INCIDENCE OF MIDDLE-EAR CHOLESTEATOMA WITH ANALYSIS OF ITS LOCATIONS, EXTENSIONS, AND COMPLICATIONS FROM 1993 TO 2009

Suzan Al Kole, Peter Florentin Nieland, Morten Søvsø, Kjell Tveterås, Michael Gaihede

Department of Otolaryngology, Head & Neck Surgery, Aarhus University Hospital, Aalborg, Denmark

Introduction

Middle-ear (ME) cholesteatoma represents a relatively common and clinically challenging form of chronic otitis media (OM). Formation of a cholesteatoma is associated with atrophic parts in the tympanic membrane, which form the basis of a retraction pocket together with negative ME pressure related to poor function of Eustachian tube and/or the mastoid. Retraction pockets may form in the pars flaccida, in the sinus or in the pars tensa of the TM, and they frequently result in accumulation of cellular debris, which causes recurrent infection with otorrhea and invasion of the ME cavity and neighboring areas with erosion of bony structures.13

Studies on the incidence rate (IR) of ME cholesteatomas are relatively scarce. In older studies, an IR between 2.8 and 13.2 per 100,000 inhabitants has been reported.4,5 More studies have investigated the hypotheses that the IR has been decreasing during the last two decades, and that such decrease may be related to a simultaneous increase in the usage of ventilation tubes (VT) during childhood OM. More of these studies have documented a significant decrease in the IR of cholesteatomas together with an increase in the numbers of VT insertions,68 whereas one study finds the IR unchanged.5

In our department we also have had the impression of a decrease in the occurrence of ME cholesteatomas referred for surgical treatments over the recent years, but in addition we also have felt that these cases may have been less extensive and with less complications than earlier. If such observations are related to childhood VT insertions, it should be noticeable in our department, since we seem to have the highest IR of VT insertions reported in the literature, amounting to 375 procedures per 10,000 children per year (< 16 years);9 this IR is around six to seven times higher than comparable countries like Norway and Finland.10,11 Therefore, the purpose of this study was to determine the IR of primary ME cholesteatomas in our region during the last 17 years including an analysis of their locations, extensions and complications.

Material and methods

The study was based on a longitudinal retrospective survey of primary acquired ME cholesteatoma from January 1, 1993 to December 31, 2009. Information about these surgeries was obtained from our department’s otosurgical database, which has been used since 1993 for complete prospective registration of all otosurgical procedures; information was occasionally supplemented by reviewing patient records. The information retrieved contained the number of primary cholesteatoma procedures per year, their locations and local extensions as well as related complications.

Our department is the only one in its specialty serving the North Denmark Region, hence all cholesteatomas were referred to our Department. The North Denmark Region has the merit of a relatively stable population with approximately 600,000 inhabitants; we obtained data about the exact population sizes for all years for both adults and children from Denmark’s Statistics.12 Conventional descriptive statistics were used for description of the basic data, whereas the IR of cholesteatoma was investigated by correlation analysis.

Results

Cholesteatoma incidence

Based on the population the overall mean annual IR was determined at 8.75 new cases per 100,000 inhabitants (range 4.5 to 12.3) (95% CI: -7.68 to 5.56). The overall annual IR during the study period has been illustrated in Figure 1 displaying a statically significant decrease (p = 0.016, r = 0.33; N = 756).

image

Fig. 1. The overall annual incidence rate (IR) per 100,000 inhabitants from 1993 to 2009. The straight line indicates the regression line; correlation analysis shows a statistically significant decrease during the period (p = 0.016, r = 0.33; N = 756).

Locations and extensions

Overall flaccida or attic cholesteatoma was found in 298 cases (39.3%), sinus cholesteatoma in 216 cases (28.8 %), and tensa cholesteatoma in 156 cases (20.6 %). In 85 cases (11.3 %) the origin of the cholesteatoma could not be assessed (mixed cholesteatoma).

The three types of cholesteatomas with well-defined origin showed different regional extensions, so that for instance sinus and tensa cholesteatomas were predominantly restricted to the ME cavity (58% and 63%, respectively), whereas flaccida cholesteatoma showed a more dispersed pattern of growth. These regional extensions have been illustrated in Figure 2. There were no systematic changes in the locations and extensions of the cholesteatomas during the study period.

Complications

Complications encountered before or during surgery comprised of ossicular erosions with affection of the crus longum in 50% of the cases; additional ossicular erosions included the stapes supra-structure in 21% of these. A separate analysis of these erosions showed no systematic variation during the study period. However, various more rare complications were found; these have been outlined in Table 1.

In total 114 of these complications were encountered in 86 cases; thus, the overall frequency of one or more of these complications was 86/756 (11%). The annual numbers of the individual complications were few, and it was not meaningful to investigate these separately; thus, the overall number of complications per year was analyzed during the study period and related to the numbers of surgeries per year; there was a significant decrease over time in the frequency of the overall complications per year (p < 0.05, r = 0.255; N = 756).

image

Fig. 2. Distribution of the extensions of sinus, flaccida and tensa cholesteatomas into adjacent regions of the ME and mastoid cavity.

Table 1. Complications of cholesteatomas

Complication type

Cases (%)

Facial nerve or dura exposure

90 (79.9)

Fistula (semicircular canal, cochlea, footplate)

17 (14.7)

Facial paralysis

4 (3.7)

Acute mastoiditis

2 (1.8)

Intracranial abscess and/or meningitis

1 (0.9)

Sinus thrombosis

0 (0)

Deafness

0 (0)

Total

114

The total number of complications appeared in 86 cases; thus, the risk of one or more complications was 86/756 = 0.11.

Discussion

Cholesteatoma incidence and VT’s

VT insertion is still one of the most frequently performed procedures in otology. Insertions of VT’s often results in permanent TM perforations; other complications include otorrhea, tympanosclerosis, and formation of cholesteatoma.13,14 In many countries there has been an increasing rate of VT insertions during the last 30 years despite the fact that its justification remains rather controversial, because its long-term effects have not been documented. During the same period more studies have focused on possible changes in the IR of cholesteatomas related to the usage of VT’s.

A number of studies have described an increased risk of cholesteatoma arising behind an intact TM or in a perforation of the pars tensa resulting from previous VT insertion; the IR of these secondary developed cholesteatoma are ranging from 0.48 to 1.1%.1517 However, other studies have shown a decline in incidence of cholesteatoma formation despite multiple VT insertions.68 Further, some studies have been unable to demonstrate any changes in the IR of cholesteatoma, despite dramatic increases in the use of VT’s.5,1820

In a recent, larger epidemiological study, Spillsbury et al. reported that insertion of VT at an early age, insertion of subsequent VT without any delay and adenoid removal were related to a reduced rate of choles-teatoma development.16 Whereas epidemiological results may not prove causal relations, it may still indicate some connections.

In our country we have a very high incidence of VT insertions, around 375 procedures per 10,000 children per year (< 16 years), and at the same time we found a statistically significant decrease in the incidences of cholesteatoma; similar results have been reported by Djurhuus et al. 21 The insertion of VT’s tends to ensure a better development of the mastoid,22 and the mastoid has been shown to play an important part in ME pressure regulation distinct from the Eustachian tube.23 Thus, VT insertions may contribute to a normal development of the mastoid which may ensure a normal pressure regulation in later life, and thus prevent underpressures resulting in retraction pockets and formation of cholesteatomas. This basic explanation is supported by the epidemiological findings by Spillsbury.16

Location and extensions

The majority of cholesteatomas involved the pars flaccida (48%), whereas the tensa (20.6%) and sinus choles-teatoma (28.8%) were less common. Tensa and sinus cholesteatomas presented with a mass in the tympanic cavity and only a few percent had epitympanic spread (Fig. 2). Flaccida cholesteatoma had more tendencies to spread into both the ME and the antrum including the mastoid, and thus, it showed a more dispersed pattern of growth. However, the size of the cholesteatomas as well as their locations has been stable during the study period.

Complications

The most common complication found was erosion of the crus longum in 50% of the cases, followed by additional erosion of the stapes supra-structure in 21%. The ossicular chain pathology did not change during the study period. However, this meant that ossicular chain reconstruction was very often required. In accordance, ossicular destructions have previously been reported to be the most common complication in cholesteatomas.24 Thus, an intact ossicular chain has been described in only 26% of flaccida cholesteatomas; the most affected part is the crus longum, followed by the incus body and the malleus head.24

One of the less frequent complications of ME cholesteatoma is labyrinthine fistula, which has been reported in 3.6–15% of cases.25,26 A fistula may not show symptoms or signs, but it poses a risk of sensory-neural hearing loss; our findings of 14.7% of cases with complications corresponded overall to 17 among 756 cases, i.e., 2.2%. A previous study from our department reported a higher rate of labyrinthine fistula (8.9%) during 1979 to 1990;27 this may corroborate our overall findings that complications seemed to decrease over the study period. We encountered no cases with deafness before or after surgery. Altogether, we found various complications in only 11% of the cases, and on an overall basis this number showed a significant decline during the study period.

References

1.Michaels L. Biology of cholesteatoma. Otolaryngol Clin North Am 22:869–881, 1989

2.Sudhoff H, Tos M. Pathogenesis of attic cholesteatoma: Clinical and immuno-histochemical support for combination of the retraction and proliferation theory. Am J Otol 21:786–782, 2000

3.Semaan MT, Megerian CA. The pathophysiology of cholesteatoma. Otolaryngol Clin North Am 39:1143, 2006

4.Tos M. A New Pathogenesis of Mesotympanic (Congenital) Cholesteatoma. Laryngoscope 110:1890–1897, 2000

5.Padgham N, Mills R, Christmas H. Has the increasing use of grommets influenced the frequency of surgery for cholesteatoma? J Laryngol Otol 105:1034–1035, 1989

6.Thomson, ISD. Exudative otitis media, grommets and cholesteatoma. J Laryngol Otol 88:947–953, 1974

7.Roland NJ, Phillips DE, Rogers JH, Singh SD. The use of ventilation tubes and the incidence of cholesteatoma surgery in the paediatric population of Liverpool. Clin Otolaryngol 17:437–439, 1992

8.Rakover Y, Keywan K, Rosen G. Comparison of the incidence of cholesteatoma surgery before and after using ventilation tubes for secretory otitis media. Int J Pediatr Otorhinolaryngol 56(1):41–44, 2000

9.Jespersen JBB, Bruhn MA, Tveterås K, Nørgaard M, Nielsen RB, Gaihede M. Incidence of ventilation tube treatments. The largest number of VT insertions in the world? In: Proceedings of the 10th International Symposium on Recent Advances in Otitis Media, New Orleans, USA. Hamilton, Ontario, Canada: BC Decker, in press, 2013

10.Karevold G, Haapkylä J, Pitkäranta A, Nafstad P, Kvaerner KJ. Paediatric otitis media surgery in Norway. Acta Otolaryngol 127:29–33, 2007

11.Haapkylä J, Karevold G, Kvaerner KJ, Pitkäranta A. Finnish adenoidectomy and tympanostomy rates in children; national variation. Int J Pediatr Otorhinolaryngol 70:1569–1573, 2006

12.Http://www.statistikbanken.dk/bef1a

13.Riley DN, Herberger S, McBride G, Law K. Myringotomy and ventilation tube insertion: a ten-year follow-up. J Laryngol Otol 111:257–261, 1997

14.Mangat KS, Morrison GAJ, Ganniwalla TM. T-tubes: a retrospective review of 1274 insertions over a 4-year period. Int J Pediatr Otorhinolaryngol 25:119–125, 1993

15.Golz A, Goldenberg D, Netzer A, Westerman L, Westerman S , Fradis M, Joachims HZ. Cholesteatomas associated with ventilation tube insertion. Arch Otolaryngol Head Neck Surg 125:754–757, 1999

16.Spillsbury K, Miller I, Semmens JB, Lannigan FJ. Factors associated with developing cholesteatoma: a study of 45,980 children with middle ear disease. Laryngoscope 120:625–630, 2010

17.Al Anazy FH. Iatrogenic cholesteatoma in children with OME in a training program. Int J Pediatr Otorhinolaryngol 70:1683–1686, 2006

18.Pappas JJ. Middle ear ventilation tubes. Laryngoscope 84:1098, 1974

19.Leek JH. Middle ear ventilation in conjunction with adenotonsillectomy. Laryngoscope 89:1760–1763, 1979

20.Kinsella JB. Ventilation tubes and cholesteatoma. Ir Med J 89:223, 1996

21.Djurhuus BD, Faber CE, Skytthe A. Decreasing incidence rate for surgically treated middle ear cholesteatoma in Denmark 1997–2007. Dan Med Bul 57(10):1–5, 2010

22.Valtonen HJ, Dietz A, Qvarnberg YH, Nuutinen J. Development of mastoid air cell system in children treated with ventilation tubes for early-onset otitis media: a prospective radiographic 5-year follow-up study. Laryngoscope 115:268–273, 2005

23.Gaihede M, Dirckx JJJ, Jacobsen H, Aernouts JEF, Søvsø M, Tveterås K. Middle ear pressure regulation – Complementary active action of the mastoid and the Eustachian tube. Otol Neurotol 31:603–611, 2010

24.Swartz JD. Colesteatomas of the middle ear. Diagnosis Etiology and Complications. Radiol Clin North Am 22:15–34, 1984

25.Watanabe N, Bundo J, Mogi G. Surgical management of labyrinthine fistula in chronic otitis media with cholesteatoma. In: Na-kado Y (ed.), Proceedings of the 4th International Conference Nigata, Japan on Cholesteatoma and mastoid surgery, pp 547–550. Amsterdam: Kugler Publications, 1992

26.Manolidis SL, Themelis CS, Manolidis LS. Comparative epidemiological study of the pathophysiology of cholesteatomas. In: Nakado Y (ed.), Proceedings of the 4th International Conference Nigata, Japan on Cholesteatoma and mastoid surgery, pp 237–240. Amsterdam: Kugler Publications, 1992

27.Rosborg J. Fistula of the labyrinth as a complication of cholesteatoma. In: Nakado Y (ed.), Proceedings of the 4th International Conference Nigata, Japan on Cholesteatoma and mastoid surgery, pp 541–545. Amsterdam: Kugler Publications, 1992


Address for correspondence: Suzan Al Kole, MD, Department of Otolaryngology-Head and Neck Surgery, Aarhus University Hospital, Hobrovej 18–22, DK-9000 Aalborg, Denmark. suak@rn.dk

Cholesteatoma and Ear Surgery – An Update, pp. 297–301

Edited by Haruo Takahashi

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