Abstract
A unilateral mixed deafness with congenital ptosis and the Goldenhar syndrome were seen in a family.
The patient was a five-year-old boy with congenital ptosis of the right lid and right severe mixed deafness. His father also has slight ptosis of the right lid. His paternal aunt was diagnosed as the Goldenhar syndrome.
These two conditions, unilateral mixed deafness with congenital ptosis and the Goldenhar syndrome, were considered as not appearing by chance in a family, because relatives of the Goldenhar syndrome patients reported in the literature sometimes have such anomalies as syndactyly, spina bifida and anomalies of the external ear. Moreover, congenital ptosis and unilateral deafness are symptom of the Goldenhar syndrome.