Otolaryngologic Manifestations of Frontometaphysial Dysplasia: The Gorlin-Holt Syndrome
- 1 January 1974
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA Otolaryngology–Head & Neck Surgery
- Vol. 99 (1) , 52-58
- https://doi.org/10.1001/archotol.1974.00780030056009
Abstract
The otolaryngologic aspect of two cases of a recently described osseous dysplasia called frontometaphysial dysplasia, or Gorlin-Holt syndrome, is elaborated. Particular emphasis is given to the oto-audiologic evaluation of these patients, including polytomography of the temporal bone. Both patients demonstrated a progressive mixed hearing loss demonstrated by serial audiogram with audiologic follow-up of nine and six years. The distinct ossicular chain abnormality including a fixed malleus demonstrated by polytomography was confirmed by an exploratory atticotympanotomy.Keywords
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