The association of cleidocranial dysostosis with hearing loss.

Abstract

Three new cases of cleidocranial dysostosis with hearing loss are reported in this paper. The significant points concerning this association are: (1) the hearing deficit is predominantly a middle ear conduction problem secondary to structural abnormalities of the ossicles; (2) there is sometimes a small bone conduction deficit indicating either a cochlear or an eighth nerve problem; (3) the middle ear hearing loss was corrected surgically in one reported case; (4) there is dense sclerosis of the temporal bone which makes a middle ear operation technically difficult; and (5) hearing loss with cleidocranial dysostosis may be more common than the number of cases in the literature suggests.