title: Hereditary congenital unilateral deafness: a new disorder?
abstract: Congenital unilateral deafness is a rare disorder. The prevalence rates are unknown. The prevalence of children with severe to profound hearing losses that are congenital (or acquired before the development of speech and language) is 0.5 to 3 per 1,000 live births. Evidently, congenital unilateral deafness must have a lower prevalence. The purpose of this research was to present a new disorder, hereditary congenital unilateral deafness. A pedigree is presented in which both male and female members display symptoms of congenital unilateral deafness. Two affected persons and a normal-hearing member of the family have vestibular abnormalities without dysequilibrium. The inheritance pattern of this new syndrome is not clear. We hypothesize that the disorder might be new. A family like this has never before been presented in the medical literature.
authors.affiliation: Department of Otorhinolaryngology, University Medical Center Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
authors.forename: Frederik G
authors.initials: FG
authors.lastname: Dikkers
authors.forename: Joke B G M
authors.initials: JB
authors.lastname: Verheij
authors.forename: Monique
authors.initials: M
authors.lastname: van Mechelen
mesh_terms: D000328:Adult; D003638:Deafness / Q000151:congenital* / Q000235:genetics*; D007758:Ear, Inner / Q000002:abnormalities / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D010375:Pedigree; D014057:Tomography, X-Ray Computed; D014724:Vestibular Function Tests