Part I Gene therapy.
Chapter 1 Gene therapy for hearing disorders – AK Lalwani, GJ Carvalho, JJ Han and AN Mhatre.
Part II Radiology.
Chapter 2 Radiology of inner ear defects – PD Phelps.
Part III Computer Systems
Chapter 3 An Internet database on genetic non–syndromal hearing impairments – M. Mazzoli, L. Saggin, SD Hatzopouilos and A Martini.
Chapter 4 A decision support system for the diagnosis of syndromal genetic hearing impairment – S Crino, A D′Amico, S Grisanti and G Grisanti.
Part IV Epidemiology
Chapter 5 Epidemiology of hereditary hearing impairment in childhood – preliminary estimates from the European Union – A Parving, RJC Admiraal, F Apaydin, E Arslan, A Davis, O Dias, H Fortnum, G Grisanti, M Gross, M Hess, K Konradsson, G Lina–Granade, VE Newton, C O′Donovan, E Orzan, M Sorri, D Stephens, MD Tsakanikox, M Waagenaar and K Welzl–Müller.
Chapter 6 The German Registry for Hearing Impairment in Children: preliminary results – A Cherechevskaia, A Costa, E Rosztok, M Hess and M Gross.
Chapter 7 Recessive hearing impairment in two birth cohorts in western Sicily – G Grisanti, AM Amodeo, S Crino and E Martines.
Chapter 8 Aetiology of hearing impairment in children borth in northern Finland ijn 1975–1979 and in 1985–1989 (Abstract) – E Maki–Torkko, P Lindholm, M Vayrynen and M Sorri.
Part V Audiovestibular tests.
Chapter 9 Audiometric criteria for linkage analysis in genetic hearing impairment – FL Wuyts, PH Van de Heyning an F Declau.
Chapter 10 Audioscan notches in carriers of genetic hearing impairment – F Zhao, D Stephens, R Meredith and VE Newton.
Chapter 11 Cochlear irregulatities in obligate carriers of recessive genetic hearing impairment and in control subjects – G Lina–Granade, M Kreiss, T Gelas, L Collet and A Morgon.
Chapter 12 Three–dimensional video–oculography for the detection of genetic vestibular dysfunction at the level of the threee semicircular canals and the otoliths (Abstract) – FL Wuyts, PH Van de Heyning, H Kingma, L Bourmans and D Van Dyck.
Part VI Non–syndromal autosomal recessive hearing impairment.
Chapter 13 Homozygosity mapping applied to hereditary hearing impairment – localizing recessive deafness genes – RJH Smith, A Ramesh, CR Srikumari Srisailapathy, K Fukushima, S Wayne, A Chen, L Van Laer, J Ashley, RIZ Zbar, M Lovett and G Van Camp.
Chapter 14 A Turkish kindred with autosomal recessive non–syndromal hearing impairment segregates DFNB9 (Abstract) – SM Leal, E Vitale, F Apaydin, Y Hu, C Barnwell, M Iber, T Kandogan, U Braendle, HP Zenner, M Schwalb and O Cura.
Chapter 15 Assessment of the contribution of the loci DFNA1–10 and DFNB1–9 in inherited hearing impairment in two populations: The United Arab Emirates and the British Pakistani populations (Abstract) – KA Brown, G Karban, G Parry, LL Moynihan, AH Janjua, LI Al–Gazali, VE Newton, AF Markham and RF Mueller.
Part VII Non–syndromal autosomal dominant hearing impairment.
Chapter 16 Hereditary dominant non–syndromal progressive hearing impairment in a large family in southern Italy – A Bojano, L Califano and P Capparuccia.
Chapter 17 Autosomal dominant non–syndromal progressive sensorineural hearing impairment: audiological evalution of a Dutch DFNA2 family (Abstract) – H Kunst, HAM Marres, PLM Huygen, P Coucke, P Willems and CWRJ Cremers.
Chapter 18 Autosomal dominant congenital severe sensorineural hearing impairment – localization of a disease gene to chromosome 11q by linkage in an Austrian family (Abstract) – K Kirschhofer, JBN Kenyon, DM Hoover, P Franz, K Weopoltshammer, F Wachtler and WJ Kimberling.
Part VIII X–Linked hearing impairment
Chapter 19 Identification of a novel locus for non–syndromal X–linked sonsorineural impairment (DFN6) on Xp22 (Abstract) – I del Castillo, M Villamar, M Sarduy, L Romero, C Herraiz, F Javier Hernandez, M Rodriguez, I Borras, A Montero, J Bellon, M Cruz Tapia and F Moreno.
Part IX Mitochondrial hearing impairment
Chapter 20 Nuclear candidate genes for ′mitochondrial deafness′ – HT Jacobs, ZH Shah, V Migliosi, SK Lehtinen, A Rovio and K O′Dell.
Chapter 21 A mitochondrial point mutation at position 7472 causes early onset hearing impairment and late onset neurological symptoms. Report of a Dutch and a comparison with a Sicilian family – RJH Ensink, PLM Huygen, HAM Marres, K Verhoeven, G Van Camp and GW Padberg.
Chapter 22 Genetic study of mitochondrially inherited sensorineural hearing impairment in eight large families from Spin and Cuba – M Sarduy, I del Castillo, M Villamar, L Romero, C Heraiz, F Javier Hernandex, M Cruz Tapia, C Magarino, D Menendez del Castillo, I Menendez–Alejo, R Ramirez, B Arellano, C Morales, J Bellon and F Moreno.
Chapter 23 Hearing impairment in mitochondrial point mutation – E Orzan, L Bartolomei, V Magnavita and E Arslan.
Chapter 24 Prevalence of the base pair 3243 mutation of the tRNALeu gene in the mitochondrial DNA in a population–based cohort of patients with sensorineural hearing impairment (Abstract) – S Uimonen, I Hassinen, M Sorri and K Majamaa.
Part X Syndromal conditions
Chapter 25 Otorhinolaryngological manifestations of Stickler syndrome linked to chromosome 6 near the COL11A2 gene – RJC Admiraal, HG Brunner, PLM Huygen and CWRJ Cremers.
Chapter 26 Dominant hemifacial microsomia in a four–general pedigree – A McInerney, R Winter and M Bitner–Glindzicz.
Chapter 27 Variability of expression of sensorineural hearing loss in Usher syndrome: report of a family – D Zanetti and AR Antonelli.
Chapter 28 Mpv 17 – Glomerulosclerosis gene is essential for inner ear function – AM Meyer zum Gottesberge, B Eschen, A Reuter, L Kintrup and H Weiher.
Chapter 29 Cloning of a cadidate gene for hearing defects in CATCH 22 syndrome (Abstract) – A Pizzuti, G Novelli, A Ratti, F Amati, A Mari, G Calabrese, S Nicolis, V Silani, B Marino, G Scarlato, S Ottolenghi, R Mingarelli and B Dallapiccola.