In children, congenital deafness presents not infrequently. The majority of cases are sensorineural in nature and congenital conductive deafness is far less common. The causes range from abnormalties and fixation of ossicles, ankylosed malleo-incudal joint and absence of one or more ossicles. We describe a case of congenital ossicular abnormality, presenting with conductive deafness.
A patient with Turner's syndrome presented with a rare anomaly of absent oval window, inferiorly placed facial nerve, and abnormal stapes. To our knowledge, this is the first report of this combination of malformations.