At the site there is only focal nodular tissue in the subcutaneous location. The middle ear cavity is markedly narrowed with a small amount of fluid. The ossicles are markedly dysplastic with a single hook-shaped ossicle possibly representing a fusion of the incus and head of the malleus or representing a dysmorphic incus with malleus separately fused to the wall of the tympanic cavity. The long process of the incus does not reach the site of the stapes suprastructure. There may be a hypoplastic stapes.