Document Detail


Prevalence and geographical distribution of Usher syndrome in Germany.
MedLine Citation:
PMID:  12107518     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: To estimate the prevalence of Usher syndrome in Heidelberg and Mannheim and to map its geographical distribution in Germany. METHODS: Usher syndrome patients were ascertained through the databases of the Low Vision Department at the University of Heidelberg, and of the patient support group Pro Retina. Ophthalmic and audiologic examinations and medical records were used to classify patients into one of the subtypes. RESULTS: The database of the University of Heidelberg contains 247 Usher syndrome patients, 63 with Usher syndrome type 1 (USH1) and 184 with Usher syndrome type 2 (USH2). The USH1:USH2 ratio in the Heidelberg database was 1:3. The Pro Retina database includes 248 Usher syndrome patients, 21 with USH1 and 227 with USH2. The total number of Usher syndrome patients was 424, with 75 USH1 and 349 USH2 patients; 71 patients were in both databases. The prevalence of Usher syndrome in Heidelberg and suburbs was calculated to be 6.2 per 100,000 inhabitants. There seems to be a homogeneous distribution in Germany for both subtypes. CONCLUSION: Knowledge of the high prevalence of Usher syndrome, with up to 5,000 patients in Germany, should lead to increased awareness and timely diagnosis by ophthalmologists and otologists. It should also ensure that these patients receive good support through hearing and vision aids.
Authors:
Ulrich H M Spandau; Klaus Rohrschneider
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2002-05-23
Journal Detail:
Title:  Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie     Volume:  240     ISSN:  0721-832X     ISO Abbreviation:  Graefes Arch. Clin. Exp. Ophthalmol.     Publication Date:  2002 Jun 
Date Detail:
Created Date:  2002-07-10     Completed Date:  2002-10-03     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8205248     Medline TA:  Graefes Arch Clin Exp Ophthalmol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  495-8     Citation Subset:  IM    
Affiliation:
Department of Ophthalmology, Clinical Faculty of Mannheim, University of Heidelberg, Mannheim, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Audiometry
Child
Databases, Factual
Diagnostic Techniques, Ophthalmological
Female
Germany / epidemiology
Hearing Loss, Sensorineural / diagnosis,  epidemiology*,  genetics
Humans
Male
Middle Aged
Prevalence
Retinitis Pigmentosa / diagnosis,  epidemiology*,  genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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