People suffering from the Usher Syndrome usually are born deaf or hard of hearing. Some people lose their hearing only at a later stage in life. Thanks to the screening of the hearing with new-born babies and DNA tests, deafness or loss of hearing can be discovered at an early stage already. In case of deafness and seriously bad hearing balance problems may occur, which will make learning to walk or to ride a bike take longer for children. As the eyesight increasingly deteriorates, lip reading in support of understanding of speech can become increasingly complicated and even impossible eventually.
THE AUDITORY SYSTEM
The hearing system consists of three elements, which together enable us to hear sounds. The external part of the ear (the auricle), the middle ear and the inner ear receive vibrations and convert these into signals that the auditory nerve transfers to the brains. The brains recognise these signals as sound.
Being able to hear means hearing many thousands of different sounds and tones. People do not just hear a sound. We observe a combination of various frequencies (pitches) and the various sound pressure levels of these tones.
LEVELS OF LOSS OF HEARING
Deafness and being hard of hearing includes various levels.
There are six levels of loss of hearing, varying from slight loss of hearing to complete deafness.
‘Deaf’ is a broad concept. Sometimes a person is called ‘deaf’ when he or she does not correctly hear one word in a conversation. Someone wearing hearing aids who can correctly hear speech is also often called deaf or considers him/herself as being deaf. And someone who can hear almost nothing can also be called deaf.
HARD OF HEARING
Children diagnosed for Usher Syndrome who were born hard of hearing or whose hearing deteriorated during childhood or at adult age, have a (progressive) perceptive loss of hearing. The low sound frequencies are heard better than the high sound frequencies.
In order to be able to make the diagnosis for Usher Syndrome, you or your child will have to undergo various hearing tests. After having received the diagnosis for Usher, you or your child will regularly have to go to an audiologist in the Audiological Centre for optimum revalidation of hearing for the whole of life.
The various hearing tests give a good indication of the development of the loss of hearing.
Early intervention of hearing revalidation is crucial. A child that was born deaf or hard of hearing will, with hearing aids and if necessary with the help of sign language, be able to learn the spoken language and fully develop this. Wearing hearing aids or cochlear implants also contributes to the stimulation of the auditory nerve, so that this will optimally keep functioning. As the eyesight deteriorates, more accurately setting of the hearing aids and CIs will become increasingly important.
Not hearing well often makes it difficult to come into contact with other people. You do not always understand the other and this makes you insecure. In a poorly lit room it is really difficult to read the lips or see the signs of the other person. Still there are very many (innovative) solutions and aids that can make communication easier. There are communication techniques, useful apps and we give tips and tricks.
IS THERE ANY TREATMENT?
At this moment there is no effective treatment for Usher Syndrome yet. A DNA test can discover changes in the genes (mutations). Various research institutes all over the world are very busy finding a solution for Usher Syndrome.
Also investigations and studies are conducted in the world that do not specifically offer a solution for people suffering from Usher Syndrome, but that may be of significance for them in the future.
WHAT CAN YOU DO YOURSELF?
- Also protect your ears against prolonged and loud noise
- Daily wear hearing aids and/or cochlear implants in order to keep stimulating the auditory nerve
- Ensure the very best and optimum hearing rehabilitation to make sure that although your eyesight is deteriorating, you can still get as much as possible information from what is going on around you.
- Be aware of the negative (temporary) side-effects of some medicines on hearing, tinnitus and hyperacusis.