Deafness, Conductive, With Ptosis And Skeletal Anomalies

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Jackson and Barr (1978) described 2 sisters with conductive hearing loss from combined atresia of the external auditory canal and the middle ear space, complicated by chronic infection, ptosis, thin, pinched-nose facial appearance, ectodermal dysplasia manifested by delayed hair growth and dysplastic teeth, and skeletal abnormalities (internal rotation of hips, dislocation of the radial heads and fifth finger clinodactyly). Lowden (1980) observed an identical case.