Abstract
A study of prevalence and aetiology was performed on 63 children and 127 adults in Denmark with congenital deafblindness. Using a Scandinavian definition of deafblindness, the prevalence of congenital deafblindness was found to be 1:29 000. Thirty-five different aetiological causes of deafblindness were found. Causes of congenital deafblindness were different among adults compared to causes among children. Rubella syndrome (28%, n = 36) and Down syndrome (8%. n = 10) were the largest groups among people above 18 years of age. Among children CHARGE syndrome (16%, n = 13) was the largest group. Ethnicity was also evaluated. Among children 72% were Danish, but among the adults 98% were Danish. Implications of difference in aetiology and ethnicity are discussed in relation to the Scandinavian traditions of clinical practice and identification of congenital deafblindness.SumarioSe llevó a cabo un estudio de prevalencia y etiología en 63 niños y 127 adultos con sordoceguera congénita en Dinamarca. Utilizando la definición escandinava de sordo-ceguera, se encontró que la prevalencia de sordo-ceguera congénita es de 1:29,000. Se encontraron 135 causas de sordo-ceguera. Las causas de sordo-ceguera congénita fueron diferentes entre los adultos comparadas con las de los niños. El síndrome de Rubeola (28% n=36) y el síndrome de Down (8% n=10) fueron los grupos más numerosos entre las personas mayores de 18 años. Entre los niños el síndrome CHARGE (16% n=13) fue el grupo más numeroso. También se evaluó la etnicidad. Del grupo de niños el 72% era daneses, mientras que el 92% de los adultos eran daneses. Se discuten las implicaciones sobre las diferencias etiológicas y étnicas en relación con las tradiciones escandinavas en la práctica clínica y la identificación de la sordo-ceguera congénita.
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