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Deformities of the external and middle ear with conductive deafness are common in mandibulo-facial dysostosis but cochlear function is nearly always normal. Twelve patients with the typical appearance and characteristics of the lesion are described. All had some degree of atresia of attic and antrum with absent or deformed ossicles but 4 had external auditory meatuses of normal calibre. The characteristic tomographic appearances of the ear are described.
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Lloyd et al. (1979) studied this question.
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