A dominant hereditary ossicular anomaly: bilateral incus anomaly and stapes fixation

Abstract

Although several cases of familial ossicular anomaly have been reported, this is the first report of an incus anomaly and stapes fixation combined with a facial nerve anomaly.

JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance

“…Radiological imaging is not available. Reports in the literature of ossicular chain deformities include stapes fixation, ossification of the stapedial tendon, missing long process of the incus, and malleus fixation, but no deformity of the malleus as seen in this family [16][17][18][19][20][21].…”

Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management

“…7 One hundred thirtyone type IIb patients exhibited stapes suprastructure deformities (91 patients [69.5%]), missing or slim formation of the long process of the incus (76 patients [58.0%]), or aberrant facial nerves (13 patients [9.9%]). 6,25,30,[35][36][37][38] Their surgical operations included ossicular chain reconstruction with Teflon piston, 6,7,31,36,38 piston placement, 14,30,34 wire piston, 13,35 Teflon wire piston, 12 modified cause prosthesis, 19 cupped piston prosthesis placements, 36 partial resection of stapes footplate and TORP, 25 Teflon shaft, 7 or autologous bone implantations. 12,13 Type III, Oval Window Bony Atresia or Aplasia, With or Without Round Window Atresia.…”