Cholesteatoma

Definition
Cholesteatoma is defined as the presence of keratinising squamous epithelium within the middle
ear, or in other pneumatised areas of the temporal bone. [1] This keratinising epithelium exhibits
independent growth, leading to expansion and to resorption of underlying bone. [2] Focal erosion
of external canal bone with accumulation of keratin is called external canal cholesteatoma. [3]

Summary
Accumulation of squamous epithelium and keratin debris that usually involves the
middle ear and mastoid.
Although benign, it may enlarge and invade adjacent bone.
Often presents with a malodorous ear discharge with associated hearing loss.
Diagnosis is clinical based on history and otoscopic findings. CT scan provides lesion
definition and extent.
Treatment is surgical removal. Adjunctive topical antimicrobial treatment may help
reduce acute symptoms preoperatively.
Complications include recurrence, meningitis, facial palsy, and a labyrinthine fistula.
Epidemiology

Aetiology
Cholesteatoma may be considered acquired or congenital.

Acquired cholesteatoma occurs in several ways. In many cases, it is due to retraction of an area of the
pars flaccida with or without associated atrophy of the pars tensa. [4] The epithelium becomes trapped
and infected, and proliferates to form a cholesteatoma. Alternatively, squamous epithelium may migrate
through a defect in the tympanic membrane, or the cholesteatoma may form due to implantation of
viable keratinocytes into the middle ear cleft following otological surgery or after a traumatic blast
injury. Children with cleft palates, craniofacial abnormalities, or Turner's or Down's syndrome have an
increased risk of developing cholesteatoma. [14] [18] [19] This increased risk is secondary to poor
eustachian tube function.

Congenital cholesteatoma is considered to be present if there is no history of previous ear surgery and
no perforation or retraction of the tympanic membrane. It is believed to arise from developmental
epidermoid rests present before birth that persist in the middle ear space. [7] [8] Alternative theories
include: invagination of squamous epithelium from the developing ear canal; [20] seeding of the middle
ear by squamous cells in the amniotic fluid; [21] [22] epithelial ingrowth from the surface of the
tympanic membrane after infection; and micro-perforation. [12]

P athophysiology
A retraction pocket is an area of invagination of the tympanic membrane that becomes pulled into the middle
ear space because of the negative pressure (vacuum-like) effect of eustachian tube dysfunction. [23] These
pockets are initially self-cleansing. They are graded or staged according to the degree of severity: [24]

Stage I: retracted membrane

Stage II: retraction onto the incus

Stage III: middle ear atelectasis

Stage IV: adhesive otitis media.

They are often found as the initial step in acquired cholesteatoma in adults or children; it is difficult to predict
which pockets will develop into a cholesteatoma. [25] View image Clinical and histological evidence suggest
that cholesteatoma in children tends to be more aggressive. [17] The neck of the pocket may narrow and trap
squamous cells with keratin accumulation and retention; these proliferate, leading to expansion and
cholesteatoma formation. [4] View image This may occur as papillae migrating through a temporary defect in
the pars flaccida or around a pars tensa perforation (marginal perforation). Less commonly there is proliferation
of the basal layers of keratinising epithelium of the pars flaccida. [26] Bacterial infection and super-infection of
the trapped debris form a biofilm and cause chronic infection and epithelial proliferation, [27] leading to further
lesion expansion and cholesteatoma formation. [4] Infection with Pseudomonas aeruginosa strains is often
involved. [28]
Once formed, the cholesteatoma sac is associated with enzymatic bony destruction due to cytokine-induced
inflammatory changes, with activation of osteoclasts and lysozymes. [29] [30] Cholesteatoma is often
associated with destruction of the ossicles causing a conductive hearing loss, and may be associated with
destruction of the semicircular canals (with resulting vertigo), cochlea (with resulting sensorineural hearing
loss), and facial canal (with resulting facial palsy).
Congenital cholesteatoma may present as an epidermoid cyst behind an intact tympanic membrane. It is often
found in the anterior superior aspect of the middle ear. This frequently arises above the eustachian tube orifice
and obstructs it early in its course, leading to a middle ear effusion. [23] With time, however, the cyst can
perforate, presenting as an acquired cholesteatoma. [25]

Classification
Aetiopathological classification [4]
Acquired cholesteatoma is usually associated with eustachian tube dysfunction, often with a previous history of
middle ear disease.

Primary: occurs as a consequence of retraction pocket formation within the tympanic membrane
subsequent to eustachian tube dysfunction, with invagination of squamous cells into the middle ear.

Secondary: occurs due to migration of squamous epithelium through an established tympanic

membrane defect (marginal perforation), or due to implantation of viable keratinocytes into the middle
ear cleft following otological surgery or after a traumatic blast injury.

Congenital cholesteatoma is believed to arise from developmental epidermoid rests present before birth that
persist in the middle ear space. [5] [6] [7][8]

History & examination

Key diagnostic factorshide all
presence of risk factors (common)

Key factors include middle ear disease, eustachian tube dysfunction, prior otological surgery,
traumatic blast injury to ear, congenital anomalies (e.g., cleft palates, craniofacial
abnormalities, Turner's or Down's syndrome). [14] [18] [19]

hearing loss (common)

Commonly presents with a conductive hearing loss. There may be a mixed conductive and
sensorineural hearing loss in patients with cochlear damage or in those with a pre-existing
hearing loss (e.g., congenital or presbycusis). Hearing may be normal in some patients.

ear discharge resistant to antibiotic therapy (common)

Recurrent or chronic purulent aural discharge that may be unresponsive to antibiotic therapy is
common in acquired cholesteatoma.

Discharge is malodorous and may be scant.

attic crust in retraction pocket (common)

Otoscopy typically shows crust or keratin in the attic (upper part of the middle ear), the pars
flaccida, or the pars tensa (usually posterior superior aspect), with or without a perforation of
the tympanic membrane. View imageView image

white mass behind intact tympanic membrane (uncommon)

Occurs in congenital cholesteatoma.

Risk factorshide all

Strong
middle ear disease

Acquired cholesteatoma is usually associated with eustachian tube dysfunction, often with a
previous history of middle ear disease such as otitis media.

eustachian tube dysfunction

Typically due to otitis media. Eustachian tube dysfunction promotes invagination of the
tympanic membrane, which becomes pulled into the middle ear space because of the
negative pressure (vacuum-like) effect. [23] This forms a retraction pocket.

These pockets are initially self-cleansing. However, the neck of the pocket then narrows and
traps squamous cells, expands in size, and leads to cholesteatoma formation. [4]

otological surgery

May result due to implantation of viable keratinocytes into the middle ear cleft.

traumatic blast injury to ear

May result due to implantation of viable keratinocytes into the middle ear cleft.

congenital anomalies

Children with cleft palates, craniofacial abnormalities, or Turner's or Down's syndrome have an
increased risk of developing cholesteatoma. [14] [18] [19] This increased risk is secondary to
poor eustachian tube function.

Weak
family history

Children with a family history of middle ear disease and/or cholesteatoma have an increased
risk of developing cholesteatoma. [23] [31]

Other diagnostic factorshide all

tinnitus (common)

May be associated with hearing loss; occurs in affected ear.

otalgia (uncommon)

Uncommonly found. Pain may be a feature of advanced disease.

altered taste (uncommon)

Occurs due to involvement of the facial nerve (VII).

dizziness (uncommon)

Occurs if there is erosion of the semicircular canal with the disease.

facial nerve weakness (uncommon)

A rare complication of cholesteatoma if the disease involves the facial nerve (VII). [39] [40]

Diagnostic tests
1st tests to orderhide all
Test

Result

pure tone audiogram

variable; may show a conductive hearing loss

Should be performed in all patients to

determine the patient's hearing status.
The test may also be normal or there
may be a mixed conductive and
sensorineural hearing loss in patients
with cochlear damage or in those with
a pre-existing hearing loss (e.g.,
congenital or presbycusis).
CT scan of the petrous temporal
bones

High-resolution CT scan of the petrous

temporal bones is recommended as part
of the initial work-up of patients with
middle ear cholesteatoma. [34] It can
provide confirmation of disease in
patients with an atypical presentation,
and can be used to assess the ear for
mastoid pathology and complications

opacification of the middle ear or mastoid; erosion

of the scutum, ossicular chain, labyrinth, facial
canal, tegmen, or bony capsule of the sigmoid sinus

such as cochlear, semicircular canal, or

intracranial involvement. [25] View
imageView image

Tests to considerhide all

Test

Result

fistula test

variable; positive in presence of semicircular canal fistula; falsenegatives may occur

Using tympanometry, pressure is

applied to the tympanic membrane and
eye movements are recorded with
electronystagmography (ENG)
electrodes.
Positive result is the production of
nystagmus secondary to positive
pressure applied to the tympanic
membrane.

MRI scan of the petrous temporal bones

opacification of the middle ear and mastoid; may show evidence of
intracranial complications such as meningoencephalic infection, intracranial extension,
Should be ordered if there are
or sigmoid sinus thrombosis
suspected intracranial complications
such as intracranial abscess,
meningitis, or inner ear
complications. [34]
A non-echo-planar, fast-spin echo, or
based diffusion-weighted MRI has been
shown to be useful in the diagnosis of
cholesteatoma. It is predominantly used
in patients who have already had
surgery in order to rule out recurrence

or residual disease. [35] [36] [37] [41]

positive for micro-organisms

bacterial culture
Order if ear discharge is unresponsive
to antimicrobial therapies.

Differential diagnosis
Condition

Differentiating signs/symptoms

Differentiating tests

chronic
suppurative
otitis media

On otoscopy there is perforation of the

pars tensa but no evidence of
cholesteatoma.

Diagnosis is clinical.

otitis
externa

On otoscopy there is swelling of the

external canal and a scant discharge.
The tympanic membrane is not visible
or, if visible, appears inflamed, but no
evidence of cholesteatoma.

Diagnosis is clinical.

benign
necrotising
otitis
externa

The patient reports severe otalgia; a

history of diabetes or other
immunosuppression.

CT scan demonstrates soft tissue

swelling of the ear canal with or
without erosion of the petrous
temporal bone.

On otoscopy there are granulations in

the ear canal but no evidence of
cholesteatoma.

On otoscopy there is inflammation of

the tympanic membrane with or
without granulations, but no evidence
of cholesteatoma.

Diagnosis is clinical.

myringitis

Step-by-step diagnostic approach

Diagnosis is based on the history and clinical findings. Imaging is not often necessary for diagnosis, but is of
value in assessment of disease extension and treatment planning.

History and examination

Patients typically present with hearing loss or tinnitus. A history of, or presentation with, recurrent or chronic
purulent aural discharge, which may be unresponsive to antibiotic therapy, is common in acquired
cholesteatoma. Discharge is malodorous and may be scant. Less commonly, patients present with symptoms of
otalgia, vertigo, or facial nerve (VII) involvement (altered taste or facial weakness). These often signify more
advanced disease.
Patients should be questioned for any pre-existing ear disease or causes of eustachian tube
dysfunction. [4] [23] A co-existing diagnosis of cleft palate, craniofacial abnormalities, or Turner's or Down's
syndrome increases the risk of acquired cholesteatoma. [14] [18] [19] Less commonly, family members may
also have had middle ear disease or cholesteatoma. [23] [31]
On examination there may be evidence of an ear discharge. Otoscopy typically shows crust or keratin in the
attic (upper part of the middle ear), the pars flaccida, or the pars tensa (usually posterior superior aspect), with
or without a perforation of the tympanic membrane. [32] View imageView image A white mass behind an
intact tympanic membrane may be observed in congenital cholesteatoma.
If there is significant aural discharge, the patient may require examination with an otomicroscope and microsuctioning of the ear. A zero- or thirty-degree endoscope may also be useful in examination of the ear. This can
help to differentiate a retraction pocket from cholesteatoma. [33] In patients who are difficult to examine (e.g.,
small children, those with learning difficulties), it may be necessary to examine the ears under a general
anaesthetic. This allows complete micro-suctioning of the ear, determination of the cause of the discharge
(whether or not a perforation or retraction pocket is present), and confirmation of the presence of a
cholesteatoma.
A fistula test is sometimes performed, using tympanometry to apply positive pressure and recording eye
movements. A positive result is nystagmus following positive pressure. However, the test may give a falsenegative result.

Audiology
Audiometry should be performed in all patients to determine the patient's hearing status. A hearing test may be
normal but more commonly demonstrates a conductive hearing loss. There may be a mixed conductive and
sensorineural hearing loss in patients with cochlear damage or in those with a pre-existing hearing loss (e.g.,
congenital or presbycusis).

Imaging
High-resolution CT scan of the petrous temporal bones is recommended as part of the initial work-up of
patients with middle ear cholesteatoma. [34] It can provide confirmation of disease in patients with an atypical

presentation, and can be used to assess the ear for mastoid pathology and complications such as cochlear,
semicircular canal, or intracranial involvement. [25] In patients with cholesteatoma, CT shows opacification of
the middle ear or mastoid, or erosion of the scutum, ossicular chain, labyrinth, facial canal, tegmen, or bony
capsule of the sigmoid sinus.View imageView image
MRI is of limited use in the diagnosis of cholesteatoma. This is because it gives no temporal bone detail. It is,
however, helpful when intracranial complications are suspected, such as a temporal lobe abscess or meningitis.
It may demonstrate opacification of the middle ear and mastoid, or show evidence of intracranial complications
such as meningoencephalic infection, intracranial extension, or sigmoid sinus thrombosis. [34] A non-echoplanar based diffusion-weighted MRI has been shown to be useful in patients who have already had surgery in
order to rule out recurrence of disease.[35] [36] [37]

Microbiology
Ear cultures are obtained mainly in patients who present with an aural discharge unresponsive to antimicrobial
therapies. Swabs from the ear demonstrate bacterial infection, commonly Pseudomonas aeruginosa, and may
dem onstrate other pathogens such as Staphylococcus aureus and anaerobic bacteria. [38]

Case history #1
A 37-year-old man presents with hearing loss and a painful intermittent purulent discharge from his right ear. He
also reports intermittent dizziness, and tinnitus in the right ear. On otoscopy, he has an attic crust on his right
tympanic membrane. Examination with an otomicroscope and micro-suctioning reveals keratin in an attic
retraction pocket. An audiogram demonstrates a conductive hearing loss on the right side.

Case history #2
A 12-year-old girl presents with a history of a recurrently discharging left ear for several months. She complains
of an offensive discharge and hearing loss. She reports a previous history of grommet insertion. Otoscopy
reveals a posterosuperior perforation of the tympanic membrane with a white keratin-like discharge. She has a
conductive hearing loss on audiometry.

Other presentations
Congenital cholesteatoma is often an incidental finding. The patient may have hearing loss, and is found to
have a white mass behind the tympanic membrane, with a normal pars tensa and pars flaccida of the tympanic
membrane, and no prior history of ear discharge, tympanic membrane perforation, or surgical procedures on
the ear. Patients (more commonly adults) may also present with complications of a cholesteatoma such as
meningitis, facial palsy, sensorineural hearing loss, or vertigo, due to destruction of the cochlea or vestibular
anatomy.

Treatment options
Consult your local pharmaceutical database for comprehensive drug information including contraindications,
drug interactions, and alternative dosing.

Acute
Patient group
Treatment line
Treatmenthide all
all patients
1st
surgery

The definitive treatment of cholesteatoma is surgery.

A canal wall up mastoidectomy allows removal of cholesteatoma but leaves the canal wall intact. The procedure
involves removal of the mastoid air cells lateral to the facial nerve and otic capsule, leaving the posterior and
superior parts of the external canal wall intact. [47] This necessitates a second-look procedure after 9 to 12 months
to ensure that the cholesteatoma has not recurred. Alternatively, a non-echo-planar diffusion-weighted MRI may be
used in some patients. [35] [36] Patients will require continued follow-up. [50] [51] [52] This technique is often
preferred for children, as it avoids long-term complications of a mastoid cavity.

A canal wall down mastoidectomy aims to remove the disease by drilling from the attic wall posteriorly. The size of
the resultant cavity will depend on the extent of the cholesteatoma. A less invasive procedure resulting in a minimal
cavity is called an atticotomy or atticoantrotomy; a more invasive procedure resulting in a larger cavity is called a
modified radical mastoidectomy. A canal wall down procedure may allow examination of the cavity for recurrence
but if the attic has been reconstructed, a second-look procedure may be necessary to examine the middle ear for
recurrent disease.

The use of endoscopy has been shown to positively impact the management of cholesteatoma and it is
recommended that it be used during surgery. [42]

Controlled hypotension during anaesthesia is often required in middle ear surgery. A study has demonstrated that
both magnesium sulfate and remifentanil in combination with sevoflurane can be used for this purpose but that
magnesium sulfate gives better postoperative analgesia and reduces shivering and postoperative nausea and
vomiting.[43]

Studies have also demonstrated that a dose of 8 mg dexamethasone, administered just before induction, is more
effective than a dose of 4 mg at controlling postoperative nausea and vomiting and patients required less postoperative analgesia. [44]Additionally, the combination of dexamethasone and midazolam may also reduce
postoperative nausea and vomiting. [45] [46]

Primary options
canal wall up mastoidectomy
OR

canal wall down mastoidectomy

adjunct
preoperative topical antibiotics + aural care

On initial presentation, if aural discharge is evident, it may be appropriate to treat the aural discharge with topical
antibiotics prior to definitive surgical treatment.

Agents containing quinolone are effective, in adults[C Evidence] and in children,[B Evidence] either alone or in
combination with a topical corticosteroid.

Aural cleaning may also reduce symptomatic discharge.

Prior to the use of topical ear drops, the ear canal may need to be cleaned of any debris or wax.

Patients who have severe swelling of the ear canal may have difficulty applying ear drops. A wick should be
inserted in the ear canal to allow for drug delivery.

Debridement of granulation tissue may be necessary.

Primary options
ciprofloxacin/dexamethasone otic: (0.3%/0.1%) 4 drops into the affected ear(s) twice daily for 7-10 days
OR
ofloxacin otic: (0.3%) 10 drops into the affected ear(s) once daily for 7 days

adjunct
second-look surgery or MRI

A canal wall up mastoidectomy allows removal of cholesteatoma but leaves the canal wall intact. This necessitates
a second-look procedure after 9 to 12 months to ensure that the cholesteatoma has not recurred. A meta-analysis
found an increased incidence of postoperative cholesteatoma when using an intact wall approach rather than canal
wall down approach. [48]

Alternatively, a non-echo-planar diffusion-weighted MRI may be used in some patients. [35] [36] [37] There
continues to be debate regarding which type of MRI best examines for recurrent cholesteatoma. Some authors
advocate routine MRI scans (such as non-echo-planar, fast-spin echo, or diffusion-weighted sequences) for followup, but caution that a negative scan may not be completely accurate as residual or recurrent disease may not yet
be detectable. Non-echo-planar imaging was found to be more reliable compared with echo-planar imaging in
identifying residual/recurrent cholesteatoma in one systematic review. [49] Another systematic review found that
non-echo-planar diffusion-weighted MRI is highly sensitive and specific in identifying middle-ear
cholesteatoma. [37]

Patients will require continued follow-up. [50] [51] [52]

A canal wall down procedure may allow examination of the cavity for recurrence but if the attic has been
reconstructed, a second-look procedure may be necessary to examine the middle ear for recurrent disease.

reatment approach

Patients suspected of having cholesteatoma should be referred to ENT surgeons on a semi-urgent basis. If
facial palsy is present with suspected cholesteatoma, those patients should be referred urgently because early
treatment is associated with better outcomes and treatment delays can result in a poorer
prognosis. [32] [39] [40]
The definitive treatment of cholesteatoma is surgery. The main goal is to remove the disease; provide a dry,
safe ear that does not discharge; and prevent potential complications. Treatment is also aimed at improving the
hearing threshold. This may not always be possible at initial surgery but may be possible following a second
procedure. There are different surgical approaches: the canal wall up mastoidectomy or the canal wall down
mastoidectomy. The use of endoscopy has been shown to positively impact the management of cholesteatoma
and it is recommended that it be used during surgery. [42]
Controlled hypotension is often required in middle ear surgery. A study has demonstrated that both magnesium
sulfate and remifentanil in combination with sevoflurane can be used for this purpose but that magnesium
sulfate gives better postoperative analgesia and reduces shivering and postoperative nausea and vomiting. [43]
Studies have also demonstrated that 8 mg dexamethasone is more effective than 4 mg dexamethasone at
controlling postoperative nausea and vomiting and providing analgesia in adult patients after middle ear
surgery. [44] Additionally, the combination of dexamethasone and midazolam may also reduce postoperative
nausea and vomiting. [45] [46]

Pre-surgical care
On initial presentation, prior to definitive surgical treatment, it may be appropriate to treat the aural discharge
with topical antibiotics. Agents containing quinolone (e.g., ciprofloxacin and ofloxacin) are effective, in adults[C
Evidence] and in children,[B Evidence] either alone or in combination with a topical corticosteroid. Aural
cleaning may also reduce symptomatic discharge. The ear canal may need to be cleaned of any debris or wax
prior to the use of topical ear drops.
Patients who have severe swelling of the ear canal may have difficulty applying ear drops. A wick should be
inserted in the ear canal to allow for drug delivery. In some patients, debridement of granulation tissue may be
necessary.

Canal wall up mastoidectomy (combined approach tympanoplasty)

This procedure allows removal of cholesteatoma but leaves the canal wall intact. The procedure involves
removal of the mastoid air cells lateral to the facial nerve and otic capsule, leaving the posterior and superior
parts of the external canal wall intact. [47] It is often preferred for children, as it avoids long-term complications
of a mastoid cavity. However, it necessitates a second-look procedure after 9 to 12 months to examine for

residual/recurrent disease. A meta-analysis found an increased incidence of postoperative cholesteatoma when

using an intact wall approach rather than canal wall down approach. [48]
Alternatively, a non-echo-planar diffusion-weighted MRI may be used in some patients. [35] [36] [37] There
continues to be debate regarding which type of MRI is best to examine for recurrent cholesteatoma. Some
authors advocate routine MRI scans (such as non-echo-planar, fast-spin echo, or diffusion weighted
sequences) for follow-up, but caution that a negative scan may not be completely accurate as residual or
recurrent disease may not yet be detectable. Non-echo-planar imaging was found to be more reliable compared
with echo-planar imaging in identifying residual/recurrent cholesteatoma in one systematic review. [49] Another
systematic review found that non-echo-planar diffusion-weighted MRI is highly sensitive and specific in
identifying middle-ear cholesteatoma. [37] Patients will require continued follow-up. [50] [51] [52]

Canal wall down mastoidectomy

This procedure aims to remove the disease by drilling from the attic wall posteriorly. The size of the resultant
cavity will depend on the extent of the cholesteatoma. A less invasive procedure resulting in a minimal cavity is
called an atticotomy or atticoantrotomy; a more invasive procedure resulting in a larger cavity is called a
modified radical mastoidectomy. A canal wall down procedure may allow direct examination of the cavity for
recurrence but if the attic has been reconstructed, a second-look procedure or a non-echo-planar diffusionweighted MRI may be necessary to examine the middle ear for recurrent disease.

Monitoring
Patients are followed up in the ENT clinic to monitor for symptoms of cholesteatoma recurrence. During
otoscopy, the cavity is examined for signs of a keratinous ear discharge. The mastoid cavity is often dry and
may initially require wax removal in an aural care clinic.
A canal wall up mastoidectomy necessitates a second-look procedure at 9 to 12 months to examine for
residual/recurrent disease. Alternatively, a non-echo-planar imaging diffusion-weighted MRI may be used in
some patients. [35] [36] There continues to be debate regarding which type of MRI best examines for recurrent
cholesteatoma. Some authors advocate routine MRI scans (such as non-echo-planar, fast-spin echo, or
diffusion-weighted sequences) for follow-up, but caution that a negative scan may not be completely accurate
as residual or recurrent disease may not yet be detectable. Non-echo-planar imaging was found to be more
reliable compared with echo-planar imaging in identifying residual/recurrent cholesteatoma in one systematic
review. [49] Patients will require continued follow-up. [50] [51] [52]
A canal wall down procedure may allow examination of the cavity for recurrence but if the attic has been
reconstructed, a second-look procedure or a non-echo-planar imaging diffusion-weighted MRI may be
necessary to examine the middle ear for recurrent disease.

Patient instructions
Patients should be made aware of the risk of cholesteatoma recurrence, which may occur several years later.
They should be instructed to seek specialist advice if ear discharge recurs or if there is a worsening hearing
loss. Patients with an open mastoid cavity may experience vertigo if water gets into the ears while swimming,

due to the temperature changes transmitted to the bony labyrinth. If this occurs, swim plugs can be used to
avoid these temperature changes.

Complications

Complicationhide all

Timefra Likelih
me
ood

recurrence of cholesteatoma

variable high

Cholesteatoma has a tendency to recur despite treatment, and may occur long after
the initial surgical excision.
The risk of recurrence after surgery in children has been shown to be higher if the
disease involves the posterior mesotympanum, and if the ossicular chain is
interrupted after excision, or both. A relative lack of experience of the surgeon
(and presumed incomplete excision of the disease) may also contribute to disease
recurrence. [55]
Recurrent disease may be detected on follow-up otoscopy or present as a
persistent aural discharge.
Treatment is by further surgery.
hearing loss

see our comprehensive coverage of Assessment of hearing loss

Patients with cholesteatoma commonly present with a conductive hearing loss.
There may be a mixed conductive and sensorineural hearing loss in patients with
cochlear damage or in those with a pre-existing hearing loss (e.g., congenital or
presbycusis).

variable high

Complicationhide all

Timefra Likelih
me
ood

labyrinthine fistula

variable high

One of the most common complications of cholesteatoma (occurs in 7% of

patients). [56] The horizontal semicircular canal is most often involved (90%).
Symptoms include vertigo, and patients may have a positive fistula test. Diagnosis
can be made preoperatively with a CT scan of the petrous temporal bone (60%
sensitivity). [57]
Treatment is closure of the fistula at the time of mastoidectomy, but surgical
manipulation may result in sensorineural hearing loss.
facial palsy

variable medium

see our comprehensive coverage of Bell's palsy

Cholesteatoma may cause facial paralysis via involvement of a dehiscent facial
nerve or via bone erosion of the facial canal. Facial paralysis is often slowly
progressive and has a worse prognosis. [58]
Diagnosis is clinical. A CT scan of the petrous temporal bone can be useful in
surgical planning as well as demonstrating other potential complications such as a
labyrinthine fistula. Treatment involves mastoidectomy for removal of the
cholesteatoma in contact with the facial nerve. [39] [40]
meningitis

see our comprehensive coverage of Overview of meningitis

Meningitis occurs in approximately 0.1% of patients. [59] [60]
Common symptoms are fever, persistent headache, nausea and vomiting, lethargy,
and irritability. Clinical signs include nuchal rigidity, new-onset seizures, ataxia,
and decreased mental status.

variable low

Complicationhide all

Timefra Likelih
me
ood

If confirmed on cranial CT or MRI brain and lumbar puncture, treatment includes

intravenous antibiotics and corticosteroids.
brain abscess

variable low

see our comprehensive coverage of Brain abscess

This often occurs in the temporal lobe of the brain or cerebellum.
Proteus species is the most common causative organism, but many abscesses are
sterile on culture.
Clinical signs include the initial encephalitic change with nausea and vomiting,
fever, headache, seizures, and change in mental state, followed by a quiescent
phase of lethargy, and a final stage of worsening of the acute symptoms. [58] [59]
Treatment includes intravenous antibiotics and corticosteroids with drainage of the
abscess and mastoidectomy.
sigmoid sinus thrombosis

variable low

see our comprehensive coverage of Cavernous sinus thrombosis

Involvement of the internal jugular vein may cause septic pulmonary emboli.
Classical presenting symptom is spiking temperatures (picket fence pattern) with
headache and lethargy. Diagnosis is made with a cranial contrast CT or MRI.
Treatment includes intravenous antibiotics and mastoidectomy. [10] [61]
mastoiditis

This is an acute infection of the temporal bone that occurs more commonly in
acute otitis media but may occur in chronic otitis media with cholesteatoma.
Patients present with post-auricular erythema, swelling, tenderness, fever, and an

variable low

Complicationhide all

Timefra Likelih
me
ood

inferiorly and laterally displaced pinna.

Treatment is intravenous antibiotics and mastoidectomy to remove revitalised
bone and cholesteatoma.
petrous apicitis

Variable low

Inflammation may spread to the petrous apex of temporal bone, resulting in

localised osteomyelitis and reactive meningitis.
Classic symptoms include deep retro-orbital pain, ear discharge, and sixth nerve
palsy (Gradenigo's syndrome). Diagnosis is made via a CT scan of the petrous
temporal bone.
Treatment includes intravenous antibiotics, corticosteroids, and a
tympanomastoidectomy to remove the cholesteatoma and granulations. [58]
epidural abscess

variable low

see our comprehensive coverage of Epidural abscess

Clinical signs may be subtle and include increased otalgia or headache. Diagnosis
is made via a CT scan with contrast or an MRI scan.
Treatment is surgical and the abscess may be drained via the mastoidectomy if
found intra-operatively. [62]

Prognosis
Some cavities persistently discharge despite topical medical therapy. This may be treated with revision mastoid
surgery or, if the cavity discharges despite multiple procedures, the cavity may be obliterated. [53] Topical
mitomycin has been found to be more effective than chemical cautery (with topical acetic acid) in achieving a
dry cavity. [54]

Cholesteatoma has a tendency to recur despite treatment, and may recur long after the initial surgical excision.
The risk of recurrence after surgery in children has been shown to be higher if the disease involves the
posterior mesotympanum, and if the ossicular chain is interrupted after excision, or both. A relative lack of
experience of the surgeon (and presumed incomplete excision of the disease) may also contribute to disease
recurrence. [55]