Otosclerosis is an abnormal bone growth of the bones in the middle ear. It is a common cause of hearing loss and one of the most common causes of progressive hearing loss in younger adults.

What is Otosclerosis?

The name and definition of otosclerosis come from the greek words “oto” (ear) and “skleros” (hard). Otosclerosis is caused by an abnormal growth of bone tissue in the ears, which causes hearing loss. In its early stages, the sufferer will normally not be affected. In most cases, a surgical operation will restore the hearing.

How does otosclerosis cause hearing loss?

Otosclerosis has to do with the three small bones in the middle ear, more specifically the stapes. A part of the stapes bone will grow abnormally and this abnormal bone growth will prevent the stapes from vibrating normally in response to sound. The stapes are vital for transmitting sound waves from the outer ear, through the middle ear and into the inner ear. When the stapes do not move properly, the sound that is sent from the middle ear into the inner ear is reduced which causes a hearing loss.

What causes otosclerosis?

Otosclerosis is often inherited, although isolated cases do occur. For instance,  measles and pregnancy have been observed as potential causes for worsening the case of otosclerosis.

The most famous otosclerosis victim is said to have been Beethoven. Deafness made him unable to hear his late compositions.

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What are the symptoms of otosclerosis?

An otosclerosis diagnosis should be made by a specialist, but some signs can be detected by the patient or their relatives.

Strong background noise can add to the confusion of people with nerve deafness, but in the case of otosclerosis, this confusion does not often occur. Sometimes the person may even hear better in noisy surroundings (paracusis), possibly because of the high frequency and loudness of other people's voices in those circumstances. Otosclerosis tends to affect low frequencies more than high ones.

Symptoms of otosclerosis may also appear in the form of dizziness, balance problems (vertigo) and tinnitus.

Otosclerosis often causes a progressive conductive hearing loss. A progressive hearing loss is a hearing loss that gets worse (more severe) over time.

How is otosclerosis diagnosed?

The diagnosis and treatment of otosclerosis are relatively straight-forward. It is however important to consult a specialist as soon as possible and to plan the course of treatment.

Otosclerosis is diagnosed by a medical examination of the middle ear, typically by an ENT-doctor or a hearing professional.

Treatment of otosclerosis

The treatment of otosclerosis can be divided into two areas: the use of hearing aids and surgery of the stapes. Hearing aids help people who are suffering from conductive deafness, including otosclerosis. However, a hearing aid will not cure the deafness. Since the deafness is progressive, more powerful hearing aids may be needed as time goes by. In the early stages, hearing aids are a great help for those who do not wish to undergo surgery.

Surgery for otosclerosis

Surgical operations for alleviating otosclerosis fall into the field of otorhinolaryngology. The relatively simple procedures are widely performed to reinstate hearing and cure otosclerosis. In a stapedectomy, which is a surgery of the stapes, either the part of the stapes with the abnormal bone growth is removed in order to insert a tiny implant (stapedotomy), or the entire stapes bone is replaced by a small prosthesis (stapedectomy). Both surgeries of the stapes can restore hearing. In many of the cases the symptoms of vertigo and tinnitus will also disappear.

What should I do if I think I have otosclerosis?

If you think that you have otosclerosis, e.g. because others in your family have it, as it is often inherited, you should contact a doctor or a hearing professional to have your middle ear examined and have your hearing tested.

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