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Congenital cholesteatoma: what radiologists need to know

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Abstract

Congenital cholesteatoma is a rare, non-neoplastic lesion that causes conductive hearing loss in children. It is underrecognized and often diagnosed only when there is an established hearing deficit. In the pediatric population, hearing deficiency is particularly detrimental because it can impede speech and language development and, in turn, the social and academic well-being of affected children. Delayed diagnosis leads to advanced disease that requires more extensive surgery and a greater chance of recurrence. A need to promote awareness and recognition of this condition has been advocated by clinicians and surgeons, but no comprehensive imaging review dedicated to this entity has been performed. This review aims to discuss the diagnostic utility of high-resolution computed tomography and magnetic resonance imaging in preoperative and postoperative settings in congenital cholesteatoma. Detailed emphasis is placed on the essential preoperative computed tomography findings that facilitate individualized surgical management and prognosis in the pediatric population.

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Data Availability

No datasets were generated or analysed during the current study. Radiological images in the figures are extracted from the Picture Archiving and Communicating System of three regional hospitals within Hong Kong.

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Authors and Affiliations

  1. Department of Diagnostic and Interventional Radiology, LG1, Main Block, Princess Margaret Hospital, Kowloon, Hong Kong

    Hoi Ming Kwok, Chun Hei Lewey Cheung, Ting Fung Ng, Sun Yu Lam, Ka Hon Stephen Wong, Ho Lim Wong, Nin Yuan Pan, Lik Fai Cheng & Ka Fai Johnny Ma

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Contributions

H.M.K., N.Y.P., L.F.C., and K.F.J.M. conceptualized the study. N.Y.P., L.F.C., and K.F.J.M. supervised and supported the study. H.M.K., C.H.L.C., and H.L.W. collected the data. All authors contributed to drafting the initial manuscript. H.M.K., C.H.L.C, T.F.N., H.L.W, K.H.S.W, and S.Y.L interpreted the images and all authors prepared the figure legends. All authors substantially revised the manuscript. All authors reviewed and approved the final manuscript.

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Correspondence to Hoi Ming Kwok.

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Kwok, H.M., Cheung, C.H.L., Ng, T.F. et al. Congenital cholesteatoma: what radiologists need to know. Pediatr Radiol 54, 620–634 (2024). https://doi.org/10.1007/s00247-024-05877-w

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