Audiology and Hearing Processes

Neuronopathic Gaucher's News Contents
Gaucher's News Contents

Pauline Campbell is an Audiological Scientist at Great Ormond Street Children's Hospital. She explained the possible problems with hearing processing in children with Type 3 Gaucher disease.

'Audiology (the study of hearing) is a relatively new area since it was discovered that the brain stem is involved in NGD,' explained Dr Campbell.

Audiology's involvement is to look at peripheral and central auditory processes in NGD and to assess the auditory pathways from the external ear right through the brain.

Recently it has been identified that children with NGD have a deficient central auditory function (CAPD). This means that they have problems processing information when the environment is not optimal for listening eg background noise.


In order to assess this a series of tests were carried out to look at how these children filter out words from distorted speech, understand speech when there is a background of noise and finally how they recognise words that are being said at the same time in each ear.

Out of the ten children tested , seven had an identified problem. Following these tests a further test was carried out in conjunction with the neuropsychology department where a number of electrodes where placed on the children's head while they watched videos.

A series of sounds were played to them through earphones. These were played at different pitches and the electrodes recorded whether or not the children detected a change in the pitch of the sound.

In seven out of eight children, there was an inability to detect a change in the pitch. This test is known as mismatch negativity.

Poor Listening Skills

As a result of these tests it has been identified that these children may have poor listening skills and often experience difficulties when there is background noise such as in a classroom and playground.

Therefore it is important that teachers are made aware of these difficulties and look at practical ways to help these children. These problems can be overcome with training the child to listen, increase practical exercises for conversation skills, grammar and vocabulary. Other ways to manage these difficulties could be a speaker system within the classroom.


'In summary it is very important to carry out a series of objective tests on patient with NGD before enzyme replacement therapy and to monitor on a regular basis the effects and efficiency of enzyme replacement therapy on the auditory system.

Finally Dr Campbell said: 'How must the world seem to our children? It must be a difficult place, challenging and threatening. Even more challenging is the fact that, to the outsider and people working with our children, they look normal.

Neuronopathic Gaucher's News Contents
Gaucher's News Contents

Source: Gaucher's News February 2000. © Copyright Gauchers Association 2000