnosis and come up with a personalized treatment plan.