May be associated with hearing loss and secondary speech disorder. Head and neck manifestations of this abnormality include flame nevus of the forehead and characteristic sulci of the ear lobe. Ear located on the cheek. May represent lack of aural ascent due to underdevelopment of the auricle. An ear that is set below an arbitrary line drawn between the lateral canthus of the eye and the occipital protruberance is considered low-set.
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.