title: Features of autosomal recessive non-syndromic hearing impairment: a review to serve as a reference.
abstract: OBJECTIVE
  Non-syndromic sensorineural hearing impairment is inherited in an autosomal recessive fashion in 75-85% of cases. To date, 61 genes with this type of inheritance have been identified as related to hearing impairment, and the genetic heterogeneity is accompanied by a large variety of clinical characteristics. Adequate counselling on a patient's hearing prognosis and rehabilitation is part of the diagnosis on the genetic cause of hearing impairment and, in addition, is important for the psychological well-being of the patient.
  
  
  METHODS
  Traditional literature review.
  
  
  METHODS
  All articles describing clinical characteristics of the audiovestibular phenotypes of identified genes and related loci have been reviewed.
  
  
  CONCLUSIONS
  This review aims to serve as a summary and a reference for counselling purposes when a causative gene has been identified in a patient with a non-syndromic autosomal recessively inherited sensorineural hearing impairment.
authors.affiliation: Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands. Anne.Oonk@radboudumc.nl.|Donders Institute for Brain Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands. Anne.Oonk@radboudumc.nl.
authors.forename: A M M
authors.initials: AM
authors.lastname: Oonk
authors.affiliation: Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: P L M
authors.initials: PL
authors.lastname: Huygen
authors.affiliation: Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands.|Donders Institute for Brain Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: H P M
authors.initials: HP
authors.lastname: Kunst
authors.affiliation: Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands.|Nijmegen Centre for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.|Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: Kremer
authors.affiliation: Department of Otorhinolaryngology, Hearing & Genes, Radboud University Medical Center, Nijmegen, The Netherlands.|Donders Institute for Brain Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: R J E
authors.initials: RJ
authors.lastname: Pennings
mesh_terms: D003376:Counseling; D005808:Genes, Recessive; D020022:Genetic Predisposition to Disease; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000534:rehabilitation; D006801:Humans; D010641:Phenotype; D011379:Prognosis