title: Congenital cholesteatoma.
abstract: OBJECTIVE
  Congenital cholesteatomas have historically been considered a rare disorder. However, a review of the literature reveals an incidence ranging from 4 to 24%, and these values are probably underestimated. This article summarizes the general diagnostic criteria of congenital cholesteatomas, their usual presenting symptoms and findings, perioperative studies, a proposed staging system to assist with predicting postoperative outcomes, general surgical procedures, and recidivism rates.
  
  
  RESULTS
  Congenital cholesteatomas have been defined as evidencing no prior history of otorrhea, tympanic perforation, or previous otologic procedures; a normal pars flaccida and tensa; and a pearly white mass medial to the tympanic membrane. Their cause remains controversial; however, the epithelial rest theory is most commonly accepted. The incidence of this disorder seems to be on the rise; however, this may be a result of increased awareness and reporting. The most common sites of presentation on physical examination are the anterior-superior and posterior-superior quadrants of the tympanic membrane. Conductive hearing loss is the most common presenting symptom. There is a lack of uniformity of reporting and classifying congenital cholesteatomas. Staging systems have recently been proposed. One in particular is reported and correlates with outcomes of treatment. Treatment of congenital cholesteatomas is still surgical. A comparison is made between canal wall up and canal wall down tympanomastoidectomy for the treatment of nonlocalized cholesteatoma pearls. Postoperative hearing results are associated with the status of the ossicular chain perioperatively.
  
  
  CONCLUSIONS
  Heightened awareness and early diagnosis of congenital cholesteatomas is imperative. Early treatment decreases the extent of the disease and reduces the risk of recidivism and complications. Management of this disease is surgical. Preoperative computed tomography should be considered to assist in preoperative planning and prediction of postoperative issues. Consideration of a standard staging system and classification is important for consistent reporting and comparison.
authors.affiliation: Department of Pediatric Skull Base Surgery and the Cochlear Implant Program, Division of Pediatric Otolaryngology, 34th Street & Civic Center Boulevard, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. kazahaya@email.chop.edu
authors.forename: Ken
authors.initials: K
authors.lastname: Kazahaya
authors.forename: William P
authors.initials: WP
authors.lastname: Potsic
mesh_terms: D001299:Audiometry / Q000379:methods; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D013506:Otologic Surgical Procedures / Q000379:methods*; D011379:Prognosis; D020127:Recovery of Function; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome