Otosclerosis and mucopolysaccharidosis

By means of a literature review, clinical observations and temporal bone histopathology in the Hunter syndrome, we could show a reason for otosclerosis in childhood, a very rare observation. Therefore we do not believe that the presence of otosclerotic foci in temporal bones is mere coincidence in MPS II. It seems there is a causal connection between the generalized metabolic disorder and otosclerosis on the basis of enzyme deficiency.