Cholesteatoma and mastoid surgery

Mr James W Fairley BSc MBBS FRCS MS
Consultant ENT Surgeon

© 2007 – 2014 JW Fairley Last updated 16 August 2014

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What is cholesteatoma?

cholesteatoma-leftearan150pxhiCholesteatoma left ear. The disease has eroded the bone above and behind the upper part of the eardrum, the attic. The eardrum is also known as the tympanic membrane. I took this picture in 1988 as part of a research project into the development of cholesteatoma using a Storz Hopkins rod tele-otoscope, Xenon 300W light source, and an Olympus OM-1 SLR camera with Fujichrome 400ASA 35mm slide film.
Key:
c = cholesteatoma
tm = tympanic membrane (eardrum)

Cholesteatoma (ker-less-tea-a-toe-ma) is a progressive destructive ear disease. Most cases occur in children and young adults, but it can affect any age. Skin builds up in layers and erodes the bone of the middle ear and mastoid. In its early stages, cholesteatoma tends to attack the ossicles, the small bones conducting sound from the eardrum to the inner ear. This causes partial deafness, sometimes with unpleasant smelling discharge and pain. If the disease progresses, it can erode the inner ear causing total and permanent deafness and tinnitus. The inner ear also contains the balance organ. If cholesteatoma erodes into the balance organ, vertigo, a severe form of dizziness, results. Cholesteatoma can also attack the facial nerve causing facial paralyis. In rare cases the disease erodes upwards. The roof of the ear is the floor of the brain. If this thin plate of bone is breached, meningitis, brain abscess and death can result. The cholesteatoma is made of layers of dead skin, like an onion. Only the outer layer, known as the matrix, contains live growing skin cells. Cholesteatoma is the most serious form of chronic ear infection. It is not a tumour, though it can behave like one. It is not cancer and never spreads widely throughout the body – though it can cause quite enough trouble by its local destructive effects. In most cases, the progress of cholesteatoma is slow. It can take years or even decades to eat its way slowly through the structures of the ear. Rapidly progressive disease, over a time course of a few months and sometimes weeks, is commoner in children and in the presence of active acute infection.

eardiagramaticborderannotatedtall200pxw

gramophone200cpxw
The outer and middle ear work like an old mechanical gramophone in reverse. They collect sound energy, and concentrate it onto the small area of the stapes footplate

The normal ear and hearing

The human ear is divided into three parts:

  • outer ear
  • middle ear
  • inner ear

Outer ear

The outer ear consists of the pinna and the ear canal. The outer ear funnels sound waves in air to the eardrum.

Eardrum (tympanic membrane)

left-normal-tympanic-membrane150pxhiNormal left eardrum (tympanic membrane)

The eardrum is a paper-thin membrane, shaped like a miniature satellite dish, 8-10 mm diameter. The tympanic membrane forms the boundary between outer and middle ear.

Middle ear

The eardrum vibrates when sounds arrive through the external ear canal. The vibrations are transmitted to the inner ear via three small bones (ossicles) suspended in the middle ear.

Ossicles

atelectatic-right-tm-ossicles-annotated150pxhi
Abnormally thin right eardrum damaged by glue ear and showing ossicles – malleus incus and stapes

The three little bones (oss-i-culls) are

  • malleus (mal-ee-us) shaped like a hammer
  • incus (ink-us) shaped like an anvil
  • stapes (stay-peas) shaped like a stirrup

Their job is to concentrate the sound energy, collected by the relatively large area of the eardrum, onto the tiny footplate of the stapes.

The outer and middle ear work like an old mechanical gramophone in reverse. The gramophone needle picks up vibrations from the grooves in the record, passes them to a vibrating membrane, then into the large horn, and so to the outside world. The outer and middle ear collect sound from the outside world and concentrate it down to the stapes footplate. The footplate moves like a piston in the oval window, the opening of the inner ear.

Inner ear

The inner ear has two parts, the cochlea and the vestibular labyrinth.

Cochlea

The cochlea is the hearing part of the inner ear. It is a biological microphone. Sound vibrations are turned into electrical signals and sent to the brain in the nerve of hearing.

Vestibular labyrinth

The vestibular labyrinth of the inner ear is concerned with balance. Disturbance of the balance organ of the inner ear can cause vertigo.

Eustachian tube

The Eustachian tube connects the middle ear with the back of the nose. To hear normally, the eardrum and ossicles must be able to move easily. For this to occur, the middle ear must contain air at the same atmospheric pressure as the outer ear. Air in the middle ear comes from the back of the nose, via the Eustachian tube. The job of the Eustachian tube is to ventilate the middle ear, keeping the pressure in the middle ear the same as in the outer ear. Most middle ear diseases, including cholesteatoma, are associated with poor Eustachian tube function. The health of the middle ear depends on the Eustachian tube working properly.

Causes of cholestatoma

Cholesteatoma is skin in the wrong place. The skin that should be on the outside of the eardrum is in the middle ear. Like skin all over the body, the surface skin cells of the eardrum are dead. They are constantly replaced by new cells growing underneath. A snake sheds its outer layer of dead skin all in one go. We humans shed it a bit at a time, in flakes. Most of the dust in your house is made up of flakes of dead skin that have dropped off. If flakes of skin kept dropping off inside your ear canal, they would end up blocking it. So, the normal ear has a special way to get rid of the dead skin layers. The skin grows in such a way that the outer layers are slowly moved outwards, like a conveyor belt. If you paint a dot of ink in the middle of someone’s eardrum, then go back a week later and look at it, it will have moved outwards. Gradually, over a few weeks, it will work its way all the way out along the ear canal. In cholesteatoma, this normal outward movement of dead skin fails. Layer upon layer of dead skin builds up. But there shouldn’t be any skin in the middle ear, it should be on the outside of the eardrum, in the outer ear. So how does the skin get into the middle ear?

Atelectasis - The eardrum is thin and sucked inwards, like cling film over the ossicles, by long term failure of the Eustachian tube to ventilate the middle ear. The patient had a long history of glue ear. This is not yet cholesteatoma, there is no build-up of skin layers, normal outward migration of skin is still happening and the retraction is self-cleaning.Atelectasis (ate-eel-ECK-ta-sis) The eardrum is thin and sucked inwards, like cling film over the ossicles, by long term failure of the Eustachian tube to ventilate the middle ear. The patient had a long history of glue ear. This is not yet cholesteatoma, there is no build-up of skin layers, normal outward migration of skin is still happening and the retraction is self-cleaning. An ear like this might or might not progress to cholesteatoma.
Key:
m = malleus
i = incus
s = stapes (STAY-peas)
  • The commonest way for it to get there is for it to be sucked in. A partial vacuum sucks the eardrum inwards. The air pressure in the middle ear falls if the Eustachian tube isn’t working properly. This sucks the eardrum inwards. The upper part of the eardrum, the attic, is thinner and weaker than the the lower part. It gets sucked in and forms a retraction pocket. Any part of the eardrum can form a retraction pocket, but they are commonest in the attic because the eardrum is naturally thinner and weaker there. Not all retraction pockets will progress to cholesteatoma, but some will. Normally, the skin of the eardrum migrates slowly outwards, like a very slow moving conveyor belt. The retraction pockets that progress to cholesteatoma are those in which the conveyor belt stops. Shed layers of dead skin begin to build up, forming layer upon layer, like an onion. Sometimes the whole of the eardrum is weak and becomes sucked in, covering the ossicles like cling film. This is called atelectasis. Most cholesteatomas start from a damaged, thinned and weakened eardrum that has been sucked inwards.
  • Another way that skin can get into the middle ear is for it to be blown in, by a pressure wave such as an explosion. Cholesteatoma does occur in bomb blast victims, who have had their eardrums perforated and blown inwards.
  • Another way that skin from the outside of the eardrum could get into the middle ear is for it to be pushed in by surgery, such as fitting grommets. This is rare. Cases of cholesteatoma associated with grommets are more likely to be due to an early cholesteatoma being missed when the grommets are fitted. It is not unusual for retraction pockets and glue ear to co-exist, since both conditions are caused by failure of the Eustachian tube to properly ventilate the middle ear.
  • Occasionally a cholesteatoma develops behind a healthy eardrum from some skin cells left behind during the development of the ear in the womb. This second kind of congenital cholesteatoma is much rarer, it presents in early childhood.
  • Some specialists believe that cholesteatoma can develop from non-skin cells in the middle ear that turn into skin because they are chronically irritated by infection. This theory is not widely accepted.

Symptoms

  • Sometimes Cholesteatoma has no symptoms and is found incidentally.
  • The damage to the eardrum usually starts in childhood. There is often a long history of repeated ear infections.
  • There may be discharge from the ear. This is usually smelly, a bit like cheesy feet. In fact, the build up of layers of greasy dead skin that forms the cholesteatoma is very similar to what happens if you don’t clean between your toes.
  • There is usually some loss of hearing.
  • A slow progressive loss of hearing, especially if it is only in one ear, can creep up on you, so isn’t always noticed.
  • If the ear is kept dry, there may be little or no infection for months or years.
  • Ear infections following swimming, dunking the head in the bath, or ear syringing, are common in cholesteatoma.
  • Occasionally, the first symptom of cholesteatoma is when it gives rise to serious complications.
  • Sudden onset of severe vertigo may be due to the disease eroding into the lateral semicircular canal of the inner ear.
  • Sudden onset of severe deafness can be due to the disease eroding into the inner ear.
  • A paralysed face could be due to the disease affecting the facial nerve in the ear.
  • Meningitis – severe headache, stiff neck and photophobia – may be the presenting feature of the disease.

An attic crust, right ear, seen with a tele-otoscope. The brown flaky material at the top of the eardrum is not wax, but the dried outer layers of dead skin of a cholesteatoma. This is one of the most difficult and subtle signs to spot in clinical medicine. The attic crust must be removed to see what is going on underneath.An attic crust, right ear, seen with a tele-otoscope. The brown flaky material at the top of the eardrum is not wax, but the dried outer layers of dead skin of a cholesteatoma. An attic crust is one of the most difficult and subtle signs to spot in clinical medicine. This picture was taken with a tele-otoscope, which gives a wide angle view of the whole eardrum and the surrounding deep ear canal. The view from a normal auriscope (as used in normal practice) is much narrower, and unless the examiner moves the auriscope around to see the whole of the drum, the attic may not be seen. Even if this brownish crust were seen, very few non-specialists would recognise it, most would simply think they were looking at a piece of wax. The attic crust must be removed to see what is going on underneath.Otitis externa. Layers of wet soggy white shed skin are partially blocking the ear canal. Red swollen inflamed skin is preventing a proper view of the eardrum. The debris must be cleaned to see the eardrum. Until the eardrum has been fully examined, it is impossible to know whether or not there is an underlying middle ear disesase such as cholesteatoma.Otitis externa. Layers of wet soggy white shed skin are partially blocking the ear canal. Red swollen inflamed skin is preventing a proper view of the eardrum. The debris must be cleaned to see the eardrum. Until the eardrum has been fully examined, it is impossible to know whether or not there is an underlying middle ear disesase such as cholesteatoma.
Suspicious for infected cholesteatoma. Red granulation tissue overlying the bone of the ear canal, behind the eardrum. Layers of wet soggy white shed skin are stuck to the eardrum.
Suspicious for infected cholesteatoma. Red granulation tissue overlying the bone of the left ear canal, behind the eardrum. Layers of wet soggy white shed skin are stuck to the eardrum. The debris must be cleaned to see the eardrum. In this case it was too painful to complete the cleaning in out-patients. Microsuction under a general anaesthetic confirmed the diagnosis.

Signs

Medical signs are things that can be seen or noticed during examination by the doctor. There are several signs of cholesteatoma, but no one single appearance. Cholesteatoma can take many forms as it evolves.

  • One classic sign of cholesteatoma is an attic crust. This is a brown flake of dried skin in the upper part of the eardrum.
  • An attic crust is an extremely difficult and subtle sign to spot.
  • Very few non-specialists would notice it.
  • Even if they saw it, they would think it just a piece of dried skin or wax.
  • ENT specialists are taught to “never trust an attic crust”
  • Unless it becomes actively infected, cholesteatoma is usually missed altogether on examining the ear.
  • If it becomes infected, then there may be signs of otitis externa (inflammation of the skin of the outer ear canal). Signs of otitis externa include:
    • discharge
    • build up of debris
    • swelling
    • reddening
    • narrowing of the ear canal
  • Until otitis externa has been treated, it is impossible to know if we are dealing with an underlying cholesteatoma, because the eardrum is not visible. The ear canal is is swollen and blocked with infected material and dead skin.

Diagnosis

Apparently normal left tympanic membrane as seen through an operating microscope. Only the pars tensa - the lower three quarters of the eardrum - is visible with the speculum pointing in this direction.Apparently normal left tympanic membrane as seen through an operating microscope as it would be lined up to fit a grommet. Only the pars tensa – the lower trhee quarters of the eardrum – is visible with the speculum pointing in this direction. Same left ear with microscope swivelled 60 degrees upward to view attic. Severe attic retraction with perforation and possible early cholesteatoma - skin edges are on the body of the incus.Same left ear with microscope swivelled 60 degrees upward to view attic. Severe attic retraction with perforation and possible early cholesteatoma – skin edges are on the body of the incus. This young man had normal hearing and no history of any problems with the left ear. He came about his right ear, with a long history of recurrent infections with smelly discharge. A large active cholesteatoma was found on the right. The left ear was only examined for the sake of completeness. It shows early signs of developing the same condition.

Shah grommet in position lower part of left eardrum, seen through the operating microscope. The attic is not visible when the speculum is lined up to look at the grommet

Shah grommet in position lower part of left eardrum, seen through the operating microscope. The attic is not seen when the speculum is lined up to look at the grommet

Cholesteatoma is very difficult to diagnose in its early stages, even for ENT specialists. It is rare for a General Practitioner (primary care, family doctor) to be able to diagnose this condition. Most times, it isn’t even suspected. Cholesteatoma is a rare condition. Ear infections in children are common. Most children will get some ear infections, hardly any of them will develop cholesteatoma. The average GP will see hundreds of ear infections, but only one cholesteatoma every ten to twenty years. Hence, it is not at the top of the list of diagnostic possibilities. Usually, it is not even suspected. The diagnosis usually comes to light only after many years of repeated infections. It is made by an ENT specialist examination, using an operating microscope. Cholesteatoma cannot be diagnosed without seeing the whole of the eardrum in close-up detail. Anything blocking the ear canal, such as wax or infective debris, must be cleaned away to get a proper view of the eardrum. In adults, this can usually be done awake as an out-patient microsuction. Microsuction can be painful, especially if the ear is infected. In children, a general anaesthetic is usually needed. Sometimes the diagnosis of cholesteatoma is discovered on the operating table, when a child has been admitted for grommets insertion. Sometimes, it is discovered on the operating table when an adult patient is admitted for a myringoplasty (repair of perforated eardrum). A perforated eardrum may be seen in the out-patient clinic, and the patient advised to have it repaired. It is only when the patient is under the anaesthetic, and the eardrum is lifted up to repair it, that the cholesteatoma is discovered.

Even under the operating microscope and with the patient fully anaesthetised, a cholesteatoma can easily be missed. Most cholesteatomas start in the attic – the upper part of the eardrum. Grommets are fitted in the lower part. The attic is not in view when the speculum is lined up to fit the grommet. The opening into the cholesteatoma sac can be very tiny – a millimetre in diameter – and if the cholesteatoma is not actively infected it will not be obvious unless the surgeon makes a point of looking for it. I have taught over a hundred junior doctors ear surgery, some at quite an advanced stage in their training, and have been surprised at how many of them fail to check the attic properly during grommet surgery. I believe that many of the cholesteatomas said to be caused by grommet insertion (a rare complication) were present at the time of the grommet fitting but were not noticed.

Audiometry (Hearing tests)

Child hearing test in a sound proofed boothChild hearing test in a sound proofed boothTuning fork test - part of Rinne's test. Bone conduction of sound is often better than air conduction if the ossicles have been destroyed by cholesteatoma.Tuning fork test – part of Rinne’s test. Bone conduction of sound is often better than air conduction if the ossicles have been destroyed by cholesteatoma.

Hearing tests are part of the assessment of any ear condition. Pure tone audiometry with air conduction and bone conduction is the main test we use. The test doesn’t diagnose the condition, but does tell us how much hearing has been lost, and whether it is a conductive hearing loss (usually due to damage to the eardrum and ossicles) or a sensorineural hearing loss due to damage to the inner ear. We need to measure and know how much hearing has been lost, and how much remains, to help advise on the likely outcome of treatment. If the loss of hearing is due to damage to the inner ear, it will definitely be permanent. A conductive loss can sometimes be improved, but it is not usually possible to improve the hearing by treating cholesteatoma. In most cases we can prevent things from getting worse. Hearing tests before and after treatment are important in assessing the results of what we do.

CT scan

A CT scan is a form of X-Ray imaging which allows us to see the body in much clearer detail than plain X-Rays. The CT scan splits the image into thin layers, sliced like a salami, so that we can see much greater detail and pinpoint what is happening at any given point. It is only in recent years that scanners have been able to produce slices thin enough to give us useful information on the state of the middle ear. Some ear surgeons always require a CT scan before operating. In the days before CT scans, some ear surgeons always wanted mastoid X-rays before operating. I have never found them all that useful. Even with the best scanners, we don’t get anywhere near as good a view as we get with the operating microscope during surgery – full colour, three dimensional and up to 40 times magnification. It is impossible to tell with a scan whether some part of the ossicular chain is fixed – this can only be determined by trying to move it during surgery. Many of the patients referred to me for ear surgery have already had scans. They rarely make any difference to what I plan on doing. I do not need a scan as a matter of routine. A CT scan is needed if we suspect complications, especially if we suspect there may be spread of disease into the brain.

Medical treatments

Cholesteatoma can only be cured by surgery. No drug will remove the disease. If the ear becomes infected, the infection can be treated medically with antibiotics. Inflammation can be treated medically with steroids. Drugs for infection and inflammation of the ear are given in three main ways:

  • topical – ear drops and sprays, ointments
  • by mouth – medicine and tablets
  • by injection – intravenous antibiotics may be needed in more severe infections, especially if the inflammation spreads into the surrounding structures.

If you are prescribed ear drops, make sure you use them properly, otherwise they probably won’t work.

Microsuction treatment

Microsuction of the ear as an out-patient procedureMicrosuction of the ear as an out-patient procedureWax removal using microsuctionWax removal using microsuction
Wax removal using a fine round ended hook
Wax removal using a fine round ended hook
Cleaning the ear without microsuction
An alternative to microsuction often used in children with runny, discharging ears is to gently mop out the ear with a cotton wool applicator. Illumination is from light shone over the child’s shoulder and focussed onto the ear by a head mirror worn by the surgeon. The view is nothing like as good, and we can’t normally reach right down to the eardrum, but it is less scary for the child.

Microsuction is an examination and treatment of the ear using a high powered binocular operating microscope. We use very fine delicate instruments, including a miniature vacuum cleaner. This Hoovering of the ear is usually done as an out-patient procedure. Sometimes, and especially in younger children, it is done as a day case surgery under general anaesthetic. Microsuction of the ear is carried out

  • to diagnose the condition of the ear accurately using
    • binocular vision for 3-d stereoscopic view
    • high power magnification
    • very bright illumination
  • to remove material blocking the ear canal such as
    • wax
    • infected debris, pus and fungal material
    • dead skin layers including cholesteatoma
    • foreign bodies
    • to apply medicationto the ear

You may be asked to use ear drops beforehand. This will make the treatment easier for you. You do need to lie very still. Despite all our efforts to be as gentle as possible, it will be noisy, and may be painful. It usually causes some short lived vertigo, lasting no more than a minute or so. Don’t get up until the nurse says so – you may be dizzy. You should be ok to drive afterwards but may need to wait until any dizziness has settled.

Repeated microsuction is important in the the long term follow up cholesteatoma patients. Rather like going to the dentist, very few people look forward to the experience, but it is effective and necessary.

Mastoidectomy

Combined approach mastoidectomy and tympanoplasty for cholesteatoma, right ear, in an 8 year old child. Key: C = cholesteatoma e = external ear canalCombined approach mastoidectomy and tympanoplasty for cholesteatoma, right ear, in an 8 year old child. From behind the ear, the outer ear has been moved forward. Most of the mastoid air cells have been drilled out to expose the disease in the attic. The bony wall separating the ear canal from the mastoid has been preserved. The middle ear is actively inflamed and filled with red granulation tissue. The most difficult part of the operation is yet to come, the disease has to be carefully taken off the underlying ossicles. At this stage we don’t know the state of the ossicular chain. It will be difficult because of the active inflammation, which is likely to bleed. Even a drop of blood is like a lake under the high powered microscope. The laser will help remove the granulations with minimal bleeding.
Key:
C = cholesteatoma
e = external ear canal.

Mastoidectomy (mass-toyed-ECK- tuh-mee, mastoid for short) is the operation to remove cholesteatoma. The mastoid is the bone behind the ear. It is part of the temporal bone, which forms the base of the skull and contains all the structures of the ear, as well as the facial nerve and some big blood vessels that go into the brain. The normal mastoid contains a honeycomb arrangement of air cells, which connect with air in the middle ear. The attic, just above the eardrum, is where the middle ear meets the mastoid. The attic contains the head of the malleus and the body of the incus. These two ossicles are very commonly surrounded by cholesteatoma. To remove cholesteatoma, we usually need to drill out the mastoid. This gets us behind the disease so we can remove it. The whole area is quite small, about the size of your thumb, and it contains some very delicate clockwork.

The aims of cholesteatoma surgery are

  1. To remove the disease
  2. To prevent future complications such as facial paralysis, total and permanent deafness, dizziness, spread of infection into the brain
  3. To give you a dry ear, that doesn’t keep getting infected
  4. As far as possible, to preserve what remains of the normal structures of the ear
  5. To give you as good hearing possible

Removing the disease takes priority. The surgeon has to tread a fine line between getting rid of the disease while preserving what he can of the hearing mechanism. Although cholesteatoma is not a tumour, it behaves like one. Unless the cholesteatoma is removed completely, it will come back. The surest way to get rid of the disease would be to drill out everything – be radical. But that would make you very deaf, and could damage the other structures we are trying to protect, like the balance organ and facial nerve. Cholesteatoma tends to infiltrate in lots of different directions. Sometimes it wraps itself around the ossicles. The most reliable way to make sure we don’t leave any behind on the ossicles would be to remove them altogether – but that would make you more deaf. In a radical mastoidectomy, all the ossicles are removed, except the footplate of the stapes. In some severe cases of cholesteatoma, the ossicles have already been eaten away by the disease so it makes no difference, they are gone anyway. Some of the most difficult areas to remove cholesteatoma are

  • facial nerve
  • stapes footplate
  • lateral semicircular canal

There is a risk of damaging the very structures we are trying to save. But if we don’t remove the disease, it would very likely cause damage anyway.

Techniques of mastoid surgery

Examination and cleaning of the ear with the operating microscope, speculum and suction prior to mastoid surgery
Examination and cleaning of the ear with the operating microscope, speculum and suction prior to mastoid surgery

Drilling right mastoid
Drilling right mastoid

Teaching mastoid surgery with binocular operating microscope and KTP LASER
Teaching mastoid surgery with binocular operating microscope and KTP LASER

Before antibiotics, mastoid surgery was commonly done in desparate circumstances for acute infection, a mastoid abscess. Our predecessors had nothing better than a hammer and gouge, and no magnification other than some spectacle loups. It was counted a success if the patient – usually a young child – survived. No delicate work could be done, and most survivors were deafened. It was only after the introduction of the binocular operating microscope in the 1950′s that modern delicate controlled microsurgery of the ear became possible. Even with all the latest high powered microscopes, lasers and modern anaesthetics, mastoid surgery is very difficult. Surgeons have to train for years to get good at it. Like all ear surgeons trained since the 1960′s I did my basic training (in the 1980′s) on temporal bones from cadavers (dead bodies). Although some might find that macabre, I’d prefer the learning curve to be on my dead granny, rather than on my live child. The margin of error in mastoid surgery is measured in fractions of a millimetre. Anatomy varies considerably, and a surgeon needs to practice on lots of bones before embarking on live patients. Simulators and plastic bones just aren’t up to it. Unfortunately, in the UK, a public attitude has become established against the use of post-mortem tissues, which has led to a severe shortage of temporal bones for the next generation of ear surgeons to train on. I teach trainee surgeons ear surgery on live patients every week, sometimes two or three cases. The operations take anywhere between one to six hours. The average is around three hours.

Approaches

The two main approaches for cholesteatoma surgery are the endaural, also known as canal wall down or modified radical mastoidectomy, and the postauricular, also known as the canal wall up or combined approach mastoidectomy and tympanoplasty.

Endaural / canal wall down / small cavity / modified radical mastoidectomy

In the endaural approach, the cut is made in the roof of the ear canal, extending up in front of the ear into the hairline. The disease is followed from where it is visible (usually the upper part of the eadrum) into the mastoid. Bone of the canal wall is drilled away to expose the disease. This creates a mastoid cavity. We try and keep the cavity as small as possible, and make the opening to the ear as large as possible, to help with ventilation and cleaning afterwards. A shallow, smooth walled cavity is much easier to keep clean, and there is less risk of cholesteatoma coming back afterwards.

Postauricular / canal wall up / combined approach tympanoplasty technique

In the postauricular approach, the cut is behind the ear. The mastoid is opened by drilling into the bone overlying it. The ear canal wall, separating the external ear canal from the mastoid, is preserved. This gives a more normal looking ear and avoids a cavity which can be difficult to clean, especially in children. This method is technically a little more difficult, and is less reliable in getting rid of all the cholesteatoma at the first attempt. It is usually necessary to have a second look operation, about a year afterwards, if this technique is used.

I use both of these methods for mastoid surgery. The choice depends on various factors. The site of the disease and the degree of destruction that has already happened are important. The age and circumstances of the patient are also taken into consideration. By and large, we prefer the postauricular approach initially in children and younger adults, provided there hasn’t already been too much destruction. For older patients with medical problems causing difficulties with repeated operations we prefer the endaural modified radical approach. In both cases, we use the KTP laser to help get rid of skin cells from from awkward areas like the ossicles.

Tympanoplasty

Tympanoplasty (TIM-pan-o-plas-tea) is surgery to rebuild the damaged structures of the middle ear, including the eardrum and ossicles. There are limits to what can be achieved. A rebuilt middle ear seldom works as well as the original. Repairing the eardrum is myringoplasty (mi-RING-o-plas-tea). Rebuilding or replacing the ossicles is ossiculoplasty (oss-SICK-you-low-plas-tea). Tympanoplasty is the general term that covers both. Often, we don’t know how much reconstruction will be needed, or possible, until we are part way through the operation. Sometimes, tympanoplasty is done together with mastoidectomy, as part of the same operation. Other times it is done at a later stage, as a second operation, once we are satisfied that the cholesteatoma has been removed and any infection has settled.

Ossiculoplasty

Artificial ear bone in place, left ear. Titanium metal prosthesis positioned between stapes head and cartilage reinforced eardrum. Kurz Variac system, trimmed to 1.75mm length.Artificial ear bone in place, left ear. Titanium metal prosthesis positioned between stapes head and cartilage reinforced eardrum. Kurz Variac system, trimmed to 1.75mm length.
Key:
m = malleus head
i = incus body
c = cartilage slice reinforcing eardrum
s = stapes head
L = Lateral semicircular canal

Ossiculoplasty (oss-SICK-you-low-plas-tea) is an attempt to rebuild the damaged chain of tiny bones that conduct sound from the eardrum to the inner ear. The surgeon is faced with a three dimensional, microscopic jigsaw puzzle, where the pieces don’t interlock, and some are missing. The aim is to achieve a stable but mobile mechanical linkage, reconnecting the reconstructed eardrum to the inner ear. There are many techniques for ossiculoplasty, depending on the exact situation found at surgery.

  • Damaged bones can sometimes be taken out, cleaned, re-shaped and put back in a different way. The results not very reliable, because the little bone tends to fall off where it was put.
  • Missing pieces can sometimes be replaced with materials taken from nearby, such as cartilage from the outer ear.
  • Artificial bones – prostheses made of various plastics, cements or metals – can also be used.

Fractions of a millimetre will determine success or failure. We then have the healing process to contend with.

  • Even if initially successful, in the weeks and months following surgery, there will be scarring.
  • This can easily pull things apart, or alternatively stiffen up and imobilise the reconstruction.
  • Further ear infection can break down the repair.
  • Cholesteatoma might recur.
  • If the eardrum has been repaired, but the Eustachian tube is still not working, there may be middle ear effusion, retraction, and / or atelectasis.
  • If a foreign material has been used in the reconstruction, there is a fair chance that the body will reject it and push it out.

All these things take their toll on the long-term results. Even in the best hands, less than 50% achieve socially acceptable hearing from ossiculoplasty. If the stapes (the third and most delicate bone in the chain) has been damaged, the prospects of restoring useful hearing by ossiculoplasty are very poor indeed. Many UK ear surgeons won’t even attempt ossiculoplasty, since the chances of success are low, and there are signficant risks of damaging the inner ear permanently by manipulating the ossicular chain in the attempt to rebuild it. My own view is that it is reasonable to attempt ossiculoplasty, if the situation on the table looks favourable. If the ossiculoplasty fails to give satisfactory hearing, we have the BAHA (Bone Anchored Hearing Aid – osseointegrated titanium auditory implant) to fall back upon.

KTP LASER

KTP LASER in use during mastoid surgery, right earKTP LASER in use during mastoid surgery, right earKTP LASER in right mastoid. Surgeon's view down high power microscope. Green light is turned red by a filter to protect the surgeon's eyes. The tip of the LASER fibre measures 0.2mm across. A fine suction tube is used to evacuate smokeKTP LASER right mastoid. Surgeon’s view down microscope. Green light is turned red by a filter to protect the surgeon’s eyes. The tip of the LASER fibre measures 0.2mm across. A fine suction tube is held near the tip to evacuate smoke

The KTP laser is a very useful tool in cholesteatoma surgery. It allows us to vapourise disease from the ossicles without touching them. That is better than having to physically scrape disease off the ossicles, which is like hitting the microphone and risks noise damage to the inner ear. The laser can be passed down extremely fine fibreoptic fibres. We use a 0.2mm fibre in ear surgery. The fibre is placed inside a fine hollow hand-held instrument. The curved tip can be placed with extreme accuracy into narrow awkward tiny crevices where cholesteatoma lurks. There are some areas where we can’t use the laser. If the cholesteatoma is directly on the facial nerve, we can’t use laser to burn it off because that would injure the nerve. I have been using the KTP laser in ear surgery since 1996. I only carry out mastoid surgery in hospitals where the KTP laser is available.

What are the risks of surgery for cholesteatoma?

Consultant anaesthetist, anaesthetic machine and monitoring equipment, Chaucer HospitalConsultant anaesthetist, anaesthetic machine and monitoring equipment, Chaucer Hospital

All operations carry risks. There are also risks of not operating, especially in a progressive destructive disease like cholesteatoma. At worst, you could die or suffer brain damage – but you are more likely to be injured in a road accident. A general anaesthetic carries a minimal risk, with consultant anaesthetists using modern drugs and monitoring equipment.

General risks of a long operation under general anaesthetic include

  • Heart and lung problems
  • Deep vein thrombosis
  • Stroke

None of these are likely unless there is some pre-existing medical condition. All these risks are higher in smokers, and you should stop smoking before any operation. If there are worries about your particular medical condition, we recommend a pre-operative assessment by the anaesthetist before deciding on surgical treatment. Sometimes we may need pre-operative tests such as X-Rays, electrocardiogram and blood tests. These investigations are not usually needed in younger patients.

Specific risks of Mastoid surgery

The same structures that are at risk from the disease are at risk from the operation to remove it. The operation has serious potential risks including:

  • total and permanent deafness in the operated ear
  • severe tinnitus
  • balance disturbance and vertigo
  • facial nerve paralysis
  • meningitis and / or brain abscess

None of these serious complications are likely, but they can and do happen occasionally. The chances of further damage from a controlled operation by a skilled surgeon using modern equipment are less that the risk of leaving the disease to progress for the rest of your life – unless your life expectancy is short.

There are also side effects of the surgery. The following aren’t really risks as such, they are just things that are going to happen that you probably won’t like.

  • Numbness or tingling of the side of the tongue on the operated side. This is due to damage to the corda tympani, a small nerve that takes a detour through the middle ear on its way from the brain to the side of the tongue. The corda is always stretched and often cut during mastoid surgery.
  • The ear itself will feel numb, as will the scalp above the ear. This is because the nerves that run in the skin are cut during the operation. The numbness usually gets better after a year or so.
  • The shape of your outer ear may alter. The opening to the ear canal is often deliberately enlarged, a meatoplasty (me-ATE-o-plass-tea). Sometimes the ear moves position slightly on the head, and may fold either tighter against the head or stick out a little more depending on how the scar heals up.
  • Infection can set into the wound, this could progress to a cauliflower ear.

What is the alternative to mastoid surgery?

  • Nothing except mastoid surgery can cure cholesteatoma.
  • But it may be possible to keep it under reasonable control by repeated microsuction treatment in out patients.
  • This option is sometimes advised for older infirm patients, especially if their general medical condition makes a long operation difficult and risky, and whose shorter life expectancy makes the probability of developing serious complications less.
  • This strategy can include the option of carrying out mastoid surgery, to try and salvage the situation, only if serious complications actually happen.

Before coming into hospital for ear surgery

  • If you smoke, you should give up, because smokers are more likely to suffer complications after operation.
  • Parents should also consider giving up, as children must not be exposed to passive smoking during recovery.
  • Make sure you have supplies of soluble paracetamol (Calpol for children) for when you come home.
  • Do not plan anything important during the two weeks after operation.
  • If you normally wear a hearing aid in the ear that is to be operated, remember you won’t be able to wear it for several weeks or months. Also, the shape of your ear canal is likely to change and it may no longer fit you afterwards.
  • Wearing spectacles could be difficult in the first 24 hours because of the head bandage. You might need to bend the arm outward, or temporarily remove it and wear them over the bridge of the nose.

Before the operation

Pre-operative checklists and handover of patient from ward sister to operating theatre nurse, Chaucer HospitalPre-operative checklists and handover of patient from ward sister to operating theatre nurse, Chaucer Hospital

Remember to bring any medicines with you to hospital. You will not be allowed anything to eat for about six hours before operation, but you can drink clear fluids up to two hours before. The six hour rule does not apply to medicines – these should be taken as usual. When you come into hospital, you will be seen by the nurse who will ask various questions about your general health and attach an identity bracelet to your wrist. Similar questions will be asked by the Resident Medical Officer, and possibly by the anaesthetist. Please don’t get upset if you are asked the same question several times. This is a routine to help avoid mistakes – like an airport checking your travel documents more than once. You will be examined and checks made to ensure you are fit for anaesthetic. If you have any worries or questions, this is a good time to ask.

After the operation

After the operation, you will wake up in the recovery area, where a nurse will look after you. You will be asked to spit out the plastic tube in your mouth. You will probably have a head bandage on. You may feel sick and dizzy. This is quite normal and will stop after a while. When you are sufficiently awake, you will return to the ward. You will stay in bed for several hours. You will feel thirsty and tired, and you may be sick. The nurse will attend you frequently to check your pulse and breathing. If you are in any discomfort, please let the nurse know as she can you an injection to help relieve it. You will be allowed to drink as soon as the nurse is happy with your condition. You will be advised not to have too much initially as it might make you sick. Food is started as soon as you are able.

How long will I be in hospital?

Most patients operated in the morning are able to go home later the same day. If your operation is done in the afternoon, or if your recovery is slow, you may need to stay in overnight. Your head bandage will normally be taken down before you go home, and you will be given a stretch headband with disposable gauze pads to be changed each day.

What restrictions are there afterwards?

  • You will need two weeks off school or work.
  • You mustn’t drive for at least 48 hours, and longer if you are still feeling dizzy or tired.
  • You must strictly avoid getting any water in the ear for at least three months, maybe longer
  • You shouldn’t fly for three months

What can I expect at home after the operation?

  • You can expect to feel tired.
  • You may be a little unsteady on your feet.
  • You won’t be able to hear very well from the operated ear, it will be packed with a dressing.
  • You will probably hear tinnitus, various sounds in the operated ear.
  • Ear surgery is not especially painful.
  • You will get the odd twinge of pain on chewing or if you happen to lie on the wound.
  • Strong painkillers aren’t normally needed, you may use paracetamol as required. Be careful you don’t take more than maximimum recommended dose.

Looking after the wound and your dressings

  • Keep the ear dry. You can have your hair washed, carefully by someone who can keep the water away from your operated ear. They may have to miss out the part immediately next to the ear.
  • Protect the wound by wearing your stretch headband and disposable dressing pads, you should receive a supply of these on discharge from the hospital.
  • You may get some seeping of yellow or brownish fluid from the ear, or slight bloodstaining. This is normal.
  • You should change the disposable pads under your stretch headband at least daily, more often if there is a lot of seepage.
  • The dressing we put in the ear is BIPP. This stands for Bismuth Iodoform Paraffin Paste.
  • It has a distinctive hospital antiseptic smell, which some find reassuring and others dislike.
  • BIPP is a very good antiseptic and can be left safely in wounds for months without going off, unlike some other dressings.
  • The paste is impregnated on a ribbon gauze. We usually place several pieces of gauze on top of one another.
  • If the BIPP ribbon gauze dressing starts coming out – sometimes it gets stuck onto your outer dressing – just cut it off near the ear with a clean pair of scissors. There will be plenty more inside.
  • If you get increasingly severe pain, or a smelly, nasty discharge, that might mean infection and you should contact us urgently for advice.
  • If the outer ear begins to itch severely, and starts to go red and blisters, that usually indicates you have become allergic to BIPP. This tends to happen only to patients who have had previous ear surgery and become sensitised to BIPP.
  • BIPP allergy with red swelling and blistering of skin around ear dressingBIPP allergy with red swelling and blistering of skin around ear dressing
  • You should contact us urgently for advice if you think you might have developed a BIPP allergy. The dressing will have to be removed, and you will need treatment with antihistamines and maybe steroids.

Looking after yourself

  • After a day or two of rest, begin gentle exercise.
  • Start just pottering around the house, then short walks outside, but avoid strenuous physical activity.
  • Keep away from crowds or anyone with a cold, ‘flu or other infection.
  • Keep strictly away from cigarette smoke.
  • Avoid dirty or dusty environments.
  • Take any antibiotics as prescribed for the full course.

First post-operative visit

Healing postauricular incision two weeks following mastoid surgery. Stitches ready for removal.Healing postauricular incision two weeks following mastoid surgery. Stitches ready for removal.BIPP ribbon gauze and silastic sheet dressing in ear canal viewed under operating microscope two weeks following mastoid surgeryYellow / brown BIPP ribbon gauze and blue silastic sheet dressing in ear canal, seen under operating microscope two weeks following mastoid surgery

Your first out-patient visit will normally be two to three weeks after the operation.

  • We will remove your stitches, and packs from the ear.
  • This is done under the microscope.
  • It is very delicate.
  • You will need to lie still.
  • Sometimes there is a brief twinge of pain as we take out the dressing.
  • We may need to admit younger children to hospital as a day case, to carry out this treatment under a short general anaesthetic.

The operated area is very delicate intitially. Depending on the method of surgery, it may take several weeks or even up to three months for it to heal. During this time, there is a risk of picking up an infection.

  • You must strictly avoid getting any water in the ear, especially bath water which is a soup of germs from the rest of the body.
  • Do not under any circumstances dunk your head in the bath.
  • For a few weeks after the dressing has been removed, we recommend protecting the ear from cold winds by placing a fresh piece of cotton wool in the canal when outdoors.
  • Indoors, at home, you may leave it uncovered.
  • We don’t normally recommend any medication as a routine.
  • If, however, you get an increasingly severe earache, or if your ear develops an unpleasant smelly discharge, that ususally means an infection has set in.
  • This would normally be treated with antibiotic / steroid combination ear drops or spray.

If you are going away or think you might have difficulities in attending for urgent treatment, we will prescribe a supply of the medication for you to have on standby. We would expect you to contact us as soon as possible if you have to start using the medication, as you may need to have your appointment brought forward.

Long term follow-up and second stage surgery

A pearl of cholesteatoma seen through a rebuilt eardrum 9 months following mastoid surgeryA pearl of cholesteatama. Routine follow-up ear examination under the microscope in out-patients, 9 months following mastoidectomy and tympanoplasty surgery in which the ossicles were preserved and the eardrum rebuilt. The small white area at the upper part of the rebuilt eardrum is a pearl of cholesteatoma. The pearl had formed from a tiny fragment of cholesteatoma that hadn’t been removed at the first operation. The patient had no symptoms from it at this stage. A second operation was advised, and the pearl was removed, it was limited to a small area and the ossicles were once again preserved. If it had been neglected until it caused symptoms, it is unlikely the hearing could have been saved.
Cartilage graft used to reconstuct the outer attic wall, left ear, five years following mastoid surgeryCartilage graft used to reconstuct the outer attic wall, left ear, five years following mastoid surgery. To the non-specialist it looks just like the cholesteatoma, but it isn’t. This ear is safe and healthy.
Key:
m = malleus handle
C = cartilage graft

Having mastoid surgery is not the end of the matter. Cholesteatoma can come back, many years or even decades after initially successful surgery. It is essential for cholesteatoma patients to have long term follow up by a specialist who knows how to diagnose the disease. There are two ways the disease can come back:

  1. Residual cholesteatoma. It wasn’t completely removed at the first surgery. It can be very difficult to remove every last skin cell when we are trying to avoid further damage to the ear. Usually, residual cholesteatoma will show up within a year or two, but it may be much longer. With some techniques, especially if we do reconstruction that hides where the disease was, you may be advised to have a second stage operation a year after the first. This will detect a small cholesteatoma that developed from the odd stray skin cell. It forms a pearl. If this pearl is discovered at an early stage, it might be possible to simply winkle it out. If it was left until it became symptomatic, there may be further loss of hearing.
  2. Recurrent cholesteatoma. A second, new cholesteatoma develops, because the underlying problems that caused the first cholesteatoma, such as poor Eustachian Tube function, the eardrum getting sucked in, and failure of the normal outward migration of skin, are still there. This may not show up for decades.

As well as looking out for recurrent disease, many cholesteatoma patients need regular cleaning of the ear in order to prevent that build-up of dead skin layers. Cleaning is best done by microsuction. Ear syringing is not very good at removing adherent layers of dead skin, and might well set off infection by getting them wet. Patients vary considerably in how often they need their ears cleaned. Some will manage with only once a year, others may need it every two or three months. The average is between six and nine months. It’s not so different from having to go to the dentist to get your teeth de-scaled. Neglected mastoid cavities are dangerous. Some of the most serious complications I have seen were in patients who neglected to have their ears cleaned following mastoid surgery for cholesteatoma.

Hearing aids and BAHA osseointegrated auditory implant

BAHA components - diagram courtesy of Cochlear / Entific

Most cholesteatoma patients have hearing loss. Even after successful surgery, it is very unlikely that hearing will be 100%. If only one ear is affected, you might just cope with having a deaf side. But if both ears are affected, or if hearing properly on both sides is important to you, some form of hearing aid is usually needed. Normal hearing aids can help, but the sound quality isn’t always great, and they often cause infection. Blocking the ear canal with a hearing aid mould makes it moist. This encourages germs. Just imagine the state of your feet if you wore plastic shoes all day. Many cholesteatoma patients find that they can’t wear hearing aids because they cause infection, with painful, discharging ears. They have to stop wearing the hearing aid, get treatment for the infection with microsuction and antibiotic / steroid ear drops, and are unable to hear properly for weeks. Once the infection clears up, they go back to wearing the hearing aid, then the whole miserable cycle repeats itself. The situation is worse if both ears have been operated. Some audiologists try to get around the problem of blockage by using loose fitting or vented moulds. This often results in feedback and whistling when the volume is turned up. Also, even partial blockage of the ear canal reduces ventilation and encourages infection. A bone conductor hearing aid uses bone conduction to put sound directly into the inner ear, bypassing the middle ear problem. Old fashioned bone conductor hearing aids are placed on metal springs like an Alice band, or sometimes built into a pair of spectacles. They are cumbersome, and very uncomforatble to wear for any length of time, because they have to press very hard. They can cause pain, headaches and ulceration of the skin. Sound quality is muffled by the soft tissue of the scalp. The most effective way of getting around this problem is to put sound directly into the bone of the skull, by the titanium screw of a BAHA osseointegrated auditory implant.

Disclaimer

All information and advice on this website is of a general nature and may not apply to you. There is no substitute for an individual consultation. We recommend that you see your General Practitioner if you would like to be referred.