Genetic counseling of the deaf. Medical and cultural considerations

Genetic counseling is a communication process where information is provided in a nondirective way. Genetic counseling emphasizes informed decision making and provision of medical, psychological, and social support; genetic counseling is not advice giving. Individuals who are members of the deaf community (culturally deaf) present unique challenges to the ability of genetic counselors to be nondirective. In contrast to the medical model which considers deafness to be a pathological condition, many deaf people do not consider themselves to be handicapped but define themselves as being part of a distinct cultural group with its own language, customs, and beliefs. Cultural and linguistic factors have a strong influence on the success of genetic counseling with deaf people. Strategies for effective genetic counseling to deaf people include the recognition that perception of "risk" is very subjective and that some deaf individuals may prefer to have deaf children. Other considerations for successful service provision include the use of appropriate questionnaires or history-taking tools, the use of qualified interpreters, and the revision of counseling materials and terminology to eliminate cultural bias. Nondirective counseling is also very dependent on the educational background and level of understanding of the consultants. There is a desperate need for education within the deaf community about genetics, particularly the less well educated, and a need for training of genetic counselors who have an appreciation of the linguistic and cultural differences of the deaf. Additionally, as with any other cultural, racial, or ethnic group, it would be very appropriate for deaf persons to be trained as genetic counselors to provide genetic counseling to deaf people. Unfortunately, there are at present no culturally deaf genetic counselors in the United States. Genetic counseling for deaf couples can also be complicated by complex family trees with mating between several deaf people and by the potential presence of other complicating features that may be associated with syndromic types of deafness. This requires careful history taking by trained geneticists and often long, complex explanations to families when the mode of inheritance cannot be confirmed. Even though deaf people most often do not pursue genetic counseling because of concerns for reproductive outcome, there is great enthusiasm among members of the deaf community about genetic services when they are provided in a manner that is sensitive to their linguistic and cultural differences. Genetic counseling for culturally deaf people can have great personal benefits for these individuals, can increase general knowledge regarding hereditary types of deafness and can help in developing strategies for providing appropriate genetic counseling for individuals with all degrees of hearing loss.