Bilateral primitive cholesteatoma of external auditory canal with congenital stenosis

• Primitive cholesteatoma of external auditory canal (EAC) is very rare and little known disease.
• There are no specific clinical symptoms which can lead to confusion with other EAC pathologies.
• Its diagnosis is clinical, but in case of stenosis, evaluation requires very close radiological analysis.
• The treatment is surgical and depends on the extent of the lesions.