A family with autosomal dominant inherited dysmorphic small auricles, lip pits, and congenital conductive hearing impairment

Design: We examined 3 generations in a family for congenital conductive hea ring impairment, dysmorphic small auricles, and lip pits. Setting: Department of Otorhinolaryngology, University Hospital Nijmegen, N ijmegen, the Netherlands. Results: Seven members of the family had bilateral dysmorphic auricles. Thr ee subjects had either a pit or dimple in the lip. Two subjects had congeni tal conductive hearing impairment. Conclusion: Using gene linkage, we confirmed that these autosomal dominant inherited branchial anomalies present a new separate branchial arch syndrom e.