In which of the following condition there are congenital anomalies of the middle ear?

Congenital anomalies of the middle ear may be associated with other general congenital deformities. There are a number of branchial arch syndromes, e.g. Pierre Robin's craniofacial dysostosis, Down's and Treacher Collins' syndromes. If there is an external ear abnormality it should raise suspicion of an underlying middle ear deformity. Middle ear deformity can be assessed by high-resolution CT scanning, if the inner ear is normal, reconstructive surgery of the middle ear can be successful.