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Korean Journal of Otorhinolaryngology-Head and Neck Surgery > Volume 24(4); 1981 > Article
Korean Journal of Otorhinolaryngology-Head and Neck Surgery 1981;24(4): 649-56.
Mondini Dysplasia in Potter's Syndrome
Chong Sun Kim, MD1, and Je Gun Chi, MD2
1;Department of Otolaryngology, 2;Pathology, College of Medicine, Seoul National University, Korea
Potter 症候群에서의 Mondini 形成不全症
金宗善1 · 池堤根2
서울大學校 醫科大學 耳鼻咽喉科學敎室1;病理學敎室2;

A lethal syndrome of bilateral renal agenesis, pulmonary dysgenesis and renal face, known as Potter's syndrome has been reported in the literature. However, temporal bone findings of this syndrome have not been reported in the world literatures. Authors experienced a case of Potter's syndrome in a 36+4 week stillborn baby from a 28-year-old mother, and left temporal bone was prepared for histopathologic study. External ear was deformed and the external canal was underdeveloped. The middle ear space was filled with loose mesenchymal tissue with small air space in the hypotympanic area. The ossicular structure was well developed and the Fallopian canal was dehiscent on the pyramidal area. Inner ear showed wide osseous lateral semicircular canal with normal vestibular sensory organs. However, the cochlea was developed only on the basal turn forming flat cochlea with underdeveloped modiolus and osseous spiral lamina, showing Mondini's dysplasia. The endolymphatic sac is underdeveloped. Other findings were rupture of the saccular wall, wide internal auditory meatus and wide cochlear aqueduct.

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