Cholesteatoma of

GARY L. THOMAS, M.D.,

the

Ear

Sacramento

* Cholesteatoma is a hazardous condition because of the erosion and

tissue destruction, which result in deafness, and the complications which
threaten life. Early diagnosis and treatment provide the best opportunity for eradication of the disease and preservation of hearing.
The patient usually complains of intermittent or persistent ear drainage and of diminished hearing acuity. Close examination of the tympanic membrane reveals a perforation, which at times may be quite
small, with epithelial extension into the middle ear space. In most
instances surgical intervention is necessary for eradication of the disease.
CHOLESTEATOMA IS ONE of the most dangerous
diseases involving the ear. It is dangerous because
of its often insidious nature, often present a number of years before a serious complication occurs,
and often goes unrecognized. The complications
of cholesteatoma are of considerable magnitude,
among them cerebral abscess, meningitis, lateral
sinus thrombosis, facial paralysis and deafness.

Pathology
The disease is a result of the presence of squamous epithelium in the middle ear space or the
mastoid process. There are at least two ways in
which squamous epithelium gains access to these
locations (where normally it is not present). One
is by invagination of epithelium, usually in Shrapnell's area (pars flaccida), secondary to negative
pressure in the middle ear space2'4'5,6 (Figure 1 ).
The other is by migration of epithelium through a
tympanic membrane perforation.
Investigators also feel that desquamating epithelium may arise in the tympanum or mastoid as
a result of epithelial cell arrest during embryological development",8 or as a result of metaplasia of
the normal pavement epithelium of the epitympanic mucosa secondary to chronic infection.5'6
Reprint requests to: 5301 F Street, Sacramento 95819.

Cholesteatoma of the ear, then, is a squamous

epithelial-lined area (cavity) in the middle ear
space or the mastoid or both. As the epithelium
desquamates, there is no place for this material to
go, and a cystic mass filled with debris is formed.
The debris frequently contains crystals of cholesterol, hence the name cholesteatoma.
The presence of desquamated material in the
moist crevices of the middle ear and mastoid cells
provides an ideal medium for bacterial growth, so
that the ear is usually chronically infected, although at certain stages it may be perfectly dry.
Pressure necrosis and chronic osteitis result in progressive destruction of the areas involved. The destruction is slow and insidious; often there is a
history of many years' duration. This is one of
the dangerous aspects of the problem.
Both physicians and patients fall into the trap
of reasoning that, since this has been a problem of
many years and has not produced any serious
complications, it probably never will. This is a
false impression. Once the epithelium dissects into
a cell or crevice and the infection becomes walled
off by edematous mucosa or granulation tissue or
debris, a potentially dangerous situation exists.
The drainage is going to find a way out and if
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Figure 1.-Progressive stages in development of cholesteatoma secondary to invagination of pars flaccida.

emptied intracranially the result will be intracranial abscess, meningitis or lateral sinus thrombosis. Any of these complications is serious and a
fatal outcome is common.
Facial paralysis may be the first sign to cause
real alarm on the part of the patient or his physician. This complication is a result of osteitis or
pressure necrosis involving the facial nerve as it
courses through the temporal bone. Inflammatory
swelling of the nerve occurs, and as the nerve is
imbedded in a bony canal the swelling causes
ischemia, with nerve damage and loss of function
resulting.

Report of a Case
The patient, a 37-year-old man, was first seen
in the office five years before the onset of the
present illness. At that time he had inadvertently
gotten water in his right ear while skiing and sub206

MARCH 1968 * 108 * 3

sequently had had purulent drainage from it. The

ear dried after a period of four months of intensive
therapy. At that time, it was noted that there was
a decided retraction in the epitympanic space, and
the patient was advised of the necessity of continued close observation. Because there was no
further drainage or pain, the patient did not return
until, five years later, he was seen because of pain
and drainage from the right ear of one week's
duration.
Examination revealed an epitympanic perforation with epithelium extending posterosuperiorly
(cholesteatoma). X-ray films of the mastoid area
demonstrated an enlargement of the attic posteriorly, with poorly defined margins. Audiometric
examination revealed a 30 decibel conductive
hearing impairment. The patient was advised that
surgical intervention would be necessary. Because
of a previously scheduled vacation, he elected to

defer operation for several weeks, during which

time he was to return at weekly intervals. Drug
therapy was initiated and the drainage and pain
began subsiding. However, on the second visit, a
definite lid lag on the right side was noted. The
patient had been unaware of this and had never
had facial nerve weakness or neurologic symptoms
at any time in the past. During the next two days
a pronounced peripheral seventh nerve paresis
developed, whereupon mastoidectomy was undertaken. At operation it was found that damage to
the ossicular chain had destroyed the incus and a
portion of the head of the malleus and had resulted in exposure of the horizontal portion of the
facial nerve. Cholesteatoma and granulation tissue
were completely excised, and with this eradication
of the source of damage to the facial nerve it was
felt that further decompression was not indicated.
On the second postoperative day, the patient began
to note an improvement in his facial function and
by the time the dressing was removed a week after
operation, recovery was almost complete. By the
second week the facial nerve function was normal.
At the end of six weeks the ear was dry and fully
healed and hearing was at a satisfactory 15 decibel
level.
Labyrinthitis with dizziness is not uncommon in
cholesteatoma of the ear, especially during an
acute exacerbation of chronic infection. Fortunately, this is usually due to irritative or serous
labyrinthitis rather than to suppurative labyrinthitis, and usually the dizziness is not severe. The
labyrinth becomes irritated due to its exposure
as a result of cholesteatomatous destruction. There
results a fistula of the semicircular canal and secondary labyrinthitis. Thrombophlebitic extension
by way of the perilabyrinthine vascular system
may also result in labyrinthitis.1 7 If the irritative
or serous labyrinthitis progresses to suppurative
labyrinthitis, permanent total destruction of the
sensory elements results, with total deafness and
potential extension in the form of meningitis.
The extent and direction of destruction are
unpredictable. In an occasional case the cholesteatoma brings about spontaneous radical "mastoidectomy," erosion having occurred into the auditory
canal from the mastoid antrum. In others there
may be only a tiny perforation in the epitympanic
space and an otherwise normal appearing ear, yet
the patient have one of the serious complications
previously mentioned.

History and Examination

Cholesteatoma commonly is manifested by intermittent or persistent ear drainage and conductive hearing impairment. The hearing impairment
will vary with the extent of the disease and, in
early cholesteatoma, may be absent. As the chronic
disease progresses, chronic osteitis and pressure
necrosis will eventually disrupt the ossicular chain
and finally progress to complete destruction with
a minimum of 60-decibel hearing loss.
Pain may be a complaint if the infection becomes walled off by edematous mucosa or granulation tissue or if a secondary external otitis results
from the purulent ear drainage.
Dizziness may occur intermittently. It is most
likely to appear with cleaning of the ear during
aspiration and, particularly, when a fistula into the
semicircular canal has resulted from the destruction. Since applying positive and negative pressure
with a pneumatic (Siegel's) otoscope will often
produce nystagmus, the procedure is used as a
diagnostic test. It is useful to the surgeon to know
of the presence of a fistula preoperatively.
Detailed examination of the tympanic membrane is the critical point in diagnosis of cholesteatoma. Just looking at the light reflex is not adequate. The light reflex is often present in a cholesteatoma that is not infected and, in fact, the tympanic membrane may be quite normal in appearance. Shrapnell's membrane (pars flaccida) often
holds the clue to danger. A severe retraction or
perforation here almost always is accompanied by
cholesteatoma to one degree or another. If there
is only moderate retraction, danger of complications is minimal. One must see the innermost
limits of the retracted sac, however, to be certain
the cholesteatoma does not extend far beyond the
confines of the area visualized. If the limits can be
visualized, the lesion is of the type that can be
safely watched without surgical intervention.
The second area that must be closely inspected
is the posterosuperior quadrant of the tympanic
membrane. Here a marginal perforation is a danger
sign, for epithelium frequently will have migrated
from the canal or tympanic membrane posterosuperiorly into the tympanum with progression
into the mastoid additus and antrum.
Cholesteatoma can come about from central
perforation but this is uncommon. Usually in such
a case an atrophic retracted tympanic membrane
has dropped into contact with the promontory of
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the middle ear, allowing migration of the squamous epithelium into the middle ear space.
Cholesteatomas occasionally are found in patients with tympanic membranes intact. In these
situations -far more frequent in children than
adults - they are felt to be due to embryonic cell
rests or to metaplasia of the epitympanic mucosa.
The disease originally makes its appearance as resistant serous otitis media with conductive hearing
impairment. Later, suppuration and destruction
occur and findings are typical of cholesteatoma.
Unfortunately, information obtained from mastoid x-ray studies is frequently misleading unless
extensive bone destruction has occurred. Tomograms will provide more detailed information, but
even these studies may be misleading in early
cholesteatoma, for at that stage there may be very
little or no radiologically demonstrable bone
destruction.
Examination of the ear under high magnification, therefore, is by far the most significant factor
in the evaluation of cholesteatoma. This requires
fastidious cleansing of the entire tympanic membrane and great care not to overlook even a small
perforation which might be covered by a dried
mucous crust especially in the region of the pars
flaccida or in an atrophic retracted posterosuperior
portion of the tympanic membrane.

Treatment
In most cases surgical intervention will be necessary to arrest the progress of the disease and preserve the patient's hearing. Sometimes, however,
mechanical removal of the epithelial debris and

208

MARCH 1968 * 108 * 3

having the patient avoid getting water in the ear

will suffice, provided the extent of the epithelial
extension into the epitympanic space or posteriorly into the tympanum can be determined with
absolute certainty.
Not often is there need to operate immediately,
but occasionally surgical intervention must be carried out as soon as a diagnosis is reached to prevent calamitous damage. (In the case herein reported, the patient could have had irreversible
facial deformity had operation been delayed.)
The surgical approach is designed to eradicate
the disease, thereby providing the patient with a
safe, dry ear and preserving the hearing that is
present. Reconstructive procedures may, in fact,
improve hearing significantly, the degree depending upon the ossicular structures remaining. The
earlier the diagnosis, the greater the opportunity
for successful therapy.
REFERENCES
1. Bagby, R. A., and Farrior, J. B.: The treatment of
the chronic middle ear in the pathogenesis of cholesteatoma, Southern Med. J., 46:712, July 1953.
2. Day, K. M.: Primary pseudocholesteatoma of the
ear, Arch. Otolaryng., 34:1144, Dec. 1941.
3. McKenzie, D.: Pathogeny of aural cholesteatoma,
J. Laryng., 46:163, Feb. 1931.
4. Nager, F.: The cholesteatoma of the middle ear,
Ann. Otol., 34:1249, Dec. 1925.
5. Ruedi, L.: Pathogenesis and treatment of cholesteatoma in chronic suppuration of the temporal bone, Ann.
Otol., 66:283, June 1957.
6. Saxen, A., and Ojala, L.: Pathogenesis of middle ear
cholesteatoma arising from Shrapnell's membrane, Acta
Oto-laryng., Suppl. 100, p. 33, Apr. 1952.
7. Shambaugh, G. E.: Surgery of the Ear, W. B. Saunders Co., Philadelphia, 1959, p. 162.
8. Teed, R. W.: Cholesteatoma verum tympani, Arch.
Otolaryng., 24:455, Oct. 1936.