Ears are low-set with or without abnormal auricles. Koretzy et al. described an unusual type of pulmonary valvular dysplasia which showed a familial tendency with either affected parent and offspring or affected sibs. Clenched hand, short sternum, low arch dermal ridge patterning on fingertips and low-set malformed auricles. Low set ear in renal agenesis 10. The external ears assume a horizontal position with the lobules located near the midline.
Severe degree is found in the cyclopia series.
Most are harmless, occasionally they can get infected, form retention cysts and cause chronic discharge. Autosomal dominant inheritance with variable expressivity and incomplete penetrance. Many occur sporadically and are associated with hereditary deafness. Report of large kindreds such as that of Bhalla et al , in which there are no associated features or lateral cervical sinuses suggests that this is a distinct mendelian dominant. The pedigree was traced through seven generations indicated in the expression of the occurred bilaterally as a small pit just anterior to the crus at the root of the ascending helix.