Keskin Gürkan, Ustündağ Emre, Almaç Ahmet
Department of Otolaryngology, Medicine Faculty of Kocaeli University, Kocaeli, Turkey.
Kulak Burun Bogaz Ihtis Derg. 2003 Dec;11(6):175-8.
Congenital absence of the stapes and the oval window is an anomaly reported in only sporadic cases. We present a 17-year-old male patient with congenital bilateral conductive hearing loss. The external auditory canal and the tympanic membrane appeared normal on both sides. An exploratory tympanotomy in the right ear revealed dehiscence of the the horizontal segment of the facial nerve, which was displaced inferiorly, occupying the area of the absent oval window. The stapes was totally absent and a malformed long process of the incus was attached by a fibrous band to the promontory. Manipulation of the facial nerve in association with stapedectomy or vestibulotomy was avoided in order not to injure the nerve. Instead, amplification with hearing aids was recommended to the patient.
先天性镫骨及卵圆窗缺如是一种仅在散发病例中报道过的异常情况。我们报告一名17岁男性先天性双侧传导性听力损失患者。双侧外耳道及鼓膜外观正常。右侧耳的探查性鼓室切开术显示面神经水平段裂开，其向下移位，占据了缺失的卵圆窗区域。镫骨完全缺失，砧骨的长突畸形并通过一条纤维带附着于岬部。为避免损伤神经，避免在镫骨切除术或前庭切开术时对面神经进行操作。相反，建议患者使用助听器进行听力放大。
