Is there a relationship between U-shaped audiograms and mutations in Connexin 26?

References

• Antoniadi T, Grønskov K, Sand A, Papanos A, Brøndum-Nielsen K, Petersen MB. Mutation analysis of the GJB2 (connexin 26) gene by DGGE in Greek patients with sensorineural deafness. Hum Mutat 2000; 16:7–12.
   PubMed Web of Science ®Google Scholar
• Billings KR, Kenna MA. Causes of pediatric sensor-ineural hearing loss. Arch Otolaryngol Head Neck Surg 1999;125:517–21.
   PubMed Web of Science ®Google Scholar
• Bussoli TJ, Steel KP. The molecular genetics of inherited deafness — current and future applications. Molecular Biology Series. J Laryngol Otol 1998;112: 523–30.
   PubMed Web of Science ®Google Scholar
• Cawthorne TE, Hinchcliffe R. Familial perceptive deafness. Pract Otorhinolaryngol 1957;19:68–83.
   Google Scholar
• Davis AC. The prevalence of hearing impairment and reported hearing disability among adults in Great Britain. Int J. Epidemiol 1989;18:911–17.
   PubMed Web of Science ®Google Scholar
• Denoyelle F, Marling S, Well D, et al. Clinical features of the prevalent form of childhood deafness. DFNB1, due to a connexin-26 gene defect: implications for genetic counselling. Lancet 1999;35: 1298–303.
   Google Scholar
• Elberling C, Ludvigsen C, Lyregaard PE. DANTALE: a new Danish speech material. Scand Audiol 1989;18: 169–75.
   PubMed Web of Science ®Google Scholar
• Estivill X, Fortina P, Surrey S, et al. Connexin-26 mutations in sporadic and inherited sensorineural deaf-ness. Lancet 1998; 351:394–8.
   PubMed Web of Science ®Google Scholar
• Fortnum H, Davis A. Epidemiology of permanent childhood hearing impairment in Trent Region. Br J Audiol 1997;31:409–46.
   PubMed Web of Science ®Google Scholar
• France EA, Stephens SDG. All Wales audiology and genetic service for hearing impaired young adults. J Audiol Med 1995;4:67–84.
   Google Scholar
• Gasparini P, Rabionet R, Barbujani G, et al. High carrier frequency of the 35delG deafness mutation in European populations. Eur J Hum Genet 2000;8:19–23.
   PubMed Web of Science ®Google Scholar
• Gorlin RJ, Toriello 1W, Cohen MM, eds. Genetic hearing loss with no associated abnormalities. In: Hereditary hearing loss and its syndromes. New York: Oxford University Press, 1995, pp. 43–61.
   Google Scholar
• Govaerts PJ, De Ceulear G, Daemers K, et al. A new autosomal-dominant locus (DFNA12) is responsible for a nonsyndromic, midfrequency, prelingual and non-progressive sensorineural hearing loss. Am J Otol 1998; 19:718–23.
   PubMedGoogle Scholar
• HEAR: European work group on genetics of hearing impairment. Infoletter, 1996.
   Google Scholar
• ISO 389–3. Acoustics - reference zero for the calibration of audiometric equipment. Part 3: Reference equivalent threshold force levels for pure tones and bone vibrators. Geneva: International Organization for Standardization, 1994.
   Google Scholar
• ISO 389. Acoustics - standard reference zero for the calibration of pure-tone air-conduction audiometers. Geneva: International Organization for Standardization, 1991.
   Google Scholar
• ISO 8253–1. Acoustics - audiometric test methods. Part I. Basic pure-tone air and bone conduction threshold audiometry. Geneva: International Organization for Standardization, 1989.
   Google Scholar
• Kunst II., Huybrechts C., Manes H., Huygen P., Van Camp G., Cremes C. The phenotypes of DFNA13/ COL11A2: Nonsyndromic autosomal dominant mid-frequency and high-frequency sensorineural hearing impairment. Am J Otol 2000;21: 181–7.
   Google Scholar
• Königsmark BW. Dominant mid-frequency nerve deaf-ness. Acta Otolaryngol 1970;79:42–54.
   Google Scholar
• Lalwani AK, Castelein CM. Cracking the auditory genetic code: non syndromic hereditary hearing im-pairment. Am J Otol 1999;20:115–32.
   PubMedGoogle Scholar
• Lench N, Houseman M, Newton V, Van Camp G, Mueller R. Connexin-26 mutations in sporadic non-syndromal sensorineural deafness. Lancet 1998;351: 415.
   Google Scholar
• Morell RJ, Kim HJ, Hood LJ, et al. Mutations in the connexin 26 gene (GJB2) among Ashkenazi Jews with nonsyndromic recessive deafness. N Engl J Med 1998; 11:1500–5.
   Web of Science ®Google Scholar
• Olsen S. Evaluation of the list of numerals in the Danish speech audiometry material: Dantale. Scand Audiol 1996;25:103–7.
   PubMed Web of Science ®Google Scholar
• O'Neill ME, Marietta J, Nishimura D, et al. Hum Mol Genet 1998;5:853–6.
   Web of Science ®Google Scholar
• Orzan E, Polli R, Madella M, Vinanzi C, Leonardi M, Murgia A. Molecular genetics applied to clinical practice: the Cx26 hearing impairment. Br J Audiol 1999;33:291–5.
   PubMed Web of Science ®Google Scholar
• Parving A. The value of speech audiometry in hearing-aid rehabilitation. Scand Audiol 1991;20:159–64.
   PubMed Web of Science ®Google Scholar
• Parving A. Factors causing hearing impairment - some perspectives from Europe. J Am Acad Audiol 1995;63: 87–95.
   Google Scholar
• Parving A, Biering-Sorensen M, Bech B, Christensen B, SOrensen MS. Hearing in the elderly >80 years of age-prevalence of problems and sensitivity. Scand Audiol 1997;26: 99–106.
   Google Scholar
• Parving A, Hein HO, Suadicani P, Ostd B, Gyntelberg F. Epidemiology of hearing disorders. Some factors affecting hearing. The Copenhagen Male Study. Scand Audiol 1993;22:101–7.
   Google Scholar
• Parving A, Newton V. Editorial guidelines for descrip-tion of inherited hearing loss. J Audiol Med 1995;4–1.
   Google Scholar
• Parving A, Ostri B, Poulsen J, Gyntelberg F. Epide-miology of hearing impairment in male adult subjects at 49-69 years of age. Scand Audiol 1983;12:191–6.
   PubMed Web of Science ®Google Scholar
• Pedersen ICE. Presbyacusis. An epidemiological study. Dissertation. Goteborg: The University of GOteborg, 1990.
   Google Scholar
• Rosenhall U, Jönsson MH, SOderlin 0. Self-assessed hearing problems in Sweden: a demographic study. Audiology 1999;38: 328–34.
   Google Scholar
• Sakihara Y, Christensen B, Parving A. The prevalence of hereditary hearing impairment in adults. Scand Audiol 1999;28:39–46.
   Google Scholar
• Steel K. The benefits of recycling. Science 1999;285: 1363–4.
   PubMed Web of Science ®Google Scholar
• Tseng CJ, Lalwani AK. Cracking the auditory genetic code: Part II. Syndromic hereditary hearing impair-ment. Am J Otol 2000;21:437–51.
   Google Scholar
• Uimonen S, Huttunen ICH, Jouni-Ervasti K, Sorri M. Do we know the real need for hearing rehabilitation in the population level? Hearing impairment in the 5-75-year-old cross-sectional Finnish population. Br J Audiol 1999;33: 53–9.
   Google Scholar
• Van Camp G, Schmidt R. http:dnalab-www.uia.ac.bed-nalab/hhh. 2000.
   Google Scholar
• Verhoeven K, Van Laer L, Kirschhofer K, et al. Mutations in the human alpha-tectorin gene cause autosomal dominant non-syndromic hearing impair-ment. Nat Genet 1998;19:60–2.
   PubMed Web of Science ®Google Scholar
• Willems PJ. Genetic causes of hearing loss. N Engl J Med 2000;15:1101–9.
   Google Scholar