What Is Otosclerosis?

Overview

• Otosclerosis is a term derived from 2 words oto means, “of the ear” and sclerosis means, “abnormal hardening of body tissue.” It is a condition that is caused by abnormal bone remodelling in the middle ear which causes hearing loss. Mild to severe hearing disablement is caused by otosclerosis, but it rarely causes total deafness
• Bone-remodelling is a lifelong process where the old bone tissue is replaced by new bone tissue thus there is a continuous renewal of bone tissue. However, in the case of otosclerosis, there is abnormal remodelling of the bone in the middle ear which results in the disruption of the ability of sound to travel from the middle ear to the inner ear resulting in hearing loss
• Almost three million Americans are affected by otosclerosis. It is thought to be inherited in most of the cases. White middle-aged women are most at risk of developing otosclerosis

Causes of otosclerosis

• According to the NIH, otosclerosis is most often caused when one of the bones named stapes is stuck in the middle ear which leads to the immovable bone, thus the sound is not able to travel through the ear and hearing ability diminishes  
• The exact reason behind the cause of otosclerosis is still not apparent, but it is believed by scientists that previous measles infection, fractures to the bony tissue surrounding the inner ear, stress or immune disorders may lead to otosclerosis. Otosclerosis also tends to run in families
• It's also believed by some researchers that otosclerosis may also be related to three different immune-system cells known as cytokines. When there is a proper balance between these three immune-system cells then it results in proper bone remodelling, but when there is an imbalance in the levels of these three immune-system cells it results in abnormal remodelling of bone which is seen in otosclerosis

Signs and symptoms of otosclerosis

According to cleveland clinic the symptom most commonly shown by people suffering from otosclerosis is hearing loss that happens gradually over time. People suffering from otosclerosis usually observe that they cannot hear whispering or low-pitched/tone sounds. In most cases, people with otosclerosis have hearing loss in both ears and approximately 10 -15% of individuals with otosclerosis have hearing loss in one ear.

Other possible symptoms of otosclerosis include:

• Balance problems
• Vertigo
• Ringing in ears (tinnitus)
• Dizziness 
• Hissing,  buzzing or roaring in the ears or head ( that sometimes occurs with hearing loss.)

Management and treatment for otosclerosis

There are two methods of treatment for otosclerosis:

1. Hearing aid: this line of treatment is used when there is mild otosclerosis, thus by the use of hearing aids helps to amplify sound, but in most cases surgery is suggested
2. Stapedectomy: This is the second line of treatment, it is the surgical way of treating otosclerosis. The surgeon inserted a prosthetic device in the middle ear to bypass the abnormal bone thus allowing the travel of the sound waves in the middle ear, thus resulting in the restoring of hearing ability

Currently, there is no effective drug treatment for otosclerosis right now, although there is hope to identify potential new therapies through ongoing bone-remodelling research.

It is very important to discuss any surgical procedure with an ear specialist to clarify the potential risk and limitations of the operation. For example, in some of the cases, hearing loss may even persist after the stapedectomy, and in rare cases, surgery can even worsen hearing loss.

Diagnosis of otosclerosis

Ear, nose and throat specialists (ENT) are the ones who usually diagnose otosclerosis. First, they rule out other health problems that have similar symptoms. Then a hearing test is performed to determine the extent of hearing loss. These tests may include an audiogram, which measures your hearing across a range of frequencies, and a tympanogram, which tells to your provider how well your eardrums are working.

The healthcare provider may request for CT scan as well. This imaging test helps your medical team see the bones and tissue inside your ear in great detail.

FAQs

Can otosclerosis be prevented?

Like other hearing problems, there are no preventable risk factors for otosclerosis. Some people simply have a genetic predisposition for this condition. As a result there’s no way to prevent otosclerosis from developing. 

Can otosclerosis be cured?

Otosclerosis cannot be cured. The hearing loss caused by otosclerosis can be improved gently by performing surgery. Hearing aids can successfully manage hearing loss as well. 

How common is otosclerosis?

More than three million Americans are affected by otosclerosis. It affects approximately 10% of the adult Caucasian population. On average, a person who has one parent with otosclerosis has a 25% chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50%.

Who are at risk of otosclerosis?

Women aged between 15 and 30 years are most commonly affected by otosclerosis. The cause is unknown, but risk factors include family history, gender and pregnancy.  

When should I see a doctor?

When one experiences a change or decrease in hearing ability, it’s important to see a doctor. Prompt diagnosis and treatment can help ease troublesome symptoms so you can return to normal life. 

Summary

Otosclerosis is a condition in which there is abnormal remodelling of bone in the middle ear which results in the inability  of sound to travel from the middle ear to inner ear, thus resulting in hearing loss. There is no exact known cause of otosclerosis, but it is believed by the scientist that people with previous measles infection, stress, immune disorders or fracture of the bony tissue surrounding the inner ear can result in the occurrence of otosclerosis. The other symptoms observed by the patients are dizziness, balance problem, tinnitus, roaring or hissing in the ear. Hearing tests such as tympanogram and audiograms are used for the diagnosis of otosclerosis. In some of the cases CT scans are also recommended by the healthcare provider. Otosclerosis can be treated with the help of stapedectomy surgery or with the help of hearing aids. More than three million Americans are affected by otosclerosis. White middle-aged women, pregnant women and people with a family history of otosclerosis are at high risk to develop otosclerosis.