Rare Disease that Erodes the Ear, Twins Operated at the Bambino Gesù

They were suffering from giant cholesteatoma, an aggressive disease that "eats" bones, nerves and tissues to the point of deafness and other serious complications: two 5-year-old Calabrian twins were successfully operated on at the Bambino Gesù Children's Hospital


Cholesteatomatous otomastoiditis (cholesteatoma) is a condition characterized by the abnormal accumulation of flaky skin in the middle ear (behind the eardrum).

This mass of epithelial debris tends to expand over time and destroy all the structures it encounters: the inner ear, the facial nerve, the meninges, the temporal bone of the skull (mastoid portion) until it creeps into the brain. The development of cholesteatoma is manifested by the symptoms of chronic recurrent otitis, i.e. hearing loss, dizziness, intense pain and discharge of pus from the ear.

If left undiagnosed and not surgically treated for mass removal, the disease can cause complete deafness, facial paralysis, meningitis, and brain abscesses.
It is estimated that cholesteatoma affects about 1 in 10,000 people, mainly in children.


The twins had developed – both in the right ear – a very voluminous and aggressive epithelial formation that had eroded several internal structures reaching up to the posterior cranial fossa. Once the diagnosis of cholesteatoma was confirmed by CT, they underwent a delicate operation to remove the mass radically.

The girls underwent a delicate microsurgery carried out by the team of the Audiology and Otosurgery Unit led by Prof. Pasquale Marsella. With more than 6,000 operations of this type to its credit, the Holy See Children’s Hospital manages one of the largest cases of children with complex diseases of the hearing organ at the national level.

For one of the girls, it was also possible to reconstruct some of the damaged structures, thus preserving the hearing capacity of the ear. A few days after the surgery, the twins returned home with their families.


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