Congenital dystrophy or absence of the nails are rare anomalies which occur in a variety of forms. A few or all of the nails may be involved. The abnormality may be the only malformation present, or it may be associated with developmental defects of teeth and the pilosebaceous apparatus as part of a more widespread congenital ectodermal defect.1 The association of defective nails with absent or rudimentary patellae and deformities of the elbows, fingers, and toes is known as the nailpatella syndrome or hereditary onychoosteodysplasia.2
An account follows of 2 siblings in each of whom congenital nail dystrophy was associated with deafness. No previous report of such an association has been found in a search of the available literature.
Report of Cases
A 10-year-old girl, whose parents were first cousins on the maternal and second cousins on the paternal side, was examined because of deafness. She was the second