Wetmore Stephen J, Gross Andrew F
Department of Otolaryngology, West Virginia University School of Medicine, PO Box 9200, Morgantown, WV 26506-9200, USA.
Ear Nose Throat J. 2011 Aug;90(8):360-6. doi: 10.1177/014556131109000809.
We conducted a retrospective chart review at a university hospital on a family that exhibited a rare cause of conductive hearing loss. Four male patients of this family had congenital conductive hearing loss. Three of these family members underwent bilateral ossiculoplasty, a father and two of his three sons. All three were found to have mild or moderate conductive hearing loss due to fixation of the head of the stapes by a bony bar that extended from the head of the stapes to the posterior ear canal wall. Surgical removal of the bony bar improved hearing in all cases. This family exhibits autosomal-dominant inheritance of this abnormality. Fixation of the head of the stapes by a bony bar that extends to the posterior ear canal wall is a rare cause of congenital, autosomal-dominant, mild or moderate conductive hearing loss that can be surgically corrected.
我们在一家大学医院对一个表现出罕见传导性听力损失病因的家族进行了回顾性病历审查。该家族的四名男性患者患有先天性传导性听力损失。其中三名家族成员接受了双侧听骨成形术，即一名父亲及其三个儿子中的两个。所有三人都被发现因镫骨头被一根从镫骨头延伸至外耳道后壁的骨棒固定而患有轻度或中度传导性听力损失。手术切除该骨棒后，所有病例的听力均得到改善。这个家族表现出这种异常的常染色体显性遗传。镫骨头被延伸至外耳道后壁的骨棒固定是先天性、常染色体显性、轻度或中度传导性听力损失的罕见病因，可通过手术矫正。
