Hereditary Deafness in Man

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Abstract
Hereditary Deafness Associated with Skeletal DiseaseAbout 12 different hereditary diseases have been described in which there is an association between hearing loss and disease of the skeletal system. The skeletal abnormalities include symphalangism, craniostenosis, facial deformities, abnormalities of the extremities and dysgenesis of bone.Dominant Proximal Symphalangism and Hearing LossAlthough four types of hereditary symphalangism have been described, in only one is there an associated hearing loss. Strasburger et al.77 and Vessel78 studied several families with dominant proximal symphalangism. Characteristics of this syndrome included ankylosis of the proximal interphalangeal joints and a conductive hearing loss. The number of . . .