Understanding Otosclerosis: Causes and Treatment

Otosclerosis is a progressively harmful disorder of the ear that is characterized by abnormal bone growth in the middle ear. The effect of this abnormal growth, which is primarily hereditary, is to impede or halt the conveyance of sound waves. The first signs of otosclerosis most often become apparent in the late teens to early 40s, and it may affect both men and women of any race.

Otosclerosis is the abnormal growth of bone in the middle of the ear, which restricts the stapes bone from moving. In otosclerosis, the stapes bone gets fixated to the underlying bone in such a way that it does not vibrate, blocking sound transmission through the inner ear and hence to the eardrum and cochlea. This results in progressive deafness. Read on to understand the causes of otosclerosis.

What precisely causes otosclerosis is still not well understood. Here are some of the contributing factors.

• Genetics: A family history of otosclerosis increases the risk. Several genes have been associated with the condition.
• Hormonal factors: Some researchers believe that hormonal changes, such as those that occur during pregnancy, may trigger the onset or progression of otosclerosis in people who are genetically predisposed to the disorder.
• Measles virus: Some studies have suggested a possible link between the measles virus and otosclerosis. One theory is that the virus may play a role in remodeling bone in the ear.
• Immune response: Some scientists think an abnormal immune response or autoimmune factors might cause otosclerosis, although this idea isn't widely accepted.
• Other factors: Although not strongly correlated, other possible triggers or contributors to otosclerosis include stress fractures of the bone tissue surrounding the inner ear and exposure to loud noises for prolonged periods.

Common early symptoms of otosclerosis include the following:

1. Progressive Hearing Loss: One of the major early signs of Otosclerosis is a gradual decrease in hearing that comes and goes initially.

2. Vertigo: Vertigo is a spinning or rocking sensation that affects balance, especially when you tilt your head.

3. Tinnitus: Tinnitus refers to a ringing, buzzing, roaring, or hissing sound in your head not caused by an external source.

4. Difficulty hearing low-pitched sounds: Firstly, people with otosclerosis have difficulty hearing low-pitched sounds. A common complaint, for example, might be that “I can’t hear a whisper,” or “I can’t hear soft sounds.”

If you experience the above-mentioned symptoms, make sure that you consult an ENT for expert opinion.

A diagnosis of otosclerosis is generally based upon a combination of these procedures:

1. Medical History Review and Physical Examination

• The physician will inquire as to the particulars of the patient's hearing loss, whether or not it has progressed, and if anyone else in the family has had to get hearing aids or has had otosclerosis. An examination using the otoscope can be performed on the same day, allowing the physician to look in the patient's ear and verify that the eardrum and middle ear appear healthy.

2. Audiometric Testing (Audiogram)

• An audiogram can check hearing and test a person's ability to hear different sounds, pitches, or frequencies. People with otosclerosis often have a certain regular type of conductive hearing loss shown on their audiogram.

3. Tympanometry

• Tympanometry tests the mobility of the tympanic membrane (eardrum) and the tiny bones of the middle ear (ossicles). An abnormal finding in some patients with otosclerosis is decreased mobility of the stapes bone that may be seen on tympanometry.

4. Computed Tomography (CT) Scan

• Occasionally, otosclerosis can be diagnosed by finding its telltale signs with a CT of the temporal bone. However, imaging is not essential in making this diagnosis, so it is not required for most patients. 

5. Stapedial Reflex Testing

• A useful test for otosclerosis is the stapedial reflex test. The stapedial reflex is a reflex that occurs in the middle ear in response to loud sounds. The reflex itself is the contraction of the muscle attached to the stapes bone of the middle ear.

6. Tuning Fork Tests

• Another test that can be useful is a tuning fork test. This test can determine if a loss of hearing is conductive or sensorineural and is applied when a patient presents with impaired hearing as an initial measure.

Treatment for otosclerosis aims to improve hearing and reduce the symptoms. The choice of treatment is determined by the severity of the condition and the specific needs of each patient. Therapeutic options include:

1. Observation: Milder cases of otosclerosis, may simply need to be monitored with regular hearing tests.

2. Hearing Aids: For many people with otosclerosis, amplifying sound with a hearing aid can help make up for hearing loss. This non-invasive option is for people who are not candidates for surgery or who prefer not to have surgery.

3. Surgery: Most people with otosclerosis regain their hearing through surgery. The most common procedure is called a stapedectomy. During a stapedectomy, part or all of the immobilized stapes bone is removed and replaced with a prosthetic device, such as a wire, a loop, or a spring. Removing the immobilized bone and replacing it with a prosthetic device restores the movement of the bones in the middle ear, and this should improve or restore hearing. During a similar surgery called a stapedotomy, a tiny hole is made in the stapes and a prosthesis is inserted. The success rate for both of these surgeries is very high. Of course, all surgeries come with risks that should be considered and discussed with an otolaryngologist.

4. Sodium Fluoride Therapy: Various studies have suggested that supplemental Sodium Fluoride can slow otosclerotic progression, especially the sensorineural hearing loss component. This treatment might originate its beneficial effects by inducing the strengthening of otospongiotic bone or damping down active otosclerotic foci. The efficacy of treatment with Sodium Fluoride has been controversial, and it has not become universally accepted in practice.

5. Bone-Anchored Hearing Aids (BAHA): This option is for people with substantial mixed hearing loss for whom regular hearing aids are not effective or appropriate. A BAHA is a surgically implanted device that sends sound vibrations directly to the inner ear through the skull bone.

6. Cochlear Implants: These electric devices are generally reserved for use in patients with severe to profound sensorineural hearing loss in both ears who do not benefit enough from hearing aids.

If otosclerosis is not adequately treated or controlled, it may result in several problems, including:

• Progressive Hearing Loss: The most significant complication of otosclerosis is a continued decrease in hearing. Over time, the hearing loss can progress from being conductive (due to fixation of the stapes bone) to mixed or sensorineural with involvement of the inner ear.
• Tinnitus: The persistent ringing, buzzing, or hissing in the ears can become more pronounced and bothersome.
• Vertigo: Some individuals may experience episodes of dizziness or the room spinning. However, this is rare.
• Poor Quality of Life: Progressive hearing loss and other symptoms can affect the ability to communicate, work, and socialize. These difficulties can eventually lead to feelings of depression, anxiety, or isolation.

There is no specific way to prevent otosclerosis because the exact cause is not known. However, several general steps and methods can help reduce the risk or delay its onset:

• Early Detection: Regular hearing check-ups can identify the early stages of otosclerosis, especially for people who have a family history of the condition. Early intervention can bring about better outcomes.
• Avoid Loud Noises: Although there's no conclusive proof that continuous loud noise could lead to otosclerosis, it is always a good idea to protect your ears from the noise to prevent other types of hearing loss.
• Vaccination: There is some speculation that otosclerosis may be caused by the measles virus. Making sure you and your children are up to date with measles vaccinations may offer some level of protection from otosclerosis but that is not definitive.
• Staying Informed on Medications: Though there is no direct association between otosclerosis and medication, there are certain drugs that destroy hearing. Always consult about any potential side effects of any medication from your healthcare provider.
• Choosing a Healthy Lifestyle: They are not directly preventive but do help to maintain a well-being and healthy lifestyle. Regular exercise and a well-balanced diet can promote good health and prevent disease. Yoga can help increase the blood circulation in the body and make the internal body system work properly. The overall well-being of a person can approximately tell about a person's hearing ability.

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