Light and electron microscopic studies of a case with simultaneous Mondini and Michel deformities of the inner ears
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We performed light and electron microscopic studies on the temporal bones of a patient with genetic aplastic deafness, in which the right ear had a Mondini-type defect and the left ear a Michel-type anomaly. The round window of each ear was absent. The stapedial foot plate of the right ear was depressed at its central part and was covered by thin membrane. The course of the internal auditory meatus of the left ear was deviated ventrally. There was total aplasia of the cochlea in the left ear, while that of the right ear showed only three-quarters of a turn. In this ear, three sites of spherical bulging were found and were believed to represent outer hair cells. Other structures of the normal cochlear duct were not present. The modiolus of the left ear was round and dome-shaped, contained sparse nerve fibers and a primitive spiral ganglion. The saccule, utricle and semicircular canals of each ear were missing, so that a common cavity of the vestibular system was present. Several otoliths could be seen under the surface of the membrane covering the common cavity. No sensory cells or their related tissues could be found in either ear.
Key wordsHuman inner ear Congenital anomalies Electron microscopy
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- 1.Fraser J (1972) A case of congenital deafness with malformation of the bony and membranous labyrinths on both sides. Epidioscope demonstration. Proc R Soc Med 20:475–477Google Scholar
- 2.Gussen R (1968) Mondini type of genetically determined deafness. J Laryngol 82:41–55Google Scholar
- 3.Igarashi M, Filippone MV, Alford BR (1976) Temporal bone findings in Pierre-Robin syndrome. Laryngoscope 86:1779–1787Google Scholar
- 4.Illum P (1972) The Mondini type of cochlear malformation: a survey of the literature. Arch Otolaryngol 96:305–311Google Scholar
- 5.Nomura Y, Kawabata I (1980) Scheibe dysgenesis of the inner ear. J Laryngol Otol 94:1345–1352Google Scholar
- 6.Ormerod FC (1960) The pathology of congenital deafness. J Laryngol Otol 74:919–950Google Scholar
- 7.Paparella MM (1972) Mondini's deafness. Arch Otorhinolaryngol 95:134–140Google Scholar
- 8.Sando I, Takahara T, Ogawa A (1984) Congenital anomalies of the inner ear. Ann Otol Rhinol Laryngol [Suppl] 112:110–118Google Scholar
- 9.Schmidt JM (1985) Cochlear neuronal populations in developmental defects of the inner ear. Arch Otorhinolaryngol 99:14–20Google Scholar
- 10.Schuknecht HF (1980) Mondini dysplasia: a clinical and pathological study. Ann Otol Rhinol Laryngol [Suppl 65] 89:3–23Google Scholar
- 11.Windle-Taylor PC (1982) The Mondini defect in Turner's syndrome. A temporal bone report. Clin Otolaryngol 7:75–80Google Scholar