Dafydd Stephens & Lesley Jones 
The Effects of Genetic Hearing Impairment in the Family 

There has been an explosion of studies in the field of genetic
hearing impairment in the past decade, associated with major
advances in our understanding of the mechanisms and conditions
involved. However, a recent review has highlighted the very limited
number of studies on the effects of such hearing impairment on the
individuals and families of those concerned.

In The Effects of Genetic Hearing Impairment in the
Family, under the aegis of the European Union GENDEAF
programme, the editors have taken the first steps to address this
deficit in our knowledge and understanding of this topic. The book
addresses the problem by secondary analyses of existing large scale
population studies, by prospective investigation of individuals
with a family history of hearing impairment and by specific studies
on patients with otosclerosis and neurofibromatosis 2 and their
families.

In addition several chapters look at the specific impact of deaf
culture, ethnicity and religion on reactions to deafness and the
specific needs in genetic counselling.

This book represents an important first step in this field and
should be an invaluable resource for all professionals involved
with people with hearing impairments.

Table of Content

Contributors.

Preface.

Acknowledgements.

PART I: RETROSPECTIVE STUDIES.

1. The impact of having a family history of hearing loss inelderly people (Dafydd Stephens, Peter Lewis and Adrian Davis).

2. The impact of a family history of hearing loss in the Blue Mountain Study (Doungkamol Sindhusake, Dafydd Stephens, Phillip Newall and Paul Mitchel).l

3. The impact for children of having a family history of hearingimpairment in a UK-wide population study (Heather Fortnum, Garry Barton, Dafydd Stephens, Paula Stacey, A. Quentin Summerfield).

4. Early childhood hearing impairment and family history – along-term perspective (Per-Inge Carlsson and Berth Danermark).

5. Effects of a history of hearing problems in the family oforigin on the working life (Lotta Coniavitis Gellerstedt and Berth Danermark.) Prospective studies – late onset hearingimpairment.

PART II: PROSPECTIVE STUDIES-LATE ONSET HEARING IMPAIRMENT.

6. Effects of a family history on late onset hearing impairment:results of an open-ended questionnaire (Sophia E. Kramer, Adriana A. Zekveld and Dafydd Stephens).

7. Effects of a family history on late onset hearing impairment:results of an open-ended questionnaire (Sophia E. Kramer, Adriana A. Zekveld and Dafydd Stephens).

8. The impact that a family history of hearing impairment has onthose with the condition themselves (Sarah Coulson).

9. Influence of a family history of hearing impairment onparticipation restriction, activity limitation, anxiety anddepression (Angeles Espeso and Dafydd Stephens).

10. Does a family history of hearing impairment effect helpseeking behaviour and attitudes to rehabilitation (Claire Wilsonand Dafydd Stephens).

11. The impact of a Family History of Hearing Impairment onrehabilitative intervention: A one-year follow-up (Christophesaglier, Fernando Perez-Diaz, Lionel Collet and Roland Jouvent).

PART III: CONDITIONS ASSOCIATED WITH FAMILIAL HEARINGIMPAIRMENT.

12. The influence of a family history of hearing loss and/or oftinnitus on tinnitus annoyance and distress (Sylviane Chéry-Croze and Hung Thai-Van).

13. Tinnitus -The Impact of Family History (Veronica Kennedy and Dafydd Stephens).

14. Psychosocial aspects of Neurofibromatosis type 2 reported byaffected individuals (Wanda Neary, Dafydd Stephens, Richard Ramsden, and Gareth Evans).

15. Psychosocial aspects of Neurofibromatosis type 2 reported byrelatives/ significant others (Wanda Neary, Dafydd Stephens, Richard Ramsden, and Gareth Evans).

16. Attitudes of Adults with Otosclerosis Towards Issues Surrounding Genetics and the Impact of Hearing Loss (Anna Middleton, Ioannis Moumoulidis, Graeme Crossland, Mallappa Raghu, Pranay Kumar Singh, Evan Reid, Patrick Axon).

17. Peoples reaction to having a family history of Otosclerosis(Dafydd Stephens and Nele Lemkens).

PART IV: GENETIC COUNSELLING AND FAMILY REACTIONS.

18. Genetic Counselling and the d/Deaf Community (Anna Middleton).

19. Seeing Chromosomes: improving access to culturally-sensitivegenetic counselling through the provision of genetic information in British Sign Language (Rachel Belk).

20. Ethnicity, spirituality and genetics services (Lesley Jones, Ghazalla Mir and Rohanna Khan(.

21. Living with NF2 (Peter Crawshaw and Cynthia Crawshaw(.

22. The meaning of hearing loss in the same family over nearly200 years (Anna-Carin Rehnman).

PART V: RESEARCH NEEDS.

23. Family History of Hearing Impairment and its psychologicaland social consequences – what next (Berth Danermark, withcontributions from Per-Inge Carlsson, Lesley Jones and Dafydd Stephens).

Index.

About the author

Prof Dafydd Stephens (Ed) Welsh Hearing Insitute, University
Hospital of Wales, Cardiff, Wales

Dr Lesley Jones (Ed) Hull-York Medical School, University
of York