Learning Objectives: In this presentation different forms of congenital cholesteatomas will be presented together with management strategies.

Between 2003–2016 author performed 817 tympanoplasties. 318 of these had cholesteatoma. 38 of these are classified as congenital cholesteatoma. 29 are primary cases and remaining 9 are revision cases. While three of the revision cases belong to the author, in remainging cases original operation had been performed in another center.

Youngest patient operated was 7 months old diagnosed with hearing screening.

Congenital cholesteatomas confined to the middle ear are usually attached to the neck of the malleus necessitating the removal of the neck and head of the malleus for complete removal. This can be managed without damaging the tympanic membrane. Ossicular chain is reconstructed with bone cement.

Larger cholesteatomas necessitate mastoidectomy with or without open cavity.

There is a group of congenital cholesteatoma extending into petrous bone. Resulting cavity can be managed by blind sac closure of the ear canal with subtotal petrosectomy. These cases usually expose carotid artery, jugular vein dura and have a high rate of recurrence.

Videos will be provided for each pathology showing the technique of removal and hearing reconstruction.