The association of cleidocranial dysostosis with hearing loss.
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.
D000328:Adult; D002973:Cleidocranial Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D004432:Ear, Middle / Q000002:abnormalities / Q000000981:diagnostic imaging; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008297:Male; D010375:Pedigree; D011859:Radiography