Otosclerosis usually causes conductive hearing loss. This happens when the bone of the stirrup (stapes) of the middle ear doesn’t move properly.
It’s not clear what makes otosclerosis happen. But it seems to run in families and may be linked with hereditary factors. It also appears to be more common after you have certain medical conditions, such as measles or otitis media.
Symptoms
The condition mainly affects one of the three tiny bones (ossicles) in your middle ear. To have normal hearing, the ossicles must move freely in response to sound waves. This allows them to pass sound vibrations to the cochlea in your inner ear, where they turn into electrical signals that travel to your brain. In otosclerosis, abnormal bone tissue grows around the stapes, reducing its ability to move and therefore limiting sound transmission.
In many people with otosclerosis, the loss of hearing is gradual and often occurs in both ears, though it may begin in one ear first. In addition, otosclerosis can cause dizziness and balance problems. It is important to tell your otolaryngologist about any new hearing loss or dizziness so that you can be evaluated for otosclerosis and ruled out for other health issues.
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People with otosclerosis experience trouble hearing low-pitched sounds and whispering. This can make it difficult to follow conversations or understand others’ voices in noisy environments. Eventually, the condition can cause severe hearing loss.
The exact cause of otosclerosis isn’t known, but it is believed to be related to abnormal remodeling of the middle ear bone tissue. Researchers believe that this is due to a combination of factors, including a previous measles infection, stress fractures in the bone tissue surrounding your inner ear, and immune system disorders. The condition also tends to run in families.
The most common symptom of otosclerosis is a reduction in your ability to hear lower-pitched sounds and higher-frequency noises. This type of hearing loss is called conductive hearing loss because it keeps sound vibrations from being conducted from the middle ear to the inner ear. The most common cause of this is that the stapes bone becomes fixed in place, which prevents it from vibrating and transmitting sound. Otosclerosis is much less likely to cause tinnitus, but it can occasionally cause this as well. Other symptoms of the condition include a feeling of fullness in the ear and ringing in the ear. The condition is more common in women and older adults.
Diagnosis
Otosclerosis happens when bone tissue in your middle ear changes abnormally. This usually affects the stapes (stirrup) bone, which normally passes sound vibrations to your inner ear. This can lead to a hearing loss, especially in lower tones and in one or both ears. Otosclerosis also can cause dizziness and a ringing in your ears or head called tinnitus.
It’s not clear what causes otosclerosis, but it may run in families. If you have a parent with the disorder, you have about a 25 percent chance of developing it yourself. Having the measles or other viral infections in childhood might increase your risk. Other causes include stress fractures to bone tissue in the ear or to the bony structure around the cochlea and an immune system condition where the body attacks its own tissues.
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To make a diagnosis, your healthcare provider will examine you and look at your ears. They might ask about your family’s history of hearing loss and any medications you take. They may order an imaging test such as a CT scan or an MRI of your ear to see how the bones and other structures in your ear are working.
Your health care provider will probably refer you to an otolaryngologist or audiologist, who are doctors who specialize in diseases of the ear, nose, and throat. These specialists can help you manage your symptoms and treat them as needed.
They will likely give you a hearing test that measures your ability to hear across a range of frequencies. They may also do a test called a tympanogram that measures your middle ear’s ability to pass sound waves.
Your otolaryngologist may perform surgery in one or both ears. This is called a “stapedectomy.” The surgeon removes the damaged stapes bone and replaces it with a plastic or metal implant that bypasses the faulty bone. The procedure is very successful, and your hearing should improve afterward. Dizziness often goes away as well, but tinnitus can return or get worse in some people. It’s important to have regular follow-up visits with your otolaryngologist.
Treatment
Symptoms of otosclerosis typically begin to occur between the ages of 10 and 45, with most people experiencing them in their twenties. It is thought that the condition is triggered by a combination of genetic, environmental, hormonal and other factors. It is more common in women than men, and tends to run in families.
Deep inside the middle ear are three tiny bones (ossicles) that vibrate and amplify sound waves to convert them into nerve signals that can be sent to your brain for processing. Otosclerosis most often occurs when one of these bones, the stapes bone, fuse together and prevent the ear from hearing. It is a type of conductive hearing impairment and can be treated by surgery.
In some cases the condition can spread to the inner ear, which may cause sensorineural hearing loss and unsteady balance. In these instances a person will need to use a hearing aid or can take medication to control symptoms.
Treatment options for otosclerosis include medication, hearing aids and surgery. Medications can help improve symptoms and are often prescribed by an otolaryngologist or otologist, both of which are doctors who specialize in diseases of the ears. They can prescribe medications to slow the progression of otosclerosis, reduce inflammation and suppress inflammatory cells that are responsible for the condition.
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Hearing aids are a great option for those suffering from otosclerosis, and can help restore some or all of your hearing. These devices can help with a wide range of hearing issues associated with the disease, including distorted or muffled sounds, and trouble hearing in noisy environments.
Surgical treatment for otosclerosis involves removing the non-functioning stapes bone and replacing it with a titanium implant. This is known as a stapedectomy and can be performed under local or general anesthesia, depending on the needs of each individual patient. The procedure is highly successful and, according to the NLM, usually only requires an overnight stay in hospital or clinic.
The National Institute on Deafness and Other Communication Disorders (NIDCD) has an online resource to find more information on otosclerosis, including symptoms and treatment options. The site also has a directory of organizations that provide additional resources on otosclerosis and related disorders.
Prevention
A person with otosclerosis experiences gradual hearing loss, often starting in one ear. This usually affects low-pitched sounds such as car engines and men’s voices first, and can get progressively worse over time. Those with the condition may also experience dizziness and other balance issues. Sodium fluoride is sometimes prescribed to slow down the progression of otosclerosis by inhibiting bone resorption and encouraging new bone growth, but the effectiveness of this can vary from person to person.
There are no preventable risk factors for otosclerosis, though it does tend to run in families. Certain medical problems such as measles, post-pregnancy conditions, stress fractures to the inner ear bone tissue and immune disorders can all raise your chances of developing this condition.
Gender and ethnicity are also important considerations when it comes to otosclerosis. Women are at a greater risk of developing the disorder, and research suggests that they lose their hearing faster than men do. Caucasians are at a higher risk as well, though it is rare for African Americans to develop the condition.
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Although it can be difficult to diagnose otosclerosis in its early stages, regular check-ups with your GP, audiologist or ENT department will help you keep track of your progress. A simple physical exam and a hearing test will give you a good idea of whether the disease is present and if treatment is needed.
If you have otosclerosis, it’s essential to see an ear, nose and throat (ENT) specialist for a comprehensive evaluation. These specialists can help you manage the condition with hearing aids and/or surgical intervention such as a stapedectomy. This surgery involves removing the affected portion of the stapes and replacing it with a prosthetic device to allow sound vibrations to pass through to the inner ear, improving or even restoring your hearing.
While you can’t prevent otosclerosis, regular visits to an ENT specialist will keep the condition under control. These doctors will monitor the symptoms and adjust treatment as necessary. Otosclerosis can cause significant hearing loss over time, so it’s important to seek treatment if you notice signs of the disorder.
