Head and Neck Imagingvan Buchem's disease
(Francis Steven Peter van Buchem, 20th century, Dutch physician), generalized cortical hyperostosis; an inherited, autosomal recessive skeletal dysplasia, with thickening and sclerosis of the mandible, skull base and calvaria, as well as the long and short bones. An increased serum alkaline phosphatase level is present. Similar to other bone dysplasias with proclivity of involvement of the skull base and calvaria, such as
osteopetrosis
and
progressive diaphyseal dysplasia
, bony encroachment on the skull base foramina may lead to various neurological deficits, including
sensorineural hearing loss
.
Conductive hearing loss
has been attributed to narrowing of the external auditory canal and middle ear cavity.
RH