Kumar Gaurav, Castillo Mauricio, Buchman Craig A
Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC 27599-7510, USA..
AJNR Am J Neuroradiol. 2003 Jun-Jul;24(6):1130-2.
A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted stapedectomy and explains the clinical findings. The findings in male patients are fairly typical X-linked congenital deafness.
一名31岁男性患者自出生以来出现进行性混合性听力损失。曾尝试进行镫骨切除术，但因外淋巴涌出而未成功。颞骨CT显示内耳道底部呈球囊状扩张，且缺乏将其与耳蜗底部隔开的骨板。这种异常情况是在尝试镫骨切除术后发现的，并解释了临床症状。男性患者的这些表现为相当典型的X连锁先天性耳聋。
