title: [Congenital cholesteatoma of the petrous bone. Etiopathogenic discussions apropos of 11 cases].
abstract: The authors report on 11 cases of congenital cholesteatomas, treated between 1979 and 1988. In all cases, there was a long-standing history of deafness. The diagnosis revealed 6 cophoses, 5 cases of mixed deafness, 9 cases of impairment of the facial nerve (7 cases of paralysis including 4 with sudden onset and 2 hemispasms), 2 facial neuralgias with facial hypesthesia, one Gradenigo-Lannois syndrome, 2 leaks of the C.S.F. (1 nasal, 1 very abundant auricular leak). In 6 cases, the surgical approach route was trans-cochlear, and in 5 cases surpa-petrosal, two of which were extended to a petrectomy. Of the 5 cases of partial deafness, only one was preserved. In 5 cases, the facial nerve was destroyed at the level of the ganglion of the facial nerve, a region where a frankly epidermal tissue is always found. In 4 cases the facial nerve was repaired (three hypo-glosso-facial anastomoses and one end-to-end suture, after re-routing). The authors share the opinion of Fisch that the origin of the intra-petrosal congenital cholesteatoma always occurs at the level of the ganglion of the facial nerve.
authors.forename: M
authors.initials: M
authors.lastname: Cannoni
authors.forename: A
authors.initials: A
authors.lastname: Pech
authors.forename: S
authors.initials: S
authors.lastname: Fuchs
authors.forename: M
authors.initials: M
authors.lastname: Zanaret
authors.forename: J M
authors.initials: JM
authors.lastname: Thomassin
authors.forename: P
authors.initials: P
authors.lastname: Dessi
authors.forename: J M
authors.initials: JM
authors.lastname: Triglia
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002781:Cholesteatoma / Q000151:congenital* / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010579:Petrous Bone; D012888:Skull Neoplasms / Q000151:congenital* / Q000209:etiology; D013997:Time Factors
vernacular_title: Cholestéatomes congénitaux du rocher. Discussion étiopathogénique à propos de 11 observations.