Cholesteatoma in the pediatric patient

International Journal of Pea;btric Otorhinolatyngology, 14 (1987) 101-112

Elsevier

101

POR 00468

ph F.

etmore, an F. Kodde, and Steven D. Handler

Department of Otorhinolatyngologv and Human Communication, Children’s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelptia, PA 19104 (U.S.A.) (Received 16 April 1987) (Revised version received 15 September 1987) (Accepted 27 September 1987)

Key wrak

Pediatric cholesteatoma; Intact canal wall procedore; Open cavity procedure

A review of patients with cholesteatoma at the Children’s ospital of Philadelphia from 1981 to 1986 yielded 161 children. Analysis of data from both outpatient and hospital charts revealed a higher incidence of males to females, and of cholesteatoma appeared to be in the 6-10 year age group. on symptom; and on physical examination, time was most often affected. Both intact canal wall and open cavity procedures were employed depending upon the site and extent of disease. Seventy-six percent of patients underwent an intact canal wall or middle ear procedure initially, with the remainder requiring an open cavity procedure. rent or residual cholesteatoma was found in 32% of 148 patients at the second procedure, and in 7% of 148 patients if a third procedure was necessary. Pre- and post-operative audiometric findings for 91 patients revealed 21% with decreased hearing post-surgically, 50% with no change in hearing and 29% with improved hearing. Post-operative complications were minimal.

uction Because of the high incidence of otitis media in children, a complication such as cholesteatoma may be encountered occasionally. Although most cholesteatomas in

* Presented at the Annual Meeting of the American Society of Pediatric Otolaryngology, Colorado Springs, CO, May 2,1987. Correspondence: R.F. Wetmore, Department of Otolaryngology, Children’s Hospital of Philadelpha, 34th Street and Civic Center Blvd., Philadelphia, PA 19104, U.S.A.

01655876/87/$03.50

0 1987 Elsevier Science Publishers B.V. (Biomedical Division)

102

children are acquired either as the result of an attic retraction pocket or associated with a marginal perforation, cholesteatomas also occur behind an iutact tympanic membrane where they are either congenital or the result of an inflammatory event. Improved recognition of cholesteatoma by primary care physicians in conjunction with auditory and immittance mass screening have resulted in early referral and prompt intervention. Treatment of concurrent infection with antibiotics has caused a decrease in regional and intracranial complications associated with cholesteatoma 141.

Several authors [3,6,14] believe that cholesteatoma in children differs from the adult disease, and that surgical treatment should be altered as a result of these differences. Currently, there is a paucity of recently published data on cholesteatoma in just children [3,6,12,17]. The purpose of this study, therefore, was to review demographic data and surgical results from a series of patients with cholesteatoma seen over a S-year period at the Children’s Hospital of Philadelphia (CHOP). Since it is difficult to assess treatment modalities in patients with cholesteatoma due to variability in both the extent of disease and surgical techniques, this review was not intended to compare the efficacy of different surgical procedures. Rather, the following report summarizes our experience and emphasizes our goals of preserving hearing and maintaining the posterior canal wall whenever possible. Materials and Methods

A review of operative cases performed at the CHOP from July 1, I981 to June 30, 1986 yielded 161 patients who underwent surgery for cholesreatoma. Both the outpatient and hospital charts were examined in order to categorize demographic information, numbers and types of operative procedures and results of surgery. Audiometric data from both sets of records were also analyzed. Results Demographic findings

Of the 161 children, 102 (63%) were male and 59 (37%) were female; 146 (91%) were white, 13 (8%) were black and 2 (1%) were oriental. There was a total of 168 ears involved with cholesteatoma: the right ear was involved in 68 patients (42%), the left ear in 86 patients (53%) and both ears in 7 patients (4%). In determining the age at presentation, only 142 children whose initial surgery was at the CHOP were included. The age at presentation ranged from 5 months to 20 years, with a mean age of 8.4 years. There were 31 patients (22%) that presented between the ages of 0 and 5 years, 67 children (47%) were between 6 and 10 years of age, 35 (25%) were in the age range of 11-15 years, and only 9 patients (6%) were older than 15 years. A history of either acute otitis media or otitis media with effusion was present in I38 patients (86%), was absent in 12 patients (7%) and could not be determined in 11 patients (7%). Eighty-nine patients (55%) had a history of at least one set of

103 TABLE I TABLE II

Symptoms at presentation

Tympanic membrane cholesteotoma

(n = 161 patients) __ Otorrhea Hearing loss Pain Facial nerve weakness Middle ear mass Acute mastoiditis No symptoms

52 9 4 2 2 1 91

quadrants

inoolved with

(n = 159 ears) None One TWO

Three Four

8 90 31 2 28

tympanostomy tubes. Seven children (4%) had a history of cleft palate, an-d 3 had Down’s syndrome. ms at the time of presentation are listed in Tab symptom, whereas other symptoms were unto 91 children (57%) were free of symptoms at the initial presentation. On physical examination during the initial presentation, the con~tion of the tympanic membrane could be determined for 167 ears (one ear had steno& of the external canal and the tympanic membrane could not be visualized). The tympanic membrane was intact in 50 ears (30%) and perforated in the re.maining 113 ears (70%). In only 159 ears was there a record of tympak membrane involvement with cholesteatoma. The number of quadrants of the tymptic membrane which appeared to be involved with cholesteatoma on examination are listed in Table II. Further analysis of the data in Table II by quadrant revealed posterior-superior quadrant to be involved most commonly (60%), followed by the anterior-superior (50%), posterior-inferior (31%) and anterior-inferior (29%) quadrant. Mastoid films were obtained in 25 patients and were abnormal in 21. Abnormalities were noted in all 6 patients who underwent mastoid tomography and in 30 of 31 children z
TABLE III Major procedures for cholesteatom (n = 294

procedures)

Middle ear or intact canal wall procedures

?I

Open cavityprocedures

n

1. Tympanomastoidectomy 2. Tym&oplasty

118 14

1. Radical mastoidectomy 2. Modified radical mastoidectomy 3. Revision mrs!&ktomy

40

3. Tympanoplasty and excision of choksteatoma 4. Bxploratory tympanotomy 5. T’ympanoplasiyand mastoidplasty

Total

35 39

9

215 (73%)

13 19

4. Tympanoplasty and atticutomy 5. Tympanoplasty and modified radical mastoidectomy 6. Revision mastoidectomy and either tympanoplasty or exploratory tympanotomy Total

3 2

2 79 (27%)

TABLE IV Types of procedures Pr&ures

#l-4

Operation

Procedures #I

(n = 148)

Middle ear or intact canal wall 1. Tympanomastoidectomy 2. Tympanoplasty 3. Tympanoplasty and excision of cholesteatoma 4. Exploratory tympanotomy 5. Exploratory tympanotomy and excision of cholesteatoma 6. Tympanoplasty and mastoidplasty Total Open cavity procedures 1. Radical mastoidectomy 2. Modified radical mastoidectomy 3. Revision mastoidectomy 4. Tympanoplasty and atticotomy 5. Tympanoplasty and modified radical mastoidectomy 6. Revision mastoidectomy and exploratory tympanotomy Total

#2 (n = 80)

66 7

36 3

36

2

#3 (n = 21)

4 3

6 11

113 (76%) 23 8

#4 (n = 2)

2

16 1 62 (78%) 9 2 5

1 14 (67%)

2 (100%)

3 1 3

3 1

35 (24%)

1 1 18 (22%)

7 (33%)

-

PO5

histories of 142 patients (1 8 ears) who were treated exclusively at the C analyzed. Table IV lists the types of operations performed as a function of the various procedures. For Procedure no. 1, the middle ear was solely involved in /7 ears and the mastoid solely in one ear; both the le ear and attic were involved in 24 ears and the middle ear, attic and mastoid 111 ears. The malleus was eroded or removed in 65 cases, the incus in 85 and the stapes in 27. A second procedure was necessary in 80 ears (see Table IV); and in 48 cases (32% of the original I48 ears), there was evidence of recurrent or residual disease. Thirty patients (38%) had cholesteatoma in the middle ear, 7 (8%) in the mastoid and 11 (14%) had involvement in both areas. The malleus was removed or eroded in 6 cases, the incus in 4 and the stapes in 4. A third procedure was performed’in 21 cases (see Table IV), and 10 ears (7% of the original I48 ears) had evidence of cholesteatoma. The middle esr was involved in 6 (29%), the mastoid in 3 (14%) and both in one child (5%). The malleus and incus were removed or eroded in one case each. The stapes were not affected. A fourth procedure was performed in two cases (see Table IV), both of which were explomtory tympanotomy. Cholesteatoma was not found in either patient. Intact tympanic membrme Cholesteatoma was discovered behind an intact tympanic membrane in 40 (41 ears) of the total 161 patients. This subgroup of patients was analyzed separately. I, The right ear was involved in 21 patients (53%), the left ear in I8 patients (45%) and both ears in one patient (2%). The average age was 5.7 years with a range of 5 months to I7 JKZWS. TlGrty-eight children had a documented history of either acute otitis media or otitis media with effusion, while two did not. Nine patients had undergone myringotomy with tube placement in the past; cholesteatoma was discovered at myringotomy in 11 patients. On physical examination, cholesteatoma in this group of patients was not visible in 5 ears. The anterior-superior quadrant was involved most commonly (46%), followed by all 4 quadrants (Is%) and the posterior-superior quadrant (12%). The remaining patients had variable involvement. In the subgroup of patients with cholesteatoma behind an intact tympanic membrane, 25 underwent one surgical procedure, 11 children required two procedures, 3 children need 3 procedures and one &Id h&d 4 operations. Table V lists the type of operative procedures performed in this subgroup of patients. Of the 40 patients who presented with cholesteatoma behind an intact tympanic membrane, 22 (55%) were under 5 years of age. For these younger children, all but one had a history of acute otitis media or otitis media with effusion, and cholesteatomm LL~smq .,- “I”+.VI A;cpe*rered at myringotomy in 8. Two patients had a history of a prior myringotomy. Right ears (59%) were involved more than left ears (36%) and one child had involvement of both ears. Cholesteatoma was noted in the anterior-superior quadrant in 65%, in all quadrants in 17% and in the osterior-superior quadrant in 9%. In this group of children under 5 years of age, I5 required one opera procedure, 6 needed two operations, and one child with bilateral cholesteatomas

TABLE V Cholesteatoma behind cznintact tympanic membrane Types of procedures

Operatioris

Total (n = 60)

Middle earor intact canal wall procedures 1. Tympanomastoidectomy 20 2. Tympanoplastyand excision of cholesteatoma 15 3. Exploratorytympanotomy and excisionof cholesteatoma 19 Total 54 (90%) Open cawy procedures 1. Radicalmastoidectomy 5 2. Modifiedradical mastoidectomy 1 ,rnaz\ vc {AVru, Total

undergone

3 procedures.

The

Ofwr 5 years (n = 30)

Under 5 years (n = 30)

;j

7

8

7

6 27 (90%)

13 27 (9’3%)

3

2

0 3 (10%)

1 3 (10%)

type of operative procedures in this group under five

years is listed in Table V. Reconstructive procedures

In 19 patients the posterior canal wall was reconstructed with Proplast. patients an ossicular replacement prosthesis (PORP/TORP) was utilized.

In 25

Complications

The most common post-operative complication was bleeding from the wound oarr ear. Two children had bleeding from either the post-auricular wound or the external meatus. Two other children had bleeding as a result of traumatic dehiscence of the wound. All episodes were self-limited except in one patient who needed operative management of bleeding. Two children developed transient facial nerve paralysis secondary to infiltration with local anesthesia. Both resolved without incident. One child suffered operative injury to the facial nerve which resolved almost completely following immediate surgical decompression. Hemiation of brain tissue into the mastoid was found in t\irOpatients who had undergone a previous mastoidectomy. Follow-up data for both intact canal wall and open cavity procedures are listed in Table Vk Follow-up intervals are from the last operative procedure which explains why 63%‘of children had one year or less over the 5-year period of the review. Several patients were transferred to the care of other physicians or failed to return for follow-up. Audiometric data for pure tone air conduction thresholds obtained prior to initial surgery for dholesteatoma and after the last operation were available for 59 ears in which an intact canal wall procedure was performed and for 32 ears where surgery resulted in an open cavity. Hearing status for each of the 91 ears was examined

107

TABLE VI Long-term follow-up (n = 148 ears] Follow-up

Intact canal wall with no disease (n = IQ4 ears)

3 months 6 months 9 months 12 months 18 months 2 years 3 years 4 or moreyears

26 12 10 2G 6 20 8 2

pre-operatively

by reducing

‘_

Open cavity (n = 44 ears) 7 Q 4 8 4 9 1 5

the pure tone tl~eshold

(PTA = 500 + 1000 + 2OOO/3) and as a consequence

data to pure tone averages of surgical intervention by

4s. comparing the pre- and post-operative The pre-operative PTAs for the intact canal subgroup averaged 29 dB Nearing Threshold Level (HTL) and ranged from 5 to 55 dB. The mean FTA for the open cavity subgroup was 41 dB HTL with a range of 5-105 dB. Regardleess of s&group, substantial conductive comport existed for all pre-operative cases of hearing loss. Post-operatively, s for the intact canal wall cavity subgroups were 24 dB from 5 to 58 15 and 4 with a range from 10 to 85 d pectively. Thus there was an approximate 5 dB improvement in the mean for the intact canal wall ears following surgery, but the mean PTA remained unchanged after surgery for the open cavity subgroup. Table VII lists the number of ears, within each subgroup, as functions of pre-operative PTA air con Zi:d post-operative changes in hearing 40% (23 ears) of the intact canal wall sensitivity. Pre-operatively, TL as compared to 12% (4 cars) subgroup demonstrated PTAs better than 20 who had surgery resulting in an for the open cavity subgroup. Conversely, for HTL whereas only open cavity, the PTA for 16 ears (50%) was poorer than 40 ee of prsoperative 27% (16 ears) of the intact canal group suffered a similar impairment. The overall magnitude of pre-operative hearing loss, therefore, tende q to be greater for the open cavity compared to the intact canal wall subgroup. Hearing status for the 91 ears as a consequence of surgical intervention was determined by subtracting post- from the pre-operative PTAs. Hence, the number of ears displayed under the post-operative section of Table VII denote relative changes in hearing status from pre-operative data. Hearing sensitivity remained unchanged for 31 of the 59 intact canal wall ears (53%) and for 15 of the 32 open cavity ears (47%). For the intact canal wall subgroup, hearing decreased (i.e. post-operative hearing sensitivity was poorer than pre-operative thresholds) for 9 ears (15%). It should be noted that for 7 of these 9 ears, however, pre-operative hearing sensitivity was better than 20 dB EITL, but in no case did hearing decrease by more than 30

108

‘I’ABLEVII Numberofear-3 4sa function of pre-operativehearing loss and post-operativechmge in hearing I,

improvedpost-ophearing; D, decreased post-op hearing. Past-ophearing change

Pre-op HTL

n

Intact canalwall O-20dB 23

+lOdE

s IO-20 dB D I

>20-30dB I D

>30-4Odl3 I D

>40-5OdB I D

> 20-30 dB > 30-40dB > 40-50 dB >SOdB Subtotal Open cavity O-20 dB > 20-30 dB > 30-40 dB > 40-50 dB >SOdB Subtotal

10 10 8 8 59

16 4 5 2 4 31

0 3 3 4 0 10

5 2 0 0 0 7

0 ! 2 1 1 5

2 0 0 0 0 2

0 0 0 1 1 2

0 0 0 0 0 0

0 0 0 0 2 2

0 0 0 0 0 0

4 5 7 9 7 32

1 4 3 3 4 15

0 0 0 3 0 3

2 1 1 1 1 6

0 0 0 1 0 1

1 0 2 0 0 3

0 0 0 1 2 3

0 0 0 0 0 0

0 0 0 0 0 0

0 0 1 0 0 1

Total

91

46

13

13

6

5

5

0

2

1

dB. Within the same subgroup, hearing increased (i.e. post-operative hearing was improved compared to pre-operative findings) for 19 ears (32%) with the majority of improvement seen in those ears where pre-operative hearing was poorer than 30 dB. There were 26 ears in the intact canal subgroup. for example, where pre-operative hearing was poorer than 30 dB, yet I5 of these ears had improved post-operative PTAs. When surgery resulted in an open cavity, &ere were 7 ears (22%) with improved hearing post-operatively as compared to 10 ears (31%) that had poorer hearing. Interestingly, all 7 ears with post-surgical open cavities and improved hearing had pre-operative PTAs poorer than 40 dB HTL. Conversely, 8 of the 10 ears with open cavities and poorer post-operative hearing had pre-operative PTAs better than 40 dB. Discussion

In reviewing the epidemiologic statistics from this study, it is apparent that there is a significant difference in the incidence of cholesteatoma according ‘to sex. A similar (2 : 1) male:female ratio has been reported by Glasscock 161.In at least one study, there is a higher incidence of otitis media in ma!es than females [16], and Solomon and Harris [15] have reported a higher incidence of myringotomies and tympanoplasties in males. These findings coupled with the male:female ratio noted in our data suggest that male children with more frequent middle ear infections may have a higher incidence of cholesteatoma than female children. A counterargument

is the low incidence of cholesteatoma in Es o natives who have a high incidence of otitis media [8]. The marked difference in racial incidence in this study is a reflection of the raciaf mix of the tertiary referral patterns to the OP. While chdesteatoma was found most commonly in the left ear in this s his finding has not been verified by data from other reviews. The age distribution of our data shows a peti incidence in the 6-10 year age group which differs from at least one other review of cholesteatoma that suggested a higher incidence in the lo-19 year age group [7]. There was a significant history of either acute otitis media or otitis media with effusion in 86% of children; however, the role of otitis media in the pathogenesis of cholesteatoma remains uncertain. Only one-third of patients presented with a history of drainage, a much lower figure than reported in children under 16 years of age The role of myringotomy tube placement as a causal factor in t cholesteatoma also remains uncertain. uckingham [2] cited a 0.5% incidence of cholesteatoma in a review of series of patients who had undergone middle ear intttbation for otitis media. Approximately 50% of the children in our series had a history of a prior m . When compared to the total num of myringotomies performed at t over the same 5-year period cover report, the incidence of cholesteatoma in our series was similar to that reported by Buckingbam (approximately 0.4%). While otitis media and cholesteatoma are common sequelae in cleft palate patients, our incidence of cholesteatoma in cleft palate patients (4%) seems low, especially in view of the large number of such patients in al examination, cholesteatoma involved one tympamc membrane quadrant, the posterior-superior most commonly. This finding was previolzsly noted by Palva [12]. Conversely, the anterior-superior quadrant was most co only an intact tympanic me_mbrane. involved in those patients with cholesteato ither to eradicate or exterioriie The goal of the otologic surgeons at the cholesteatoma while retaining as much hearing as is safely possible. Intact canal wall procedures are performed most frequently unless there has been either a complicating factor (i.e. mastoiditis or facial nerve paralysis) or cholesteatoma which cannot be removed without risk to the facial nerve or labyrinth. Second-look procedures are performed if residual or recurrent disease is suspected or as a planned procedure if disease was extensive at the initial operation. This approach to cholesteatoma is dependent upon excellent stugical expertise GZ close patient follow-up. In our series patients were informed about the need for careful follow-up and the ptissibihty of additional surgery. Since the primary goal of cholesteatoma surgery is always to provide a safe ear, open cavity mastoidectomy represents the conservative approach. Failure to eradicate or exteriorize cholesteatoma in a patient with poor follow-up could result in further hearing loss (sensorineural or conductive), regional complications such as a fection. labyrinthine fistula or labyrintbitis or intracranial extension o P, the middle ear In reviewing those cases who presented initially at ihe c alone was involved most commonly (52%) at the first procedure. Extensive involvement of the middle ear and mastoid was seen in one-third of cases. Intact CaXal wa]?

110

procedures were performed in 75% of patients as the initial procedure. Fifty-four percent of ems required a second procedure and almost half of this group had either residual or recurrent disease. The ratio of intact cad wall to open cavity procedures remain4 the same for the second procedure as in the first procedure. Of the original 148 ears, 14% required a third procedure, and 50% of this group had cholesteatoma. In this third group, there was a slightly higher incidence of open cavity procedures. These findings support our philosophy that, if attempts to eradicate disease by an intact canal wall procedure are unsuccessful, consideration should be given to an open cavity approach. A fourth procedure was necessary in only two patients, and in each case no cholesteatoma was found. utibtion of an intact canal wall approach or an open cavity to manage cholesteatoma is controversial in adults and even more so in children [2]. Proponents of the intact canal wall procedure cite the goal of maintaining as much functional hearing as possible, in addition to avoidance of a bowl that requires perk& cleaning [2]. Supporters of the open cavity stress the high recurrence rates of cholesteatoma and the aggressive nature of pediatric cholesteatoma as reasons to avoid the intact canal wall procedure [2]. Palva [12] performed open cavity procedures with obliteration of the mastoid in 65 patients and had a recurrence rate of 5%. Tos [17] showed slightly better results when comparing a modified canal wall up technique with a canal wall down technique. Tos’ overall recurrence rate was 12% in 122 children. Glasscock [6], who employed intact canal wall procedures in 95% of cases, had a 23% residual rate and 23% recurrence rate.’ In the current study, no attempt is made to compare these two approaches since, in most cases, our goal was to maintain functional hearing and. avoid a bowl that required cleaning. Radical mastoidectomy was employed ,usually in cases with extensive disease. Of the 148 ears operated upon initially at the’CHOP, 48 (32%) had recurrent or residual disease at the second procedure. At the third procedure, 10 (7%) of the original 148 ears still had cholesteatoma. In the 91 patients where good pre- and post-operative audiometric data could be obtained, 21% had a loss, 50% had no change and 29% showed improvement in hearing. Both Glasscock [6] and Tos [17] employed a large number of intact canal wall procedures and have analyzed their hearing results. Glasscock reported 58% improvement in hearing in the under-&year age group while only 5% showed a decrease of hearing. Hearing was unchanged in 37%. Tos noted no change in hearing in 9%, hearing loss in 7% and hearing improvement in 84%. Palva [15] utilized open cavity procedures and an average of his ears showed a 3 dB loss from pre- to post-operatively. Thirty-one percent of his ears had poor hearing results due to post-operative adhesions or unreconstructed middle ears. Cholesteatoma behind an intact tympanic membrane has received wider recognition in the medical literature in the past few years, and the presumed increase in its incidence is probably a result of greater surveillance. Most of these cholesteatomas fit Meyerhoff’s f’ll] description of a tertiary cholesteatoma, that is cholesteatoma behind a normal appearing tympanic membrane which may result from inflammation or trauma. A small number of cholesteatomas fit Derlacki’s [5] original criteria for congenital cholesteatoma: (1) cholesteatoma behind an intact tympanic men+

111

brane; (2) no history of aural infections; or (3) lesion arising from squamous epithelium during embryonic development. In our review, only two children had a negative history of otitis media or previous ear surgery and could fulfill Derlacl$s criteria for congenital lesions. Ievenson [9], however, has suggested that the presence of infection or previous otorrhea does not rule out a congenital lesion since secondary infection can occur. Thus, other cholesteatomas in our series which oaxrred behind an intact tympanic membrane may represent congenital lesions. 0f the patients with cholesteatoma behind an intact tympanic membrane, there is a slightly higher incidence in the right ear, especially in view of the greater number found in the left ear in the total study. McDonald [lo] noted a similar right-sided dominance, but Schwartz [13] cited a higher incidence in the left ear. Previous studies [9,10,13] have found a higher male incidence by a 3 : 2 ratio; the current review had a higher male incidence but not as large a ratic. In contrast to the total study group, children in this category had involvement more commonly in the anterior-superior quadrant on physical exam, a finding previously supported by Levins011[9]. Most olesteatomas behind an intact t membrane were man tially wi either an intact canal wall ear procedure (90%). probably the result of early diagnosis and greater involvement of the anterior half of the middle ear. Over half of these patients were under 5 years of age, and their experience appeared to mirror the group as a whole. Reconstruction of the posterior canal wall or ossicular chain was performed in a total of 39 patients. While the implants had to be removed or extruded in 6 patients, the remainder had no problems. Reconstruction of the posterior canal wall allows a child to participate in water activities without concern for infection, and placement of an ossicular prosthesis contributed to improvement in hearing. Post-operative complications in this series were minimal, and most involved bleeding from the wound. In two children, this involved trauma to the wound several days after surgery. Transient facial paralysis, seen in two patients, was secondary to infiltration of too much local anesthetic and resolved without consequence. One child suffered a more serious injury to his facial nerve during a radical mastoidectomy. Immediate recognition of the injury and decompression of the Fallopian canal medial and lateral to the site of injury resulted in only a slight residual defect. Brain hernias were discovered incidentally in two patients who had previous mastoid surgery. These typically result from a defect in the tegmen in the presence of chronic infection. One was managed through the mastoid without difficulty, while the other re+ired a temporal craniotomy to repair the defect in the tegmen.

Cholesteatoma is not rare in children, and presents a challenge to the otologic

surgeon. Because it may be more aggressive than in adults, there is controversy over its management. Certainly, the primary goal should be either to eradicate or

112

exteriorize disease. The secondary goal of maintaining or improving hearing can be met in many patients depending upon the extent and location ‘of disease. Several operative procedures may be necessary to meet these goals; but in experienced hands, the results can be excellent and the complications few. References 1 Abramson, M., Open or closed tympanomastoidectomy for cholesteatoma in children, Am. J. Otol., 6 (1985) 167-169. 2 Bu&ngham, RA., Cholesteatoma and chronic otitis media following middle ear intubation, Laryu goscope, 91(1981) 1450-1456. 3 Chamchon, R. and Gratacap, B., The surgical treatment of cholesteatoma in children, Ciin. Otolaryngol., 10 (1985) 177-184. 4 Counrige, C.B. and Nielsen, J.M., Symposium intracranial complications of otitis media and mastoiditis in the antibiotic era, Laryngoscope, 65 (1955) 31-52. 5 DerIa&i, E.L. and Clemis, J.D., Congenital cholesteatoma of the middle ear and mastoid, Ann. Otol. Rhinol. Laryngol., 74 (1965) 706-727. 6 Glasscock, M.E., Dickins, J.R.E. and Wiet, R., Cholesteatoma in children, Laryngoscope, 91 (1981) 2743-1753. 7 Marker, L.A. and Koontz, F.P., The bacteriology of cholesteatoma. In B.F. McCabe, J. Sade and M. Abramson (Eds.), Cholesteatoma: First International Conference. AescuIapius Publ., New York, 1977, pp. 264-267. 8 Hinchciiffe, R., Cholesteatoma: epidemiological and quantitative aspects. In B.F. McCabe, J. Sade and M. Abramson (Eds.), Cholesteatoma: First International Conference, Aesculapius Publishers, New York 1977, pp. 277-286. 9 Levenson, M.J., Paris&, S.C., Chute, P., Wenig, S. and Juarbe, C., A review of twenty congenital cholesteatomas of the middle ear in children, Otolaryngol. Head Neck Surg., 94 (1986) 560-567. 10 McDonald, T.J.. Cody, D.T.R. and Ryan, R.E., Congenital cholesteatoma of the ear, Ann. Otol. Rhinol. Laryngol., 93 (1984) 637-640. 11 Meyerhoff, W.L. and Truelson, J., Cholesteatoma staging, Laryngoscope, 96 (1986) 935-939. 12 PaIva, A., Karma, P. and Karja, J., Cholesteatoma in chiidren, Arch. Otolaryngol., 103 (1977) 74-77. 13 Schwartz, R.H., Grundfast, K.M., Feldman, B., Linde, R.E. and Hermansen, K.L., Cholesteatoma medial to an intact tympanic membrane in 34 young chihlren, Pediatrics, 74 (1984) 236-240. 14 Sheehy, J.L., Cholesteatoma surgery in chiIdren, Am. J. Otol., 6 (1985) 170-172. 15 Solomon, NE and Harris, L.J., Otitis Media in Children. Assessing the Quality of Medical Care Using Short-Term Outcome Measures. Quality of Medical Care Assessment Using Outcome Measures: Eight DiseasbSpecific Applications, Rand Corp., Sam Monica CA, 1976, p. 589. 16 Teele, D.W., Klein, J.O. and Rosner, B.A., Epidemiology of otitis media in children, Ann. Otol. Rhinol. Laryngol., 89 (1980) 5-6. 17 Tos, M., Treatment of cholesteatoma in children: a long-term study of results, Am. J. Otol., 4 (1983) 189-197.