title: Familial superior canal dehiscence syndrome.
abstract: OBJECTIVE
  The etiology of superior canal dehiscence (SCD) involving the arcuate eminence is not completely understood, but genetic factors may play a role. One hypothesis is that patients are born with a defect of the superior canal, and an acute event (such as head trauma) or progressive loss of bone (eg, due to dural pulsations) may result in the onset of SCD symptoms. Familial SCD has only been briefly mentioned in the literature to date.
  
  
  METHODS
  We report 3 families that each had 2 members with SCD syndrome. We found that first-degree relatives presented with similar complaints and that temporal bone computed tomography scans between relatives showed very similar skull base topography and anatomic SCD defects.
  
  
  CONCLUSIONS
  The presence of symptomatic SCD among first-degree relatives and similar skull base topography suggests that genetics may play a role in the etiology of SCD.
authors: Department of Otorhinolaryngology-Head and Neck Surgery, University Medical Center, Utrecht, the Netherlands2Braincenter Rudolf Magnus, Utrecht, the Netherlands3Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts4Departmen.
authors: Marlien E F
authors: ME
authors: Niesten
authors: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston.
authors: Sarah
authors: S
authors: Lookabaugh
authors: Department of Radiology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston.
authors: Hugh
authors: H
authors: Curtin
authors: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts4Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston.
authors: Saumil N
authors: SN
authors: Merchant
authors: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts4Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston.
authors: Michael J
authors: MJ
authors: McKenna
authors: Department of Otorhinolaryngology-Head and Neck Surgery, University Medical Center, Utrecht, the Netherlands2Braincenter Rudolf Magnus, Utrecht, the Netherlands.
authors: Wilko
authors: W
authors: Grolman
authors: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts4Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston.
authors: Daniel J
authors: DJ
authors: Lee
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007759:Labyrinth Diseases / Q000000981:diagnostic imaging / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012665:Semicircular Canals / Q000000981:diagnostic imaging / Q000473:pathology*; D013577:Syndrome; D013701:Temporal Bone / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed