CHOLESTEATOMA IN CHILDREN WITH CLEFT PALATE – DOES ROUTINARY TYMPANOSTOMY MAKE ANY DIFFERENCE?

Martin A. Fernandez

Department of Otolaryngology, Clinica de Marly, Bogota DC, Colombia

Introduction

Reports in the literature establish a higher incidence of otologic abnormalities (cholesteatoma, acute otitis media, retraction, tympanosclerosis, perforation and middle-ear effusion) among cleft palate patients.13 Since ear pathology plays a crucial role in language, speech and educational development, regular otologic care is more than desirable. It is a wide accepted statement that patients with cleft palate have a Eustachian-tube dysfunction due to muscular abnormalities. It seems logical to establish a middle-ear aeration inserting tubes through the tympanic membrane to serve as pressure equalizer.4 However, recent studies suggest a secondary role of Eustachian tube dysfunction in the genesis of the cholesteatoma5 and the routine placement of ventilation tubes in cleft-palate patients remains controversial.68 In the current climate of limited health-care funding, evidence of clinical and cost effectiveness is becoming increasingly necessary.

The objective of this study present study was to determine the incidence of ear problems in the cleft palate population and the long-term effectiveness of tympanostomy among these patients.

Methods

Study design

This was a prospective, controlled, open clinical trial conducted between August 1998 and December 2011. The study was approved by the Institutional Board of the Operation Smile Foundation Colombia.

Selection of patients and controls

A cohort of 151 patients born with cleft palate was included in this study; all of them underwent ENT evaluation before primary palatoplasty was performed. According to the reconstructive-surgery protocol, the first surgery should be done after the age of one year, when the child has reached enough weight and maturity of the pharynx tissues. However, in some unfortunate cases, patients from distant rural areas are evaluated as late as eight years old.

Sixty-three patients (126 ears) underwent preventive bilateral tympanostomy at the time of the primary palatoplasty. A Paparella No. 2 or Sheehy-type tube (Medtronic-Xomed, Jacksonville, FL) is placed in a normal area of the membrane, usually through an incision in the anterior-superior quadrant because the epithelial migration is, in this part of the tympanic drum, the slowest to allow a late extrusion of the tube 9. T-type tubes are not used currently due to a higher incidence of persistent perforation and a negative impact in the conductive hearing level that we have seen in a previous group of patients. During a period of at least ten years, a year-base follow up is conducted and a replacement of the tube is done every time we identified the extrusion of the previous. These patients are called the ‘Tympanostomy group’.

In 88 patients (176 ears), no surgical treatment for the ear was proposed and a year-base follow up was performed. This group of patients is called the ‘Control Group’.

Patients with a history of general or neurological disease were excluded. All the patients belong to the lowest socio-economical levels in Colombia, where a lack of medical resources is evident.

Outcome measures

At the time of the last evaluation (at least ten years follow up), a record of the next information was done:

Otoscopy findings: This information described the oto-microscope evaluation and the information analyzed were: acceptable otoscopy, tympanic retraction, effusion, perforation and cholesteatoma;

Hearing Assessment: Audiometric studies included pure-tone thresholds and SRTs, with special focus in the air-bone gap;

Number of acute otitis media episodes: Parents are previously requested to record every episode of ear infection determined either by the pediatrician, general physician or the otolaryngologist;

Number of tubes: Describes the number of surgical procedures to replace the tube in the tympanostomy-group patients.

After completion of studies in all patients, differences and their significance were determined and calculated among both groups.

Results

Bilateral tympanostomy as a routine procedure was used in 63 patients (38 male and 25 female), ranging in age at the time of the last evaluation from 11 to 19 years, with a mean of 13.1 years. The mean follow up was 11.3 years (range, 10.1 y-13.2 y).

Eighty-eight patients (50 male and 38 female) were used as a control group and no tympanostomy was performed. The age at the end of the study ranged from 11 to 20 years (mean 14.5). The mean follow up was 11.1 years (range, 10.1–12.9 y).

Patients who had been lost to follow up were excluded from the present study.

Each patient in this study underwent microscope examination of the ear and hearing screening at least at the end of the follow up. In only a few patients a CT scan was performed, so those data are not included in the present study.

When describing the appearance of the middle ear, there is a group of patients with an acceptable otoscopy encompassing findings of tympanosclerosis and mild retraction (I and II according to the Sáde classification) with no clinical and audiological relevance. In such cases, further treatment was not indicated in the opinion of the author.

Figure 1 shows the results of the otoscopic findings in both groups, suggesting better outcomes in the tympanostomy group. Figure 2 suggest a better long-term outcome in terms of middle-ear function in patients with prophylactic tympanostomy. However, there is a slight but significant difference in the incidence of cholesteatoma, indicating a more favorable outcome in the control group. Figure 3 reveals an important decrease in the number of acute otitis media episodes in cleft-palate patients with a routine tympanostomy, during the first decade of their life. The average number of tubes required in these patients was 2.8 over a ten-years period of time.

Discussion

The face is developed from five processes which fuse with one another. Development disturbances of these processes appear as cleft. These clefts may occur in a wide variety of types, the most common of which is the cleft lip and palate. Complete clefts of the palate may occur on the left or right side or also bilaterally in different degrees of severity; bilateral total cleft is the most extreme form. All these patients suffer an abnormal insertion of the levator and tensor veli palatini muscles into the posterior margin of the hard palate, which are responsible for the Eustachian-tube function. As a consequence, the middle ear is not aerated and a vacuum process begins inciting an inflammatory reaction in the middle ear and tympanic membrane. During the last century, several authors have suggested the role of retraction pockets and secondary bacterial inflammation in the pathogenesis of acquired cholesteatoma.10,11 In an attempt to aerate the middle ear, tubes are inserted through the tympanic membrane to serve as pressure equalizer and prevent middle-ear pathologies. However, recently, Karmody12 reviewing the pathogenesis of acquired cholesteatoma, suggested a secondary role of retraction pockets supporting the hypothesis of the medial migration of squamous epithelium from tympanic membrane. Some other authors have reported similar findings that agree with a secondary role of the Eustachian tube dysfunction in the origin of cholesteatomas.1315

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Fig. 1. Percentage of abnormal ear findings.

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Fig. 2. Air-bone gap and cholesteatoma incidence.

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Fig. 3. Number of acute otitis-media episodes during a ten-year period.

Children born with cleft palate are an in-vivo and excellent model to observe the long-term consequences of the Eustachian-tube dysfunction. The prevalence of cleft palate in a general population varies from 0.5 to 2.5 in 1000 births.

Our study population is a group of cleft-palate patients observed for the author for more than ten years in order to establish the ear pathologies during their childhood. Another objective of the present study was to evidence the benefits and drawbacks of the routine tympanostomy, with special focus on cholesteatoma.

We found enough clinical evidence to suggest that a tympanostomy in cleft-palate patients, significantly reduces the incidence of middle-ear effusion, tympanic-membrane retraction, perforation and acute otitis media episodes. Our results showed a trend toward reduction of conductive hearing loss in cleft-palate patients with ventilation tubes. However, the incidence of cholesteatoma in our study population was higher in the tympanostomy group, supporting the hypothesis of a secondary role of Eustachian-tube dysfunction in the genesis of cholesteatoma and addressing the question of a possible iatrogenic role of the ventilation tubes.16

The author suggests that the ventilation tube placement decision should be taken case by case on the basis of recurrent infection, hearing loss or evidence of abnormalities detected in a regular follow up combined with the surgeon’s seasoned judgment, depending on anatomic, pathologic and socio-economic conditions.

References

1.Bluestone CD. Eustachian tube obstruction in the infant with cleft palate. Ann Otol Rhinol Laryngol 80(suppl 2):1–30, 1971

2.Flynn T, Möller C, Jönsson R, Lohmander A. The high prevalence of otitis media with effusion in children with cleft lip and palate as compared to children without clefts. Int J Pediatr Otorhinolaryngol 73(10):1441–1446, 2009

3.Bennett M. Symposium on ear diseases.III. The older cleft palate patient. (A clinical otologic-audiologic study). Laryngoscope 82:1217–1225, 1972

4.Valtonen H, Dietz A, Qvamberg Y. Long-term clinical, audiologic and radiologic outcomes in palate cleft children treated with early tympanostomy for otitis media with effusion: a controlled prospective study. Laryngoscope 115(8):1512–1516, 2005

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9.Reiter R, Haase S, Brosch S. Repaired cleft palate and ventilation tubes and their associations with cholesteatoma in children and adults. Cleft Palate Craniofac J 46(6):598–602, 2009

10. Nadol JB, Schuknecht HF. Surgery of the Ear and Temporal Bone. New York: Raven Press, 1993

11. Dominguez S, Harker LA. Incidence of cholesteatoma with cleft palate. Ann Otol Rhinol Laryngol 97(6):659–60, 1988

12. McCabe BF, Sadé J, Abramson M. Cholesteatoma: First International Conference. Birmingham: Aesculapius, 1977

13. Karmody CS, Northrop C. The pathogenesis of acquired cholesteatoma of the human middle ear: support for the migration hypothesis. Otol Neurotol 33:42–47, 2012

14. Michaels L. Biology of cholesteatoma. Otolaryngol Clin North Am 22:969–981, 1989

15. Welkoborsky HJ. Current concepts of the pathogenesis of acquired middle ear cholesteatoma. Laryngorhinootologie 90:38–48, 2011

16. Yamamoto-Fukuda T, Hishikawa Y, Shibata Y , Kobayashi T, Takahashi H, Koji T. Pathogenesis of middle ear cholesteatoma: a new model of experimentally induced cholesteatoma in Mongolian gerbils. Am J Pathol 176:2602–2606, 2010

17. Spilsbury K, Miller I, Semmens JB , Lannigan FJ. Factors associated with developing cholesteatoma: a study of 45,980 children with middle ear disease. Laryngoscope 120(3):625, 2010

Address for correspondence: Martin Fernandez, MD, Otorrinolaringologia, Otologia & Neurotologia, Centro de Especialidades Clinica de Marly, Calle 50 # 7–36 piso 5, Bogota DC, Colombia. clinicadeloido@marly.com.co

Cholesteatoma and Ear Surgery – An Update, pp. 313–316

Edited by Haruo Takahashi

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