Temporal bone histopathology of osteopetrosis.
The histopathology of the temporal bone of an eight and one-half-year-old girl with osteopetrosis (Albers-Schönberg disease) was studied to evaluate the pathogenesis of its frequent complications in hearing and facial nerve function. The patient was blind and had acute otitis media, but facial paralysis was not noted. Although the size of the temporal bone pyramid was markedly increased due to enormously thickened periosteal layer, changes in the endosteal and endochondral layers  were less marked. Exostotic growth of periosteal bone was seen in the middle ear wall, and the tympanic cavity appeared to be narrowed. At the oval window region, the facial nerve was pushed down towards the stapes due to extreme overgrowth of periosteal bone of the epitympanum, and the superstructure of the stapes was imbedded deeply into the dislocated facial nerve and had strongly compressed it. Although the footplate was free from ankylosis, the crus of the stapes appeared to be immobilized because it was lodged in the facial nerve. Such changes appeared to be the pathogenesis of one form of facial nerve paralysis and conductive hearing loss associated with osteopetrosis.
D002675:Child, Preschool; D003051:Cochlea / Q000473:pathology; D004432:Ear, Middle / Q000473:pathology; D005154:Facial Nerve / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010022:Osteopetrosis / Q000473:pathology*; D013701:Temporal Bone / Q000473:pathology*; D014722:Vestibule, Labyrinth / Q000473:pathology