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Otosclerosis

Published by Bupa's health information team, August 2008.

This factsheet is for people who have otosclerosis, or who would like information about it.

Otosclerosis is a disease in which the bone structure inside your ear changes, leading to hearing loss. It may also be called otospongiosis. Otosclerosis is a common cause of deafness in young adults.

• About otosclerosis
• Symptoms of otosclerosis
• Complications of otosclerosis
• Causes of otosclerosis
• Diagnosis of otosclerosis
• Treatment of otosclerosis
• Further information
• Questions and answers
• Related topics
• Sources

About otosclerosis

Your ear is made up of three parts: the outer ear, the middle ear and the inner ear. Inside the middle ear, there are three tiny bones called the ossicles. When sound waves enter your ear, they hit your eardrum, causing the three ossicles to move backwards and forwards. This transmits the sound waves to your inner ear, where they are converted into nerve signals and sent to your brain.

The structures of the middle ear

In otosclerosis, the bone structure of your inner ear starts to change, with one of the three ossicles (called the stapes) becoming fused together with the surrounding bone. Eventually, this causes the stapes to become fixed in a certain position, so that the ossicles are unable to move. Sound waves can no longer be transmitted into the inner ear. When sound waves cannot get through to the inner ear, it is called conductive hearing loss.

Otosclerosis usually develops when you are in your 20s or 30s. It is commoner in white people and in women.

Symptoms of otosclerosis

The main symptom of otosclerosis is deafness. You might notice this before you reach the age of 30, but it can take a number of years to become obvious. You may feel you can hear better in noisy surroundings.

You might also get tinnitus (a ringing sound in your ears) if you have otosclerosis. Some people may get mild vertigo (a feeling of giddiness even when you are standing still), but this is rare.

Although not necessarily a result of otosclerosis, if you have these symptoms, you should visit your GP.

Complications of otosclerosis

On rare occasions, the abnormal bone growth that occurs in otosclerosis can spread further into the inner ear, affecting a structure called the cochlea (the part of the inner ear responsible for hearing). This damages the tiny hair cells in the cochlea that are responsible for turning sound waves into electrical signals, causing further hearing loss. This type of hearing loss is known as sensorineural deafness and cannot be helped by surgery.

If otosclerosis spreads to the area of the inner ear responsible for balance, it can also cause vertigo.

Otosclerosis and pregnancy

Otosclerosis can react to female hormones. This means that if you are a woman and become pregnant, you may find that your condition gets worse. There is also some suggestion that taking the contraceptive pill or hormone replacement therapy (HRT) can worsen otosclerosis. If you have otosclerosis, your GP should discuss this with you before prescribing the contraceptive pill or HRT.

Causes of otosclerosis

Otosclerosis is usually caused by a faulty gene, inherited from one of your parents. However, it can also occur without either of your parents having the disease.

If a family member has the disease, you are likely to have similar symptoms, with the disease starting at a similar age and progressing at about the same rate.

Diagnosis of otosclerosis

Your GP will ask about your symptoms and examine you. He or she may also ask you about your medical history and carry out some initial basic tests to assess your hearing.

If your GP thinks you have signs of hearing loss, he or she will refer you to an ear, nose and throat (ENT) surgeon at your local hospital. You will need to have the following tests before your doctor can diagnose otosclerosis.

• Pure tone audiogram. This test takes place in a soundproof room. You will be asked to wear headphones, through which notes of different pitches and volume will be played to you. You will be asked to press a button when you can hear the sounds.
• A tuning fork will be placed on the bone behind your ear, and you will be asked if you can hear the sound. This can help your doctor work out whether your hearing loss is due to otosclerosis or another problem with your ears.

You may also have the following tests.

• Speech audiogram. This test also takes place in a soundproofed room. A recording of a person saying various words or phrases will be played to you through headphones or loudspeakers at different volumes. Background noise may also be played into the room to try to make the test true to life. You will be asked to repeat the words that you hear.
• Computerised tomography (CT) scan. A CT scan uses X-rays to make a three-dimensional image of part of your body. In otosclerosis, the scan is done in a way that allows your doctor to measure how dense the bone around your ears is, showing which parts have been affected by the disease.

Treatment of otosclerosis

Having a hearing aid fitted or having surgery are the two treatment options for otosclerosis, which can help to improve hearing. Your ENT surgeon will discuss both options with you. Which treatment you have will depend on how severe your symptoms are and your own individual circumstances. If your hearing loss is mild, you may decide that you can manage without any treatment at all.

Hearing aids

Having a hearing aid may be enough if you have only a slight hearing loss. A hearing aid is a small electronic device that fits in or behind your ear, and works by making sound loud enough for you to hear.

If you decide to have a hearing aid, you will see an audiologist - a health professional specialising in helping people who have problems with their hearing. Your audiologist will discuss the different types of hearing aid available with you, and advise which is the best one for you. Newer types of hearing aid can transmit sound waves straight through the bone in your skull, and may be particularly useful in people with otosclerosis.

Surgery

You will probably only be advised to have surgery if your hearing loss has reached a certain level and you are having trouble understanding other people speaking, even with a hearing aid.

Surgery for otosclerosis is often able to improve your hearing, but carries a risk of complications - such as losing more, or all of your hearing. Your surgeon will discuss the benefits, potential risks and possible alternatives to the procedure with you before you have the operation. You will also have a chance to ask any questions you have about the procedure.

The operation may be done under general or local anaesthesia. If you have a general anaesthetic, you will be asleep during the procedure. If you have a local anaesthetic, you will stay awake during the procedure but feeling from your ear will be blocked.

During the surgery (called a stapedotomy), a small cut will be made just above the opening to your ear, or inside your ear canal. Your surgeon will then make a small opening in the footplate of the stapes bone with tiny instruments, or sometimes with a laser. Sometimes your surgeon may completely remove the stapes bone. An artificial piece of bone, made out of plastic or metal (called a prosthesis) is then inserted into your ear and connected to the other two ossicles. The procedure allows the three ossicle bones to move freely again, so that sound can be transmitted to your inner ear.

Further information

• Deafness Research UK
  0808 808 2222 (Helpline)
  www.deafnessresearch.org.uk
• British Tinnitus Association
  0800 018 0527 (Helpline)
  www.tinnitus.org.uk

 

Related topics

• Hearing loss

Sources

• Otosclerosis. Deafness Research UK. www.deafnessresearch.org.uk, accessed 7 May 2008
• Otosclerosis. British Tinnitus Association. www.tinnitus.org.uk, accessed 7 May 2008
• Kumar P, Clark M. Clinical Medicine. 6th ed. Elsevier, 2005: 1155
• Roland NJ, McRae RDR, McCombe AW. Key topics in otolaryngology. Oxford: Bios Scientific Publishers Ltd, 1995
• Otosclerosis. ENT.UK - the British Association of Otorhinolaryngologists - Head & Neck Surgeons. www.entuk.org, accessed 7 May 2008
• Bone conduction hearing aids. Royal National Institute for Deaf People. www.rnid.org.uk, accessed 18 June 2008

This information was published by Bupa's health information team and is based on reputable sources of medical evidence. It has been peer reviewed by Dr Sebastian Hendricks, MSc, Consultant in Audiovestibular Medicine, Barnet & Chase Farm Hospitals NHS Trust, Edgware Community Hospital, and by Bupa doctors. It has also been reviewed by Deafness Research UK. The content is intended for general information only and does not replace the need for personal advice from a qualified health professional.

Publication date: August 2008.