先天性内耳奇形症例の走査および透過電子顕微鏡的観察

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Light microscopic and scanning and transmission electron microscopic findings from a patient with Mondini type at right ear and Michel type at left ear genetic aplastic deafness are described.<BR>The round window of each ear was absent. The medial surface of stapedial foot plate of right ear was depressed roundly at central part and it was covered by thick membrane to make a partition of the middle and the inner ear.<BR>The course of the internal auditory meatus of left ear was deviated ventrally. The cochlea was presented by only three forths of turn at right ear and total aplasia at left ear. Three lows of spherical buldging thought to be outer hair cell were found between outer pillar cells and Hensen's cells. The other superstructures of cochlear duct were not present. Round dome shaped modiolus of the left ear contained some nerve fibers and spiral ganglions sparsely. In the spiral ganglion cell, a small number of neurofibril and a few Nissle Bodies were present in the cytoplasm of it. The saccule, utricle and semicircular canals of each ear were missing to make a common cavity of vestibular system. There were several otolith under the surface of membrane covered the common cavity. The sensory cells and its related tissues were not found in the both ear.
耳鼻咽喉科臨床学会の論文