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.
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications*; D016532:Mucopolysaccharidosis II / Q000150:complications; D009087:Mucopolysaccharidosis VI / Q000150:complications; D010040:Otosclerosis / Q000209:etiology*