vavilovВавиловский журнал генетики и селекцииVavilov Journal of Genetics and Breeding2500-3259Institute of Cytology and Genetics of Siberian Branch of the RAS10.18699/VJ16/098vavilov-517Research ArticleГЕНЕТИКА ЧЕЛОВЕКАHUMAN GENETICSРоль социально-демографической структуры сообществ глухих людей в распространенности наследуемых форм потери слухаImpact of socio-demographic structure of the deaf people communities in prevalence of hereditary hearing lossПосухО. Л.PosukhO. L.posukh@bionet.nsc.ruБады-ХооМ. С.Bady-KhooM. S.ЗыцарьМ. В.ZytsarM. V.МихальскаяВ. Ю.MikhalskaiaV. Yu.ЛашинС. А.LashinS. A.БарашковН. А.BarashkovN. A.РомановГ. П.RomanovG. P.Федеральное государственное бюджетное научное учреждение «Федеральный исследовательский центр Институт цитологии и генетики Сибирского отделения Российской академии наук», Новосибирск, Россия Федеральное государственное автономное образовательное учреждение высшего образования «Новосибирский национальный исследовательский государственный университет», Новосибирск, РоссияРоссияInstitute of Cytology and Genetics SB RAS, Novosibirsk, Russia Novosibirsk State University, Novosibirsk, RussiaRussian FederationФедеральное государственное бюджетное научное учреждение «Федеральный исследовательский центр Институт цитологии и генетики Сибирского отделения Российской академии наук», Новосибирск, Россия Государственное бюджетное учреждение здравоохранения Республики Тыва «Перинатальный центр Республики Тыва», Кызыл, РоссияРоссияInstitute of Cytology and Genetics SB RAS, Novosibirsk, Russia Perinatal Center of the Tyva Republic, Kyzyl, RussiaRussian FederationФедеральное государственное бюджетное научное учреждение «Федеральный исследовательский центр Институт цитологии и генетики Сибирского отделения Российской академии наук», Новосибирск, Россия Федеральное государственное автономное образовательное учреждение высшего образования «Новосибирский национальный исследовательский государственный университет», Новосибирск, РоссияРоссияInstitute of Cytology and Genetics SB RAS, Novosibirsk, Russia Novosibirsk State University, Novosibirsk, RussiaRussian FederationФедеральное государственное бюджетное научное учреждение «Якутский научный центр комплексных медицинских проблем», Якутск, Россия Федеральное государственное автономное образовательное учреждение высшего профессионального образования «Северо-Восточный федеральный университет им. М.К. Аммосова», Якутск, РоссияРоссияYakut Scientific Center of Сomplex Мedical Рroblems, Yakutsk, Russia M.K. Ammosov North-Eastern Federal University, Yakutsk, RussiaRussian Federation201611032016201715Copyright © Посух О.Л., Бады-Хоо М.С., Зыцарь М.В., Михальская В.Ю., Лашин С.А., Барашков Н.А., Романов Г.П., 20162016Посух О.Л., Бады-Хоо М.С., Зыцарь М.В., Михальская В.Ю., Лашин С.А., Барашков Н.А., Романов Г.П.Posukh O.L., Bady-Khoo M.S., Zytsar M.V., Mikhalskaia V.Y., Lashin S.A., Barashkov N.A., Romanov G.P.This work is licensed under a Creative Commons Attribution 4.0 License.https://vavilov.elpub.ru/jour/article/view/517

Потеря слуха, обусловленная как средовыми, так и генетическими причинами, в той или иной степени затрагивает более чем 10 % населения мира, приводит к инвалидности и существенно снижает качество жизни глухих людей. В среднем 1 из 1 000 новорожденных рождается глухим, и в 50–60 % случаев патология имеет генетическую причину. Несиндромальная наследственная потеря слуха – моногенное заболевание с уникально высокой генетической гетерогенностью. Частота форм «генетической глухоты» варьирует в разных регионах мира и может определяться, как и для многих других моногенных заболеваний, этническим составом населения, изоляцией, эффектами основателя и «бутылочного горлышка», долей близкородственных браков, возможным селективным преимуществом гетерозигот. Предполагается, что в распространенности наиболее частой генетической формы потери слуха, обусловленной мутациями гена GJB2 (Сx26), важную роль играли и определенные социальные факторы: долговременная традиция заключения ассортативных браков между глухими людьми в сочетании с ростом их социальной адаптации и биологической приспособленности (genetic fitness). Стартом для этих событий явился «прорыв» глубокой социальной изоляции глухих людей, произошедший около 300 лет назад, когда в Европе (а позднее и в США) возникли школы для глухих с обучением жестовому языку как единому средству коммуникации (лингвистическая гомогамия). Компьютерное моделирование и сравнительное ретроспективное исследование показали, что в США за 200-летний период эти социальные процессы могли привести к удвоению частоты глухоты, обусловленной мутациями гена GJB2. Сведения о социально-демографической структуре сообществ глухих людей в прошлом крайне ограничены практически полным отсутствием соответствующих архивных данных. Тем не менее изучение социально-демографических и медико-генетических характеристик современных сообществ глухих людей имеет важное значение как для прогнозирования распространенности различных форм наследственной глухоты, так и понимания роли социальных факторов в эволюционных процессах, происходящих в популяциях человека.

Hearing loss caused by environmental or genetic factors concerns more than 10 % of the world population. It leads to disability and considerably reduces the life quality of deaf people. On average, 1 in 1,000 newborns are born deaf, and 50-60 % of cases are due to genetic causes. Nonsyndromic hereditary deafness is a monogenic disease with uniquely high genetic heterogeneity. The prevalence of some forms of genetic deafness varies in different populations and could be determined, as for many other genetic diseases, by the ethnic composition of a population, isolation, founder and «bottleneck» effects, the proportion of consanguineous marriages, and probable heterozygote advantage. It is assumed that high prevalence of hearing loss due to mutations in the GJB2 (Cx26) gene was also influenced by some social factors: a long-standing tradition of assortative marriages between deaf people, combined with growth of their social adaptation and genetic fitness. The start for these events was the breakdown of the deep social isolation of deaf people, which occurred about 300 years ago in Europe, and later in the US, when special schools for the deaf with learning sign language as a common tool for communication were established (linguistic homogamy). Computer simulations and comparative retrospective study showed that over the past 200 years these social processes can have doubled the frequency of deafness in the US caused by the GJB2 gene mutations. Information about the sociodemographic structure of deaf communities in the past is extremely limited by an almost complete lack of relevant archival data. Nevertheless, studies of sociodemographic and medical-genetic characteristics of deaf people’s contemporary communities are important for predicting the prevalence of inherited forms of deafness, as well as for understanding the impact of social factors on the evolutionary processes occurring in human populations.

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The authors declare that there are no conflicts of interest present.