Introduction
An ear that is constantly releasing discharge alongside hearing loss can often be the first symptoms of cholesteatoma. But what is that? Cholesteatoma is a condition that results from an abnormal growth of skin cells within your middle ear.1 There are 2 types: Congenital cholesteatoma and Acquired Cholesteatoma.congenital cholesteatoma is where there is a growth behind an intact eardrum and this develops while you are a embryo. Congenital cholesteatomas also block the eustachian tube causing a build of fluid within the middle ear.1, 2 The eustachian tube is a tube that connects your ear to your throat and is responsible for controlling pressure in the ear and draining fluid. An acquired cholesteatoma however is the result of changes that occur due to eustachian tube dysfunction. Eustachian tube dysfunction causes a pocket to form and it is here where the cells begin to accumulate and eventually become a cholesteatoma.3 Eustachian tube dysfunction has many causes however the main one is multiple ear, nose, or throat infections.
There are three parts to your ear. The first is the part, your outer ear, includes the visible part of your ear and the ear canal that leads up to your eardrum. The middle ear lies behind your eardrum and is made up of three small bones called ossicles that are constantly vibrating as sound waves hit your eardrum. The vibration of these bones within your middle ear is transferred into your inner ear which contains structures responsible for processing those vibrations. This is how you hear. Cholesteatomas as they grow will erode the structures of the ear, effectively interfering with the normal process of hearing.1, 4 This is why the common symptoms are hearing loss and discharge. If they get large enough they can also cause symptoms such as facial paralysis as the condition can damage the facial nerve, ringing in your ears, and balance problems as they start to damage the structures of the inner ear more directly and at their worst can lead to brain abscesses.
Diagnosis
Physical examination of the ear
On examination the outer ear might appear to be normal however the main hallmark symptom is foul smelling discharge. This discharge is also normally painless.1 A cholesteatoma can also often look like a condition called otitis media which is a common middle ear infection which can on occasion cause your eardrum to burst causing fluid to drain out the ear.
Hearing tests
In the actual physical examination, there may be two tests called Rinne’s and Weber’s tests. This involves using an instrument called a tuning fork which rings and is placed in front of your ear behind your ear and on your forehead and being asked where you hear the ringing loudest. This will allow the doctor to know if hearing loss is due to an issue with the transfer of sound waves from your eardrum to your middle ear bones (conductive hearing loss) or if there is an issue with actually sensing the sound waves (sensorineural hearing loss). You will then have a more objective hearing test called pure tone audiometry which will show hearing loss in the affected ear.
Imaging tests, including CT scan or MRI
The imaging that is often used to diagnose this condition is a CT scan of the petrous temporal bones which will allow the doctor to see the extent of the cholesteatoma.5
Microscopic examination of the ear
Using an instrument called an otoscope the doctor will look inside your ear canal, typically a cholesteatoma will look like a waxy growth above the ear drum.5
Treatment options
Antibiotic ear drops or oral antibiotics
Antibiotic ear drops can be used to treat the discharge that is draining out of the ear.6
Surgical removal of the cholesteatoma
Surgery isr the only definitive treatment and typically a wall-up mastoidectomy is performed. This is surgery that removes the cholesteatoma but maintains the structure of the ear canal walls. The main aim of the surgery is to remove all of it as this is the only way to prevent any recurrence. Patients will have to be informedthat potential complications of this surgery include worse hearing and damage to the facial nerve.(1)
Hearing aids or implantable devices for hearing loss
Following cholesteatoma surgery, a repeat hearing test will need to be done to identify any hearing loss. In the event of hearing loss following surgery a hearing aid may be used or in more profound cases a cochlear implant. This will not restore hearing to what it was however this can still greatly alleviate the effects of hearing loss.6
Facial nerve reconstruction surgery for facial weakness
The facial nerve is responsible for the muscles that control your facial movements. Branches of the facial nerve can be damaged both by the mass and because of the trauma from surgery. This can cause weakness in the affected areas as a result and is a serious complication that does not often occur. However, in the event it does, facial nerve surgery will be done to retain as much function as possible.7
Complications
Infection of the surrounding bones
The main bone that could be affected is the mastoid causing a severe infection called mastoiditis
Hearing loss or deafness
Deafness occurs as a result of the erosion of the middle ear bones and may even progress to causing damage to the inner ear causing sensorineural hearing loss.
Facial nerve paralysis
Branches of the facial nerve pass through the ear so could be damaged by the development of the cholesteatoma.
Meningitis or brain abscess
The mastoid lies very close to the brain so if it gets completely eroded the brain will be exposed and significantly more susceptible to a meningitis infection or brain abscess. These are life threatening conditions and must be treated urgently.8
Prevention
Timely treatment of ear infections
This will prevent eustachian tube dysfunction that could lead to cholesteatoma formation. Middle ear infections are particularly common in young children and can be chronic.
Avoiding exposure to loud noises
Loud noises can perforate your eardrum which could potentially create a pocket for the accumulation of cells
Using earplugs while swimming or diving
Swimming is a very common cause of an outer ear infection known as otitis externa and earplugs can prevent this
Avoiding trauma to the ear
Similar to loud noises this can perforate your eardrum but can also predispose you to infection. This includes avoiding the use of cotton buds as this can cause you to push your ear wax further down the ear and cause infection.
Good ear hygiene practices
Making sure your ears are clean can prevent infection. Your ears generally drain wax themselves so there isn’t a need to clear wax yourself unless you are producing too much. You can see your doctor in this case and olive oil drops can soften the wax to make it fall out easier.
Summary
In conclusion, cholesteatoma is a very serious ear condition that can often look like and be caused by repeated ear infections. If you have experienced hearing loss with a constantly discharging ear you will need to see a doctor so you can receive treatment as soon as possible.
References
- Kennedy KL, Singh AK. Middle Ear Cholesteatoma. StatPearls. Treasure Island (FL): StatPearls Publishing. Copyright © 2023, StatPearls Publishing LLC.; 2023.
- Abdul Jalil S, Md Daud MK. Congenital cholesteatoma: Different clinical presentation in two cases. Malaysian family physician : the official journal of the Academy of Family Physicians of Malaysia. 2016;11(2-3):30-2.
- Urík M, Tedla M, Hurník P. Pathogenesis of Retraction Pocket of the Tympanic Membrane-A Narrative Review. Medicina (Kaunas, Lithuania). 2021;57(5).
- Harvard. Chronic Otitis Media, Cholesteatoma and Mastoiditis 2022 [Available from: https://www.health.harvard.edu/a_to_z/chronic-otitis-media-cholesteatoma-and-mastoiditis-a-to-z.
- NICE. When should I suspect cholesteatoma? 2020 [Available from: https://cks.nice.org.uk/topics/cholesteatoma/diagnosis/diagnosis/.
- Geerse S, Bost TJM, Allagul S, de Wolf MJF, Ebbens FA, van Spronsen E. Hearing and hearing rehabilitation after obliteration of troublesome mastoid cavities. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2020;277(12):3307-13.
- Ryu NG, Kim J. How to Avoid Facial Nerve Injury in Mastoidectomy? Journal of audiology & otology. 2016;20(2):68-72.
- Zhang L, Niu X, Zhang K, He T, Sun Y. Potential otogenic complications caused by cholesteatoma of the contralateral ear in patients with otogenic abscess secondary to middle ear cholesteatoma of one ear: A case report. World journal of clinical cases. 2022;10(28):10220-6.