Cannoni M, Pech A, Fuchs S, Zanaret M, Thomassin J M, Dessi P, Triglia J M
Rev Laryngol Otol Rhinol (Bord). 1989;110(1):33-42.
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.
