Phelps P D, Proops D, Sellars S, Evans J, Michaels L
Royal National Throat, Nose and Ear Hospital, Cape Town, South Africa.
J Laryngol Otol. 1993 Jun;107(6):492-5. doi: 10.1017/s0022215100123552.
Congenital deformities of the labyrinth of the inner ear can be associated with a fistulous communication between the intracranial subarachnoid space and the middle ear cavity. We describe seven such cases, six confirmed by high resolution CT and one by postmortem histological section. The seven patients all presented with meningitis although a cerebrospinal fluid fistula was demonstrated at subsequent surgery or postmortem. The lesions were bilateral in three patients, unilateral in three and probably bilateral in the postmortem case although only one temporal bone was obtained. In every case there was a dilated sac instead of the normal two and a half turn cochlea on the affected side and this was confirmed at surgery. The demonstration of the basal cochlear turn is of paramount importance in any deaf child presenting with meningitis. A true Mondini deformity with a normal basal turn and some hearing is not at risk of developing a fistula.
先天性内耳迷路畸形可能与颅内蛛网膜下腔和中耳腔之间的瘘管相通有关。我们描述了7例这样的病例，其中6例经高分辨率CT确诊，1例经尸检组织切片确诊。这7例患者均表现为脑膜炎，尽管在随后的手术或尸检中证实存在脑脊液瘘。3例患者病变为双侧，3例为单侧，尸检病例可能为双侧，但仅获得一块颞骨。在每例病例中，患侧均有一个扩张的囊，而非正常的2.5圈耳蜗，这在手术中得到了证实。对于任何患有脑膜炎的失聪儿童，显示耳蜗底转至关重要。具有正常底转且有一定听力的真正蒙迪尼畸形不会有发生瘘管的风险。
