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The objective of the study was to estimate the prevalence at birth and epidemiologic characteristics of patients/cases with both isolated and multiple “syndromic” external ear congenital abnormalities (CAs) in Hungary. The Hungarian Congenital Abnormality Registry, 1980–1996, included 649 cases with isolated external ear CAs, while the number of cases with unclassified multiple CA, including ear CAs, was 331. Thus the prevalence at birth of cases with isolated external ear CAs and unclassified multiple CAs was 0.30 and 0.15, respectively, for a total 0.46 per 1000 births. After reevaluation of reported 354 cases with isolated external ears CAs in the Hungarian Case-Control Surveillance of Congenital Abnormalities, 74 (20.9%) and 236 (66.7%) were affected with mild and severe microtia, while 24 (6.8%) had anotia. The fourth group included 20 cases with the combination of anotia/microtia and external/middle ear CAs. Isolated ear CAs showed a slight male excess (54.0%) and strong predominance of unilateral manifestation (93.4%). Multiple ear CAs showed a stronger male excess (65.4%) and less frequent unilateral affection (62.2%). In conclusion, ear CAs had a low diagnostic validity; thus it was necessary to reassess the data and to reclassify several cases.

Prevalence at birth of congenital abnormalities of external ears in Hungary