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Frequently asked questions about Otosclerosis

Blog post by Dallas Ear Institute

Frequently asked questions about Otosclerosis

What is Otosclerosis?

Otosclerosis is a disorder of bone metabolism that affects only the ear.

The bone where the inner ear and the third hearing bone (stapes) meet is called the oval window. This is where otosclerosis affects the ear. Bone is constantly being broken down and rebuilt by our body. In otosclerosis, the rate of rebuilding is increased resulting in the formation of new bone at the oval window.

What is Otosclerosis?

What symptoms are caused by otosclerosis?

Hearing loss is the main symptom.

Unlike many problems with the ear that can cause hearing loss, ear drainage, infections, or dizziness, otosclerosis mainly causes hearing loss. Sometimes the hearing loss is accompanied by tinnitus (ringing in the ear or head noise). The hearing loss is usually conductive, meaning sound is blocked from reaching the inner ear. The inner ear, hearing nerve, and brain often process the limited sound that reaches them completely normally.

People affected by otosclerosis are usually born with normal hearing. They may develop hearing loss in their 20s but this can occur from teens to midlife. Women are more commonly affected than men.

How did I get otosclerosis?

Otosclerosis is inherited from your parents.

The pattern of inheritance is autosomal dominance. This means that if one parent has it, the children have a 50% chance of getting it. However, there is variable penetrance. This means that if you get the gene, you are only at risk for otosclerosis. There is no guarantee of hearing loss. The trigger may be a viral infection, such as measles. Although measles vaccine protects against contracting measles symptoms, it does not protect against triggering otosclerosis.

Will otosclerosis cause deafness?

Deafness from otosclerosis is unlikely.

Because it typically only blocks the conduction of sound, there is a limit to the hearing loss that it can cause. It will progress until the hearing bones are completely stuck. At this point hearing loss is around 50%. It commonly does affect both ears in two out of three cases. Rarely, it progresses from the oval window into the inner ear. This is called cochlear otosclerosis. In these cases, it decreases processing of sound in the inner ear as well. In these instances, hearing loss is more severe.

How do I know if I have otosclerosis?

If you have hearing loss, you need to first have a hearing test to find the cause.

A neurotologist (hearing, balance, and skull base surgery specialist) or an otolaryngologist (ear, nose, and throat specialist) will provide a hearing test and evaluation. They will examine your ear to check for other causes of hearing loss and evaluate your ability to hear a tuning fork to further differentiate your hearing loss.

What can be done to help me hear better with otosclerosis?

The three options to treat otosclerosis are medical therapy, hearing aids, and stapes surgery.

The goal of medical therapy is to prevent the disease from advancing. Fluoride is one option. It can be given as a pill or can be obtained from drinking tap water. It prevents the buildup of new bone at the oval window. Bisphosphonates are a class of medications commonly used in osteoporosis. They decrease bone destruction. Since otosclerosis is a problem of bone breakdown and repair, they may help slow progression. Neither of these reverse the hearing loss that has already occurred.

Hearing aids are an option to treat many forms of hearing loss, including hearing loss caused by otosclerosis. They are a great option for patients who would like to hear better and prefer to avoid surgery or are not surgical candidates.

The goal of surgical therapy is to correct the conductive hearing loss associated with otosclerosis. During surgery, an incision is made in the ear canal allowing the surgeon to elevate the ear drum and look behind it. The stapes (third hearing bone) is removed and a prosthesis is placed to connect the incus (second hearing bone) and the inner ear. The ear drum is then returned to its normal position. The procedure is called a stapedotomy.

Are there any risks associated with treatment?

There are minor risks associated with any form of treatment.

Medications such as fluoride and bisphosphonates may cause stomach upset, allergic reaction, or joint pain. Rarely break down of the ear canal bone or jaw bone is a side effect.

Hearing aids are generally well tolerated. Occasional discomfort associated with poor fitting molds or ear wax buildup may be experienced.

General risks of surgery apply to stapedotomy surgery. Specific to the procedure, there is a 1% chance of hearing loss. After surgery, there may be a metallic taste in the mouth temporarily. Very rarely, injury to the facial nerve may occur.

Which options is best for me?

Stapedotomy is preferred under normal circumstances.

Successful stapedotomy prevents further conductive hearing loss. It also allows the patient to have restored hearing without the use of a hearing aid. The risks are minimal. It is the usual option for patients who are candidates for surgery. For nonsurgical candidates, a hearing aid avoids all the risks of surgery and works very well to amplify the hearing loss at specific frequencies needed. Medical therapy is usually not the standard treatment as it only halts progression at best and does nothing to restore hearing loss caused by otosclerosis.

How do I make an appointment?

At the Dallas Ear Institute, there are three experienced ear specialists. Please call Call (972) 566-7600 to schedule an appointment.