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Audiology Volume 38, 1999 - Issue 3
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Original Article

Autosomal Dominant Non-syndromal Low-frequency Sensorineural Hearing Impairment Linked to Chromosome 4p16 (DFNA14): Statistical Analysis of Hearing Threshold in Relation to Age and Evaluation of Vestibulo-ocular Functions

H. Kunst Department of Otorhinolaryngology, University Hospital, Nijmegen, The Netherlands
,
H. Marres Department of Otorhinolaryngology, University Hospital, Nijmegen, The Netherlands
,
P. Huygen Department of Otorhinolaryngology, University Hospital, Nijmegen, The Netherlands
,
G. van Camp Department of Medical Genetics, University of Antwerp, Belgium
,
F. Joosten Department of Radiology, University Hospital, Nijmegen, The Netherlands
&
C. Cremers Department of Otorhinolaryngology, University Hospital, Nijmegen, The Netherlands
Pages 165-173 | Received 10 Jun 1998, Accepted 05 Oct 1998, Published online: 07 Jul 2009

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Sanna Häkli, Laura Kytövuori, Mirja Luotonen, Martti Sorri & Kari Majamaa. (2014) WFS1 mutations in hearing-impaired children. International Journal of Audiology 53:7, pages 446-451.
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RONALD JE PENNINGS, PATRICK LM HUYGEN, GUY VAN CAMP & COR WRJ CREMERS. (2003) A Review of Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment. Audiological Medicine 1:1, pages 47-55.
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PATRICK LM HUYGEN, RONALD JE PENNINGS & COR WRJ CREMERS. (2003) Characterizing and Distinguishing Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment. Audiological Medicine 1:1, pages 37-46.
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Agnete Parving, Yukimi Sakihara & Birger Christensen. (2000) Inherited Sensorineural Low-Frequency Hearing Impairment: Some Aspects of Phenotype and Epidemiology. Audiology 39:1, pages 50-60.
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S. J. H. Bom, H. P. M. Kunst, P. L. M. Huygen, F. P. M. Cremers & C.W. RJ Cremers. (1999) Non-Syndromal Autosomal Dominant Hearing Impairment: Ongoing Phenotypical Characterization of Genotypes. British Journal of Audiology 33:5, pages 335-348.
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Articles from other publishers (16)

Mirko Aldè, Giovanna Cantarella, Diego Zanetti, Lorenzo Pignataro, Ignazio La Mantia, Luigi Maiolino, Salvatore Ferlito, Paola Di Mauro, Salvatore Cocuzza, Jérôme René Lechien, Giannicola Iannella, Francois Simon & Antonino Maniaci. (2023) Autosomal Dominant Non-Syndromic Hearing Loss (DFNA): A Comprehensive Narrative Review. Biomedicines 11:6, pages 1616.
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Hongbo Cheng, Qin Zhang, Wenbin Wang, Qingxia Meng, Fuxin Wang, Minjuan Liu, Jun Mao, Yichao Shi, Wei Wang & Hong Li. (2018) Whole exome sequencing identifies a pathogenic mutation in WFS1 in two large Chinese families with autosomal dominant all-frequency hearing loss and prenatal counseling. International Journal of Pediatric Otorhinolaryngology 106, pages 113-119.
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Shujiro B. Minami, Sawako Masuda, Satoko Usui, Hideki Mutai & Tatsuo Matsunaga. (2012) Comorbidity of GJB2 and WFS1 mutations in one family. Gene 501:2, pages 193-197.
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Margit Schraders, Jaap Oostrik, Patrick L.M. Huygen, Tim M. Strom, Erwin van Wijk, Henricus P.M. Kunst, Lies H. Hoefsloot, Cor W.R.J. Cremers, Ronald J.C. Admiraal & Hannie Kremer. (2010) Mutations in PTPRQ Are a Cause of Autosomal-Recessive Nonsyndromic Hearing Impairment DFNB84 and Associated with Vestibular Dysfunction. The American Journal of Human Genetics 86:4, pages 604-610.
Crossref
Margit Schraders, Kwanghyuk Lee, Jaap Oostrik, Patrick L.M. Huygen, Ghazanfar Ali, Lies H. Hoefsloot, Joris A. Veltman, Frans P.M. Cremers, Sulman Basit, Muhammad Ansar, Cor W.R.J. Cremers, Henricus P.M. Kunst, Wasim Ahmad, Ronald J.C. Admiraal, Suzanne M. Leal & Hannie Kremer. (2010) Homozygosity Mapping Reveals Mutations of GRXCR1 as a Cause of Autosomal-Recessive Nonsyndromic Hearing Impairment. The American Journal of Human Genetics 86:2, pages 138-147.
Crossref
Richard J.H. Smith & Guy Van Camp. 2009. Molecular Pathology in Clinical Practice: Genetics. Molecular Pathology in Clinical Practice: Genetics 123 131 .
Naomi F Bramhall, Jeremy C Kallman, Aimee M Verrall & Valerie A Street. (2008) A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings. BMC Medical Genetics 9:1.
Crossref
Richard J. H. Smith & Guy Van Camp. 2007. Molecular Pathology in Clinical Practice. Molecular Pathology in Clinical Practice 125 133 .
Tímea Tóth, Markus Pfister, Hans-Peter Zenner & Istvan Sziklai. (2006) Phenotypic characterization of a DFNA6 family showing progressive low-frequency sensorineural hearing impairment. International Journal of Pediatric Otorhinolaryngology 70:2, pages 201-206.
Crossref
Richard J.H. Smith. (2004) Clinical application of genetic testing for deafness. American Journal of Medical Genetics Part A 130A:1, pages 8-12.
Crossref
Kim Cryns, Theru A. Sivakumaran, Jody M.W. Van den Ouweland, Ronald J.E. Pennings, Cor W.R.J. Cremers, Kris Flothmann, Terry-Lynn Young, Richard J.H. Smith, Marci M. Lesperance & Guy Van Camp. (2003) Mutational spectrum of theWFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease. Human Mutation 22:4, pages 275-287.
Crossref
Richard J.H Smith & Stephen Hone. (2003) Genetic screening for deafness. Pediatric Clinics of North America 50:2, pages 315-329.
Crossref
Steven J. H. Bom, Guy Van Camp, Kim Cryns, Ronald J. C. Admiraal, Patrick L. M. Huygen & Cor W. R. J. Cremers. (2002) Autosomal Dominant Low-Frequency Hearing Impairment (DFNA6/14). Otology & Neurotology 23:6, pages 876-884.
Crossref
Theru A. Sivakumaran & Marci M. Lesperance. (2002) A PCR-RFLP Assay for the A716T Mutation in the WFS1 Gene, a Common Cause of Low-Frequency Sensorineural Hearing Loss . Genetic Testing 6:3, pages 229-231.
Crossref
Belen Hurle, Keara Lane, Jane Kenney, Lisa M Tarantino, Maja Bucan, Bernard H Brownstein & David M Ornitz. (2001) Physical Mapping of the Mouse Tilted Locus Identifies an Association between Human Deafness Loci DFNA6/14 and Vestibular System Development. Genomics 77:3, pages 189-199.
Crossref
S Brodwolf, IR Böddeker, A Ziegler, P Rausch & J Kunz. (2008) Further evidence for linkage of low–mid frequency hearing impairment to the candidate region on chromosome 4p16.3. Clinical Genetics 60:2, pages 155-160.
Crossref

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