[Familial bilateral atresia of the auditory canal with hypertelorism]

The incidence of malformations of the ear is 1:5000, the incidence of stenosis and atresia is 1:12,000. 30% of the malformations are bilateral. In only 0.3% it is the only malformation. Inherited bilateral atresia of the external auditory canal associated with hypertelorism as the only additional malformation has not yet been described. Basing on a case report the way of interdisciplinary rehabilitation of children affected by bilateral hearing impairment is discussed. Special emphasis is on the discussion of the necessity of radiological examinations for the exclusion of cholesteatoma in an atretic ear which could be found in a 5-year old boy of the described family.