Congenital cholesteatoma of mastoid origin

We report an extremely rare case of congenital cholesteatoma of mastoid origin.

A male patient was admitted with a one-month history of dizziness and headache, plus tinnitus in the right ear. Computed tomography scanning of the temporal bone showed destruction of the posterior wall of the external auditory canal by a lesion of soft tissue density in the right mastoid cavity, and also destruction of the bony plates of the posterior fossa and the sigmoid sinus, and of the mastoid tegmen. During surgery, a huge cholesteatoma sac was observed in the mastoid cavity, containing a large amount of keratinous material. The tegmen mastoideum and the bony plates of the posterior fossa and the sigmoid sinus were also observed to be destroyed. The skin and the tympanic membrane of the external auditory canal were intact, and the middle ear and aditus ad antrum mucosa were normal. The huge cholesteatoma sac was completely excised via a partial translabyrinthine approach, eradicating the superior and posterior semicircular canals.

This case of congenital cholesteatoma of mastoid origin was diagnosed by clinical examination, radiological evaluation and surgical findings.