Molecular biology of otosclerosis.
Otosclerosis is a bone disease of the human otic capsule, which is among the most common causes of acquired hearing loss. The pathologic process is characterized by a wave of abnormal bone remodeling in specific sites of predilection within the endochondral layer of the temporal bone. Although the cause of otosclerosis remains uncertain, there is a clear genetic predisposition with half of all cases occurring in families with more than one affected member. There is also compelling evidence that measles virus may play a role in some cases. Ultimately, how genetic factors and viral infection result in otosclerosis must be explained by effects on the molecular factors that control bone remodeling.
McKenna
Michael J
MJ
Department of Otology and Laryngology, Harvard Medical School, Boston, Mass., USA.
Kristiansen
Arthur G
AG
D016723:Bone Remodeling / Q000235:genetics; D002356:Cartilage / Q000473:pathology; D004432:Ear, Middle / Q000473:pathology; D015870:Gene Expression / Q000502:physiology; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008459:Measles virus / Q000235:genetics; D010006:Osteoblasts / Q000473:pathology; D053244:Osteoprotegerin / Q000235:genetics; D010040:Otosclerosis / Q000235:genetics* / Q000473:pathology; D053245:RANK Ligand / Q000235:genetics; D012367:RNA, Viral / Q000235:genetics; D053246:Receptor Activator of Nuclear Factor-kappa B / Q000235:genetics; D013701:Temporal Bone / Q000473:pathology
D053244:Osteoprotegerin; D053245:RANK Ligand; D012367:RNA, Viral; D053246:Receptor Activator of Nuclear Factor-kappa B; C505686:TNFRSF11A protein, human; C505688:TNFSF11 protein, human