Some Considerations on Congenital Middle Ear Cholesteatoma

Abstract

Congenital cholesteatomas (CCs) are white mass lesions in the middle ear arising from keratinizing squamous epithelium behind the intact tympanic membrane. CCs are characterized by invasive growth and osteolytic activity. The pathogenesis of CCs is unclear. CCs are estimated to account for 1–5% of all middle ear cholesteatomas. Along with improvement of office-based tools used for otologic examinations (i.e., otomicroscopy, lightening, and photodocumentation), detection of CCs among otolaryngologists and pediatricians has increased in recent years. To date, no conservative treatment for CCs has proven to be effective, except for treatment of secondary infection. The only effective treatment for CCs is therefore surgery. CCs are some surgical techniques, for example, intact canal wall mastoidectomy and canal wall down mastoidectomy using microscopy, and transcanal endoscopic ear surgery. Postoperative issues related to the effects of deafness and recurrence can have social, educational, and behavioral consequences.