[Hypo-/aplasia of the nasal bones--an autosomal dominant inherited anomaly]

We report a family with isolated hypo- or aplasia of the nasal bones as an autosomal dominant trait. The conspicuous shape of the nose resembled that of "potato nose". This anomaly is caused by a specific developmental field defect of the medial nasal processes with no genetic relation to frontonasal "dysplasia". The minimal intrafamilial variation suggests a monogenic trait with an autosomal dominant mode of transmission. For the ENT practitioner it may be added that patients with hypo- or aplasia need no treatment unless a septal deviation causes nasal obstruction.