Cholesteatoma

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Cholesteatoma
Classification and external resources

Cholesteatoma
ICD-10 H71
ICD-9 385.32
DiseasesDB 2553
MedlinePlus 001050
eMedicine ped/384 ent/220
MeSH D002781
Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous
epithelium in the middle ear and/or mastoid process. Although these are not strictly speaking
tumours or cancers they can still cause significant problems because of their erosive and expansile
properties resulting in the destruction of the ossicles as well as their possible spread through
the base of the skull into the brain. They are also often infected and result in chronically draining
ears.
Contents
[hide]
1 Classification and Pathogenesis
2 Acquired Cholesteatoma
3 Epidemiology
4 Symptoms
5 Signs
6 Treatment
o 6.1 General aims of treatment
o 6.2 Surgical strategies
7 Prognosis
8 See also
9 References
10 External links
Classification and Pathogenesis[edit]
There are two types: congenital and acquired. Acquired cholesteatomas, which are more common,
can be caused by pathological alteration of the ear drum leading to accumulation of keratin within
the middle ear.
Less commonly the disease may be a developmental abnormality, when it grows from birth behind
the ear drum, in the form of a keratin-filled cyst.
[1]

Congenital cholesteatoma- it arrises from the embryonic epidermal crest . cong cholesteatoma
occurs at three important site- middle ear Petrous appex Cerebropontine angle.
Keratin-filled cysts that grow medial to the tympanic membrane result either from birth abnormality,
trauma or metaplasia. If they fulfill the following criteria they are considered to be congenital:
[1]

mass medial to the tympanic membrane
normal tympanic membrane
no previous history of ear discharge, perforation or ear surgery
Congenital cholesteatomas are more often found in the anterior aspect of the ear drum, and a
vestigial structure, theepidermoid formation, from which congenital cholesteatoma may originate,
has been identified in this area.
[2]

Acquired Cholesteatoma[edit]
More commonly, keratin accumulates in a pouch of tympanic membrane which extends into
the middle ear space. This abnormal folding or 'retraction' of the tympanic membrane arises in one of
the following ways:
Wittmaack's theory : Invagination of tympanic membrane from the
attic or posterosuperior part of pars tensa in the form of retraction
pockets lead to the formation of cholesteatoma.
[3]

Ruedi's theory : The basal cells of germinal layer of skin proliferate
under the influence of infection and lay down keratinising squamous
epithelium.
[4]

Habermann's theory: The epithelium from the meatus or outer drum
surface grows into the middle ear through a pre-existing perforation
and form cholesteatoma.
[5]

Cholesteatoma may also arise as a result of metaplasia of the middle ear mucosa
[6]
or implantation
following trauma.
Epidemiology[edit]
In one study, the number of new cases of cholesteatoma in Iowa was estimated in 19756 to be just
under one new case per 10,000 citizens per year.
[7]
Cholesteatoma affects all age groups, from
infants through to the elderly. The peak incidence occurs in the second decade.
[7]

Symptoms[edit]
The majority (98%) of patients with cholesteatoma have ear discharge or hearing loss or both in the
affected ear.
Other more common conditions, such as otitis externa may also present with these symptoms, but
cholesteatoma is much more serious and should not be overlooked. If a patient presents to a doctor
with ear discharge and hearing loss, the doctor should consider the patient to have cholesteatoma
until the disease is definitely excluded.
Other less common symptoms (all less than 15%) of cholesteatoma may include: pain, balance
disruption, tinnitus, ear ache,headaches and bleeding from the ear. There can also be facial
nerve weakness. Balance symptoms in the presence of a cholesteatoma raises the possibility that
the cholesteatoma is eroding the balance organs, which form part of the inner ear.
Signs[edit]
On initial inspection, an ear canal full of discharge may be all that is visible. Until the doctor has
cleaned the ear and inspected the entire tympanic membrane, cholesteatoma cannot be either
confirmed or excluded.
Once the debris is cleared, cholesteatoma can give rise to a number of appearances. If there is
significant inflammation, the tympanic membrane may be partially obscured by an aural polyp. If
there is less inflammation, the cholesteatoma may present the appearance of 'semolina' discharging
from a defect in the tympanic membrane. The posterior and superior parts of the tympanic
membrane are most commonly affected. If the cholesteatoma has been dry, the cholesteatoma may
present the appearance of 'wax over the attic'. The attic is just above the ear drum.
The patient may commonly also have clinical signs of conductive hearing loss. Less frequently, there
may be signs of imbalance or facial weakness.
If untreated, a cholesteatoma can eat into the three small bones located in the middle ear
(the malleus, incus and stapes, collectively called ossicles), which can result in nerve
deterioration, deafness, imbalance and vertigo. It can also affect and erode, through the enzymes it
produces, the thin bone structure that isolates the top of the ear from the brain, as well as lay the
covering of the brain open to infection with serious complications (rarely even death due to
brain abscess andsepticemia).
Both the acquired as well as the congenital types of the disease can affect the facial nerve that
extends from the brain to the face and passes through the inner and middle ear and leaves at the
anterior tip of the mastoid bone, and then rises to the front of the ear and extends into the upper and
lower face.
Treatment[edit]
Cholesteatoma is a persistent disease. Once the diagnosis of cholesteatoma is made in a patient
who can tolerate a general anesthetic, the standard treatment is to surgically remove the growth.
General aims of treatment[edit]
The challenge of cholesteatoma surgery is to permanently remove the cholesteatoma whilst
retaining or reconstructing the normal functions of the structures housed within the temporal bone.
The general objective of cholesteatoma surgery has two parts. It is both directed against the
underlying pathology and directed towards maintaining the normal functions of the temporal bone.
These aims are conflicting and this makes cholesteatoma surgery extremely challenging.
Sometimes, the situation results in a clash of surgical aims. The need to fully remove a progressive
disease like cholesteatoma is the surgeon's first priority. Preservation of hearing is secondary to this
primary aim. If the disease can be removed easily so that there is no increased risk of residual
disease, then the ossicles may be preserved. If the disease is difficult to remove, so that there is an
increased risk of residual disease, then removal of involved ossicles in order to fully clear
cholesteatoma has generally been regarded as necessary and reasonable.
In other words, the aims of cholesteatoma treatment form a hierarchy. The paramount objective is
the complete removal of cholesteatoma. The remaining objectives, such as hearing preservation, are
subordinate to the need for complete removal of cholesteatoma. This hierarchy of aims has led to
the development of a wide range of strategies for the treatment of cholesteatoma.
Surgical strategies[edit]
The variation in technique in cholesteatoma surgery results from each surgeons' judgment whether
to retain or remove certain structures housed within the temporal bone in order to facilitate the
removal of cholesteatoma. This typically involves some form of mastoidectomy which may or may
not involve removing the posterior ear canal wall and the ossicles.
Removal of the canal wall facilitates the complete clearance of cholesteatoma from the temporal
bone in three ways:
1. it removes a large surface onto which cholesteatoma may be
adherent;
2. it removes a barrier behind which the cholesteatoma may be
hidden;
3. it removes an impediment to the introduction of instruments
used for the removal of cholesteatoma.
Thus removal of the canal wall provides one of the most effective strategies for achieving the
primary aim of cholesteatoma surgery, the complete removal of cholesteatoma. However, there is a
trade-off, since the functional impact of canal wall removal, is also important.
The removal of the ear canal wall results in:
a space, the "mastoid cavity", which is less likely than the original
ear canal to resist infection;
exposure of the ossicles, which may allow the subsequent formation
of a new cholesteatoma deep to the ossicles. To prevent this, these
ossicles must be removed, which may diminish the patient's
hearing.
The formation of a mastoid cavity by removal of the canal wall is the simplest and most effective
procedure for facilitating the removal of cholesteatoma, but may bestow the most lasting infirmity
due to loss of ear function upon the patient treated in this way.
The following strategies are employed to mitigate the effects of canal wall removal:
1. careful design and construction of the mastoid cavity. This is
essential for the health and integrity of the protective sheet of
migrating, keratising epithelium which lines the distorted ear
canal. This requires the surgeon to saucerise the cavity. A high
facial ridge and an inappropriately small cartilaginous meatus
are obstructions to epithelial migration and are particularly high
risk factors for failure of the self-cleaning mechanism of the
external ear.
[8]

2. partial obliteration of the mastoid cavity. This can be performed
using a wide range of materials. Many of these resorb in time,
which means that the long term results of such surgery are
poorer than the short term results.
[9]

3. reconstruction of the ear canal wall. Canal wall reconstruction
has been performed using ear canal skin alone, fascia,
cartilage, titanium as well as by replacing the original intact wall.
If the reconstruction is poorly performed, it may result in a high
rate of recurrent cholesteatoma.
[10]

4. preservation of the ear canal wall. If poorly performed, it may
result in a high rate of both residual and recurrent
cholesteatoma.
[11]

5. reconstruction of the chain of hearing bones.
[12]

Clearly, preservation and restoration of ear function at the same time as total removal of
cholesteatoma requires a high level of surgical expertise.
Prognosis[edit]
It is important that the patient attend periodic follow-up checks, because even after careful
microscopic surgical removal, cholesteatomas may recur. Such recurrence may arise many years, or
even decades, after treatment.
A residual cholesteatoma may develop if the initial surgery failed to completely remove the original;
residual cholesteatomas typically become evident within the first few years after the initial surgery.
A recurrent cholesteatoma is a new cholesteatoma that develops when the underlying causes of the
initial cholesteatoma are still present. Such causes can include, for example, poor eustachian
tube function, which results in retraction of the ear drum, and failure of the normal outward migration
of skin.
[13]

In a retrospective study of 345 patients with middle ear cholesteatoma operated on by the same
surgeon, the overall 5-year recurrence rate was 11.8%.
[14]
In a different study with a mean follow-up
period of 7.3 years, the recurrence rate was 12.3%, with the recurrence rate being higher in children
than in adults.
[15]

See also[edit]
Chronic suppurative otitis media
Otic polyp
References[edit]
1. ^ Jump up to:
a

b
Derlacki EL, Clemis JD (1965). "Congenital
cholesteatoma of the middle ear and mastoid". Annals of Otology,
Rhinology, and Laryngology 74 (3): 706727.PMID 5846535.
2. Jump up^ Michaels L (1988). "Origin of congenital cholesteatoma
from a normally occurring epidermoid rest in the developing middle
ear". Int J Pediatr Otorhinolaryngol 15 (1): 51
65.doi:10.1016/0165-5876(88)90050-X. PMID 3286554.
3. Jump up^ "Chronic suppurative otitis media". ENT. Retrieved 12
January 2013.
4. Jump up^ Rueedi L (1959). "Cholesteatoma formation in the
middle ear in animal experiments". Acta Oto-Laryngologica 50 (3
4): 233242. doi:10.3109/00016485909129191.PMID 13660782.
5. Jump up^ Haberman J (1888). "Zur Entstehung des
Cholesteatoms des Mittelohrs". Archiv fuer Ohrenheilkunde 27:
4351.
6. Jump up^ Sade, J; Babiacki, A; Pinkus, G (1983). "The
metaplastic and congenital origin of choesteatoma". Acta
Otolaryngologica 96 (12): 119
129.doi:10.3109/00016488309132882. PMID 6193677.
7. ^ Jump up to:
a

b
Harker LA (1977). Cholesteatoma: an incidence
study in Cholesteatoma First International Conference.
Birmingham, Alabama, USA: Aesculapius Publishing Company.
pp. 308309. ISBN 0-912684-11-9.
8. Jump up^ Wormald P,J Nilssen EL (1998). "The facial ridge and
the discharging mastoid cavity". Annals of Otology, Rhinology, and
Laryngology 108 (1): 9296. PMID 9432074.
9. Jump up^ Black B (1995). "Mastoidectomy
elimination".Laryngoscope 105 (12 pt 2 Suppl 76): 1
30.doi:10.1288/00005537-199512000-00023.PMID 7500804.
10. Jump up^ Deveze A et al (2010). "Rehabilitation of canal wall
down mastoidectomy using a titanium ear canal implant". Otol
Neurotol 31 (2): 220
224.doi:10.1097/MAO.0b013e3181c9960d.PMID 20009781.
11. Jump up^ Jansen C (1968). "The combined approach for
tympanoplasty (report on 10 years' experience)". J Laryngol
Otoll 82 (9): 779
793. doi:10.1017/S0022215100069462.PMID 4878658.
12. Jump up^ Austin DF (1971). "Ossicular reconstruction". Archives
of Otolaryngol 94 (6): 525
535.doi:10.1001/archotol.1971.00770070825007.PMID 5129224.
13. Jump up^ Fairley, James (7 November 2010). "Cholesteatoma
and mastoid surgery". entkent.com. Retrieved 29 December 2012.
14. Jump up^ Mishiro, Y.; Sakagami, M.; Kitahara, T.; Kondoh, K.;
Okumura, S. (September 2008). "The investigation of the
recurrence rate of cholesteatoma using Kaplan-Meier survival
analysis". Otology & Neurotology (American Otological Society,
American Neurotology Society [and] European Academy of
Otology and Neurotology) 29 (6): 803
6. doi:10.1097/MAO.0b013e318181337f.PMID 18636031.
15. Jump up^ Vartiainen, Eero (1995). "Factors associated with
recurrence of cholesteatoma". The Journal of Laryngology &
Otology 109 (7). doi:10.1017/S0022215100130804.
Information on Cholesteatomas
Functional Orthogonal Cholesteatoma Surgery
[hide]
V
T
E
Diseases of the ear and mastoid process (H60H99, 380389)

Outer ear
Otitis externa
Otomycosis

Middle ear
and mastoid
Otitis media
Mastoiditis
Bezold's abscess
Gradenigo's syndrome
Tympanosclerosis
Cholesteatoma
Perforated eardrum

Inner ear and
central pathways
Common pathway
Labyrinthitis/Otitis interna

Equilibrioception
Vertigo/Balance disorder: peripheral
Mnire's disease
BPPV
Vestibular neuronitis
Perilymph fistula
central (Central positional nystagmus)

Hearing
Hearing impairment
Conductive hearing loss
Otosclerosis
Superior canal dehiscence
Sensorineural hearing loss
Presbycusis
Cortical deafness
Nonsyndromic deafness

Excessive response
Tinnitus
Hyperacusis/Phonophobia

Deafblindness
Wolfram syndrome
Usher syndrome

Other
Auditory processing disorder
Spatial hearing loss


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2007 2014 JW Fairley Last updated 16 August 2014
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What is cholesteatoma?
Cholesteatoma 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 andtinnitus. 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.



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)
Normal 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

Abnormally thin right eardrum damaged by glue ear and showing ossicles malleus incus andstapes
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 someones 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 shouldnt 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 (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 isnt 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
dont 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 isnt
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. 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.

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:

o discharge
o build up of debris
o swelling
o reddening
o 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 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. 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 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
isnt 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 anout-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 booth
Tuning fork test part of Rinnes 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 doesnt 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 dont 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 wont
work.
Microsuction treatment
Microsuction of the ear as an out-patient procedure
Wax removal using microsuction

Wax removal using a fine round ended hook

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 childs shoulder and focussed onto the ear by a head
mirror worn by the surgeon. The view is nothing like as good, and we cant 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

o binocular vision for 3-d stereoscopic view
o high power magnification
o very bright illumination
to remove material blocking the ear canal such as

o wax
o infected debris, pus and fungal material
o dead skin layers including cholesteatoma
o foreign bodies
o 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. Dont 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. 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
dont 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 doesnt 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 dont 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 dont 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

Drilling right mastoid

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 1950s 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 1960s I did my basic training
(in the 1980s) on temporal bones from cadavers (dead bodies). Although some might find that
macabre, Id 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 arent 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 hasnt 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 dont 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.
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 dont 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 wont 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 ear KTP LASER right
mastoid. Surgeons view down microscope. Green light is turned red by a filter to protect the surgeons 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 cant use the laser. If the cholesteatoma is directly on the facial nerve, we cant 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 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.
Further information on General Anaesthesia for children
Further information on General Anaesthesia for adults
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 arent really risks as such, they are just
things that are going to happen that you probably wont 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 wont 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 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 dont 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 mustnt 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 shouldnt 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 wont 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 arent normally needed, you may use paracetamol as required. Be careful you
dont 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 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. Yellow / 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 dont 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 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 hadnt 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 surgery. To the non-specialist it looks just like the cholesteatoma, but it isnt. 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 wasnt 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. Its 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

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 isnt 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 cant 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.
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About us ENT Kent Partnership 2014
Specialist in Ear Surgery
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Functional Orthogonal Cholesteatoma
Surgery
John Hamilton's approach to preserving hearing in
cholesteatoma surgery

What is cholesteatoma?
Where does cholesteatoma occur?
What are the consequences of cholesteatoma?
How does cholesteatoma behave?
How is cholesteatoma diagnosed?
What structures are in the temporal bone?
What is the treatment for cholesteatoma?
What are the aims of cholesteatoma surgery?
Is it possible to measure how well cholesteatoma surgery is performed?
How effective is conventional surgery at removing cholesteatoma?
What advances may improve the situation?
How much does the laser improve the removal of cholesteatoma?
Does the laser improve hearing results after cholesteatoma?
How is cholesteatoma removed from the hearing bones?
How is cholesteatoma removed from the epitympanic gutter?
How much does the laser improve hearing after cholesteatoma surgery?
Summary

What is cholesteatoma?

In most cases, cholesteatoma results from abnormal growth of the skin of the ear drum into the skull.
The resulting sac continually generates skin cells. These stagnate causing relentless or recurrent
ear discharge. The inflammation caused by the infected skin causes erosion of the structures within
the skull.

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Where does cholesteatoma occur?
Cholesteatoma is the accumulation of skin debris
within the middle ear.
The middle ear occupies much of the temporal bone which is the main bone on each side of the
base of the skull.
Cholesteatoma does not occur anywhere else.
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What are the consequences of cholesteatoma?
In addition to ear discharge a patient with cholesteatoma may notice symptoms caused by
destruction of the structures within the skull. The commonest of these symptoms is hearing loss.
Less common symptoms include dizzyness, tinnitus and facial weakness. Most people are surprised
to learn that cholesteatoma rarely causes pain.
If an abscess forms, it causes pain and swelling around the ear and can result in meningitis or other
infection of the brain. When cholesteatoma is neglected such serious complications can cause
death.
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How does cholesteatoma behave?
Cholesteatoma causes inflammation. Inflammation causes erosion of bone, so the cholesteatoma
progressively enlarges.
It also causes scarring so that the cholesteatoma becomes firmly attached to the structures in the
temporal bone.
At any time, the indolent suppuration of the dead skin within the cholesteatoma may erupt into an
abscess.
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How is cholesteatoma diagnosed?
Other ear disorders, such as ear drum perforation and ear canal infection also cause hearing loss
and ear discharge.
The following features should raise suspicion that cholesteatoma is present:
Relentless ear discharge despite treatment
Recurrent ear discharge despite treatment
Foul smelling ear discharge
Cholesteatoma is usually diagnosed on careful inspection of the eardrum. This may be obvious or it
may be subtle. It often requires the ear to be competely clean, which may require specialist skills
and equipment.
On rare occasions, scans are required to diagnose or confirm the presence of cholesteatoma
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What structures are in the temporal
bone?
Most of the temporal bone is occupied by the air spaces of the middle ear. This air space is neede to
allow the ear drum to vibrate and transmit sound.
The ear canal, ear drum and hearing bones are also housed within the temporal bone. Their role is
also to transmit sound through to the inner ear.
The inner ear, which has two parts, the cochlea (for hearing) and the vestibule (for balance) lies
deep within the temporal bone.
Nerves from the brain to the face and neck run through the temporal bone.
Major bood vessels to and from the brain also run through the temporal bone.
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What is the treatment for cholesteatoma?
Cholesteatoma is an abnormal physical structure. It cannot be removed with medicines.
As it is progressive, it requires removal, which means surgery.
All cholesteatoma should be surgically removed if the patient is fit enough for anaesthetic.
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What are the aims of cholesteatoma surgery?
Cholesteatoma surgery has two conflicting aims. Getting the balance between these is very difficult.
This is the reason why you should consider carefully who performs your cholesteatoma surgery.
The conflicting aims of cholesteatoma surgery are:
To completely remove (and prevent the recurrence) of cholesteatoma.
To maintain or restore the normal function of the structures of the temporal bone.
The conflict arises because:
The former is facilitated by wide removal of all the structures of the temporal bone.
The latter is facilitated by preservation of the structures of the temporal bone.
There are many techniques currently used to try to achieve these aims. Older techniques tend to be
heavily biased towards achieving the first aim at the cost of the second.
This is the reason why you should consider carefully who performs your cholesteatoma surgery.
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Is it possible to measure how well cholesteatoma surgery is performed?
Mr Hamilton has proposed that four outcomes should be recorded to measure the effectiveness of
cholesteatoma surgery.
1. Complete removal of cholesteatoma
2. No new growth of cholesteatoma
3. Dry ear, even when washing or swimming
4. Socially useful hearing
The first two assess whether the disease has been completely removed. The latter pair assess
whether the ear is functioning normally. Each is a simple yes/no answer.
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How effective is conventional surgery at removing cholesteatoma?
Take a look at how well cholesteatoma is completely removed in one operation:
Famous and dedicated surgeons who have audited and published their work often have
residual cholesteatoma rates of 15-30 per cent.
Is this necessarily a measure of individual failure? If even the most skilled surgeons leave this
amount of disease, the problem resides more with the limitations imposed by the surgical
instruments than with the dexterity of the surgeons:
Conventional instruments are not up to the task of completely clearing all cholesteatoma.
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What advances may improve the situation?
Lasers vaporise disease and therefore are:
simple to use
cause no movement
stop bleeding
Some lasers can be transmitted along fibres. These can guide the laser energy around corners. In
cholesteatoma surgery, this means that the laser can be guided around the many obstacles in the
temporal bone.
Some surgeons feel that endoscopes may help by improving inspection and image capture of
cholesteatoma in regions of complex anatomy. However, the benefit of endoscopy in cholesteatoma
surgery is limited as:
endoscopes are not actually used for removing disease;
the surgeon holding an endoscope has only one free hand to perform surgery;
endoscopes are difficult to use with bleeding tissues.
problems encountered during
cholesteatoma surgery
conventional steel instruments fibre-guided laser
cochlea damaged by movement of hearing
bones
movement needed to remove disease no movement involved in disease
many obstacles
can work around corners with angled
instruments
can be guided around corners by optical fibre or off
mirror
inflamed tissues full of blood
vessels
dissection worsens bleeding laser stops bleeding
complex anatomy requires dexterity especially around corners point and shoot
Compare how well suited to the problems posed by cholesteatoma are conventional instruments, endoscopes and the fibre-
guided laser
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How much does the laser improve the removal of cholesteatoma?
Mr Hamilton compared the complete clearance of cholesteatoma in two hospitals, one with a laser
and one without. He did this by checking whether there was any residual cholesteatoma at a second
operation.
The flow chart shows how patients were involved in the study:

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Does the laser improve hearing results after cholesteatoma?
The laser does not require movement to remove cholesteatoma. This means that an entirely new
approach can be adopted to the treatment of firmly adherent cholesteatoma on the hearing bones.
Previously it was necessary to remove the hearing bones with the cholesteatoma, in order to protect
the delicate inner ear from damage.
Now the laser can remove firmly adherent cholesteatoma from the hearing bones without movement
and thereby without placing the inner ear at risk
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How is cholesteatoma removed from the hearing bones?
Cholesteatoma can be lasered from the ossicles themselves without movement.
The ossicles also present a barrier to removal of cholesteatoma.
A special view, the "orthogonal" view, has been developed to get to the cholesteatoma on the other
side of the ossicles: this enables a direct view of the deep side of the ossicles, the facial nerve and
the gap in between which Mr Hamilton has termed the epitympanic gutter. This view protects the
facial nerve and the hearing bones as the cholesteatoma is removed.

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How is cholesteatoma removed from the epitympanic gutter?
The area is approached using the orthogonal view.
The facial nerve is identified and protected.
The laser used on the ossicular side only, to protect the cochlea.
Steel instruments only are used on the facial nerve side, to protect the nerve.
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How much does the laser
improve hearing after cholesteatoma surgery?
Mr Hamilton compared the hearing of two groups of patients after cholesteatoma surgery. One group
had conventional treatment of the hearing bones, with reconstruction after removal of bones to
facilitate surgery. The other group had new, laser-assisted "functional" surgery, in which
cholesteatoma was removed from and around the hearing bones, which were left in place.
Analysis of the results showed that the risk of poor hearing in the conventional group was
approximately 3 times higher than the risk of poor hearing in the laser group.
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Summary
Lasers are better suited to the removal of cholesteatoma than conventional instruments.
Over the last decade Mr Hamilton has provided evidence that:
Cholesteatoma is removed more effectively when a laser is used
Lasers provide an opportunity to preserve hearing more effectively than any other technique
To achieve these outcomes has required the development of a new approach to cholesteatoma
surgery.
This has required new technical terms and ultimately a new operation, Functional Orthogonal
Cholesteatoma Surgery.