軽度の外耳奇形を伴った耳小骨奇形--monofocal奇形とmultifocal奇形

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Four cases of congenital ossicular malformation with congenital aural fistula and/or slight deformity of the pinna were reported. They were ncudostapedial disconnection and fixation of the stapedial footplate (Case 1, 2), total lack of ossicles with undeveloped oval window (Case 3) and lack of the incus with maldeveloped stapes (Case 4). Anomalies of the facial nerve or of the chorda tympani were also observed.The embryological consideration leads us to the following conjecture; these malformations hold at least two undeveloped processes, for example, maldeveloped "secondary continuity" to be the incudostapedial joint and "stapedial lamina" to form the annular ligament. Thus the ossicular anomalies presented in this paper might be the results of multifocal maldevelopment and this is a distinguishing feature of this type of ossicular malformation from the one without deformities of the external ear, which has a monofocal deformity.
社団法人日本耳鼻咽喉科学会の論文