PRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 95 No. 1 January 2002
Counseling for Congenital Hearing Loss
Juichi
Ito
(Kyoto University)
Since the development of clinical genetic
medicine, we have needed the knowledge and ability to provide genetic counseling
in the Department of Otolaryngology.
Genetic counseling for
congenital hearing loss plays an especially important role in medicine. We have
encountered more than 40 such cases in our hospital. Counseling for congenital
hearing loss is very complex. Even if both parents are affected by deafness, the
risk of their children developing deafness is around 10 per cent, which is very
low compared with other genetic disorders. Before the discovery of the genes
responsible for hearing loss, it was possible to predict hearing loss in the
next child only on the basis of previous data. However, in the past 10 years,
many genes responsible for congenital hearing loss have been identified. In fact
genetic diagnosis of hearing loss is possible using molecular techniques. Thus,
genetic counseling is more complicated than that for other diseases. As is the
case for other disease, ethical problems are deeply involved. We discuss not
only the molecular technique but also the importance of genetic counseling for
the happiness of the patient.
Key words : counseling, congenital hearing loss, gene, deaf mutism, syndromic deafness, non-syndromic deafness