title: Neurophysiologic, audiometric and vestibular function tests in patients with hyperostosis cranialis interna.
abstract: OBJECTIVE
  Hyperostosis cranialis interna (HCI) is an autosomal dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina. The aim of this study is to describe the value of several neurophysiological, audiometric and vestibular tests related to the clinical course of the disorder.
  
  
  METHODS
  Ten affected subjects and 13 unaffected family members were recruited and tested with visual evoked potentials, masseter reflex, blink reflex, pure tone and speech audiometry, stapedial reflexes, otoacoustic emissions, brainstem evoked response audiometry and electronystagmography.
  
  
  RESULTS
  Due to the symmetrical bilateral nature of this disease, the sensitivity of visual evoked potentials (VEPs), masseter reflex and blink reflex is decreased (25-37.5%), therefore reducing the value of single registration. Increased hearing thresholds and increased BERA latency times were found in 60-70%. The inter-peak latency I-V parameter in BERA has the ability to determine nerve encroachment reliably. 50% of the patients had vestibular abnormalities. No patient had disease-related absence of otoacoustic emissions, because the cochlea is not affected.
  
  
  CONCLUSIONS
  In patients with HCI and similar craniofacial sclerosing bone dysplasias we advise monitoring of vestibulocochlear nerve function with tone and speech audiometry, BERA and vestibular tests. VEPs are important to monitor optic nerve function in combination with radiological and ophthalmologic examination. We do not advise the routine use of blink and masseter reflex.
authors.affiliation: Department of Otorhinolaryngology - Head & Neck Surgery, Maastricht University Medical Center, PO 5800, 6202 AZ Maastricht, The Netherlands. J.Waterval@gmail.com
authors.forename: J J
authors.initials: JJ
authors.lastname: Waterval
authors.forename: M P H
authors.initials: MP
authors.lastname: Bischoff
authors.forename: R J
authors.initials: RJ
authors.lastname: Stokroos
authors.forename: L J
authors.initials: LJ
authors.lastname: Anteunis
authors.forename: D M W
authors.initials: DM
authors.lastname: Hilkman
authors.forename: H
authors.initials: H
authors.lastname: Kingma
authors.forename: J J
authors.initials: JJ
authors.lastname: Manni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001299:Audiometry; D002150:Caloric Tests; D002648:Child; D018450:Disease Progression; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005154:Facial Nerve / Q000473:pathology; D005158:Facial Paralysis; D005260:Female; D006801:Humans; D015576:Hyperostosis / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009900:Optic Nerve / Q000473:pathology; D010026:Osteosclerosis / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D017084:Otoacoustic Emissions, Spontaneous; D010375:Pedigree; D011379:Prognosis; D019291:Skull Base / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D013199:Stapes / Q000502:physiology; D014057:Tomography, X-Ray Computed; D014276:Trigeminal Nerve / Q000473:pathology; D014724:Vestibular Function Tests; D000159:Vestibulocochlear Nerve / Q000473:pathology; D055815:Young Adult
keywords: Cranial nerve palsies; Facial nerve; Hyperostosis; Hyperostosis cranialis interna; Leave hyperostosis; Trigeminal nerve; Vestibulocochlear nerve