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1791年Carlo Mondini报导了一种以耳蜗扩张、前庭扩张、蜗导水管短、螺旋器和神经不育为特征的内耳畸形。随后,不少作者对该综合征的解剖、临床、放射线学以及治疗等方面作了补充描述,Albrecht等认为该综合征是一种常染色体异常的遗传性疾病。Mondini畸形常伴有蛛网膜下腔与外淋巴间隙相通,由于迷路内压增高,可发生圆窗或卵圆窗脑脊液漏。据说蛛网膜下腔与外淋巴间隙的通路可在内耳道底,也可能在扩张了的耳蜗导水管。本文报告两例Mondini综合征。1例17岁,1例8岁,都有双侧先天性聋,有过5次脑膜炎发作史。听力学检查表现为双耳全聋、前庭无反应。颞骨X线断层相,1例证明前庭发育不良,内耳道扩大,半规管畸形;1例半规及管前庭扩大,耳蜗发育不良。第1例先后作左右耳鼓室探查,见鼓室中充满脑脊液,漏口在卵圆
In 1791 Carlo Mondini reported a deformity of the inner ear characterized by cochlear dilatation, vestibular dilatation, short snorting ducts, spirals, and neuropsychiatry. Subsequently, many authors have made a supplementary description of the anatomy, clinical, radiological and therapeutic aspects of the syndrome, Albrecht so that the syndrome is an autosomal abnormality genetic disease. Mondini deformity often accompanied by subarachnoid space and the outer lymphatic interlinked, due to labyrinth pressure, can occur round or oval cerebrospinal fluid leakage. It is said that the passage between the subarachnoid space and the perilymph space may be at the bottom of the inner ear canal, and may also be an enlarged cochlear aqueduct. This article reports two cases of Mondini syndrome. One case was 17 years old and one case was 8 years old. Both had bilateral congenital deafness and had 5 episodes of meningitis. Hearing tests showed binaural deafness, vestibular no response. The temporal bone and the X-ray showed one case of hypoplasia of the vestibular veins, the enlargement of the internal auditory canal and the semicircular canal deformity. One case of semicircular canal and vestibule enlarged and the cochlea developed dysplasia. The first case has been around the ear drum exploration, see the tympanic cavity is full of cerebrospinal fluid, leakage in the oval