Congenital cholesteatoma of the middle ear – uncommon clinical presentation
Abstract
Introduction. Congenital cholesteatoma of the middle ear is un uncommon and yet not well-defined disease. Only few cases of cholesteatoma in the fossa ovalis with unusual clinical presentation have been reported in medical literature. Case report. We reported a 16-year-old girl with congenital cholesteatoma in the fossa ovalis with minimal clinical presentation. A small mass was found occluding the fossa ovalis and mimicking otosclerotic process within tympanic cavity. The operation started as stapedotomy, and when the process was confirmed it converted to mastoidectomy via the retroauricular approach. Conclusion. The diagnosis of congenital cholesteatoma in children should always be considered, even if the clinical symptoms imitate other ear disorders, in our case otosclerosis.
References
Levine JL, Wright CG, Pawlowski KS, Meyerhoff WL. Postnatal persistence of epidermoid rests in the human middle ear. La-ryngoscope 1998; 108(1 Pt 1): 70−3.
Persaud R, Hajioff D, Trinidale A, Khemani S, Battacharya MN, Pa-padimitriou N, et al. Evidence-based review of etiopathogenic theories of congenital and acquired cholesteatoma. J Laryngol Otol 2007; 121(11): 1013−9.
Lim HW, Yoon TH, Kang WS. Congenital cholesteatoma: clini-cal features and growth patterns. Am J Otolaryngol 2012; 33(5): 538−42.
Kojima H, Tanaka Y, Shiwa M, Sukurai Y, Moriyama H. Conge-nital cholesteatoma clinical features and surgical resulats. Am J Otolaryngol 2006; 27(5): 299−305.
McGill TJ, Merchant S, Healy GB, Friedman EM. Congenital cho-lesteatoma of the middle ear in children: a clinical and histopa-thological report. Laryngoscope 1991; 101(6 Pt 1): 606−13.
Wang R, Zubick HH, Vernick DM, Strome M. Bilateral congenital middle ear cholesteatomas. Laryngoscope 1984; 94(11 Pt 1): 1461−3.
Levenson MJ, Michaels L, Parisier SC, Juarbe C. Congenital choles-teatomas in children: an embryologic correlation. Laryngos-cope 1988; 98(9): 949−55.
Iino Y, Imamura Y, Hiraishi M, Yabe T, Suzuki J. Mastoid pneu-matization in children with congenital cholesteatoma: an aspect of the formation of open-type and closed-type cholesteatoma. Laryngoscope 1998; 108(7): 1071−6.
Warren FM, Bennett ML, Wiggins RH 3rd, Saltzman KL, Blevins KS, Shelton C, et al. Congenital cholesteatoma of the mastoid temporal bone. Laryngoscope 2007; 117(8): 1389−94.
Mevio E, Gorini E, Sbrocca M, Artesi L, Lenzi A, Lecce S, et al. Congenital cholesteatoma of mastoid origin. Otolaryngol Head Neck Surg 2002; 127(4): 346−8.
Lee JH, Hong SJ, Park CH, Jung SH. Congenital cholesteatoma of mastoid origin. J Laryngol Otol 2007; 121(11): e20.