The CRUSH database is a collection of various clinical data, including audiograms, field of vision examinations and DNA results. In this way the prognosis can be better recorded and a possible explanation for the large individual differences in loss of hearing and eyesight between patients, even of the same family, can be found. This CRUSH database will be accessible for other centres, so they can store their data in the database as well.
Most patients are already known in the national RD5000 database, but this database only contains personal data and the diagnosis. The CRUSH database, in which the clinical data of patients are stored as well, is intended for all people who have been diagnosed for Usher Syndrome.
The (inter)national CRUSH database will enable the researchers of the Usher Syndrome Expert Centre of the Radboudumc to store all important data of people suffering from Usher Syndrome. Here they do not think only of research data telling something about the seriousness of the loss of hearing and vision, but also about general things, such as weight, life pattern, use of certain medicines, etc.
This database has two advantages:
- Firstly, this database can be used to map out the natural course of the disorder for each person. If it is exactly known how the impairment of hearing and vision increases in the course of time, this can be used in the evaluation of future therapies, such as gene therapy. By starting this inventory already now, the researchers will be ready to test these therapies by the time that a therapy for vision or hearing has been developed.
- Secondly, the researchers hope that this natural development study will help them explain the differences in the loss of hearing and poor eyesight of people suffering from Usher Syndrome. Strikingly, there are many differences in loss of hearing and poor eyesight between brothers and sisters suffering from Usher Syndrome. So far, they have not been able to explain these differences based on the hereditary components and they hope this database will help them clarify this. Here you may think of, for example, an oversensitiveness for certain medication, exposure to too much sunlight or too much noise.
Of course, this database complies with the important privacy and anonymity regulations that we have agreed upon internationally and therefore also in the Netherlands with respect to scientific research.
The dream of all people who are confronted with Usher Syndrome in their daily lives is that researchers will in time find a treatment to stop the deterioration of eyesight and hearing.
Our dream can come true when all patients with Usher Syndrome know their gene and mutation.
By taking action yourself and to register yourself in the international CRUSH database of the national Usher Syndrome Expert Centre you contribute to sooner finding a treatment.
Do you have any questions about the CRUSH database? We have answered the most frequently asked questions.