Middle ear cholesteatoma can generally be divided into congenital middle ear cholesteatoma and acquired middle ear cholesteatoma, with congenital middle ear cholesteatoma being uncommon in the overall incidence of middle ear cholesteatoma.
Congenital middle ear cholesteatoma is due to abnormal embryonic development and is present at birth. There is usually no history of otitis media and there may be no abnormal symptoms in the early stages. Parents usually do not notice the abnormality until infancy or childhood and bring the child to the doctor for treatment, usually with complaints of ear leakage and hearing loss.