PROGNOSIS

    At this moment, very little is known about the progress and the fastness with which eyesight and hearing will deteriorate. Most people suffering from Usher Syndrome retain their central eyesight in the 5-10 degrees tunnel for a long time. However, individual differences are really large, even within families in which several children suffer from Usher Syndrome.

    LOSS OF AUDITORY AND VISUAL FUNCTION

    It is difficult to predict the progress and the speed of the deterioration. Sometimes these will remain quite stable and at other times the deterioration seems to go faster. The deterioration of hearing does not have to run parallel with the deterioration of eyesight.

    Sometimes you may have a feeling that the eyesight strongly deteriorates because you increasingly miss information and lose sight of the overall picture. Still, a field of vision test may show little deterioration in this situation. Often measurements make clear that not the loss of eyesight but the loss of hearing has increased. Hearing less well you can give you the feeling that your eyesight has deteriorated.

    As vision gets poorer, a greater demand is made on hearing. Think for instance of hearing direction, distance and warning or signalling sounds. All this information is no longer seen and therefore they are to be compensated by the hearing. A new and better setting of hearing aids or CIs can often ‘improve’ the feeling for orientation.

    It is of major importance that both hearing and eyesight are properly supported by a professional who looks at both senses and tries to improve these simultaneously. This can be done by means of adjustments and rehabilitation. Orientation training and/or a mobility training is of little use with incorrectly set hearing aids. Computer training using voice support and/or voice-over is only useful if hearing aids and/or CIs have been correctly set. Do you want to know more about the best possible setting of your hearing aids and CIs? Read on.

    THE DEVELOPMENT

    As little is known about the progress of the loss of hearing and eyesight, a lot of research is required to accurately map out the development and to find out which factors may have influence on this. Knowledge about the natural development for each mutation improves an early diagnosis, guidance of young parents and care for people suffering from Usher Syndrome. Additionally, ENT specialists can inform their patients better about the prognosis and enable patients to better organise their lives. Some studies have already been done into loss of hearing and deterioration of eyesight.

    WHAT CAN YOU DO YOURSELF?

    There are some things that you can do yourself to maximally protect your ears, eyes and balance and so improve your quality of life.

    Join the CRUSH database

    DEVELOPMENT STUDIES

    A detailed mapping out of the natural development of the functioning of eyesight and hearing with the different types of Usher Syndrome is essential for being able to determine the effectiveness of future therapy. Only after studies have demonstrated the effectiveness and safety of certain therapy types, these will be made available to patients on a large scale after a long process of market implementation. At this moment, several natural development studies are being conducted in the Netherlands, being the CRUSH study, the RUSH 2A and a natural development study 1B.