{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T13:28:24Z","timestamp":1771075704368,"version":"3.50.1"},"reference-count":36,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2016,9,1]],"date-time":"2016-09-01T00:00:00Z","timestamp":1472688000000},"content-version":"vor","delay-in-days":5357,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["aao-hnsfjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Otolaryngol.--head neck surg."],"published-print":{"date-parts":[[2002,1]]},"abstract":"<jats:sec><jats:title>OBJECTIVES<\/jats:title><jats:p>To evaluate clinical data, extensions, residual disease rate, and functional results in cases of congenital cholesteatoma in pediatric patients compared with cases of acquired forms.<\/jats:p><\/jats:sec><jats:sec><jats:title>STUDY DESIGN AND SETTING<\/jats:title><jats:p>In a retrospective study conducted at a single tertiary care center over a decade, 34 congenital cholesteatomas (mean patient age, 6.6 years) isolated from a series of 215 cholesteatomas in children were treated surgically and followed up for an average of 83\u00a0months. Surgical treatment consisted of the closed technique (CT), open technique tympanoplasty in CT [TOT], radical mastoidectomy (RM), or Rambo's technique. The main outcome measures were surgical findings, residual lesion rate, and hearing assessment.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>The first surgical procedure was CT in 85.3%, TOT in 8.8%, RM in 2.9%, and Rambo's technique in 2.9% of patients. Two, 3, and 4 operations were necessary in 76.5%, 11.4%, and 11.4% of children, respectively. Residual lesions were observed in 34.6% of those multi\u2010operated patients (7 CT and 2 TOT), 24.1% in those treated with CT, and 29.2% in those treated with CT with a planned second look. Mean postoperative pure tone average and air\u2010bone gap were 26 and 21\u00a0dB, respectively. A speech reception threshold of less than 30\u00a0dB HL was achieved in 66% of patients. A total of 26 ossiculoplasties had to be performed (8 partial and 18 total); the mean postoperative air\u2010bone gap was lower after the former (15\u00a0dB) than after the latter (22\u00a0dB). A high\u2010frequency pure\u2010tone hearing loss of more than 10\u00a0dB was uncommon (5.8%). Unsurprisingly, the residual lesion rate seems to be higher for congenital than for acquired cholesteatomas, but hearing results are significantly better.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>In most cases, staged CT appears to be the best technique to treat these lesions, which often develop in a well\u2010pneumatized mastoid.<\/jats:p><\/jats:sec>","DOI":"10.1067\/mhn.2002.121514","type":"journal-article","created":{"date-parts":[[2003,2,4]],"date-time":"2003-02-04T21:49:09Z","timestamp":1044395349000},"page":"34-40","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Congenital Middle Ear Cholesteatomas in Children: Our Experience in 34 Cases"],"prefix":"10.1002","volume":"126","author":[{"given":"Vincent","family":"Darrouzet","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology University Hospital of Bordeaux Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Yves","family":"Duclos","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Hospital of Bordeaux Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Didier","family":"Portmann","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Hospital of Bordeaux Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Pierre","family":"Bebear","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Hospital of Bordeaux Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,9]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"557","article-title":"A large epidermal cholesteatoma of the parieto\u2010temporal region deforming the left hemisphere without cerebral 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