title: Imaging features and progression of hyperostosis cranialis interna.
abstract: OBJECTIVE
  HCI is a unique autosomal-dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina, whereby the mandible is affected to a lesser extent. The aim of this study is to describe the specific radiologic characteristics and course of the disorder.
  
  
  METHODS
  CT scans of affected individuals within 1 family were analyzed and compared with scans of their unaffected family members and with an age- and sex-matched control group. Linear measurements were performed of the inner table, the medulla, and the outer table of different skull locations, and attenuation (density) measurements of the same regions were recorded. Neuroforamina widths were recorded as well.
  
  
  RESULTS
  There was significant thickening of the skull in the frontal, parietal, temporal, and occipital regions, which was mainly due to thickening of the inner table of the skull. The attenuation of the deposited hyperostotic bone was lower than normal cortical bone.
  
  
  CONCLUSIONS
  HCI is the only genetic bone dysplasia known that is confined to the craniofacial area. The hyperostotic bone is less attenuated than normal cortical bone. The observed radiologic abnormalities explain the possible impairment of the olfactory, optic, trigeminal, facial, and vestibulocochlear nerves.
authors.affiliation: Department of Otorhinolaryngology-Head & Neck Surgery, Maastricht University, Maastricht, The Netherlands. J.Waterval@gmail.com
authors.forename: J J
authors.initials: JJ
authors.lastname: Waterval
authors.forename: T M
authors.initials: TM
authors.lastname: van Dongen
authors.forename: R J
authors.initials: RJ
authors.lastname: Stokroos
authors.forename: B-J
authors.initials: BJ
authors.lastname: De Bondt
authors.forename: M N
authors.initials: MN
authors.lastname: Chenault
authors.forename: J J
authors.initials: JJ
authors.lastname: Manni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015576:Hyperostosis / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D019291:Skull Base / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult