Cholesteatoma



Overview

Cholesteatoma is a problem involving skin of the eardrum or ear canal growing into middle ear and its surrounding areas. Its name is misleading as it is not a tumor however, if left untreated, it can become invasive and destructive. In cholesteatoma, the skin cells become trapped in a pocket and can cause inflammation and infection. This leads to destruction of parts of the surrounding areas of the middle ear as more and more skin cells become trapped causing more inflammation and infection. It occurs predominantly in people who have had multiple ear infections during childhood and ventilation tubes, although it can also occur in those who have never had an ear infection. They occur in about 1 in 20,000 people but are a common presentation to the Stanford Ear Institute. We don’t fully understand why they occur but the main theories include layers underneath the ear-drum becoming sticky or negative pressure within the middle ear pulling the eardrum inwards. The main form of management involves surgery.

Symptoms

Some cholesteatomas may not produce any symptoms early on but the earliest symptoms can include a persistently draining smelly ear. Cholesteatoma should be considered especially if the drainage persists after using antibiotic drops in someone who has had many ear infections in the past. As the cholesteatomas become larger, they can cause ear fullness or pressure, ear pain, hearing loss, tinnitus, taste dysfunction, imbalance or vertigo and rarely a weakness of the nerve that controls movement of the face. If ignored cholesteatomas can lead to very serious complications such as total hearing loss, brain fluid leak and brain infection. An ENT doctor will often look for the area of eardrum perforation, inflammation, collection of skin cells and erosion produced at the top part of the eardrum.

Diagnosis

Most of the time the patient does not have a cholesteatoma and usually the diagnosis can be made be an ENT doctor on its classic appearance seen by otoscope or after cleaning the ear with a microscope. To evaluate the hearing loss ENT doctors will order an audiogram and other accessory hearing tests. Balance tests may also be ordered in select cases. A CT is useful in determining how big the cholesteatoma is and what structures it is growing near or into. In some cases an MRI is useful to evaluate certain areas.

Treatment

Sometimes a small limited cholesteatoma can be managed with regular follow up, cleaning and eardrops but the majority of cholesteatomas are best managed using surgery. Sometimes surgery is limited to excision through the ear canal but often it requires a mastoidectomy, an incision behind the ear and opening the air cells behind the ear. This usually involves outpatient surgery under general anesthesia but in larger operations it may require an inpatient stay. The exact surgical approach depends on the size and extent of the cholesteatoma. The aim of surgery is to remove the invading skin cells and surrounding inflammation or infection to make the ear dry and safe from further damage. The main specific risks of surgery include further hearing loss, tinnitus, imbalance or vertigo, taste dysfunction and facial weakness. Time off work is usually one to two weeks and requires post-operative dressings for one to two months in the short term. After surgery, six to twelve monthly follow-ups are needed to look for a recurrence. Some patients require lifelong follow up. Some techniques require a “second look” procedure to make sure the cholesteatoma has not recurred and to reconstruct the hearing, via replacing the middle ear bones previously eroded, when the inflammation has settled.

References

Santa Maria PL, Jackler RK. The Cholesteatoma: From Principles to Practice. Chp 12. Otolaryngology Principles and Practice.

Jackler RK, Santa Maria PL, Varsak YK, Nguyen A, Blevins NH. A new theory on the pathogenesis of acquired cholesteatoma: Mucosal traction. Laryngoscope. 2015;125 Suppl 4:S1-S14.