Cholesteatoma of the Ear

GARY L. THOMAS, M.D., Sacramento

* Cholesteatoma is a hazardous condition because of the erosion andtissue destruction, which result in deafness, and the complications whichthreaten life. Early diagnosis and treatment provide the best oppor-tunity for eradication of the disease and preservation of hearing.The patient usually complains of intermittent or persistent ear drain-

age and of diminished hearing acuity. Close examination of the tym-panic membrane reveals a perforation, which at times may be quitesmall, with epithelial extension into the middle ear space. In mostinstances surgical intervention is necessary for eradication of the disease.

CHOLESTEATOMA IS ONE of the most dangerousdiseases involving the ear. It is dangerous becauseof its often insidious nature, often present a num-ber of years before a serious complication occurs,and often goes unrecognized. The complicationsof cholesteatoma are of considerable magnitude,among them cerebral abscess, meningitis, lateralsinus thrombosis, facial paralysis and deafness.

PathologyThe disease is a result of the presence of squa-

mous epithelium in the middle ear space or themastoid process. There are at least two ways inwhich squamous epithelium gains access to theselocations (where normally it is not present). Oneis by invagination of epithelium, usually in Shrap-nell's area (pars flaccida), secondary to negativepressure in the middle ear space2'4'5,6 (Figure 1 ).The other is by migration of epithelium through atympanic membrane perforation.

Investigators also feel that desquamating epi-thelium may arise in the tympanum or mastoid asa result of epithelial cell arrest during embryologi-cal development",8 or as a result of metaplasia ofthe normal pavement epithelium of the epitym-panic mucosa secondary to chronic infection.5'6

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Cholesteatoma of the ear, then, is a squamousepithelial-lined area (cavity) in the middle earspace or the mastoid or both. As the epitheliumdesquamates, there is no place for this material togo, and a cystic mass filled with debris is formed.The debris frequently contains crystals of choles-terol, hence the name cholesteatoma.The presence of desquamated material in the

moist crevices of the middle ear and mastoid cellsprovides an ideal medium for bacterial growth, sothat the ear is usually chronically infected, al-though at certain stages it may be perfectly dry.Pressure necrosis and chronic osteitis result in pro-gressive destruction of the areas involved. The de-struction is slow and insidious; often there is ahistory of many years' duration. This is one ofthe dangerous aspects of the problem.

Both physicians and patients fall into the trapof reasoning that, since this has been a problem ofmany years and has not produced any seriouscomplications, it probably never will. This is afalse impression. Once the epithelium dissects intoa cell or crevice and the infection becomes walledoff by edematous mucosa or granulation tissue ordebris, 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 intra-cranial abscess, meningitis or lateral sinus throm-bosis. Any of these complications is serious and afatal outcome is common.

Facial paralysis may be the first sign to causereal alarm on the part of the patient or his physi-cian. This complication is a result of osteitis orpressure necrosis involving the facial nerve as itcourses through the temporal bone. Inflammatoryswelling of the nerve occurs, and as the nerve isimbedded in a bony canal the swelling causesischemia, with nerve damage and loss of functionresulting.

Report of a CaseThe patient, a 37-year-old man, was first seen

in the office five years before the onset of thepresent illness. At that time he had inadvertentlygotten water in his right ear while skiing and sub-

sequently had had purulent drainage from it. Theear dried after a period of four months of intensivetherapy. At that time, it was noted that there wasa decided retraction in the epitympanic space, andthe patient was advised of the necessity of con-tinued close observation. Because there was nofurther drainage or pain, the patient did not returnuntil, five years later, he was seen because of painand drainage from the right ear of one week'sduration.

Examination revealed an epitympanic perfora-tion with epithelium extending posterosuperiorly(cholesteatoma). X-ray films of the mastoid areademonstrated an enlargement of the attic posteri-orly, with poorly defined margins. Audiometricexamination revealed a 30 decibel conductivehearing impairment. The patient was advised thatsurgical intervention would be necessary. Becauseof a previously scheduled vacation, he elected to

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defer operation for several weeks, during whichtime he was to return at weekly intervals. Drugtherapy was initiated and the drainage and painbegan subsiding. However, on the second visit, adefinite lid lag on the right side was noted. Thepatient had been unaware of this and had neverhad facial nerve weakness or neurologic symptomsat any time in the past. During the next two daysa pronounced peripheral seventh nerve paresisdeveloped, whereupon mastoidectomy was under-taken. At operation it was found that damage tothe ossicular chain had destroyed the incus and aportion of the head of the malleus and had re-sulted in exposure of the horizontal portion of thefacial nerve. Cholesteatoma and granulation tissuewere completely excised, and with this eradicationof the source of damage to the facial nerve it wasfelt that further decompression was not indicated.On the second postoperative day, the patient beganto note an improvement in his facial function andby the time the dressing was removed a week afteroperation, recovery was almost complete. By thesecond week the facial nerve function was normal.At the end of six weeks the ear was dry and fullyhealed and hearing was at a satisfactory 15 decibellevel.

Labyrinthitis with dizziness is not uncommon incholesteatoma of the ear, especially during anacute exacerbation of chronic infection. Fortu-nately, this is usually due to irritative or serouslabyrinthitis rather than to suppurative labyrinth-itis, and usually the dizziness is not severe. Thelabyrinth becomes irritated due to its exposureas a result of cholesteatomatous destruction. Thereresults a fistula of the semicircular canal and sec-ondary labyrinthitis. Thrombophlebitic extensionby way of the perilabyrinthine vascular systemmay also result in labyrinthitis.1 7 If the irritativeor serous labyrinthitis progresses to suppurativelabyrinthitis, permanent total destruction of thesensory elements results, with total deafness andpotential extension in the form of meningitis.

The extent and direction of destruction areunpredictable. In an occasional case the choleste-atoma brings about spontaneous radical "mastoid-ectomy," erosion having occurred into the auditorycanal from the mastoid antrum. In others theremay be only a tiny perforation in the epitympanicspace and an otherwise normal appearing ear, yetthe patient have one of the serious complicationspreviously mentioned.

History and ExaminationCholesteatoma commonly is manifested by in-

termittent or persistent ear drainage and conduc-tive hearing impairment. The hearing impairmentwill vary with the extent of the disease and, inearly cholesteatoma, may be absent. As the chronicdisease progresses, chronic osteitis and pressurenecrosis will eventually disrupt the ossicular chainand finally progress to complete destruction witha minimum of 60-decibel hearing loss.

Pain may be a complaint if the infection be-comes walled off by edematous mucosa or granu-lation tissue or if a secondary external otitis resultsfrom the purulent ear drainage.

Dizziness may occur intermittently. It is mostlikely to appear with cleaning of the ear duringaspiration and, particularly, when a fistula into thesemicircular canal has resulted from the destruc-tion. Since applying positive and negative pressurewith a pneumatic (Siegel's) otoscope will oftenproduce nystagmus, the procedure is used as adiagnostic test. It is useful to the surgeon to knowof the presence of a fistula preoperatively.

Detailed examination of the tympanic mem-brane is the critical point in diagnosis of choleste-atoma. Just looking at the light reflex is not ade-quate. The light reflex is often present in a choles-teatoma that is not infected and, in fact, the tym-panic membrane may be quite normal in appear-ance. Shrapnell's membrane (pars flaccida) oftenholds the clue to danger. A severe retraction orperforation here almost always is accompanied bycholesteatoma to one degree or another. If thereis only moderate retraction, danger of complica-tions is minimal. One must see the innermostlimits of the retracted sac, however, to be certainthe cholesteatoma does not extend far beyond theconfines of the area visualized. If the limits can bevisualized, the lesion is of the type that can besafely watched without surgical intervention.The second area that must be closely inspected

is the posterosuperior quadrant of the tympanicmembrane. Here a marginal perforation is a dangersign, for epithelium frequently will have migratedfrom the canal or tympanic membrane postero-superiorly into the tympanum with progressioninto the mastoid additus and antrum.

Cholesteatoma can come about from centralperforation but this is uncommon. Usually in sucha case an atrophic retracted tympanic membranehas dropped into contact with the promontory of

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the middle ear, allowing migration of the squa-mous epithelium into the middle ear space.

Cholesteatomas occasionally are found in pa-tients with tympanic membranes intact. In thesesituations -far more frequent in children thanadults- they are felt to be due to embryonic cellrests or to metaplasia of the epitympanic mucosa.The disease originally makes its appearance as re-sistant serous otitis media with conductive hearingimpairment. Later, suppuration and destructionoccur and findings are typical of cholesteatoma.

Unfortunately, information obtained from mas-toid x-ray studies is frequently misleading unlessextensive bone destruction has occurred. Tomo-grams will provide more detailed information, buteven these studies may be misleading in earlycholesteatoma, for at that stage there may be verylittle or no radiologically demonstrable bonedestruction.

Examination of the ear under high magnifica-tion, therefore, is by far the most significant factorin the evaluation of cholesteatoma. This requiresfastidious cleansing of the entire tympanic mem-brane and great care not to overlook even a smallperforation which might be covered by a driedmucous crust especially in the region of the parsflaccida or in an atrophic retracted posterosuperiorportion of the tympanic membrane.

TreatmentIn most cases surgical intervention will be nec-

essary to arrest the progress of the disease and pre-serve the patient's hearing. Sometimes, however,mechanical removal of the epithelial debris and

having the patient avoid getting water in the earwill suffice, provided the extent of the epithelialextension into the epitympanic space or posterior-ly into the tympanum can be determined withabsolute certainty.

Not often is there need to operate immediately,but occasionally surgical intervention must be car-ried out as soon as a diagnosis is reached to pre-vent calamitous damage. (In the case herein re-ported, the patient could have had irreversiblefacial deformity had operation been delayed.)The surgical approach is designed to eradicate

the disease, thereby providing the patient with asafe, dry ear and preserving the hearing that ispresent. Reconstructive procedures may, in fact,improve hearing significantly, the degree depend-ing upon the ossicular structures remaining. Theearlier the diagnosis, the greater the opportunityfor successful therapy.

REFERENCES1. Bagby, R. A., and Farrior, J. B.: The treatment of

the chronic middle ear in the pathogenesis of cholestea-toma, Southern Med. J., 46:712, July 1953.

2. Day, K. M.: Primary pseudocholesteatoma of theear, 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 cholestea-toma in chronic suppuration of the temporal bone, Ann.Otol., 66:283, June 1957.

6. Saxen, A., and Ojala, L.: Pathogenesis of middle earcholesteatoma arising from Shrapnell's membrane, ActaOto-laryng., Suppl. 100, p. 33, Apr. 1952.

7. Shambaugh, G. E.: Surgery of the Ear, W. B. Saun-ders Co., Philadelphia, 1959, p. 162.

8. Teed, R. W.: Cholesteatoma verum tympani, Arch.Otolaryng., 24:455, Oct. 1936.

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