Otosclerosis

Textbook of Otosclerosis, Co-Edited by Dr. Sperling.

Otosclerosis is an abnormal bone-remodeling condition of the ear that can cause progressive hearing loss. It most commonly affects the stapes, one of the three tiny bones in the middle ear responsible for transmitting sound vibrations to the inner ear. When the stapes becomes stiff or fixed in place, sound cannot travel efficiently to the cochlea, resulting in conductive hearing loss.

In some cases, otosclerosis can also affect the inner ear and contribute to sensorineural hearing loss.

If you have been diagnosed with otosclerosis or are experiencing unexplained, gradual hearing loss, an evaluation by an ear specialist can determine whether hearing aids, surgery, monitoring, or another treatment approach is appropriate.

What Causes Otosclerosis?

The exact cause of otosclerosis is not completely understood. However, the condition often has a genetic or hereditary component, and it can occur in families.

Researchers have also investigated possible relationships between otosclerosis and factors involving bone remodeling, immune activity, and previous infections. However, no single cause explains every case. Having a family history of otosclerosis can increase the likelihood of developing the condition.

The most common symptom of otosclerosis is gradual hearing loss. Some people initially notice difficulty hearing:

  • Quiet or whispered speech
  • Low-pitched sounds
  • Conversations when several people are speaking
  • Speech from a distance
  • Certain sounds without increasing the volume
  • Conversations in situations where background noise is present

Other possible symptoms include:

Tinnitus

Some people with otosclerosis experience tinnitus, which may sound like ringing, buzzing, roaring, hissing, or another noise in the ear.

Dizziness or Balance Problems

Some people may experience dizziness, vertigo, or balance difficulties. These symptoms are not present in every person with otosclerosis.

Hearing Loss in One or Both Ears

Otosclerosis frequently affects both ears, although one ear may be affected more than the other. Hearing loss can begin in one ear and later involve the other.

Because hearing loss can have many different causes, these symptoms do not automatically mean that a person has otosclerosis. A comprehensive ear and hearing evaluation is important for an accurate diagnosis.

Diagnosing otosclerosis involves evaluating the ear, measuring hearing, and ruling out other possible causes of hearing loss. An evaluation may include:

Medical and Hearing History

Your physician may ask about:

  • When you first noticed hearing loss
  • Whether hearing has changed gradually or suddenly
  • Whether one or both ears are affected
  • Tinnitus, dizziness, or balance symptoms
  • Previous ear problems or surgery
  • A family history of hearing loss or otosclerosis

An examination of the ear canal and eardrum can help rule out other causes of hearing loss.

A hearing test, or audiogram, measures how well you hear different frequencies and helps determine the type and severity of hearing loss. In otosclerosis, the hearing test may demonstrate a conductive component because the stapes is not transmitting sound normally.

Tympanometry evaluates the function of the eardrum and middle ear and can provide additional information about how sound is being conducted through the middle ear.

In selected patients, a high-resolution CT scan of the temporal bones may provide additional information about the structures of the middle and inner ear. The diagnosis is based on the overall clinical picture and hearing evaluation rather than on a single test.

Treatment depends on the extent of hearing loss, whether the condition primarily affects the middle or inner ear, your symptoms, and your individual goals. Treatment options may include:

  1. Observation and monitoring
  2. Hearing aids
  3. Stapes surgery, including stapedectomy or stapedotomy
  4. Cochlear implantation in selected cases involving significant inner-ear hearing loss

There is currently no medication that reliably reverses the abnormal bone remodeling associated with otosclerosis.

Hearing aids can amplify sound and may be an effective option for people with otosclerosis-related hearing loss. A hearing aid does not remove or reverse the abnormal bone growth. Instead, it compensates for the hearing loss by making sounds louder and clearer. Hearing aids may be considered when:

  • Hearing loss is mild or moderate
  • A patient prefers a nonsurgical approach
  • Surgery is not appropriate
  • A patient wants to try amplification before considering surgery
  • Hearing loss involves the inner ear in addition to the middle ear

An audiologist can help determine which type of hearing aid is appropriate based on the patient’s hearing test.

For appropriate patients with conductive hearing loss caused by fixation of the stapes, stapes surgery can improve the mechanical transmission of sound to the inner ear.

Two terms commonly used when discussing surgery are stapedectomy and stapedotomy.

A stapedectomy is a surgical procedure in which the surgeon removes part or all of the immobilized stapes and places a small prosthesis to restore the transmission of sound to the inner ear. The prosthesis bypasses the abnormal portion of the stapes and allows sound vibrations to reach the cochlea.

Stapes surgery may be considered when testing indicates that the hearing loss is primarily caused by stapes fixation and the potential benefits of surgery outweigh its risks. A specialist may consider:

  • The severity and type of hearing loss
  • The air-bone gap on the audiogram
  • Speech discrimination
  • The condition of the middle ear
  • Whether the opposite ear is affected
  • The presence of tinnitus or balance symptoms
  • Previous ear surgery
  • Other causes of hearing loss

Not everyone with otosclerosis needs surgery. Hearing aids or monitoring may be appropriate depending on the individual’s circumstances.

The procedure is performed through the ear using specialized surgical instruments. The surgeon accesses the middle-ear structures and creates a pathway that allows a small prosthesis to bypass the immobilized stapes. The prosthesis is positioned to transmit sound vibrations to the inner ear.

The details of the procedure, anesthesia, surgical approach, and recovery vary from patient to patient. Your surgeon should explain the specific technique recommended for your ear before surgery.

Recovery varies depending on the procedure and the individual. After surgery, patients may experience temporary:

  • Mild ear discomfort
  • A sensation of fullness in the ear
  • Changes in hearing while the ear heals
  • Dizziness or imbalance
  • Changes in taste

Your surgeon will provide specific instructions regarding activities, travel, keeping the ear dry, and follow-up appointments. Hearing may not return immediately. Follow-up hearing testing is generally used to evaluate the result after the ear has had time to heal.

As with any surgery, stapes surgery has potential risks and limitations. Possible complications may include:

  • Persistent hearing loss
  • Failure to achieve the expected hearing improvement
  • Worsening hearing loss
  • Tinnitus
  • Temporary or persistent dizziness or balance problems
  • Taste disturbance
  • Need for additional treatment or revision surgery

Rarely, surgery can result in a significant worsening of hearing. These risks should be discussed carefully with your ear surgeon before deciding whether surgery is appropriate.

Yes. Although otosclerosis commonly begins with conductive hearing loss caused by stapes fixation, the condition can sometimes involve the inner ear. When abnormal bone remodeling affects the cochlea, sensorineural hearing loss can occur. The type and severity of hearing loss are important when determining the most appropriate treatment.

Otosclerosis often progresses gradually, although the rate of progression varies considerably from person to person. Some people have relatively stable hearing loss, while others experience progressive hearing changes over time. Because hearing loss can also result from other conditions, a change in hearing should be evaluated rather than automatically attributed to known otosclerosis.

There is currently no medication that reverses otosclerosis. However, hearing loss caused by stapes fixation can often be managed with hearing aids or, in appropriately selected patients, stapes surgery. The goal of treatment is to improve hearing and communication while taking into account the patient’s individual anatomy, hearing profile, symptoms, and treatment preferences.

Not all conductive hearing loss is caused by otosclerosis. Other conditions can affect the middle ear or interfere with sound transmission, including:

  • Ear infections
  • Eardrum problems
  • Fluid in the middle ear
  • Ossicular chain disorders
  • Cholesteatoma
  • Trauma
  • Other abnormalities of the middle-ear bones

A detailed examination and hearing evaluation are therefore important before treatment is recommended.

Why Choose Dr. Neil Sperling for Otosclerosis Treatment

If you have been diagnosed with otosclerosis or have unexplained progressive hearing loss, an evaluation by an experienced ear specialist can help determine the cause of your hearing loss and whether treatment may be appropriate.

Dr. Neil Sperling specializes in disorders of hearing and ear surgery in New York City. His evaluation can help determine whether your hearing loss is consistent with otosclerosis and whether options such as hearing aids, monitoring, or stapes surgery should be considered.

Dr. Sperling’s practice provides care for patients with hearing disorders and conditions affecting the middle and inner ear.

Frequently Asked Questions

Otosclerosis is an abnormal bone-remodeling condition in the ear that primarily affects the stapes bone in the middle ear. When the stapes becomes stiff or fixed, it impairs the transmission of sound vibrations to the inner ear, causing conductive hearing loss. In some cases, it can also affect the inner ear, leading to sensorineural hearing loss.

The exact cause of otosclerosis is not fully understood, but it often has a genetic or hereditary component and can run in families. Researchers have also explored links to bone remodeling processes, immune system activity, and past infections, but no single cause explains all cases. A family history increases the likelihood of developing the condition.

The most common symptom is gradual hearing loss, especially difficulty hearing quiet speech, low-pitched sounds, or conversations in noisy environments. Other symptoms may include tinnitus (ringing or buzzing in the ear), dizziness, vertigo, or balance problems. Hearing loss often affects both ears but can start in one.

Diagnosis involves a comprehensive evaluation including medical and hearing history, ear examination, audiogram (hearing test), tympanometry to assess middle ear function, and sometimes a high-resolution CT scan of the temporal bones. These tests help confirm the diagnosis and rule out other causes of hearing loss.

Treatment depends on hearing loss severity and patient goals and may include observation, hearing aids, stapes surgery (stapedectomy or stapedotomy), or cochlear implants in severe cases. There is currently no medication that reverses the bone changes caused by otosclerosis.

Hearing aids are recommended for mild to moderate hearing loss, when surgery is not suitable or preferred, or when hearing loss involves the inner ear. They amplify sound to improve hearing but do not reverse the underlying bone changes.

Stapes surgery involves removing part or all of the immobilized stapes bone and replacing it with a prosthesis to restore sound transmission. Candidates typically have conductive hearing loss primarily caused by stapes fixation and are evaluated based on hearing tests, middle ear condition, and overall health to weigh benefits and risks.

Recovery varies but may include temporary ear discomfort, fullness, changes in hearing, dizziness, or taste changes. Hearing improvement may not be immediate, and patients receive specific post-operative care instructions. Follow-up hearing tests assess surgical outcomes after healing.

Yes, like any surgery, stapes surgery carries risks such as persistent dizziness, hearing loss, tinnitus, taste changes, or infection. The decision to proceed with surgery involves weighing these risks against potential hearing improvement benefits.