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Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis

  • Otology
  • Published:
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Abstract

Purpose

Congenital cholesteatoma (CC) presents as a white pearl-like lesion behind a normal tympanic membrane (TM), without a history of otorrhea, infection, perforation or previous otologic surgery. Several recent studies provided new data improving this pathology characterization. The aim of this paper is to expand the knowledge about CC and to provide new insights on its pathogenesis.

Methods

The study consisted of two main research parts: (1) systematic review and meta-analysis; (2) medical literature review englobing anatomy, histology, embryology and congenital pathology of the ear.

Results

The search strategy identified a total of 636 papers. Seventy retrospective studies were included. A total of 1497 cases were studied and the mean age was 6.58 years, with a male–female ratio of 3:1, 34% were asymptomatic, 26% had hearing loss and 2% had facial dysfunction/paralysis. The overall estimate for antero-superior quadrant involvement was 0.70 [95% confident interval (CI) 0.64–0.76], in the postero-superior quadrant was 0.60 (95% CI 0.52–0.69), in the antero-inferior quadrant was 0.32 (95% CI 0.23–0.41), in the postero-inferior quadrant was 0.38 (95% CI 0.29–0.47), in the attic was 0.53 (95% CI 0.43–0.63) and in the mastoid was 0.33 (95% CI 0.26–0.41). More advanced Potsic stages were present in older patients. The most likely inclusion place seems to be between the pars flaccida and the upper quadrants of the pars tensa.

Conclusions

During the last decades, a substantial improvement in CC diagnosis and management had been achieved. The presented mechanism seems to explain most of middle ear CC.

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Based in Carlson 2004 [98]

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Acknowledgements

The authors would like to thank to Jorge Domingues, MD and Deodato Silva, MD, for offering their help to improve this manuscript.

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  1. Department of Otorhinolaryngology, Centro Hospitalar de Lisboa Ocidental, Nova Medical School, New University of Lisbon, Lisbon, Portugal

    Nelson Gilberto, Sara Custódio, Tiago Colaço, Ricardo Santos, Pedro Sousa & Pedro Escada

  2. Department of Anatomy, Nova Medical School, New University of Lisbon, Lisbon, Portugal

    Nelson Gilberto

  3. Department of Otorhinolaryngology, Hospital das Forças Armadas, Lisbon, Portugal

    Nelson Gilberto

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  1. Nelson Gilberto
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Gilberto, N., Custódio, S., Colaço, T. et al. Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis. Eur Arch Otorhinolaryngol 277, 987–998 (2020). https://doi.org/10.1007/s00405-020-05792-4

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