Before describing the pathologic changes in the ear in a case of Niemann-Pick's disease, it is perhaps advisable first to say a word or two about the disease itself. For a more comprehensive study on the subject the reader is referred to the excellent papers by Pick,1 Niemann,2 Bloom,3 Kramer4 and others.
In brief, Niemann-Pick's disease is a condition that starts in infancy, usually at the age of 5 or 6 months. The child has a retarded physical and mental development; the abdomen is enlarged, owing to the increased size of the liver and spleen, and brownish discolorations of the skin are present. The disease is occasionally associated with amaurotic family idiocy and mongolism. A low grade fever, anemia and cachexia are present. The child succumbs to the disease, usually from associated infection of the upper respiratory tract, before the age of 2 years. A
DRUSS JG. PATHOLOGIC CHANGES IN THE EAR IN NIEMANN-PICK'S DISEASE. Arch Otolaryngol. 1932;15(4):592–598. doi:10.1001/archotol.1932.03570030611010
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