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The aetiology of ossicular chain defects in congenital cholesteatoma

Published online by Cambridge University Press:  08 September 2021

X Y Li ,
Y Liu ,
D H Zhao ,
Y Liu  and
J J Sun
X Y Li
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, Beijing, China
Y Liu*
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, Beijing, China
D H Zhao
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, Beijing, China
Y Liu
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, Beijing, China
J J Sun
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, Beijing, China
*
Author for correspondence: Dr Yang Liu, Department of Otolaryngology Head and Neck Surgery, Sixth Medical Center of The PLA General Hospital, No. 6 Fucheng Road, Haidian District, Beijing100048, China E-mail: liuyangdoc@sina.com
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Abstract

Objective

The aim of the present study was to perform a retrospective review of the lesion sites in congenital middle-ear cholesteatoma and any accompanying ossicular defects, as well as to explore the possible aetiology of congenital middle-ear cholesteatoma associated with such ossicular chain defects.

Method

The clinical characteristics and pathogenic mechanisms of ossicular chain defects were investigated in 10 patients with early-stage congenital middle-ear cholesteatoma confirmed by surgery, from August 2011 to February 2019.

Results

Monofocal cholesteatoma was located in the anterosuperior quadrant in 3 cases and in the posterosuperior quadrant in 7 cases; all 10 cases showed an absence of the long crus of incus, and 8 cases showed a complete or partial absence of stapes superstructure. The lesions were confined to the vestibular window and the stapes region and had no contact with the long crus of the incus or stapes in nine cases. None of the 10 patients had any recurrence of cholesteatoma.

Conclusion

Although cholesteatoma can cause erosion of ossicles, the present cases suggest that residual epithelium of the cholesteatoma may coexist with ossicular malformations. Therefore, the aetiology of the clinical characteristics in these patients may derive from residual epithelial hinderance of ossicle development.

Keywords

CholesteatomaEar OssiclesMalformation
Type
Main Article
Information
The Journal of Laryngology & Otology , Volume 136 , Issue 5 , May 2022 , pp. 391 - 395
Copyright
Copyright © The Author(s), 2021. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED

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Footnotes

Dr Y Liu takes responsibility for the integrity of the content of the paper

References

Derlacki, EL, Clemis, JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965;74:706–27CrossRefGoogle ScholarPubMed
Levenson, MJ, Parisier, SC, Chute, P, Wenig, S, Juarbe, C. A review of twenty congenital cholesteatomas of the middle ear in children. Otolaryngol Head Neck Surg 1986;94:560–7CrossRefGoogle ScholarPubMed
Koltai, PJ, Nelson, M, Castellon, RJ, Garabedian, EN, Triglia, JM, Roman, S et al. The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 2002;128:804–9CrossRefGoogle ScholarPubMed
Yung, M, Tono, T, Olszewska, E, Yamamoto, Y, Sudhoff, H, Sakagami, M et al. EAONO/JOS Joint Consensus Statements on the Definitions, Classification and Staging of Middle Ear Cholesteatoma. J Int Adv Otol 2017;13:1–8CrossRefGoogle ScholarPubMed
Michaels, L. An epidermoid formation in the developing middle ear: possible source of cholesteatoma. J Otolaryngol 1986;15:169–74Google ScholarPubMed
Wang, RG, Hawke, M, Kwok, P. The epidermoid formation (Michaels' structure) in the developing middle ear. J Otolaryngol 1987;16:327–30Google ScholarPubMed
Michaels, L. Origin of congenital cholesteatoma from a normally occurring epidermoid rest in the developing middle ear. Int J Pediatr Otorhinolaryngol 1988;15:51–65CrossRefGoogle ScholarPubMed
Levenson, MJ, Michaels, L, Parisier, SC. Congenital cholesteatomas of the middle ear in children: origin and management. Otolaryngol Clin North Am 1989;22:941–54CrossRefGoogle ScholarPubMed
Hough, JV. Congenital malformations of the middle ear. Arch Otolaryngol 1963;78:335–43CrossRefGoogle ScholarPubMed
Snow, JB Jr, Wackym, PA, Ballenger's Otorhinolaryngology: Head and Neck Surgery, 17th edn. PMPH, USA, 2008Google Scholar
Sade, J, Fuchs, C. Cholesteatoma: ossicular destruction in adults and children. J Laryngol Otol 1994;108:541–4CrossRefGoogle ScholarPubMed
Albera, R, Canale, A, Piumetto, E, Lacilla, M, Dagna, F. Ossicular chain lesions in cholesteatoma. Acta Otorhinolaryngol Ital 2012;32:309–13Google ScholarPubMed
Tos, M. A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope 2000;110:1890–7CrossRefGoogle ScholarPubMed
Huang, TS. Double intratemporal congenital cholesteatomas combined with ossicular anomalies. Ann Otol Rhinol Laryngol 1986;95:401–3CrossRefGoogle ScholarPubMed
Suetake, M, Kobayashi, T, Takasaka, T. Bilateral congenital cholesteatomas associated with ossicular anomalies: a case report. Am J Otol 1991;12:132–4Google ScholarPubMed