Goldenhar syndrome

Case contributed by Dinesh Brand
Diagnosis almost certain


Born with Goldenhar syndrome, 50% deafness in the right ear, inability to initiate actions, problems with retaining information.

Patient Data

Age: 20 years
Gender: Female

Right Petrous Temporal Bone
There are appearances in keeping with otic hypoplasia on the right.
The right middle ear cavity is smaller than the left.
The mastoid air cells are underaerated.
There is slight asymmetry of the skull and petrous temporal bone with downsloping of the external auditory canal.
The right middle ear ossicles appear intact but the malleus is close to the roof of the small epitympanum /middle ear cavity.

There are appearances of asymmetry of the skull with a hypoplastic right temporal bone and otic capsule in keeping with the history of Goldenhar's syndrome.

Case Discussion

A rare case of Goldenhar syndrome with associated congenital defect of incomplete development of the right ear and accompanying deafness. 

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