Cholesteatoma (Pediatric)

What is a cholesteatoma?

A cholesteatoma is a rare disorder in which skin grows or becomes trapped behind the ear drum.  It is benign, but can cause destruction of the hearing bones and lead to dangerous infections of the ear.  It most commonly presents with hearing loss or recurrent drainage of pus from the ear.  Occasionally the patient is asymptomatic upon discovery.

Cholesteatomas form in two ways.  They may be congenital in that a child may be born with skin trapped behind the eardrum or they may be acquired over time as the ear drum becomes suctioned in on itself to trap skin in the middle ear.  Either way the process is slow growing and may take years to be discovered.

How is a cholesteatoma diagnosed?

Cholesteatomas are usually discovered on clinical exam.  A CT scan may be used to confirm the diagnosis and assess the extent of the situation and may be helpful to surgical planning.  A hearing test confirms the extent of hearing loss. There is no risk to the other ear when cholesteatoma is diagnosed.  It cannot be inherited.

How is a cholesteatoma treated?

Surgical removal is the mainstay of treatment.  The extent of surgery will depend upon the extent of the disease.  Occasionally the disease may be removed completely through the ear canal avoiding any obvious incision.  More commonly though an incision behind the ear is necessary.  A mastoidectomy, or opening of the bone behind the ear, may also be necessary to access the disease process.  During these types of procedures it is important to monitor and maintain the facial nerve, a nerve running through the ear which moves the face.

Because the skin is microscopic, a second surgery is common and usually planned to assess for any cholesteatoma that has grown back or was left behind.  Second surgery also is used to correct hearing once the disease has been confirmed gone.  Occasionally the hearing will not be able to be restored and a hearing aid will be necessary.