A Review of Progressive Phenotypes in Nonsyndromic Autosomal Dominant Hearing Impairment

REFERENCES

• Bespalova IN, Van Camp G, Bom SJH, Brown DJ, Cryns K, DeWan AT, Erson AE, Flothmann K, Kunst HPM, Kurnool P, Sivakumaran TA, Cremers CWRJ, Leal SM, Burmeister M, Lesperance MM. Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing loss. Human Molecular Genetics 2001; 10: 2501–8.
   PubMed Web of Science ®Google Scholar
• Bom SJH, De Leenheer EMR, Lemaire FX, Kemperman MH, Verhagen WIM, Marres HAM, Kunst HPM, Ensink RJH, Bosman AJ, Van Camp G, Cremers FPM, Huygen PLM, Cremers CWRJ. Speech recognition scores related to age and degree of hearing impairment in DFNA2/KCNQ4 and DFNA9/COCH. Archives of Otolaryngology-Head & Neck Surgery 2001; 127: 1045—8.
   PubMedGoogle Scholar
• Bom SJH, Kemperman MH, De Kok YJM, Huygen PLM, Verhagen WIM, Cremers FPM, Cremers CWRJ. Progressive cochleovestibular impairment caused by a point mutation in the COCH gene at DFNA9. The Laryngoscope 1999b; 109: 1525—30.
   PubMed Web of Science ®Google Scholar
• Bom SJH, Kunst HPM, Huygen PLM, Cremers FPM, Cremers CWRJ. Non-syndromal autosomal dominant hearing impairment: ongoing phenotypical characterization of genotypes. British Journal of Audiology 1999a; 33: 335—48.
   PubMed Web of Science ®Google Scholar
• Chen AH, Ni L, Fukushima K, Marietta J, O’Neill M, Coucke P, Willems P, Smith RJH. Linkage of a gene for dominant nonsyndromic deafness to chromosome 19. Human Molecular Genetics 1995; 4: 1073—6.
   PubMed Web of Science ®Google Scholar
• Coucke P, Van Camp G, Djoyodiharjo B, Smith SD, Frants RR, Padberg GW, Darby JK, Huizing EH, Cremers CWRJ, Kimberling WJ, Oostra BA, Van de Heyning PH, Willems PJ. Linkage of autosomal dominant hearing loss to the short arm of chromosome 1 in two families. New England Journal of Medicine 1994; 331: 425—31.
   PubMed Web of Science ®Google Scholar
• Coucke PJ, Van Hauwe P, Kelley PM, Kunst H, Schatteman I, Van Velzen D, Meyers J, Ensink RJ, Verstreken M, Declau F, Marres H, Kastury K, Bhasin S, McGuirt WT, Smith RJH, Cremers CWRJ, Van de Heyning P, Willems PJ, Smith SD, Van Camp G. Mutations in the KCNQ4 gene are responsible for autosomal dominant deafness in four DFNA2 families. Human Molecular Genetics 1999; 8: 1321—8.
   PubMed Web of Science ®Google Scholar
• De Kok YJM, Bom SJH, Brunt TM, Kemperman MH, van Beusekom E, van der Velde-Visser SD, Robertson NG, MortonCC, HuygenPLM, Verhagen WIM, Brunner HG, Cremers CWRJ, Cremers FPM. A Pro51Ser mutation in the COCH gene is associated with late onset autosomal dominant progressive sensorineural hearing loss with vestibular defects. Human Molecular Genetics 1999; 8: 361—6.
   PubMed Web of Science ®Google Scholar
• De Leenheer EMR, Huygen PLM, Wayne S, Smith RJH, Cremers CWRJ. The DFNA10 phenotype. Annals of Otology, Rhinology & Laryngology 2001; 110: 861—6.
   PubMed Web of Science ®Google Scholar
• De Leenheer EMR, Huygen PLM, Wayne S, Verstreken M, Declau F, Van Camp G, Van de Heyning PH, Smith RJH, Cremers CWRJ. DFNA10/EYA4 — The clinical picture. In Cremers CWRJ, Smith RJH (eds) Genetic hearing impairment: its clinical presentations. Advances in Otorhinolaryngology, Basel: Karger, 2002e; Vol 61: 73—78.
   Google Scholar
• De Leenheer EMR, Huygen PLM, Coucke PJ, Admiraal RJC, Van Camp G, Cremers CWRJ. Longitudinal and cross-sectional phenotype analysis in a new, large Dutch DFNA2/ KCNQ4 family. Annals of Otology, Rhinology & Laryngology 2002b, 111: 267—274.
   PubMed Web of Science ®Google Scholar
• De Leenheer EMR, van Zuijlen DA, Van Laer L, Van Camp G, Huygen PLM, Huizing EH, Cremers CWRJ. Clinical features of DFNA5. In Cremers CWRJ, Smith RJH (eds) Genetic hearing impairment: its clinical presentations. Advances in Otorhinolaryngology, Basel: Karger, 2002c; Vol 61: 53—59.
   Google Scholar
• De Leenheer EMR, Ensink RJH, Kunst HPM, Marres HAM, Talebizadeh Z, Declau F, Smith SD, Usami SI, Van de Heyning PH, Van Camp G, Huygen PLM, Cremers CWRJ. DFNA2/KCNQ4 and its manifestations. In Cremers CWRJ, Smith RJH (eds) Genetic hearing impairment: its clinical presentations. Advances in Otorhinolaryngology, Basel: Karger, 2002a; Vol 61: 41—46.
   Google Scholar
• De Leenheer EMR, van Zuijlen DA, Van Laer L, Van Camp G, Huygen PLM, Huizing EH, Cremers CWRJ. Further delineation of the DFNA5 phenotype. Results of speech recognition tests. Annals of Otology, Rhinology & Laryngology 2002d, 111: 639—641.
   PubMed Web of Science ®Google Scholar
• Eavey RD, Manolis EN, Lubianca J, Merchant S, Seidman JG, Seidman C. Mutations in COCH (formerly Coch5b2) cause DFNA9. In Kitamura, K, Steel KP (eds) Genetics in Otorhinolaryngology. Advances in Otorhinolaryngology, Basel: Karger, 2000; Vol 56: 101—102.
   Google Scholar
• Elfenbein JL, Fisher RA, Wei S, Morell RJ, Stewart C, Friedman TB, Friderici K. Audiologic aspects of the search for DFNA20: a gene causing late-onset, progressive, sensori-neural hearing loss. Ear & Hearing 2001; 22: 279—88.
   PubMed Web of Science ®Google Scholar
• Elverland HH, Hansen PW, Fagerheim T, Tranebjrg L. Audiological variation in a family with autosomal dominant non-syndromic hearing impairment linked to the DFNA7 locus — a need for reclassification of deafness. Journal of Audiological Medicine 1998; 7: 109—19.
   Google Scholar
• Ensink RJH, Huygen PLM, Van Hauwe P, Coucke P, Cremers CWRJ, Van Camp G. A Dutch family with progressive sensorineural hearing impairment linked to the DFNA2 region. European Archives of Otorhinolaryngology 2000; 257: 62—7.
   PubMed Web of Science ®Google Scholar
• Fagerheim T, Nilssen Ø, Raeymaekers P, Brox V, Moum T, Elverland HH, Teig E, Omland HH, Fostad GK, TranebjØrg L. Identification of a new locus for autosomal dominant nonsyndromic hearing impairment (DFNA7) in a large Norwegian family. Human Molecular Genetics 1996; 5: 1187—91.
   PubMed Web of Science ®Google Scholar
• Fransen E, Van Camp G. The COCH gene: a frequent cause of hearing impairment and vestibular dysfunction? British Journal of Audiology 1999; 33: 297—302.
   Google Scholar
• Fransen E, Verstreken M, Bom SJH, Lemaire F, Kemperman MH, De Kok YJM, Wuyts FL, Verhagen WIM, Huygen PLM, McGuirt WT, Smith RJH, Van Maldergem L, Declau F, Cremers CWRJ, Van de Heyning PH, Cremers FPM, Van Camp G. A common ancestor for COCH related cochleovestibular (DFNA9) patients in Belgium and The Netherlands bearing the P51S mutation. Journal of Medical Genetics 2001; 38: 61—5.
   PubMed Web of Science ®Google Scholar
• Fransen E, Verstreken M, Verhagen WIM, Wuyts FL, Huygen PLM, D’Haese P, Robertson NG, Morton CC, McGuirt WT, Smith RJH, Declau F, Van de Heyning PH, Van Camp G. High prevalence of symptoms of Menie’re’s disease in three families with a mutation in the COCH gene. Human Molecular Genetics 1999; 8: 1425—9.
   PubMed Web of Science ®Google Scholar
• Frydman M, Vreugde S, Nageris BI, Weiss S, Vahava O, Avraham KB. Clinical characterization of genetic hearing loss caused by a mutation in the POU4F3 transcription factor. Archives of Otolaryngology-Head & Neck Surgery 2000; 126: 633—7.
   PubMedGoogle Scholar
• Fukushima K, Kasai N, Ueki Y, Nishizaki K, Sugata K, Hirakawa S, Masuda A, Gunduz M, Ninomiya Y, Masuda Y, Sato M, McGuirt WT, Coucke P, Van Camp G, Smith RJH. A gene for fluctuating, progressive autosomal dominant nonsyndromic hearing loss, DFNA16, maps to chromosome 2q23-24.3. American Journal of Human Genetics 1999; 65: 141—50.
   Google Scholar
• Gorlin RJ, Toriello HV, Cohen MM. Hereditary hearing loss and its syndromes. Oxford Monographs on Medical Genetics No. 28, New York: Oxford University Press, 1995, pp. 1—457.
   Google Scholar
• Halpin C, Khetarpal U, McKenna M. Autosomal-dominant progressive sensorineural hearing loss in a large North American family. American Journal of Audiology 1996b; 5: 105—11.
   Google Scholar
• Halpin C, Herrmann B, Whearty M. A family with autosomal-dominant progressive sensorineural hearing loss rehabilitation and counseling. American Journal of Audiology 1996a; 5: 23–32.
   Google Scholar
• Huizing EH, Van Bolhuis AH, Odenthal DW. Studies on progressive hereditary perceptive deafness in a family of 335 members. I. Genetical and general audiological results. Acta Otolaryngologica 1965a; 61: 35–41.
   Google Scholar
• Huizing EH, Van Bolhuis AH, Odenthal DW. Studies on progressive hereditary perceptive deafness in a family of 335 members. II. Characteristic pattern of hearing deterioration. Acta Otolaryngologica 1965b; 61: 161–7.
   Google Scholar
• Huizing EH, van den Wijngaart WSIM, Verschuure J. A followup study in a family with dominant progressive inner ear deafness. Acta Otolaryngologica 1983; 95: 620–6.
   PubMed Web of Science ®Google Scholar
• Huygen PLM, Bom SJH, Van Camp G, Cremers CWRJ. The clinical presentation of the DFNA loci where causative genes have not yet been cloned. DFNA4, DFNA6/14, DFNA7, DFNA16, DFNA20 and DFNA21. In Cremers, CWRJ, Smith RJH (eds) Genetic hearing impairment: its clinical presentations. Advances in Otorhinolaryngology, Basel: Karger, 2002; Vol 61: 98–106.
   Google Scholar
• Huygen PLM, Pennings RJE, Cremers CWRJ. Characterizing and distinguishing progressive phenotypes in nonsyndromic autosomal dominant hearing impairment. Audiological Medicine 2003; 1: 37–46.
   Google Scholar
• Huygen PLM, Verhagen WIM. Peripheral vestibular and vestibulo-ocular dysfunction in hereditary disorders. A review of the literature and a report on some additional findings. Journal of Vestibular Research 1994; 4: 81–104.
   Google Scholar
• Kamarinos M, McGill J, Lynch M, Dahl H. Identification of a novel COCH mutation, I109N, highlights the similar clinical features observed in DFNA9 families. Human Mutation 2001; 17: 351–6.
   PubMedGoogle Scholar
• Kasai N, Fukushima K, Ueki Y, Prasad S, Nosakowski J, Sugata K, Sugata A, Nishizaki K, Meyer NC, Smith RJH. Genomic structures of SCN2A and SCN3A — candidate genes for deafness at the DFNA16 locus. Gene 2001; 264: 113–22.
   PubMed Web of Science ®Google Scholar
• Kharkovets T, Hardelin J-P, Safieddine S, Schweizer M, ElAmraoui A, Petit C, Jentsch TJ. KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway. Proceedings of the National Academy of Sciences of the U.S.A. 2000; 97: 4333–8.
   PubMed Web of Science ®Google Scholar
• Khetarpal U. Autosomal dominant sensorineural hearing loss. Further temporal bone findings. Archives of Otolaryngology-Head & Neck Surgery 1993; 119: 106–8.
   PubMed Web of Science ®Google Scholar
• Khetarpal U. DFNA9 is a progressive audiovestibular dysfunction with a microfibrillar deposit in the inner ear. The Laryngoscope 2000; 110: 1379–84.
   PubMed Web of Science ®Google Scholar
• Khetarpal U, Schuknecht HF, Gacek RR, Holmes LB. Auto somal dominant sensorineural hearing loss. Pedigrees, audiologic findings, and temporal bone findings in two kindreds. Archives of Otolaryngology-Head & Neck Surgery 1991; 117: 1032–42.
   Google Scholar
• Kubisch C, Schroeder BC, Friedrich T, LuÄtjohann B, ElAmraoui A, Marlin S, Petit C, Jentsch TJ. KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness. Cell 1999; 96: 437–46.
   PubMed Web of Science ®Google Scholar
• Kunst H, Marres H, Huygen P, Van Camp G, Joosten F, Cremers C. Autosomal dominant non-syndromal low-frequency sen sorineural hearing impairment linked to chromosome 4p16 (DFNA14): statistical analysis of hearing threshold in relation to age and evaluation of vestibulo-ocular functions. Audiology 1999; 38: 165—73.
   PubMedGoogle Scholar
• Kunst H, Marres H, Huygen P, van Duijnhoven G, Krebsova A, van der Velde S, Reis A, Cremers F, Cremers C. Nonsyndromic autosomal dominant progressive non-specific mid-frequency sensorineural hearing impairment with childhood to late adolescence onset (DFNA21). Clinical Otolaryngology 2000; 25: 45—54.
   PubMed Web of Science ®Google Scholar
• Kunst H, Marres H, Huygen P, Ensink R, Van Camp G, Van Hauwe P, Coucke P, Willems P, Cremers C. Nonsyndromic autosomal dominant progressive sensorineural hearing loss: audiologic analysis of a pedigree linked to DFNA2. The Laryngoscope 1998; 108: 74—80.
   PubMed Web of Science ®Google Scholar
• Lalwani AK, Castelein CM. Cracking the auditory genetic code: nonsyndromic hereditary hearing impairment. American Journal of Otology 1999; 20: 115—32.
   PubMedGoogle Scholar
• Lalwani AK, Goldstein JA, Kelley MJ, Luxford W, Castelein CM, Mhatre AN. Human nonsyndromic hereditary deafness DFNA17 is due to a mutation in nonmuscle myosin MYH9. American Journal of Human Genetics 2000; 67: 1121—8.
   PubMed Web of Science ®Google Scholar
• Lalwani AK, Jackler RK, Sweetow RW, Lynch ED, RaventoÁs H, Morrow J, King MC, LeoÁn PE. Further characterization of the DFNA1 audiovestibular phenotype. Archives of Otolaryngology. Head and Neck Surgery 1998; 124: 699—702.
   PubMed Web of Science ®Google Scholar
• Lalwani AK, Linthicum FH, Wilcox ER, Moore JK, Walters FC, San Agustin TB, Mislinski J, Miller MR, Sinninger Y, Attaie A, Luxford WM. A five-generation family with late-onset progressive hereditary hearing impairment due to cochleosaccular degeneration. Audiology and Neurootology 1997; 2: 139—54.
   PubMed Web of Science ®Google Scholar
• Lemaire FX, Feenstra L, Huygen PLM, Fransen E, Devriendt K, Van Camp G, Vantrappen G, Cremers CWRJ. Progressive late-onset sensorineural hearing loss and vestibular impair ment with vertigo (DFNA9/COCH). Longitudinal analyses in a Belgian family. Otology Neurotology 2003, in press.
   Google Scholar
• LeoÁn PE, Bonilla JA, SaÁnchez JR, Vanegas R, Villalobos M, Torres L, LeoÁn F, Howell AL, RodrÁguez JA. Low frequency hereditary deafness in man with childhood onset. American Journal of Human Genetics 1981; 33: 209—14.
   PubMed Web of Science ®Google Scholar
• Leon PE, Raventos H, Lynch E, Morrow J, King M-C. The gene for an inherited form of deafness maps to chromosome 5q31. Proceedings of the National Academy of Sciences of the U.S.A. 1992; 89: 5181—4.
   PubMed Web of Science ®Google Scholar
• Lesperance MM, Hall JW III, Bess FH, Fukushima K, Jain PK, Ploplis B, San Agustin TB, Skarka H, Smith RJH, Wills M, Wilcox ER. A gene for autosomal dominant nonsyndromic hereditary hearing impairment maps to 4p16.3. Human Molecular Genetics 1995; 4: 1967—72.
   Google Scholar
• Lynch ED, Lee MK, Morrow JE, Welcsh PL, LeoÁn PE, King MC. Nonsyndromic deafness DFNA1 associated with mutation of a human homolog of the Drosophila gene diaphanous. Science 1997; 278: 1315—8.
   PubMed Web of Science ®Google Scholar
• Manolis EN, Yandavi N, Nadol JB Jr, Eavey RD, McKenna M, Rosenbaum S, Khetarpal U, Halpin C, Merchant SN, Duyk GM, MacRae C, Seidman CE, Seidman JG. A gene for nonsyndromic autosomal dominant progressive postlingual sensorineural hearing loss maps to chromosome 14q12-13. Human Molecular Genetics 1996; 5: 1047—50.
   PubMed Web of Science ®Google Scholar
• Marres H, van Ewijk M, Huygen P, Kunst H, Van Camp G, Coucke P, Willems P, Cremers C. Inherited nonsyndromic hearing loss. An audiovestibular study in a large family with autosomal dominant progressive hearing loss related to DFNA2. Archives of Otolaryngology Head & Neck Surgery 1997; 123: 573—7.
   Google Scholar
• Morell RJ, Friderici KH, Wei S, Elfenbein JL, Friedman TB, Fisher RA. A new locus for late-onset, progressive, hereditary hearing loss DFNA20 maps to 17q25. Genomics 2000; 63: 1—6.
   PubMed Web of Science ®Google Scholar
• O’Neill ME, Marietta J, Nishimura D, Wayne S, Van Camp G, Van Laer L, Negrini C, Wilcox ER, Chen A, Fukushima K, Ni L, Sheffield VC, Smith RJH. A gene for autosomal dominant late-onset progressive non-syndromic hearing loss, DFNA10, maps to chromosome 6. Human Molecular Genetics 1996; 5: 853—6.
   PubMed Web of Science ®Google Scholar
• Pennings RJE, Bom SJH, Cryns K, Flothmann K, Huygen PLM, Kremer H, Van Camp G, Cremers CWRJ. Low-frequency sensorineural hearing loss (DFNA6/14/WFS1) is progressive. Archives of Otolaryngology Head & Neck Surgery 2003, in press.
   Google Scholar
• Robertson NG, Lu L, Heller S, Merchant SN, Eavey RD, McKenna M, Nadol JB Jr, Miyamoto RT, Linthicum FH Jr, Lubianca Neto JF, Hudspeth AJ, Seidman CE, Morton CC, Seidman JG. Mutations in a novel cochlear gene cause DFNA9, a human nonsyndromic deafness with vestibular dysfunction. Nature Genetics 1998; 20: 299—303.
   PubMed Web of Science ®Google Scholar
• Robertson NG, Skvorak AB, Yin Y, Weremowicz S, Johnson KR, Kovatch KA, Battey JF, Bieber FR, Morton CC. Mapping and characterization of a novel cochlear gene in human and in mouse: a positional candidate gene for a deafness disorder, DFNA9. Genomics 1997; 46: 345—54.
   PubMed Web of Science ®Google Scholar
• Talebizadeh Z, Kelley PM, Askew JW, Beisel KW, Smith SD. Novel mutation in the KCNQ4 gene in a large kindred with dominant progressive hearing loss. Human Mutation 1999; 14: 493—501.
   PubMed Web of Science ®Google Scholar
• Teig E. Hereditary progressive perceptive deafness in a family of 72 patients. Acta Otolaryngologica 1968; 65: 365—72.
   PubMedGoogle Scholar
• Vahava O, Morell R, Lynch ED, Weiss S, Kagan ME, Ahituv N, Morrow JE, Lee MK, Skvorak AB, Morton CC, Blumenfeld A, Frydman M, Friedman TB, King M-C, Avraham KB. Mutation in transcription factor POU4F3 associated with inherited progressive hearing loss in humans. Science 1998; 279: 1950—4.
   PubMed Web of Science ®Google Scholar
• Van Camp et al. Human Mutation 2002; 20: 15—19 (see reprint).
   Google Scholar
• Van Camp G, Coucke P, Balemans W, van Velzen D, van de Bilt C, van Laer L, Smith RJH, Fukushima K, Padberg GW, Frants RR, Van de Heyning P, Smith SD, Huizing EH, Willems PJ. Localization of a gene for non-syndromic hearing loss (DFNA5) to chromosome 7p15. Human Molecular Genetics 1995; 4: 2159—63.
   PubMed Web of Science ®Google Scholar
• Van Camp G, Coucke PJ, Kunst H, Schatteman I, Van Velzen D, Marres H, van Ewijk M, Declau F, Van Hauwe P, Meyers J, Kenyon J, Smith SD, Smith RJH, Djelantik B, Cremers CWRJ, Van de Heyning PH, Willems PJ. Linkage analysis of progressive hearing loss in five extended families maps the DFNA2 gene to a 1.25-Mb region on chromosome 1p. Genomics 1997; 41: 70—4.
   Google Scholar
• Van Camp G, Kunst H, Flothmann K, McGuirt W, Wauters J, Marres H, Verstreken M, Bespalova IN, Burmeister M, Van de Heyning PH, Smith RJH, Willems PJ, Cremers CWRJ, Lesperance MM. A gene for autosomal dominant hearing impairment (DFNA14) maps to a region on chromosome 4p16.3 that does not overlap the DFNA6 locus. Journal of Medical Genetics 1999; 36: 532—6.
   Google Scholar
• Van Camp G, Smith RJH. Hereditary Hearing Loss Homepage (HHH). Van Camp G. and Smith RJH. Available at: http:// www.uia.ac.be/dnalab/hhh. Accessed January 19, 2002. van den Wijngaart WSIM, Huizing, EH, Niermeijer, MF, Verschuure, J, Brocaar, MP, Blom W. Follow-up study in a family with dominant progressive hereditary sensorineural hearing impairment. II. Clinical aspects. Audiology 1985b; 24: 336—42.
   Google Scholar
• van den Wijngaart WSIM, Verschuure J, Brocaar MP, Huizing EH. Follow-up study in a family with dominant progressive hereditary sensorineural hearing impairment. I. Analysis of hearing deterioration. Audiology 1985a; 24: 233–40.
   PubMedGoogle Scholar
• Vanderbilt University Hereditary Deafness Study Group. Dominantly inherited low-frequency hearing loss. Archives of Otolaryngology 1968; 88: 242–50.
   Google Scholar
• Van Hauwe P, Coucke P, Van Camp G. The DFNA2 locus for hearing impairment: two genes regulating K+ ion recycling in the inner ear. British Journal of Audiology 1999b; 33: 285–9.
   PubMed Web of Science ®Google Scholar
• Van Hauwe P, Coucke PJ, Declau F, Kunst H, Ensink RJ, Marres HA, Cremers CWRJ, Djelantik B, Smith SD, Kelley P, Van de Heyning PH, Van Camp G. Deafness linked to DFNA2: one locus but how many genes? Nature Genetics 1999a; 21: 263.
   PubMed Web of Science ®Google Scholar
• Van Hauwe P, Coucke PJ, Ensink RJ, Huygen P, Cremers CWRJ, Van Camp G. Mutations in the KCNQ4 K+ channel gene, responsible for autosomal dominant hearing loss, cluster in the channel pore region. American Journal of Medical Genetics 2000; 93: 184–7.
   PubMed Web of Science ®Google Scholar
• Van Laer L, Huizing EH, Verstreken M, van Zuijlen D, Wauters JG, BossuytPJ, Van de Heyning P, McGuirtWT, Smith RJH, Willems PJ, Legan PK, Richardson GP, Van Camp G. Nonsyndromic hearing impairment is associated with a mutation in DFNA5. Nature Genetics 1998; 20: 194–7.
   PubMed Web of Science ®Google Scholar
• Van Laer L, Van Camp G, van Zuijlen D, Green ED, Verstreken M, Schatteman I, Van de Heyning P, Balemans W, Coucke P, Greinwald JH, Smith RJH, Huizing E, Willems P. Refined mapping of a gene for autosomal dominant progressive sensorineural hearing loss (DFNA5) to a 2-cM region, and exclusion of a candidate gene that is expressed in the cochlea. European Journal of Human Genetics 1997; 5: 397—405.
   PubMed Web of Science ®Google Scholar
• Verhagen WIM, Bom SJH, Fransen E, Van Camp G, Huygen PLM, Theunissen EJJM, Cremers CWRJ. Hereditary cochleovestibular dysfunction due to a COCH gene mutation (DFNA9): a follow-up study of a family. Clinical Otolaryngology 2001; 26: 477–83.
   PubMed Web of Science ®Google Scholar
• Verhagen WIM, Bom SJH, Huygen PLM, Fransen E, Van Camp G, Cremers CWRJ. Familial progressive vestibulocochlear dysfunction caused by a COCH mutation (DFNA9). Archives of Neurology 2000; 57: 1045–7.
   PubMedGoogle Scholar
• Verhagen WIM, Huygen PLM. Familial progressive vestibulocochlear dysfunction. Archives of Neurology 1991; 48: 262.
   PubMed Web of Science ®Google Scholar
• Verhagen WIM, Huygen PLM, Bles W. A new autosomal dominant syndrome of idiopathic progressive vestibulocochlear dysfunction with middle-age onset. Acta Otolaryngologica 1992; 112: 899–906.
   PubMed Web of Science ®Google Scholar
• Verhagen WIM, Huygen PLM, Joosten EMG. Familial progressive vestibulocochlear dysfunction. Archives of Neurology 1988; 45: 766–8.
   PubMed Web of Science ®Google Scholar
• Verhagen WIM, Huygen PLM, Theunissen EJJM, Joosten EMG. Hereditary vestibulo-cochlear dysfunction and vascular disorders. Journal of the Neurological Sciences 1989; 92: 55—63.
   PubMed Web of Science ®Google Scholar
• Verhoeven K, Fagerheim T, Prasad S, Wayne S, De Clau F, Balemans W, Verstreken M, Schatteman I, Solem B, Van de Heyning P, Tranebjrg L, Smith RJH, Van Camp G. Refined localization and two additional linked families for the DFNA10 locus for nonsyndromic hearing impairment. Human Genetics 2000; 107: 7–11.
   PubMed Web of Science ®Google Scholar
• Verstreken M, Declau F, Schatteman I, Van Velzen D, Verhoeven K, Van Camp G, Willems PJ, Kuhweide EW, Verhaert E, D’Haese P, Wuyts FL, Van de Heyning PH. Audiometric analysis of a Belgian family linked to the DFNA10 locus. American Journal of Otology 2000; 21: 675—81.
   PubMedGoogle Scholar
• Verstreken M, Declau F, Wuyts FL, D’Haese P, Van Camp G, Fransen E, Van den Hauwe L, Buyle S, Smets REM, Feenstra L, Van der Stappen A, Van de Heyning PH. Hereditary otovestibular dysfunction and MeÁnie’re’s disease in a large Belgian family is caused by a missense mutation in the COCH gene. Otology and Neurotology 2001; 22: 874—81.
   PubMed Web of Science ®Google Scholar
• Wayne S, Robertson NG, DeClau F, Chen N, Verhoeven K, Prasad S, Tranebjrg L, Morton CC, Ryan AF, Van Camp G, Smith RJH. Mutations in the transcriptional activator EYA4 cause late-onset deafness at the DFNA10 locus. Human Molecular Genetics 2001; 10: 195—200.
   PubMed Web of Science ®Google Scholar
• Young T-L, Ives E, Lynch E, Person R, Snook S, MacLaren L, Cator T, Griffin A, Fernandez B, Lee MK, King M-C. Nonsyndromic progressive hearing loss DFNA38 is caused by heterozygous missense mutation in the Wolfram syndrome gene WFS1. Human Molecular Genetics 2001; 10: 2509–14.
   PubMed Web of Science ®Google Scholar