Cholesteatoma Treatment & Management

Medical therapy is not a viable treatment for cholesteatomas. Patients who refuse surgery or whose medical condition makes a general anesthetic too hazardous should have their ears cleaned regularly. Regular cleaning can help control infection and may slow growth, but it does not stop further expansion and does not eliminate risk. The mainstay of microbial therapy should be topical, but systemic therapy is occasionally a helpful adjunct.

Surgical therapy consists of removing the cholesteatoma. In certain circumstances, the surgeon can make the decision to use a canal-wall-up (closed) or canal-wall-down (open) technique preoperatively. If the patient has had several episodes of recurrence of the cholesteatoma and wishes to avoid future operations, the canal-wall-down technique is most applicable. For those who are unwilling or unable to return for a second-look procedure, a canal-wall-down operation is safer.^{[15, 16, 17]}

A large meatoplasty is simply unacceptable under any circumstances for some patients. Such patients can be treated with a closed (canal-wall-up) technique, provided they understand that disease recurrence is more likely and they may require multiple, serial surgical procedures.

A variety of factors help determine which operation is best. Sometimes, this decision is not possible until the operation has begun and a clear understanding of the extent of disease has been obtained. Canal-wall-down operations have the highest probability of permanently ridding the patient of the cholesteatoma. Canal-wall-up procedures have the advantage of maintaining a normal appearance, but they have a higher risk of persistent or recurrent cholesteatomas. The risk of recurrence is sufficiently high so that most surgeons advise an obligatory second-look tympanomastoidectomy 6 months to 1 year following the initial operation. At the time of the second look, small amounts of residual disease can frequently be removed prior to the development of either complications or massive recurrence.

No operation can be successful unless the goals of the procedure are kept clearly in mind. These goals include the following:

• To make the ear safe by eliminating the cholesteatoma and chronic infection
• To make the ear problem-free for all the usual activities of daily living, including swimming
• To conserve residual hearing
• To improve hearing when possible
• To provide an acceptable cosmetic appearance

A careful discussion of the advantages and disadvantages of canal-wall-up (closed) tympanomastoidectomies and canal-wall-down (open) procedures is useful. If a canal-wall-down procedure is considered pre-operatively, showing the patient a picture of a well-crafted meatoplasty, as shown below, can help the patient form a clear understanding of the expected postoperative appearance.

Characteristics of the canal-wall-up procedure include the following:

• Normal appearance
• Hearing aids easily fit
• No routine cleaning required
• Relatively high rate of recurrent or persistent cholesteatomas
• High tolerance for water exposure
• Usually a staged procedure

Characteristics of the canal-wall-down procedure include the following:

• Enlarged meatus
• Hearing aids difficult to fit
• Annual or semiannual canal cleaning needed
• Low rate of persistent or recurrent cholesteatomas
• Occasional problem with water exposure: Dizziness may develop after exposure to water or cold air.
• Usually a single procedure

Often, the best plan is to reserve the decision about how to manage the canal wall until the operation has begun and a better understanding of the extent of disease has emerged. Some intraoperative findings that favor a canal-wall-down, or open, technique, including the following:^[15]

• Involvement of the sinus tympani
• Involvement of the medial end of the canal wall, with the cholesteatoma wedged laterally between the heads of the ossicles in the epitympanum and medial canal wall
• Ostitis or irremovable cholesteatomas in the protubal area or hypotympanum: This situation sometimes mandates a true radical mastoidectomy.
• Large defects of the canal wall: Small defects in canal wall are readily repaired. Larger defects are harder to repair, and, frequently, the best plan is simply to convert the operation to an open-cavity technique.
• The presence of labyrinthine fistula: If the cholesteatoma matrix is left over the fistula so as not to expose it, remove the posterior wall of the external auditory canal. To do otherwise would be to trap epithelium within the mastoid cavity, which would simply result in cholesteatoma recurrence. If the cholesteatoma matrix is removed from the fistula, some surgeons are comfortable with leaving the canal wall up.

Most surgical procedures for cholesteatomas in the United States are now performed through an incision behind the ear combined with an incision in the external auditory canal, but the procedure can be performed through an extended incision starting in the ear canal. Completely remove the air cells of the mastoid. Elevate the tympanic membrane in the middle ear space, and carefully evaluate the mastoid. Remove the cholesteatoma. If ossicles are involved, they must be removed to avoid recurrence. Reconstruct the ossicular chain either at the end of the primary procedure or as part of a secondary operation. The tympanic membrane is usually reconstructed. If a canal-wall-up technique is used, replace missing bone with a cartilage graft. If a canal-wall-down procedure is selected, create a large meatoplasty to allow adequate air circulation into the cavity that arises as a consequence of removing the posterior canal wall.

Essential features of a canal-wall-up procedure include the following:^[16]

• Removal of all infected air cells
• A functioning eustachian tube
• A well-maintained middle ear space
• Adequate communication between the mastoid and the middle ear space through the aditus ad antrum or a generous facial recess
• Elimination of any bony attic defect with a cartilage or bone graft

Essential features of a canal-wall-down procedure include the following:

• Removal of all air cells, including those within the retrofacial, retrolabyrinthine, and subarcuate air cell tracts
• Removal of the lateral and the posterior walls of the epitympanum so that the tegmen mastoideum and tegmen tympani become a smooth, featureless plane
• Amputation of the mastoid tip is usually advisable.
• Saucerization of the lateral margins of the cavity
• Lowering of the posterior bony external auditory wall to the level of the facial nerve
• Exteriorization of the anterior epitympanic recess by removing the cog
• Enlargement of the meatus (at least twice as large as normal) by removing conchal cartilage
• Lowering of the medial end of the external auditory canal toward the floor of the hypotympanum

Some surgeons prefer a subcortical technique that starts by removing bone medially through the ear canal. The attic is progressively enlarged, and the posterior canal wall is removed from "inside-out." Bone removal is limited to that necessary to remove all of the cholesteatoma. Such a "subcortical" or "inside-out" mastoidectomy combines some of the advantages of canal-wall-up and canal-wall-down techniques but commits the surgeon to a canal-wall-down operation if the cholesteatoma has extensive mastoid involvement.^{[18, 19]}

Regular office visits are necessary following surgery. Some surgeons prefer to see the patient on the following day and remove the dressing themselves. Others prefer to wait 7-10 days; these surgeons allow the patient to remove the dressing at home after 12-48 hours. If nonabsorbable sutures or staples are used, they must be removed at 7-10 days. If suture or staple removal is unnecessary, some surgeons delay the first postoperative visit for as long as 2-3 weeks. Open cavities heal slowly, and 12-24 weeks are often required for full epithelialization.

Postoperative treatment regimens for canal-wall-down procedures vary considerably. For canal-wall-down procedures, the wick in the external auditory canal (EAC) is removed at the first postoperative visit, 7-10 days after surgery. Antibiotic otic drops are prescribed twice daily for 10 days.

For canal-wall-down cavities, the author prefers to fill the cavity with antibiotic ointment at the first postoperative visit (7-10 d after surgery). The first cavity cleaning occurs at the postoperative visit 2-3 weeks after the procedure. Often, the patient is asked to fill the cavity with peroxide daily, starting 1-2 weeks postoperatively, or to irrigate the cavity with an alcohol-vinegar solution. This limits the amount of superficial infection.

Frequently, granulation tissue forms in the open cavity as it heals. Granulation tissue may be helpful because it can contract the size of the cavity, resulting in a much smaller postoperative cavity than would be the case if the initial defect epithelialized. On the other hand, granulation tissue can obliterate medial portions of the cavity, including the newly grafted drum, and compromise the hearing outcome. Exuberant granulation tissues can significantly delay epithelialization.

Steroid creams, steroid-containing drops, and regular applications of gentian violet can be used to help control the development and extent of granulation tissue. Silver nitrate cautery effectively eliminates or reduces granulation tissue, but care must be taken. Silver nitrate produces a chemical burn, the depth of which is difficult to control. So extra caution must be used around delicate or sensitive structures such as the horizontal semicircular and facial nerve in order to avoid injury from the chemical burn produced by the silver nitrate. Open cavities require variable amounts of cleaning over the first 4-8 postoperative weeks. Cleaning can be accomplished regularly in an office setting every 2-3 weeks, depending on how quickly the cavity epithelializes.

Often, a superficial infection develops that can be effectively controlled using topical antibiotic drops. Occasionally, the causative organisms are predominantly gram-negative, with Pseudomonas species as the single most common pathogens, but gram-positive organisms, including Streptococcus and Staphylococcus species, account for 20-30% of recovered organisms.

Both aminoglycoside and fluoroquinolone topical preparations provide appropriate antibacterial spectra for these organisms. The complete absence of potential ototoxicity among the fluoroquinolones makes them appealing. If a nonsteroid otic preparation is desired, either ciprofloxacin (Ciloxan) or ofloxacin (Floxin Otic) are excellent choices.

If granulation tissue is present, steroid-containing drops may be preferred. In such cases, ciprofloxacin/hydrocortisone (Ciprodex) is an excellent choice. Antibacterial creams containing fluoroquinolones can also be used. Occasionally, if granulation tissue appears to be overwhelming, steroid creams alone are useful. If a superficial fungal infection or candidiasis develops, nystatin-triamcinolone (Mycolog) or clotrimazole (Mycelex) cream is usually sufficient to eradicate it.

Long-term care

An open cavity should be problem-free once it is fully healed, provided that the patient returns every 6-12 months for microscopic cleaning in the office. At the cleanings, variable amounts of desquamated epithelium and/or cerumen fill the depths of the cavity. Occasional areas of crusting may be present, behind which superficial areas of infection have developed. Usually, removing the crust and exposing these areas to circulating air is sufficient to resolve the infection. Home care may consist of regular installations of hydrogen peroxide or a one-to-one mixture of alcohol and white vinegar. Such installations may be administered daily, every other day, or weekly. The alcohol-vinegar combination is desiccating.

Because the skin of the epithelialized cavity is normal squamous epithelium, filling the cavity once or twice a month with an emollient is sometimes helpful. Baby oil or any other mineral oil product is effective. This may help soften cerumen and reduce itching. Most patients swim comfortably with an open cavity. Some develop vertigo when cold water enters the ear and stimulates the exposed horizontal semicircular canal. In such circumstances, the use of an earplug can effectively the block circulation of water over the exposed canal and eliminate vertigo. Children should be watched carefully the first several times they swim to make sure they do not develop dysequilibrium and vertigo while in the water.

Each patient must be monitored for many years. Recurrence can occur long after the initial surgical excision. Follow-up care should include semiannual or annual evaluations, even in patients who are asymptomatic.

Patients who have had canal-wall-down procedures may require follow-up evaluations as often as every 3 months for canal cleaning. On the other hand, some patients only need to be seen once a year. The frequency with which a patient with a canal-wall-down mastoidectomy cavity needs to be seen in order to keep the ear free of desquamated epithelium and cerumen becomes readily apparent. Patients who have had canal-wall-up operations generally need a second-look procedure 6-9 months after the original operation. Once the second-look operation is healed, regular follow-up care at intervals of 6 months to 1 year are necessary in order to identify persistence or recurrence of the cholesteatoma.

A variety of complications are possible from cholesteatomas and cholesteatoma surgery. The most feared complication is facial nerve paralysis. The incidence of permanent facial nerve injury following cholesteatoma surgery is not entirely certain, but it appears to be 1% or less. The risk probably is much less than 1% in the hands of experienced otologists who perform the operation regularly. Controversy exists (see Future and Controversies) about whether or not facial nerve monitoring is helpful in reducing the risk of postoperative facial nerve injury.

The chance of total neurosensory hearing loss associated with cholesteatoma removal is 1-2%. Cholesteatomas that produce labyrinthine fistulas or those that lie directly over the footplate are more likely to produce permanent neurosensory loss.

Many patients have an alteration in taste on the anterior ipsilateral tongue for weeks after an otologic procedure. However, this is rarely a complaint beyond a few months after surgery.

Long-term balance disturbance may occur as a consequence of labyrinthine or middle ear injury, but it is uncommon, occurring in fewer than 1% of cases.

In approximately 10-15% of cases, the graft fails resulting in a permanent tympanic membrane perforation. Such perforations can frequently be eliminated by performing a revision operation. Depending on the procedure, 5-30% of operations are unsuccessful, and cholesteatoma persistence or recurrence becomes evident at some point in the postoperative period. Persistence may manifest as early as 5-6 months, or it may be delayed for many years. Consequently, long-term follow-up is important.

Perichondritis or chondritis occurs in fewer than 1% of patients. Care should be taken at the time of surgery to minimize the amount of exposed cartilage because exposure and devascularization render it more susceptible to infection. The symptoms of perichondritis are (1) increasing pain, erythema, and edema of the skin over the involved conchal or auricular cartilage and (2) occasional fluctuance. The process is indolent and slow to develop.

Because the blood supply to cartilage is relatively sparse, perichondritis and chondritis are slow to respond to antibiotic therapy. Fluoroquinolones offer excellent coverage for these infections, achieving similar blood levels equal to those achieved with oral or IV administration. Antibiotic therapy must be continued for a minimum of 3-4 weeks; often, 6 weeks of oral antibiotic therapy is required. If fragments of devitalized cartilage become sequestered, they require operative removal. If no clinical response to antibiotic therapy occurs within 2-3 weeks or if the infection worsens during that period, consider operative drainage should be considered. Drains should be left in place for several weeks. A semicircular incision that parallels the margin of the antihelix minimizes the cosmetic impact.

Postoperative stenosis is usually a consequence of exuberant granulation tissue. It is more likely to occur when a postoperative infection is present. Principles of prevention include control of the infection and suppression of the granulation tissue. Surprisingly, the gentle pressure of the expanded packing often slowly increases the size of the canal. By keeping the packing in place for several months, a 1- to 2-mm nonfunctional medial canal can be expanded to 4-5 mm, which is large enough to conduct sound efficiently and prevent additional conductive hearing loss. If postoperative stenosis completely obliterates the cavity, revision of the cavity in an attempt to restore patency and improve hearing may be necessary. Split-thickness skin grafts limit the recurrence of postoperative stenosis.

The most dreaded complication of tympanomastoid surgery is injury to the facial nerve. Facial nerve injuries are sometimes recognized during the course of the procedure, but, other times, they are noted only after the patient has awakened in the recovery room or day surgery unit. Because even the suggestion of intraoperative facial nerve injury may produce in the surgeon in pronounced sympathetic discharge with high levels of circulating catecholamines, technical skills may be degraded and judgment impaired. Therefore, the surgeon should stop operating to assess the situation, and, if at all possible, a consultant opinion should be obtained immediately. The first step in managing a possible or recognized facial nerve injury is to decompress the nerve around the area thought to be injured. Remove bone a few millimeters proximal and distal to the damaged segment so that the injury can be clearly visualized.

Evaluation of the severity of injury is extremely difficult if the injured segment is in the middle ear, where the space in which the surgeon can operate is very limited. The horizontal semicircular canal limits exposure posteriorly and the superstructure of the stapes limits exposure anteriorly. Consequently, accurate assessment of the severity of injury is especially difficult in this area.

If more than 50% of the diameter of the nerve is deemed nonviable because it has been cut, crushed, or stretched, then resect the injured segment and re-anastomose or graft the nerve.

If a primary anastomosis can be achieved without tension, this is probably the best method of repair. However, a primary anastomosis, even with small segments of missing nerve, is possible only if the nerve can be mobilized and rerouted over a substantial portion of its length. This is not usually possible when surgery is limited to the mastoid and tympanic segment. If a primary anastomosis without tension cannot be achieved, then place an interposition graft.

If injury to the facial nerve is not recognized during the operation and the patient awakens with facial paralysis, the surgeon should wait several hours to make sure that this is not a consequence of the local anesthetic. If facial paralysis persists 3-4 hours beyond recovery from anesthesia, the packing should be removed.

If the facial nerve was clearly visualized at the time of surgery and the operating surgeon is absolutely certain that the nerve is anatomically intact, treat the injury like any other posttraumatic nerve paralysis. Begin electroneuronography testing approximately 72 hours postoperatively. If, within the first 2-3 weeks, the compound action potential on the affected side drops to less than 10% of the compound action potential on the normal side, the patient should be taken back to the operating room for exploration.

If the surgeon is not absolutely sure that the facial nerve is anatomically intact, the patient should be taken back to surgery immediately, the nerve decompressed around the area of injury, and the severity of the injury assessed. Based on such an assessment, the surgeon must decide whether or not to excise the injured segment. Once again, a consultation should be obtained if possible. Consultants should document their assessment and opinion on the chart.

Sometimes, a labyrinthine fistula is suggested based on the preoperative CT scan images; other times, a fistula is a complete surprise. Initially, the desquamated epithelium should be removed, carefully leaving the matrix over the horizontal canal. Blunt probes are used to locate the fistula by palpation. If the fistula appears superficial, the matrix is gently removed at the end of the case and the defect covered with fascia.

However, if the fistula is large and it appears that the cholesteatoma matrix is attached firmly to the membranous labyrinth itself, leaving the matrix in position should be considered. Debate continues as to whether hearing loss is more likely if the matrix is removed. If the membranous labyrinth is opened at any point during the procedure, broad-spectrum intravenous antibiotics and steroids should both be administered immediately. Bacterial labyrinthitis and inflammation pose the biggest risk to hearing.^[20] Fistulas involving the oval window area should be treated according to the same principles. Violation of the vestibule itself is much more likely to produce hearing loss than violation of one of the semicircular canals.

Occasionally, a canal fistula is created during the surgical procedure. If such a fistula involves one of the semicircular canals, then it should be sealed with soft tissue (preferably fascia) and intravenous antibiotics and steroids administered. These patients are quite likely to have significant balance disturbance in the immediate postoperative period, but a significant number recover with little or no hearing loss if appropriate antibiotic therapy and steroids are provided in the postoperative period.

Persistent drainage from a canal-wall-down cavity can arise for a number of reasons. The most common cause of persistent drainage is either a sequestered air cell that continues to harbor infection or a small area of osteitis. In such cases, the cavity as a whole heals up quite well except for a small area that remains covered with granulation tissue. The only solution is to remove the involved area. If the area of osteitis is large and postoperative otorrhea has persisted for months or years, skin grafting should be considered. Skin grafts are especially useful in the cavities that have developed, on one or more occasions, mucosal (as opposed to squamous) epithelial linings. Sometimes, persistent or recurrent drainage is due to a residual cholesteatoma. Then the only viable solution is reoperation to remove residual disease.

Foreign bodies retained within the mastoid cavity or wound must if they have become the focus for inflammation or infection. The most common foreign bodies are small metal fragments that result from the burr hitting the end of the suction irrigation during surgery. Retained pieces or fragments of packing and/or shreds of cotton can similarly serve as a nidus of infection but can usually be removed in the office.

Brain herniation through the tegmen of the middle fossa has a characteristic glistening, shiny appearance. The presence of clear fluid associated with such a lesion should raise the suggestion of a brain hernia and cerebrospinal fluid leak. MRI and CT scanning can often help clarify the diagnosis preoperatively.

Options for managing a brain hernia fall into 2 basic categories: reconstruction of the mastoid cavity or obliteration of the mastoid cavity. If reconstruction is chosen, glialized brain can be removed simply by sharply excising it and any viable brain can be pushed back up into the middle cranial fossa. If the defect is large, both intradural and extradural repair of the dural defect should be considered. The second alternative is to simply obliterate the middle ear, external auditory canal, and mastoid by removing posterior canal wall, obliterating the eustachian tube, filling the defect with abdominal fat and over sewing the external canal. This is an especially appealing option if hearing is very poor in the afflicted ear. If all the squamous epithelium has been removed, obliteration ensures a safe, well-healed, problem-free ear. Maximal conductive hearing loss is unavoidable.

Eliminating a cholesteatoma is almost always possible; however, multiple operations may be required. Because surgery is generally successful, complications from uncontrolled cholesteatoma growth are now relatively uncommon.

Canal-wall-down tympanomastoidectomy offers a very low rate of recurrence or persistence of a cholesteatoma. Reoperation for cholesteatomas occurs in 5% or fewer patients. This compares quite favorably to the 20-40% recurrence rates associated with closed-cavity techniques^[15] .

Nonetheless, because the ossicular chain and/or tympanic membrane cannot always be completely restored to normal, cholesteatomas remain a relatively common cause of permanent conductive hearing loss.

Controversies

The routine use of facial nerve monitoring remains controversial. A survey of practicing otologists in 1990 showed that most experienced otologists do not believe that facial nerve monitoring is obligatory. Many experienced otologists only use it occasionally. Facial nerve monitoring requires experience and is unlikely to provide meaningful protection to an inexperienced surgeon.

On the other hand, some surgeons believe that predicting the cases in which the facial nerve is at risk is impossible and, consequently, believe monitoring should be performed in every case. These surgeons see facial nerve monitoring as a precaution, much like ECG monitoring, that may be potentially useful in any given case.

A large percentage of surgeons do not use facial nerve monitoring for all cases but, instead, monitor only selected cases. Such cases often include revision operations or situations in which the patient has had perioperative facial nerve weakness in the past or when imaging studies show facial nerve anomalies.

Future

The applicability of using small otoendoscopes as part of the surgical management of cholesteatomas is being explored. To date, they have only an adjunctive role; however, their role may expand in the future.