Deaf Community and Genetics

Abstract

The Deaf community consists of a group of like minded people sharing a common sign language and culture. This community has a positive attitude toward being deaf. Typically, deafness is considered a strong part of linguistic and cultural identity and Deaf individuals do not wish to have treatments or a cure. Deaf people have concerns that a hearing society, with little knowledge or experience of their rich culture and language, would encourage the use of pre‐implantation genetic diagnosis and prenatal genetic testing for deafness with the ultimate aim of having hearing children. They feel strongly that deafness is a source of human variation that does not warrant the use of genetic technology in this way. Deaf (written with an uppercase 'D') refers to people who belong to the Deaf community. Deaf people use sign language (e.g. British Sign Language, American Sign Language, Auslan, etc.) as their first or preferred language. They also have a positive identity attached to being Deaf. People who consider themselves deaf (written with a lowercase 'd') or hard of hearing tend to use speech as their preferred form of communication, and may experience being deaf as a medical disability that needs to be treated. These groups often have very differing attitudes towards the use of genetic technology.

Key Concepts:

  • Deaf people often do not mind if their children are deaf or hearing; whereas hearing or hard of hearing people typically prefer to have hearing children.

  • Genetic technology can be used to reduce the number of deaf children born, for example, via the use of preimplantation genetic diagnosis with a selection of embryos that contain the genes for hearing or via the use of prenatal genetic diagnosis where a termination of pregnancy is offered if the baby is found to have the genes for deafness.

  • There is a serious concern amongst the Deaf community that genetic technology will be ‘misused’ by hearing people to select against deafness. If significant number of people choose to use genetic technologies to facilitate the selection of hearing children then they fear that this will result in decline of the Deaf community.

  • If fewer deaf babies are born, Deaf people recognise that this may result in a decimation of their language and culture, and an increasing disregard for the rights of self‐determination of deaf individuals.

  • Being deaf can arise as a result of different factors, genetics is only one of these. It is likely that there will always be environmental causes, newly arising genetic causes, and people who do not use genetic technology for diagnostic purposes, and so there will always be D/deaf people in society.

Keywords: deafness; attitudes; Deaf culture; prenatal genetic diagnosis; preimplantation genetic diagnosis

References

Bahan B (1989) What if. … Alexander Graham Bell had gotten his way? In: Wilcox S (ed.) American Deaf Culture, pp. 83–87. Silver Spring, MD: Linstock Press.

Baldwin EE, Boudreault P, Fox M, Sinsheimer JS and Palmer CG (2012) Effect of pre‐test genetic counseling for deaf adults on knowledge of genetic testing. Journal of Genetic Counseling 21(2): 256–272.

Boudreault P, Baldwin EE, Fox M et al. (2010) Deaf adults’ reasons for genetic testing depend on cultural affiliation: results from a prospective, longitudinal genetic counseling and testing study. Journal of Deaf Studies and Deaf Education 15: 209–227.

British Deaf Association (2001) Genetics Policy Statement. London, UK: BDA.

Brunger JW, Murray GS, O'Riordan M et al. (2000) Parental attitudes toward genetic testing for pediatric deafness. American Journal of Human Genetics 67(6): 1621–1625.

Burton SK, Withrow K, Arnos KS, Kalfoglou AL and Pandya A (2006) A focus group study of consumer attitudes toward genetic testing and newborn screening for deafness. Genetics in Medicine 8(12): 779–783.

Christiansen JB (1991) Sociological implication of hearing loss. Annals of the New York Academy of Sciences 630: 230–235.

Dagan O, Hochner H, Levi H, Raas‐Rithschild A and Sagi M (2002) Genetic testing for hearing loss: different motivations for the same outcome. American Journal of Medical Genetics 113(2): 137–143.

Department of Work and Pensions (2002) Research and Statistics. http://statistics.dwp.gov.uk/asd/asd5/report_abstracts/ih_abstracts/iha_102.asp. Accessed on 28 Aug 2011.

Dolnick E (1993) Deafness as culture. The Atlantic Monthly 272(3): 37–53.

Emery SD, Middleton A and Turner GH (2010) Whose deaf genes are they anyway? The deaf community challenge to legislation on embryo selection. Sign Language Studies 10(2): 155–169.

Emery SE, Blankmeyer Burke T, Middleton A, Belk R and Turner GH (2008) Reproductive liberty and deafness clause 14(4)(9) of embryo bill should be amended or deleted. British Medical Journal 336: 976.

Enns EE, Boudreault P and Palmer CG (2010) Examining the relationship between genetic counselors’ attitudes toward deaf people and the genetic counselling session. Journal of Genetic Counseling 19(2): 161–173.

Euteneuer S and Ryan A (2007) Mechanisms for hair cell protection and regeneration in the mammalian organ of Corti. In: Martini A, Dafydd S and Read A (eds) Genes, Hearing and Deafness. From Molecular Biology to Clinical Practice, pp. 305–312. London: Informa healthcare.

Grosjean F (2008) Studying Bilinguals, pp. 221–237. New York: Oxford University Press.

Hoffmeister R (1985) Families with deaf parents: a functional perspective. In: Thurman K (ed.) Children of Handicapped Parents: Research and Clinical Perspectives, pp. 111–130. Orlando, FL: Academic Press.

Israel J (ed.) (1995) An Introduction to Deafness: A Manual for Genetic Counselors. Washington DC: Gallaudet Research Institute, Gallaudet University.

Ladd P (2003) Understanding Deaf Culture: In Search of Deafhood. Clevedon: Multilingual Matters Ltd.

Levy N (2002) Deafness, culture, and choice. Journal of Medical Ethics 28(5): 284–285.

Mand C, Duncan RE, Gillam L, Collins V and Delatycki MB (2009) Genetic selection for deafness: the views of hearing children of deaf adults. Journal of Medical Ethics 35(12): 722–728.

Martinez A, Linden J, Schimmenti LA and Palmer CG (2003) Attitudes of the broader hearing, deaf, and hard‐of‐hearing community toward genetic testing for deafness. Genetics in Medicine 5(2): 106–112.

Middleton A, Emery SD and Turner GH (2010) Views, knowledge and beliefs about genetics and genetic counselling amongst people with deafness. Sign Language Studies 10(2): 170–196.

Middleton A, Hewison J and Mueller RF (1998) Attitudes of deaf adults towards genetic testing for hereditary deafness. American Journal of Human Genetics 63(4): 1175–1180.

Middleton A, Hewison J and Mueller RF (2001) Prenatal diagnosis for inherited deafness – what is the potential demand? Journal of Genetic Counseling 10(2): 121–131.

Mitchell RE, Young TA, Bachleda B and Karchmer MA (2006) How many people use ASL in the United States? Why estimates need updating. Sign Language Studies 6(3): 306–335.

Murphy TF (2009) Choosing disabilities and enhancements in children: a choice too far? Reproductive BioMedicine Online 18(Suppl. 1): 43–49.

Nance W (2003) The genetics of deafness. Mental Retardation and Developmental Disabilities Research Reviews 9: 109–119.

Nunes R (2006) Deafness, genetics and dysgenics. Medicine, Health Care and Philosophy 9(1): 25–31.

Padden C (1980) The deaf community and the culture of deaf people. In: Wilcox S (ed.) American Deaf Culture, pp. 1–16. Silver Spring, MD: Linstock Press.

Padden C and Humphries T (2005) Inside Deaf Culture. London: Harvard University Press.

Palmer CGS, Martinez A, Fox M et al. (2009) A prospective, longitudinal study of the impact of GJB2/GJB6 genetic testing on the beliefs and attitudes of parents of deaf and hard‐of‐hearing infants. American Journal of Medical Genetics Part A 149A: 1169–1182.

Pfister M and Lalwani AK (2007) Gene therapy of the inner ear. In: Martini A, Stephens D and Read A (eds) Genes, Hearing and Deafness. From Molecular Biology to Clinical Practice, pp. 299–304. London: Informa healthcare.

Robertson JA (2003) Extending preimplantation genetic diagnosis: the ethical debate. Human Reproduction 18(3): 465–471.

Schuchman J (2004) Deafness and eugenics in the Nazi era. In: Van Cleve JV (ed.) Genetics, Disability and Deafness , p. 72–78. Washington, DC: Gallaudet University Press.

Stephens D and Danermark B (2005) The international classification of functioning, disability and health as a conceptual framework for the impact of genetic hearing impairment. In: Dafydd S and Lesley J (eds) The Impact of Genetic Hearing Impairment, pp. 54–67. London and Philadelphia: Whurr Publishers.

Stern SJ, Arnos KS, Murrelle L et al. (2002) Attitudes of deaf and hard of hearing subjects towards genetic testing and prenatal diagnosis of hearing loss. Journal of Medical Genetics 39: 449–453.

Van Camp G and Smith RJH (2011) Deafness and hereditary hearing loss overview. Accessed on 28 August 2011 from http://hereditaryhearingloss.org.

Wang J, Guitton M, Ruel J, Pujol R and Puel J‐L (2007) Innovative therapeutical strategies to prevent deafness and to treat tinnitus. In: Allessandro M, Dafydd S and Andrew R (eds) Genes, Hearing and Deafness. From Molecular Biology to Clinical Practice, pp. 271–278. London: Informa healthcare.

Further Reading

Arnos KS, Israel J and Cunningham M (1991) Genetic counseling for the deaf: medical and cultural considerations. Annals of the New York Academy of Sciences 630: 212–222.

Davis DS (1997) Genetic dilemmas and the child's right to an open future. Hastings Central Report 27: 7–15.

Grundfast KM and Rosen J (1992) Ethical and cultural considerations in research on hereditary deafness. Otolaryngologic Clinics of North America 25(5): 973–978.

Jordan IK (1991) Ethical issues in the genetic study of deafness. Annals of the New York Academy of Sciences 630: 236–239.

Kaplan D (1993) Prenatal screening and its impact on persons with disabilities. Fetal Diagnostic Therapy 8(3): 64–69.

Lane HL (1989) When the Mind Hears – A History of the Deaf. USA: Vintage Books.

Padden C and Humphries T (1990) Deaf in America: Voices from a Culture. USA: Harvard University.

Resendes B, Williamson RE and Morton CC (2001) At the speed of sound: gene discovery in the auditory system. American Journal of Human Genetics 69: 923–935.

Sacks OW (2002) Seeing Voices: A Journey into the World of the Deaf. USA: Vintage Books.

Web Links

Deafhood and genetics: http://www.deafhoodgenetics.com/

Hereditary Hearing Loss home page: http://hereditaryhearingloss.org/

Overview of various BSL publications: http://www.bsluptake.org.uk/category/dr‐steve‐emery/

University of Manchester, information about genetic counselling in BSL: http://sites.mhs.manchester.ac.uk/what‐is‐genetic‐counselling

Contact Editor close
Submit a note to the editor about this article by filling in the form below.

* Required Field

How to Cite close
Middleton, Anna, Emery, Steve, Palmer, Christina, and Boudreault, Patrick(Jan 2013) Deaf Community and Genetics. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0005875.pub2]