Hostname: page-component-78c5997874-dh8gc Total loading time: 0 Render date: 2024-11-19T17:57:49.717Z Has data issue: false hasContentIssue false

Congenital cholesteatoma

Published online by Cambridge University Press:  29 June 2007

B. Laskiewicz*
Affiliation:
Consultant, Queen Mary's Hospital for Children, Carshalton, Surrey.
S. Chalstrey
Affiliation:
ENT Department, St. Bartholomew's Hospital, London.
D. J. Gatland
Affiliation:
ENT Department, St. Bartholomew's Hospital, London.
N. Jones
Affiliation:
Senior Registrar, ENT Department, Royal Berkshire Hospital, Reading.
L. Michaels
Affiliation:
Professor of Histopathology, University College and Middlesex School of Medicine, The Institute of Laryngology and Otology, London.
*
Address for correspondence: Miss B. Laskiewicz, F.R.C.S., D.L.O., ENT Department, Queen Mary's Hospital for Children, Carshalton, Surrey KT18 7EG

Abstract

We present a British series of eleven patients with primary cholesteatoma, including one patient with bilateral disease. Eight children presented with a history of hearing loss, while one child had had recurrent otitis media and another had had carache. Operative findings were: in five ears, cholesteatoma confined to the antero-superior segment with intact ossicles, in a further four, cholesteatomas extending throughout the mesotympanum with ossicular erosion in one, and in two ears posterior disease throughout the middle ear and mastoid, which had eroded the ossicles in both cases. The five cases of antero-superior cholesteatoma lend most support to Michaels' concept of epidermoid formation as a possible source of congenital cholesteatoma. With a greater awareness of the problem and careful examination of the antero-superior quadrant of the tympanic membrane, earlier diagnosis may be possible enabling removal of small intact cholesteatoma sacs and preserving the structures of the middle ear and therefore the hearing.

A screening programme for infants included as part of their routine examination which would be undertaken by examiners who are trained to be more aware of the problem and skilled at otoscopy, would help in the earlier detection of such cases as is shown by reports from the U.S.A.

Type
Main Articles
Copyright
Copyright © JLO (1984) Limited 1991

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Aimi, K. (1983) Role of the tympanic ring in the pathogenesis of congenital cholesteatoma. Laryngoscope, 93: 11401146.CrossRefGoogle ScholarPubMed
Cohen, D. (1987) Location of primary cholesteatoma. American Journal of Otology, 8: 6165.Google ScholarPubMed
Cruveilhier, (1829) Anatomic Pathologique T. II.Google Scholar
Curtis, A. (1979) Congenital middle ear cholesteatoma: two unusual cases and a review of the literature. Laryngoscope, 89: 11591165.CrossRefGoogle Scholar
Cushing, H. A. (1922) A large epidermal cholesteatoma of the parieto-temporal region deforming the left hemisphere without cerebral symptoms. Surgery, Gynaecology and Obstetrics, 34: 557566.Google Scholar
House, H. P. (1953) An apparent primary cholesteatoma: case report. Laryngoscope, 63: 712713.CrossRefGoogle ScholarPubMed
Levenson, M. J., Parisier, S. C., Chute, P., Wenig, S., Juarbe, C. (1986) A review of 20 congenital cholesteatomas of the middle ear in children. Otolaryngology—Head and Neck Surgery, 94: 560567.CrossRefGoogle Scholar
McDonald, T. J., Cody, D. T. R., Ryan, R. E. (1984) Congenital cholestetatoma of the ear. Annals of Otology, Rhinology and Laryngology, 93: 637640.CrossRefGoogle ScholarPubMed
Michaels, L. (1986) An epidermoid formation in the developing middle ear: possible source of cholesteatoma. Journal of Otolaryngology, 15:3: 169174.Google ScholarPubMed
Michaels, L. (1988a) Origin of congenital cholesteatoma from a normally occurring epidermoid rest in the developing middle ear. International Journal of Pediatric Otorhinolaryngology, 15: 5165.CrossRefGoogle ScholarPubMed
Michaels, L. (1988b) Evolution of the epidermoid formation and its role in the development of the middle ear and tympanic membrane during the first trimester. Journal of Otolaryngology, 17: 2228.Google ScholarPubMed
Paparella, M. M., Rybah, L. (1978) Congenital cholesteatoma. Otolaryngologic Clinics of North America, 11: 113120.CrossRefGoogle ScholarPubMed
Peron, D. L., Schuknecht, H. F. (1975) Congenital cholesteatomata with other anomalies. Archives of Otolaryngology, 101: 498505.CrossRefGoogle ScholarPubMed
Piza, J., Northop, E. C., Eavey, R. (1985) Amniotic fluid contents in the ears of neonates and infants. XIII World Congress of Otorhinolaryngology (Abstract), 1: 48.Google Scholar
Ruben, R. (1989) Efficacy of early diagnosis of primary cholesteatoma in infants. Proceedings of Third International Conference in Cholesteatoma and Mastoid Surgery.Google Scholar
Ruedi, L. (1959) Cholesteatoma formation in the middle ear in animal experiments. Acta Otolaryngologica, 50: 233242.CrossRefGoogle ScholarPubMed
Sade, J., Babiacki, A., Pincus, G. (1983) The metaplastic and congenital origin of cholesteatoma. Acta Otolaryngologica, 96: 119129.CrossRefGoogle ScholarPubMed
Wang, R., Zubick, H. H., Vernick, D. M., Strome, M. (1984) Bilateral congenital middle ear cholesteatomas. Laryngoscope, 94: 14611463.CrossRefGoogle ScholarPubMed
Wingert, R. H., McCurdy, J. A., Hays, L. L. (1976) Congenital tympanic cholesteatoma. Ear, Nose and Throat Journal, 55: 323327.Google ScholarPubMed