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A 72-year-old woman with congenital familiar lack of smell is described. General ENT and otoneurological examination were normal. The ability to smell was totally absent. By MR scan normal conditions were found in the nose, paranasal sinuses, the cribriform plate in the anterior cranial fossa and in the brain especially in the olfactory bulbs. Microscopic examinations of biopsy specimens from the olfactory region showed no signs of olfactory epithelium.
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