Brittle bone disease (osteogenesis imperfecta)

Written by Dr Colin R Paterson, consultant physician

What about older men?
Men do not appear to have a 'menopause' and older men with OI do not have a rise in the fracture rate in later life.

How common is hearing loss in OI?
About 50 per cent of people with OI find that they have impaired hearing with an onset mainly in the teenage years or early adult life. Not everyone is affected and, of those who have no hearing loss at the age of 50, relatively few become deaf thereafter.

Hearing loss in OI is most commonly due to problems in the small bones in the middle ear which may be fractured or deformed so that sounds are not transmitted effectively to the inner ear. There are a smaller number of people with OI whose hearing loss is caused by problems in the inner ear and some with mixed causes. It is important to investigate hearing loss properly to find out exactly what the cause is, because this influences treatment.

For most people with both types of hearing loss, hearing aids are the first line of treatment at any age. The hearing loss that is caused by problems in the middle ear may, if severe or progressive, be helped by surgery. The decision about whether or not surgery is appropriate needs discussion with an expert ENT (ear, nose and throat) surgeon, preferably someone with a special interest in OI.

What is temporary brittle bone disease?
A special type of OI has become recognised in the last 20 years. In this fractures, and often many fractures, occur in the first year of life and largely in the first six months. The identification of this disorder is still controversial, in part because the cause is not yet known. The disorder is more common in infants born before term and in twins. In some cases there are minor features of collagen abnormality in parents or other relatives.

Where can I obtain fuller information about OI?
In the UK the Brittle Bone Society has a website (www.brittlebone.org) and can be contacted by mail, e-mail or telephone (a freephone helpline is available at 08000 282459).

In the United States the corresponding society is the Osteogenesis Imperfecta Foundation (www.oif.org).

Both societies issue factsheets on different aspects of OI and may be able to give advice on appropriate specialists, welfare provision and education. There are similar societies in many European countries and in Canada, South Africa, Australia and New Zealand.

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