There is a range of congenital ear, nose and throat problems - some occurring alone and others as part of a syndrome. The underlying causes are varied but, as with any congenital disorder, they can be broadly divided into chromosomal abnormalities (mutations and inherited problems), diseases associated with prenatal infection, maternal drug abuse, environmental factors, iatrogenic causes and abnormalities of unknown aetiology. The history will generally point you in the right direction and management will be guided by the degree of functional impairment as well as by any psychological distress associated with the condition (including parental when the child is very young). These are generally uncommon conditions, occurring in <5:10,000 births for each of ear, nose and throat abnormalities.
This is a companion discussion topic for the original article at https://patient.info/doctor/history-examination/congenital-ear-problems