Abstract 99. Jahrestagung der DOG, 29. 9. - 2. 10. 01 im ICC, Berlin

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Prevalence and geographical distribution of Usher syndrome in Germany

1Spandau U., 2Rohrschneider K.

1Universitätsaugenklinik Mannheim, 2Universitätsaugenklinik Heidelberg

Background: Usher syndrome, a combination of retinitis pigmentosa and deafness (USH1) or sensorineural hearing loss (USH2) is the main reason for deafblindness worldwide. In contrast to other countries, the prevalence and geographic distribution of Usher syndrome is unknown in Germany.
Patients and methods: The databases of the Low Vision Department of the University of Heidelberg and the patient organisation Pro Retina were used to calculate the number of USH1 and USH2 patients and their geographic distribution. The prevalence was estimated by the amount of patients in the area of Heidelberg and Mannheim.
Results: The database of the University of Heidelberg comprises 238 Usher syndrome patients and herein 59 USH1 and 179 USH2. The database of Pro Retina contains 246 patients with 21 USH1 and 225 USH2. The total number of Usher syndrome patients is 246 with 72 USH1 and 341 USH2. The prevalence of Usher syndrome in Germany is estimated to be 5-6 per 100000 inhabitants or about 5000 affected. Except of a geographic dominace in the southwest of Germany, the distribution was evenly distributed throughout the country within both subtypes.
Conclusion: The high number of more than 5000 Usher syndrome patients and the even distribution all over Germany ought to increase the awareness of opthalmologists and ENT specialists towards this disease so that an adequate follow-up and support with low vision aids can be ensured in patients with Retinitis pigmentosa.




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