Otosclerosis is a conformational disease☆

Abstract 

The otosclerotic focus is a result of the normal process of bone remodelling in the otic capsule – a place where there is little if any bone remodelling. There is still no single accepted theory for the pathogenesis of otosclerosis. Sources we used were relevant clinical and basic science publications from 1973 to 2001. The data from each publication were critically reviewed. In this paper we suggest a kind of molecular predisposition in otosclerosis, like molecular instability in some key protein with regulatory effect, cell receptor for example. Also it can be a destabilasing mutation of some repressor of the normal bone remodelling. Any triggering event like mechanic distress, vascular compromise, radiation, viral infection, etc. can start a process of conformational changes with subsequent induction of long-standing bone remodelling focuses. We conclude that many unusual features of otosclerosis can be explained if we consider this entity as a conformational disease.