Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

The term cholesteatoma is actually a misnomer coined by Johannes Muller in 1838. He described "a layered pearly tumor of fat which was distinguished from other fat tumors by the presence of biliary fat or cholestrin that is interspersed among sheets of polyhedral cells." Aural cholesteatomas are best thought of as skin growing in the middle ear space. Cholesteatomas actually do not contain fat but are composed of an outer matrix which surrounds layers of desquamated epithelium. The matrix is comprised of fully differentiated keratinizing squamous epithelium resting on a collagenous perimatrix. Various theories have been advanced to account for the destructive bone resorption seen commonly with these lesions. These include activation of osteoclastic bone destruction, mechanical pressure necrosis and bone degradation by enzymes such as collagenases and lysozymes.

Cholesteatomas may be classified as either congenital or acquired. Congenital cholesteatomas develop behind an intact tympanic membrane; according to the classic teachings of Derlacki and Clemis there must be no antecedent history of infection or breach of the tympanic membrane. This definition has recently been challenged by Friedman et al who note that approximately 70% of children will have had at least one episode of otitis media. There is little doubt that patients with epidermal rests of cells in the protympanum may also have episodes of AOM. The acquired form of cholesteatoma is much more common. Primary acquired disease arises from a skin lined retraction pocket within which retained keratin debris accumulates. Primary acquired cholesteatoma occurs most commonly in the posterior-superior quadrant of the pars tensa and in the pars flaccida. Secondary acquired cholesteatoma develops from an ingrowth of skin through a tympanic membrane perforation that is then retained within the middle ear, mastoid or both.

The pathogenesis of cholesteatoma growth is still poorly understood as evidenced by the multiple theories currently found in the literature. However, a common denominator appears to be eustachian tubal dysfunction. Bluestone proposes that patients with acquired cholesteatoma have a functional obstruction of the eustachian tube and are thus predisposed to high negative middle ear pressures. The areas of the tympanic membrane most susceptible to these forces are the pars flaccida and posterior-superior regions. The expansion or growth of cholesteatomas are channeled along well defined pathways determined by ligaments and folds. Review of embryologic development is helpful in understanding these pathways. Between the 3rd and 7th months of development the gelatinous tissue of the middle ear space is absorbed. A primitive tympanic cavity develops by growth of an endothelium lined pouch extending from the eustachian tube to the middle ear cleft. Four primary sacs then bud into the cleft. The remnants of these sacs direct the growth of cholesteatoma along predictable pathways in the middle ear. Prussaks space is commonly thought of as the most common point of invasion for primary acquired cholesteatomas. From this space cholesteatomas expand in one of three directions: the posterior route is most commonly seen, and follows the superior incudal space above the incus into the epitympanum. The inferior route follows the inferior incudal space into the mesotympanum. The anterior route from Prussaks space is the route least frequently travelled. The anterior pouch of von Trolsch serves as the avenue for spread into the protympanum.

The presence of cholesteatoma requires surgical intervention unless underlying medical problems contraindicate exposure to general anesthesia. Most cholesteatomas are asymptomatic in their early development; children may come to medical attention with otorrhea but will rarely complain of decreased hearing. Microscopic examination of the ear is imperative in identifying and delineating cholesteatoma. Audiometric evaluation is part of the standard preoperative workup. Computerized tomography may be used to delineate the extent of disease, check the aeration of the mastoid and to help rule out intratemporal and /or intracranial complications.

The primary surgical goal is to achieve a safe, dry ear by removing disease with the preservation of normal anatomy. Improving hearing is a secondary goal. The principles of surgical management are based on the extent of the disease and the presence of complications arising from cholesteatoma. Much debate has emerged in the literature concerning canal wall up procedures vs canal wall down approaches. It is clear that the consensus among otologists over the last several years has been that canal wall up procedures are indicated in patients with well pneumatized mastoids and adequate middle ear clefts with cholesteatoma limited to the middle ear space or mastoid. Relative contraindications to canal wall up procedures include a sclerotic mastoid, a fistula, an only hearing ear and poor eustachian tubal function. There is no objective measurement of eustachian tubal function though several clinical observations can help predict the ability of the middle ear to ventilate itself. According to Parisier et al the appearance of the pars tensa, the amount of mastoid cellular development and the appearance of middle ear mucosa will reflect the eustachian tubal function and guide the surgical approach. The success of any given approach may be measured in terms of the rate of recidivism. Originally a term applied to criminal behavior, recidivism is the measurement of residual disease plus recurrent cholesteatoma.

Cholesteatoma in children is widely considered to be a more aggressive disease than in the adult population for two major reasons. First, very extensive disease is found more frequently in children compared to adults and second, higher rates of residual and recurrent disease have been documented in the pediatric population. Of note however is the observation that the incidence of complications arising from cholesteatoma is directly related to the duration of the disease and, as such, adults tended to have higher complication rates.

Glasscock in 1981 published a retrospective review of charts comparing rates of recidivism in patients less than 16 years old to those older than 16. Almost 90% of both groups had a canal wall up approach regardless of initial findings. Rates of recidivism were compared and it was found that patients less than 16 had an almost two-fold higher rate and a much shorter interval to recurrence. Based on these findings Glasscock concluded that pediatric cholesteatoma is more aggressive than that seen in adults. There was no statistical analysis presented and no criteria were set forth regarding canal wall up vs canal wall down approaches.

In 1977 Palva published a retrospective evaluation of 65 pediatric patients (<16 years old) matched with 65 adults with cholesteatoma. All patients underwent canal wall down procedures. The duration of disease in the pediatric population was shorter and the number of complications was higher in adults than in children. Operative findings revealed that 65% of pediatric ossicular mechanisms were disrupted compared to 84% in the adult group despite the finding that cholesteatoma was more extensive in the pediatric group. The authors calculated a 5% rate of recidivism in the pediatric group but did not calculate rates in the adult group. Based on the finding that cholesteatoma tended to be more extensive in children and have an overall shorter duration of disease these workers conclude that pediatric cholesteatoma is more aggressive. There is no mention of recurrence rates in adults however.

A study published in 1988 by Parisier illustrates the results of a single surgeon over 15 years. Operative approach was dictated by intraoperative findings with 53% of patients undergoing canal wall down procedures and 30 % of patients undergoing canal wall up approaches. The remainder of patients had a tympanotomy for localized disease. Average follow-up was 4 years and rates of recidivism did not differ significantly between the pediatric population and an adult population. Based on these findings, the authors conclude that pediatric cholesteatoma is not a different disease than that seen in adults but that surgical approaches must be individualized to the patient's disease.

A preliminary evaluation of the Baylor experience with pediatric cholesteatoma was performed. There were 26 patients (19 males and 7 females) for a total of 27 operated ears. The age at presentation averaged 8 years (range 2-15 years) with an average follow-up of 4 years (range 3 months to 15 years). The most common presenting symptoms were otorrhea (11/26), hearing loss (4/26) and tympanic membrane perforation (3/26). Five patients had congenital cholesteatoma. Pre-operative and post-operative audiograms were performed in all patients and there was no significant difference in pure tone averages (30dB pre-op compared with 27dB post-op). Fifty-four percent of patients had canal wall up procedures while 27% had canal wall down procedures. Nineteen percent of patients had a tympanotomy approach. Planned second look surgery was performed in 16 of 27 ears. Two patients underwent primary ossicular reconstruction while 9 patients had a secondary ossicular reconstruction. The rate of residual disease was 4/27 (15%) while the rate of recurrent disease was 3/27 (11%) for a recidivism rate of 26%.

In conclusion there remain many questions concerning the pathophysiology of cholesteatoma; whether or not the disease differs in children and adults is still a matter of debate. The next area of inquiry will be on a cellular level. It is clear however, from the above data, that a reasoned individualized approach to cholesteatoma is the best way to achieve low rates of recidivism.

Case Presentations

An eight-year-old white female child presented with a history of chronic eustachian tube dysfunction, numerous episodes of acute otitis media and recent bloody otorrhea from the left ear. Her otologic history dates to the age of three when she had an episode of acute otitis media which was treated with antibiotics and decongestants. The patient subsequently failed a school audiogram and continued to require intermittent courses of oral antibiotics. Subsequent school audiogram failures were attributed to "fluid in the ears." There was no history of facial nerve palsy, vertigo or otologic surgery.

Physical exam was significant for a white mass behind the posterior half of the right tympanic membrane and a central posterior, superior perforation of the left tympanic membrane. There was no evidence of keratin debris although the drum was severely retracted over the malleus. Audiometric evaluation of the right ear demonstrated a slight loss over the lower frequencies and a type C tympanogram. CTT of the temporal bones revealed a soft tissue mass involving the tympanic membrane extending toward the epitympanum without erosion of the scutum.

The child was brought to the operating room where a right tympanomastoidectomy (canal wall up) was performed. Cholesteatoma was found involving the posterior half of the eardrum, extending towards the epitympanum and antrum. The ossicular mechanism was noted to be intact although there was some attenuation of the incudostapedial joint. The cholesteatoma was excised completely, leaving the ossicles intact. A medial graft was incorporated. The patient has done well postoperatively.

Bibliography

Abramson M, Lachenbruch PA, Press BA, McCabe BF. Results of conservative surgery for middle ear cholesteatoma. Laryngoscope 1977;87:1281-1286.

Derlacki EL, Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965;74:706-727.

Edelstein DR, Parisier SC. Surgical techniques and recidivism in cholesteatoma. Otolaryngol Clin North Am 1989;22:1029-1040.

Edelstein DR, Parisier SC, Ahuja GS, Jurabe C, Clute P, Wenig S, et al. Cholesteatoma in the pediatric age group. Ann Otol Rhinol Laryngol 1988;97:23-29.

Edelstein DR, Parisier SC, Han JC. Acquired cholesteatoma in the pediatric age group. Otolaryngol Clin North Am 1989;22:955-965.

Glasscock ME III, Dickins JRE, Wiet R. Cholesteatoma in children. Laryngoscope 1981;91:1743-1753.

Jackson DG, Glasscock ME III, Nissen AJ, Schwaber MK, Bojrab DI. Open mastoid procedures: contemporary indications and surgical technique. Laryngoscope 1985;95:1037-1043.

Jahn AF. Cholesteatoma: What is it, how did it get there, and how do we get rid of it? Otolaryngol Clin North Am 1989;22:847-857.

Jansen C. Cholesteatoma in children. Clin Otolaryngol 1978;3:349-352.

McGill TJ, Merchant S, Healy GB, Friedman EM. Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report. Laryngoscope 1991;101:606-613.

Nadol JB Jr. Causes of failure of mastoidectomy for chronic otitis media. Laryngoscope 1985;95:410-413.

Palva A, Karma P, Kärjä J. Cholesteatoma in children. Arch Otolaryngol 1977;103:74-77.

Schmid H, Dort JC, Fisch U. Long-term results of treatment for children's cholesteatoma. Am J Otol 1991;12:83-87.

Schulerati N, Bluestone CD. Pathogenesis of cholesteatoma. Otolaryngol Clin North Am 1989;22:859-881.

Sheehy JL. Cholesteatoma surgery in children. Am J Otol 1985;6:170-172.

Sheehy JL. Cholesteatoma surgery: canal wall down procedures. Ann Otol Rhinol Laryngol 1988;97:30-35.

Sheehy JL, Patterson ME. Intact canal wall tympanoplasty with mastoidectomy: a review of eight years' experience. Laryngoscope 1967;77:1502-1542.

Sismanis A, Hutchinson L, Abedi E. Chronic otitis media: surgical failures and management. Am J Otol 1989;10:460-465.

Stern SJ, Fazekas-May M. Cholesteatoma in the pediatric population: prognostic indicators for surgical decision making. Laryngoscope 1992;102:1349-1352.

Toner JG, Smyth GDL. Surgical treatment of cholesteatoma: a comparison of three techniques. Am J Otol 1990;11:247-249.

Tos M. Treatment of cholesteatoma in children: a long-term study of results. Am J Otol 1983;4:189-197.

Vartiainen E, Nuutinen J. Long-term results of surgical treatment in different cholesteatoma types. Am J Otol 1993;14:507-511.

Weiss MG, Parisier SC, Han JC, Edelstein DR. Surgery for recurrent and residual cholesteatoma. Laryngoscope 1992;102:145-151.

Wullstein SR. Cholesteatoma in children: is the disease different in childhood? Clin Otolaryngol 1978;3:353-362.

Grand Rounds Archive | Department Home page

BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map |

©2001-2006 Baylor College of Medicine
Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery
Mail: One Baylor Plaza, NA102, Houston, TX 77030
Phone: 713-798-5906
E-mail: oto@bcm.edu

Last modified: Feb. 7, 2006