Am J
otolaryngol
1225~258,1991
Clinical Characteristics of Cholesteatoma BJ~)RN
ABERG,
MD,
THOMAS
WESTIN, MD, PHD, ANDERS TJELLSTR~M, MD, PHD, AND STAFFANEDSTROM,MD,PHD
Clinical characteristics of 99 consecutive patients with acquired and perioperatively established aural cholesteatoma were compared with those of 81 patients with chronic otitis without cholesteatoma (controls). The patients answered a questionnaire, and preoperative and perioperative clinical observations were recorded. There were no presenting symptoms or clinical signs that significantly distinguished the cholesteatoma patients. However, these patients had a significantly higher incidence (45%) of previous middle ear surgery than the control patients (30%). The cholesteatomas were not evident until the time of surgical exploration in as many as 38% of cases. Therefore, surgical exploration appears to be the safest way to identify middle ear cholesteatoma. AM J OTOLARYNGOL 12:254-258. Copyright 0 1991 by W.B. Saunders Company Key words: symptoms, signs, cholesteatoma, chronic otitis media.
Aural cholesteatoma consists of accumulations of desquamating keratinized epithelium within the middle ear or other pneumatized portions of the temporal bone. Cholesteatoma may arise from the external epithelium of the ear canal or the tympanic membrane, or possibly result from metaplastic changes in the mucosal membranes. It is well recognized that, although cholesteatoma tissue per se is innocuous, when localized in the middle ear it is a menacing disease. A longstanding cholesteatoma frequently results in hearing loss due to erosion of the ossicular chain. Untreated, there is a risk of labyrinth and cochlear involvement resulting in vertigo and sensorineural hearing loss. Facial nerve involvement and intracranial extension, although rarely seen today, are serious complications. It is therefore important to recognize cholesteatoma disease at an early stage to prevent the complications of this destructive process with its significant proteolytic activity.le3 Complicating the task of early detection, controlled studies on the presentation of symptoms and clinical signs characteristic of cholesteatoma
disease are sparse. The present prospective study was undertaken to identify symptoms and clinical signs that would facilitate to early diagnosis. Special attention was paid to the need to distinguish established cholesteatoma disease from chronic otitis without evidence of cholesteatoma. MATERIALS
AND METHODS
Materials. In this prospective study, all patients undergoing surgery for chronic otitis during the period from 1984 to 1988 at the Department of Otolaryngology, Sahlgrenska Hospital, Gothenburg, Sweden, were investigated according to a protocol. The patient population was divided into two groups based on the perioperative finding or absence of cholesteatoma. Only those control patients who had a history of cholesteatoma disease were excluded from the study.
Received June 13,1991, from Department of Otolaryngology, Sahlgrenska Hospital, University of Gcteborg, Gothenburg, Sweden. Accepted for publication July 15,1991. Supported by grants from the Gijteborg Medical Society (318188: 2937891. The Medical Facultv of the University of &teborg (363/88; 335/89), Sahlgrenska Sjukhuset (854/88), and The Swedish Society of Medicine (871/89). Address correspondence and reprint requests to Staffan Edstr6m, MD, PhD, Department of Otolaryngology-Head and Neck Surgery, Sahlgrenska Sjukhuset, 413 45 Gdteborg, Sweden. Copyright 0 1991 by W.B. Saunders Company 0196-0709/91/1205-0004$5.00/O 254
Methods. The investigation protocol consisted of three parts. The first was a questionnaire to elucidate the hereditary and social history of the patient. The second part assessed the preoperative physical status, hearing function, and an x-ray of the ear in most cases. The third comprised the perioperative findings. The clinical evaluation of the perioperatively established cholesteatoma patient was thus performed in comparison with both the preoperative assessment and the clinical findings of the noncholesteatoma patient. Cholesteatoma was clinically defined as a whitish mass with a keratinoid appearance. In those cases in which the diagnosis of cholesteatoma was uncertain, the specimen was subjected to histopathologic analysis. The chi-square test was used to compare variables of the study and control patients. A probability value of c.01 was accepted as a statistically significant difference. RESULTS
A total of 180 patients were operated on by seven otosurgeons. Ninety-nine patients were in-
255
ABERGETAL
eluded in the cholesteatoma group and 81 in the noncholesteatoma group with chronic otitis media (cotitrols). The mean ages of these groups were 40 years (range, 5 to 71) and 42 years (range, 6 to 88), respectively. The male to female ratios were 48 to 51 and 46 to 35, respectively. Table 1 shows the prevalence of heredity. Hereditary ear disease was recorded in 26% and 22%, respectively. Prevalence rates are also shown for skin disease, infectious disease, allergy, diabetes, and hypertension. No significant difference between the cholesteatoma patients and the controls was demonstrated. Table 2 shows the prevalence of intercurrent diseases and potentially inductive conditions for the development of middle ear disease. No significant difference was recorded except for a history of sniffing (forced nasal inhalation), which was recorded in 28% and 14% respectively. An urban or rural upbringing apparently had no influence on cholesteatoma development. Approximately 50% of the patients presented with frequent symptoms compatible with ear infection in childhood, and the average age at onset of persisting symptoms was 14 and 10 years, respectively, indicating that the average duration of symptoms of ear disease was approximately 25 years before cholesteatoma was diagnosed. Regarding the specific symptoms of secretion, vertigo, and pain, it was found that approximately 25% of the patients had no history of secretion, and that continuous secretion was rare. Disabling vertigo was a rare symptom, as was persistent pain. Minor dizziness symptoms were, however, found in approximately 30% of the cases and occasional pain was fairly common. None of these symptoms was overrepresented among the cholesteatoma patients. Previous operations on the middle ear and mastoid were significantly more frequent among cholesteatoma patients than among those with chronic otitis but without cholesteatoma (45% and 30%, respectively). Table 3 illustrates the results of the audiologic, radiologic, and laboratory investigations. The extent of hearing disorders did TABLE 1. Percentage of Hereditary Factors
Among Patients With Chronic Otitis Presenting With Cholesteatoma and Without Evidence of Cholesteatoma [Controls) FREQUENCY(%) Cholesteatoma
Ear disease Skin disease Infectious disease Allergy Diabetes Hypertension
26 19 4 19 18 24
Controls 22 20 5 19 19 30
TABLE 2. Patient History of Previous and Present Symptoms and Signs in Cholesteatoma Patients and ControIs PREVALENCE(%) Cholesteatoma Skin disease Psoriasis Diabetes Hypertension Allergy as a child Allergy as an adult Congenital face defect Smoker Sniffer Otitis prone as a child Onset, mean age (yr) Disabled hearing Episode(s) of grommet usage Urban or rural upbringing (town/village/countryside) Drainage from ear (never/occasionally/continuous) Vertigo Pain Previous ear surgery Operated for cholesteatoma Cholesteatoma seen at preoperative examination
Controls
6 4 5 8
16
16 16
11 23
2 44 28* 52
0 40
14
9.8
84
80
15
16
5 5 5
14
55
14120164
27117156
2317314 64/16/O 52/47/l 45*
25/75/O 75/25/O 56/44/O 30 0
62
-
* P < .Ol.
not differ between
groups, nor did the prevalence of a reduced dimension or sclerosis of the pneumatized mastoid differ as judged by x-ray examination. However, bony defects, demonstrated in 9% of the cholesteatoma ears, were not found in any of the ears without established cholesteatoma. In a limited number of cases, computerized tomography of the middle ear was used to assess bone destruction: bone destruction was found in eight of 14 and two of seven cases, respectively. In the preoperative assessment, cholesteatoma was evident in 62% of perioperatively established cases. Table 4 shows the distribution of cholesteatoma location as recorded perioperatively. Cholesteatoma location was rather evenly distributed in the ear. In 42% of cases the cholesteatomas occupied more than one anatomic site, the antral part of the middle ear being most involved (48%). Bone destruction considered to be a result of disease was evident in 46% of cholesteatoma cases. Breaking down the 46%, fistulation of the semicircular canal was observed in 7% and incusstapedial bone destruction in 40%. Bone destruction was also seen in 44% of the control cases, with incudostapedial bone involvement in 15%. However, fistulation of the semicircular canals was not found in any of the control ears. DISCUSSION
This study was undertaken to identify the symptoms and signs, if any, that are characteristic
256
CLINICAL CHARACTERISTICS
OF CHOLESTEATOMA
TABLE 3. Audiologic, Radiologic, and LaboratoryFindings CHOLESTEATOMA (average of frequencyrange, 500 to 2,000 Hz) Bone conduction Air conduction X-ray examination (%) Large mastoid Sclerosis Bone destruction Computed tomography Bone destruction Blood tests (mean values] Hemoglobin (g/L] Sedimentation rate (mm) Leukocyte count ( X 1,000)
CONTROLS
Hearingin dB
17 dB (max 60; min - 3) 45 dB (max 105; min 2)
CHOLESTEATOMALOCATION (PEROPERATIVEOBSERVATION) Tympanic cavity Epitympanon Antrum Mastoid Tympanic cavity and atticus Typanic cavity and antrum Tympanic cavity and mastoid Atticus and antrum Antrum and mastoid Atticus, antrum, and mastoid Typanic cavity, atticus, antrum, and mastoid Bone destruction Fistuia on the semicircular canal Erosive incus and stapes
4.
18 85 19
20 78 0
8114
Z/7
148 (110-182) 7 7.8
of cholesteatoma disease, and may be used to differentiate it from chronic otitis without cholesteatoma. The study comprised 180 consecutive patients during a period of 4 years. Fifty-five percent of the patients demonstrated an established cholesteatoma perioperatively. This occurrence is in agreement with previous reports4 We were not able to demonstrate any significant sign that distinguished cholesteatoma patients from those without cholesteatoma. The mean age of both groups was approximately 40 years, and chronic otitis with and without cholesteatoma was found in all age groups. The duration of middle ear disease until the time of surgery was approximately 25 years in both groups. This supports the suggestion that acquired cholesteatoma in the pediatric age group has a more aggressive behavior than in adults.5-g Furthermore, it emphasizes that the time required for the development of cholesteatoma varies considerably and that the duration of middle ear disease is of little value in the assessment of cholesteatoma presentation. The most striking finding was that a history of previous middle ear surgery was significantly more frequent among patients with cholesteatoma than among controls. As many as 45% of the choTABLE
15 dB (max 60; min - 3) 43 dB (max 88; min 8)
148 (121-176) 7 7.1
lesteatoma patients had previously undergone surgery. This high percentage may be the result of either residual cholesteatoma or previous surgery. Unfortunately, the patient histories and previous hospital records were insufficient to allow an analysis of the surgical modality performed. However, the preliminary results of another study at our institution demonstrate a higher incidence of residual cholesteatomas when using the canal wall up, as compared with the canal wall down, technique, a finding consistent with other reports.6,10*11 It has been suggested that grommets may induce cholesteatoma development by means of keratin invasion.‘2*‘3 Although the rate and duration of grommet use were not recorded in the present study, the percentage of previous grommet users was not significantly different from control patients. However, this does not rule out the possibility that repeated grommet insertion with subsequent fibrosis and adhesions may constitute a risk of cholesteatoma development, and such patients should therefore be followed closely.12 Cholesteatoma patients had the habit of forced nasal insufflation (sniffing] more often than control patients. It is difficult to evaluate this finding
Surgical
Findings
ISOLATED CHOLESTEATOMA 18 10 13 19 5 6 2 11 10 4 4 46 7 40
COMBINATIONS
CONTROLS
35 44 48 39
44 0 15
257
ABERG ET AL
since there is certainly broad variation in such habits. Nevertheless, previous studies have shown that cleft palate children have a high risk of developing cholesteatoma14 and such patients are known to have a eustachian tube malfuncit is tempting to speculate tion.15 Accordingly, that frequent episodes of negative middle ear pressure might contribute to keratin invasion of the middle ear cavity from the ear canal and the retracted tympanic membrane. Traditional presenting symptoms such as ear discharge were not as frequent as expected.5s’6 Thus, most patients in this study had only occasional discharge, and as many as approximately 25% had no discharge at all. Furthermore, more than 50% of cholesteatoma patients presented no history of otalgia or vertigo. We therefore conclude that there are no obligatory symptoms of cholesteatoma and that hearing loss, otorrhea, otalgia, and vertigo may be present at similar frequencies in cases of chronic otitis without cholesteatoma. The preoperative assessment of chronic otitis patients includes a radiologic survey of the middle ear and mastoid. In this study, conventional x-ray examination demonstrated sclerosis and a reduced size of the pneumatized mastoid in approximately 80% of all cases, that is, both cholesteatoma and contro1 ears. Only in 9% of the cholesteatoma ears was bone destruction demonstrated by x-ray, in spite of the fact that 45% had previously been subjected to ear surgery. In the control ears, bone destruction was not observed. We therefore agree with the view, expressed elsex-ray is of limited where,17 that the conventional value in preoperative assessment. Bone destruction was more frequently recognized in the cholesteatoma cases by means of computed tomography, but it was also recognized occasionally in controls. Computed tomography is superior to conventional x-ray because, in addition to the destruction of bony structures, it has the potential to identify damage to adjacent soft tissues. It therefore appears that computed tomography may contribute to the clinical diagnosis, 17,18 but its value decreases when previous surgery of the middle ear has been undertaken.” The role of contemporary magnetic resonance imaging in the evaluation of cholesteatoma in the middle ear has not yet been established; its major contribution so far appears to be in defining intracranial pathology associated with middle ear disease.” Bone destruction was recorded during surgery in 46% of the cholesteatoma cases, but was also a frequent finding in the control ears, as has been demonstrated previously.” In the present series, bone destruction involved the ossicles, primarily
the incus, but also the stapes. The destructive process in the cholesteatoma ears is the result of a proteolytic process initiated by the capsule of the cholesteatoma,’ while the destructive process in the control ears may be explained by the proteolytic properties of longstanding inflammation, and possibly by the fact that some of these ears had undergone previous surgery. Perioperative examination of the cholesteatoma ears revealed a variety of pathologic findings. The epitympanum and antrum were the most commonly affected sites, which is consistent with other studies.5 Strikingly, in 38% of the cholesteatoma cases, the preoperative assessment did not reveal a cholesteatoma presentation. Although an ordinary preoperative, high-resolution computed tomogram would have improved the preoperative identification of cholesteatoma, we conclude that the only way to obtain a definitive diagnosis is surgical exploration. Acknowledgment. The authors thank Eva Westin for questionnaire compilation. References 1. Aberg B, Jontell M. Edstrijm S: Analysis of class II antigen expressing cells in cholesteatoma epithelium. Acta Otolaryngo1 (Stockh) 198B; 106:186-191 2. Aberg B, Bagger-Sjdblck D, EdstrBm S, et al: A comparative study of enzyme histochemical features in the gerbilline and human cholesteatoma. Acta Otolaryngol (Stockh) 1990; 109:130-136 3. Aberg B, Bagger-SjGblck D, Heyden G, et al: Bone destruction in experimental cholesteatoma. A histopathological and histochemical study on the mongolian gerbil, in Tos M, Thomsen J, Peitersen E (eds): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini, 1989, pp 49-51 4. Smyth GDL: Tympanic reconstruction. J Laryngol Otol 1976; 90:713-741 5. Edelstein DR, Parisier SC, Cheng Han J: Acquired cholesteatoma in the pediatric age group. Otolaryngol Clin North Am 1989; 22:955-966 6. Gristwood RE, Venables WN: Factors influencing the probability of residual chalesteatoma. Ann Otal Rhino1 Laryngo1 1990; 99:120-123 7. Palva A, Karma P, Karja J: Cholesteatoma in children. Arch Otolaryngol 1977; 103:74-77 8. Jansen C: Cholesteatoma in children. Clin Otolaryngol 1978; 3:349-352 9. Tos M: Treatment of cholesteatoma in children. Am J Otol 1983; 4:189-197 10. Reimer A, Andreasson L, Harris S: Surgical treatment of cholesteatoma: A comparison of closed and open techniques in a follow-up of 164 ears. Clin Otolaryngol 1987; 12447-454 11. Parisier SP: Management of cholesteatoma. Otolaryngol Clin North Am 1989; 22:927-940 12. Herdman R, Wright JLW: Grommets and cholesteatoma in children. J Laryngol Otol 1988; 102:1OOO-1002 13. Vartiainen E, Karja J, Karjalainen S: Surgery of chronic otitis media in young patients. J Laryngol Otol 1986; 100:514519 14. Harker L, Severeid L: Cholesteatoma in the cleft palate patients, in Sad6 J (ed): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler Publications, 1982, pp 37-40
258 15. Falk B, Magnusson B: Eustachian tube closing failure. Occurrence in patients with cleft palate and middle ear disease. Arch Otolaryngol 1984; 110:67-71 16. Anniko M, Mendel L: Cholesteatoma. A clinical and morphological analysis. Acta Otolaryngol 1981; 91275-283 17. Park KH, Kwon J, Park SI, et al: High-resolution computed tomography of cholesteatomatous otitis media. Significance of preoperative information, in Tos M, Thomsen J, Peitersen E (eds): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini. 1989, pp 709-715
CLINICAL CHARACTERISTICS OF CHOLESTEATOMA 18. Liu DPC, Bergeron RT: Contemporary radiologic imaging in evaluation of middle ear-attic-ax-ma1 complex cholesteatomas. Otolaryngol Clin North Am 1989; 22:897-909 19. Oberascher G, Grobovschek M, Albegger K: The use of high resolution computed tomography to exclude a cholesteatoma recurrence. Is the second-look operation necessary? in Tos M, Thomasen J, Peitersen E (eds]: Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini, 1989, pp 717-720 20. Austin D: Ossicular reconstruction. Arch Otolaryngol 1971; 94:525-535
otolaryngol
1225~258,1991
Clinical Characteristics of Cholesteatoma BJ~)RN
ABERG,
MD,
THOMAS
WESTIN, MD, PHD, ANDERS TJELLSTR~M, MD, PHD, AND STAFFANEDSTROM,MD,PHD
Clinical characteristics of 99 consecutive patients with acquired and perioperatively established aural cholesteatoma were compared with those of 81 patients with chronic otitis without cholesteatoma (controls). The patients answered a questionnaire, and preoperative and perioperative clinical observations were recorded. There were no presenting symptoms or clinical signs that significantly distinguished the cholesteatoma patients. However, these patients had a significantly higher incidence (45%) of previous middle ear surgery than the control patients (30%). The cholesteatomas were not evident until the time of surgical exploration in as many as 38% of cases. Therefore, surgical exploration appears to be the safest way to identify middle ear cholesteatoma. AM J OTOLARYNGOL 12:254-258. Copyright 0 1991 by W.B. Saunders Company Key words: symptoms, signs, cholesteatoma, chronic otitis media.
Aural cholesteatoma consists of accumulations of desquamating keratinized epithelium within the middle ear or other pneumatized portions of the temporal bone. Cholesteatoma may arise from the external epithelium of the ear canal or the tympanic membrane, or possibly result from metaplastic changes in the mucosal membranes. It is well recognized that, although cholesteatoma tissue per se is innocuous, when localized in the middle ear it is a menacing disease. A longstanding cholesteatoma frequently results in hearing loss due to erosion of the ossicular chain. Untreated, there is a risk of labyrinth and cochlear involvement resulting in vertigo and sensorineural hearing loss. Facial nerve involvement and intracranial extension, although rarely seen today, are serious complications. It is therefore important to recognize cholesteatoma disease at an early stage to prevent the complications of this destructive process with its significant proteolytic activity.le3 Complicating the task of early detection, controlled studies on the presentation of symptoms and clinical signs characteristic of cholesteatoma
disease are sparse. The present prospective study was undertaken to identify symptoms and clinical signs that would facilitate to early diagnosis. Special attention was paid to the need to distinguish established cholesteatoma disease from chronic otitis without evidence of cholesteatoma. MATERIALS
AND METHODS
Materials. In this prospective study, all patients undergoing surgery for chronic otitis during the period from 1984 to 1988 at the Department of Otolaryngology, Sahlgrenska Hospital, Gothenburg, Sweden, were investigated according to a protocol. The patient population was divided into two groups based on the perioperative finding or absence of cholesteatoma. Only those control patients who had a history of cholesteatoma disease were excluded from the study.
Received June 13,1991, from Department of Otolaryngology, Sahlgrenska Hospital, University of Gcteborg, Gothenburg, Sweden. Accepted for publication July 15,1991. Supported by grants from the Gijteborg Medical Society (318188: 2937891. The Medical Facultv of the University of &teborg (363/88; 335/89), Sahlgrenska Sjukhuset (854/88), and The Swedish Society of Medicine (871/89). Address correspondence and reprint requests to Staffan Edstr6m, MD, PhD, Department of Otolaryngology-Head and Neck Surgery, Sahlgrenska Sjukhuset, 413 45 Gdteborg, Sweden. Copyright 0 1991 by W.B. Saunders Company 0196-0709/91/1205-0004$5.00/O 254
Methods. The investigation protocol consisted of three parts. The first was a questionnaire to elucidate the hereditary and social history of the patient. The second part assessed the preoperative physical status, hearing function, and an x-ray of the ear in most cases. The third comprised the perioperative findings. The clinical evaluation of the perioperatively established cholesteatoma patient was thus performed in comparison with both the preoperative assessment and the clinical findings of the noncholesteatoma patient. Cholesteatoma was clinically defined as a whitish mass with a keratinoid appearance. In those cases in which the diagnosis of cholesteatoma was uncertain, the specimen was subjected to histopathologic analysis. The chi-square test was used to compare variables of the study and control patients. A probability value of c.01 was accepted as a statistically significant difference. RESULTS
A total of 180 patients were operated on by seven otosurgeons. Ninety-nine patients were in-
255
ABERGETAL
eluded in the cholesteatoma group and 81 in the noncholesteatoma group with chronic otitis media (cotitrols). The mean ages of these groups were 40 years (range, 5 to 71) and 42 years (range, 6 to 88), respectively. The male to female ratios were 48 to 51 and 46 to 35, respectively. Table 1 shows the prevalence of heredity. Hereditary ear disease was recorded in 26% and 22%, respectively. Prevalence rates are also shown for skin disease, infectious disease, allergy, diabetes, and hypertension. No significant difference between the cholesteatoma patients and the controls was demonstrated. Table 2 shows the prevalence of intercurrent diseases and potentially inductive conditions for the development of middle ear disease. No significant difference was recorded except for a history of sniffing (forced nasal inhalation), which was recorded in 28% and 14% respectively. An urban or rural upbringing apparently had no influence on cholesteatoma development. Approximately 50% of the patients presented with frequent symptoms compatible with ear infection in childhood, and the average age at onset of persisting symptoms was 14 and 10 years, respectively, indicating that the average duration of symptoms of ear disease was approximately 25 years before cholesteatoma was diagnosed. Regarding the specific symptoms of secretion, vertigo, and pain, it was found that approximately 25% of the patients had no history of secretion, and that continuous secretion was rare. Disabling vertigo was a rare symptom, as was persistent pain. Minor dizziness symptoms were, however, found in approximately 30% of the cases and occasional pain was fairly common. None of these symptoms was overrepresented among the cholesteatoma patients. Previous operations on the middle ear and mastoid were significantly more frequent among cholesteatoma patients than among those with chronic otitis but without cholesteatoma (45% and 30%, respectively). Table 3 illustrates the results of the audiologic, radiologic, and laboratory investigations. The extent of hearing disorders did TABLE 1. Percentage of Hereditary Factors
Among Patients With Chronic Otitis Presenting With Cholesteatoma and Without Evidence of Cholesteatoma [Controls) FREQUENCY(%) Cholesteatoma
Ear disease Skin disease Infectious disease Allergy Diabetes Hypertension
26 19 4 19 18 24
Controls 22 20 5 19 19 30
TABLE 2. Patient History of Previous and Present Symptoms and Signs in Cholesteatoma Patients and ControIs PREVALENCE(%) Cholesteatoma Skin disease Psoriasis Diabetes Hypertension Allergy as a child Allergy as an adult Congenital face defect Smoker Sniffer Otitis prone as a child Onset, mean age (yr) Disabled hearing Episode(s) of grommet usage Urban or rural upbringing (town/village/countryside) Drainage from ear (never/occasionally/continuous) Vertigo Pain Previous ear surgery Operated for cholesteatoma Cholesteatoma seen at preoperative examination
Controls
6 4 5 8
16
16 16
11 23
2 44 28* 52
0 40
14
9.8
84
80
15
16
5 5 5
14
55
14120164
27117156
2317314 64/16/O 52/47/l 45*
25/75/O 75/25/O 56/44/O 30 0
62
-
* P < .Ol.
not differ between
groups, nor did the prevalence of a reduced dimension or sclerosis of the pneumatized mastoid differ as judged by x-ray examination. However, bony defects, demonstrated in 9% of the cholesteatoma ears, were not found in any of the ears without established cholesteatoma. In a limited number of cases, computerized tomography of the middle ear was used to assess bone destruction: bone destruction was found in eight of 14 and two of seven cases, respectively. In the preoperative assessment, cholesteatoma was evident in 62% of perioperatively established cases. Table 4 shows the distribution of cholesteatoma location as recorded perioperatively. Cholesteatoma location was rather evenly distributed in the ear. In 42% of cases the cholesteatomas occupied more than one anatomic site, the antral part of the middle ear being most involved (48%). Bone destruction considered to be a result of disease was evident in 46% of cholesteatoma cases. Breaking down the 46%, fistulation of the semicircular canal was observed in 7% and incusstapedial bone destruction in 40%. Bone destruction was also seen in 44% of the control cases, with incudostapedial bone involvement in 15%. However, fistulation of the semicircular canals was not found in any of the control ears. DISCUSSION
This study was undertaken to identify the symptoms and signs, if any, that are characteristic
256
CLINICAL CHARACTERISTICS
OF CHOLESTEATOMA
TABLE 3. Audiologic, Radiologic, and LaboratoryFindings CHOLESTEATOMA (average of frequencyrange, 500 to 2,000 Hz) Bone conduction Air conduction X-ray examination (%) Large mastoid Sclerosis Bone destruction Computed tomography Bone destruction Blood tests (mean values] Hemoglobin (g/L] Sedimentation rate (mm) Leukocyte count ( X 1,000)
CONTROLS
Hearingin dB
17 dB (max 60; min - 3) 45 dB (max 105; min 2)
CHOLESTEATOMALOCATION (PEROPERATIVEOBSERVATION) Tympanic cavity Epitympanon Antrum Mastoid Tympanic cavity and atticus Typanic cavity and antrum Tympanic cavity and mastoid Atticus and antrum Antrum and mastoid Atticus, antrum, and mastoid Typanic cavity, atticus, antrum, and mastoid Bone destruction Fistuia on the semicircular canal Erosive incus and stapes
4.
18 85 19
20 78 0
8114
Z/7
148 (110-182) 7 7.8
of cholesteatoma disease, and may be used to differentiate it from chronic otitis without cholesteatoma. The study comprised 180 consecutive patients during a period of 4 years. Fifty-five percent of the patients demonstrated an established cholesteatoma perioperatively. This occurrence is in agreement with previous reports4 We were not able to demonstrate any significant sign that distinguished cholesteatoma patients from those without cholesteatoma. The mean age of both groups was approximately 40 years, and chronic otitis with and without cholesteatoma was found in all age groups. The duration of middle ear disease until the time of surgery was approximately 25 years in both groups. This supports the suggestion that acquired cholesteatoma in the pediatric age group has a more aggressive behavior than in adults.5-g Furthermore, it emphasizes that the time required for the development of cholesteatoma varies considerably and that the duration of middle ear disease is of little value in the assessment of cholesteatoma presentation. The most striking finding was that a history of previous middle ear surgery was significantly more frequent among patients with cholesteatoma than among controls. As many as 45% of the choTABLE
15 dB (max 60; min - 3) 43 dB (max 88; min 8)
148 (121-176) 7 7.1
lesteatoma patients had previously undergone surgery. This high percentage may be the result of either residual cholesteatoma or previous surgery. Unfortunately, the patient histories and previous hospital records were insufficient to allow an analysis of the surgical modality performed. However, the preliminary results of another study at our institution demonstrate a higher incidence of residual cholesteatomas when using the canal wall up, as compared with the canal wall down, technique, a finding consistent with other reports.6,10*11 It has been suggested that grommets may induce cholesteatoma development by means of keratin invasion.‘2*‘3 Although the rate and duration of grommet use were not recorded in the present study, the percentage of previous grommet users was not significantly different from control patients. However, this does not rule out the possibility that repeated grommet insertion with subsequent fibrosis and adhesions may constitute a risk of cholesteatoma development, and such patients should therefore be followed closely.12 Cholesteatoma patients had the habit of forced nasal insufflation (sniffing] more often than control patients. It is difficult to evaluate this finding
Surgical
Findings
ISOLATED CHOLESTEATOMA 18 10 13 19 5 6 2 11 10 4 4 46 7 40
COMBINATIONS
CONTROLS
35 44 48 39
44 0 15
257
ABERG ET AL
since there is certainly broad variation in such habits. Nevertheless, previous studies have shown that cleft palate children have a high risk of developing cholesteatoma14 and such patients are known to have a eustachian tube malfuncit is tempting to speculate tion.15 Accordingly, that frequent episodes of negative middle ear pressure might contribute to keratin invasion of the middle ear cavity from the ear canal and the retracted tympanic membrane. Traditional presenting symptoms such as ear discharge were not as frequent as expected.5s’6 Thus, most patients in this study had only occasional discharge, and as many as approximately 25% had no discharge at all. Furthermore, more than 50% of cholesteatoma patients presented no history of otalgia or vertigo. We therefore conclude that there are no obligatory symptoms of cholesteatoma and that hearing loss, otorrhea, otalgia, and vertigo may be present at similar frequencies in cases of chronic otitis without cholesteatoma. The preoperative assessment of chronic otitis patients includes a radiologic survey of the middle ear and mastoid. In this study, conventional x-ray examination demonstrated sclerosis and a reduced size of the pneumatized mastoid in approximately 80% of all cases, that is, both cholesteatoma and contro1 ears. Only in 9% of the cholesteatoma ears was bone destruction demonstrated by x-ray, in spite of the fact that 45% had previously been subjected to ear surgery. In the control ears, bone destruction was not observed. We therefore agree with the view, expressed elsex-ray is of limited where,17 that the conventional value in preoperative assessment. Bone destruction was more frequently recognized in the cholesteatoma cases by means of computed tomography, but it was also recognized occasionally in controls. Computed tomography is superior to conventional x-ray because, in addition to the destruction of bony structures, it has the potential to identify damage to adjacent soft tissues. It therefore appears that computed tomography may contribute to the clinical diagnosis, 17,18 but its value decreases when previous surgery of the middle ear has been undertaken.” The role of contemporary magnetic resonance imaging in the evaluation of cholesteatoma in the middle ear has not yet been established; its major contribution so far appears to be in defining intracranial pathology associated with middle ear disease.” Bone destruction was recorded during surgery in 46% of the cholesteatoma cases, but was also a frequent finding in the control ears, as has been demonstrated previously.” In the present series, bone destruction involved the ossicles, primarily
the incus, but also the stapes. The destructive process in the cholesteatoma ears is the result of a proteolytic process initiated by the capsule of the cholesteatoma,’ while the destructive process in the control ears may be explained by the proteolytic properties of longstanding inflammation, and possibly by the fact that some of these ears had undergone previous surgery. Perioperative examination of the cholesteatoma ears revealed a variety of pathologic findings. The epitympanum and antrum were the most commonly affected sites, which is consistent with other studies.5 Strikingly, in 38% of the cholesteatoma cases, the preoperative assessment did not reveal a cholesteatoma presentation. Although an ordinary preoperative, high-resolution computed tomogram would have improved the preoperative identification of cholesteatoma, we conclude that the only way to obtain a definitive diagnosis is surgical exploration. Acknowledgment. The authors thank Eva Westin for questionnaire compilation. References 1. Aberg B, Jontell M. Edstrijm S: Analysis of class II antigen expressing cells in cholesteatoma epithelium. Acta Otolaryngo1 (Stockh) 198B; 106:186-191 2. Aberg B, Bagger-Sjdblck D, EdstrBm S, et al: A comparative study of enzyme histochemical features in the gerbilline and human cholesteatoma. Acta Otolaryngol (Stockh) 1990; 109:130-136 3. Aberg B, Bagger-SjGblck D, Heyden G, et al: Bone destruction in experimental cholesteatoma. A histopathological and histochemical study on the mongolian gerbil, in Tos M, Thomsen J, Peitersen E (eds): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini, 1989, pp 49-51 4. Smyth GDL: Tympanic reconstruction. J Laryngol Otol 1976; 90:713-741 5. Edelstein DR, Parisier SC, Cheng Han J: Acquired cholesteatoma in the pediatric age group. Otolaryngol Clin North Am 1989; 22:955-966 6. Gristwood RE, Venables WN: Factors influencing the probability of residual chalesteatoma. Ann Otal Rhino1 Laryngo1 1990; 99:120-123 7. Palva A, Karma P, Karja J: Cholesteatoma in children. Arch Otolaryngol 1977; 103:74-77 8. Jansen C: Cholesteatoma in children. Clin Otolaryngol 1978; 3:349-352 9. Tos M: Treatment of cholesteatoma in children. Am J Otol 1983; 4:189-197 10. Reimer A, Andreasson L, Harris S: Surgical treatment of cholesteatoma: A comparison of closed and open techniques in a follow-up of 164 ears. Clin Otolaryngol 1987; 12447-454 11. Parisier SP: Management of cholesteatoma. Otolaryngol Clin North Am 1989; 22:927-940 12. Herdman R, Wright JLW: Grommets and cholesteatoma in children. J Laryngol Otol 1988; 102:1OOO-1002 13. Vartiainen E, Karja J, Karjalainen S: Surgery of chronic otitis media in young patients. J Laryngol Otol 1986; 100:514519 14. Harker L, Severeid L: Cholesteatoma in the cleft palate patients, in Sad6 J (ed): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler Publications, 1982, pp 37-40
258 15. Falk B, Magnusson B: Eustachian tube closing failure. Occurrence in patients with cleft palate and middle ear disease. Arch Otolaryngol 1984; 110:67-71 16. Anniko M, Mendel L: Cholesteatoma. A clinical and morphological analysis. Acta Otolaryngol 1981; 91275-283 17. Park KH, Kwon J, Park SI, et al: High-resolution computed tomography of cholesteatomatous otitis media. Significance of preoperative information, in Tos M, Thomsen J, Peitersen E (eds): Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini. 1989, pp 709-715
CLINICAL CHARACTERISTICS OF CHOLESTEATOMA 18. Liu DPC, Bergeron RT: Contemporary radiologic imaging in evaluation of middle ear-attic-ax-ma1 complex cholesteatomas. Otolaryngol Clin North Am 1989; 22:897-909 19. Oberascher G, Grobovschek M, Albegger K: The use of high resolution computed tomography to exclude a cholesteatoma recurrence. Is the second-look operation necessary? in Tos M, Thomasen J, Peitersen E (eds]: Cholesteatoma and Mastoid Surgery. Amsterdam, The Netherlands, Kugler & Ghedini, 1989, pp 717-720 20. Austin D: Ossicular reconstruction. Arch Otolaryngol 1971; 94:525-535
