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.
Otosclerosis may fix the stapes by a tiny bridge of abnormal bone or may totally obliterate it.
It may spread to the cochlea to produce either a sensorineural loss of varying degree or deafness.
Lesions may be sclerotic, spongiotic, or fibrous but usually comprise all these histologic types.
This diversity of size and anatomic distribution coincides with the variable types of hearing loss, from a mild conductive deficit to a total loss.
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