TY - JOUR

T1 - A new approach to estimating the prevalence of hereditary hearing loss

T2 - An analysis of the distribution of sign language users based on census data in Russia

AU - Romanov, Georgii P.

AU - Pshennikova, Vera G.

AU - Lashin, Sergey A.

AU - Solovyev, Aisen V.

AU - Teryutin, Fedor M.

AU - Cherdonova, Aleksandra M.

AU - Borisova, Tuyara V.

AU - Sazonov, Nikolay N.

AU - Khusnutdinova, Elza K.

AU - Posukh, Olga L.

AU - Fedorova, Sardana A.

AU - Barashkov, Nikolay A.

N1 - Publisher Copyright: © 2020 Romanov et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Copyright: Copyright 2020 Elsevier B.V., All rights reserved.

PY - 2020/11

Y1 - 2020/11

N2 - The absence of comparable epidemiological data challenges the correct estimation of the prevalence of congenital hearing loss (HL) around the world. Sign language (SL) is known as the main type of communication of deaf people. We suggest that the distribution of SL can be interpreted as an indirect indicator of the prevalence of congenital HL. Since a significant part of congenital HL is due to genetic causes, an assessment of the distribution of SL users can reveal regions with an extensive accumulation of hereditary HL. For the first time, we analyzed the data on the distribution of SL users that became available for the total population of Russia by the 2010 census. Seventy-three out of 85 federal regions of Russia were ranked into three groups by the 25th and 75th percentiles of the proportion of SL users: 14 regions-"low proportion"; 48 regions-"average proportion"; and 11 regions-"high proportion". We consider that the observed uneven prevalence of SL users can reflect underlying hereditary forms of congenital HL accumulated in certain populations by specific genetic background and population structure. At least, the data from this study indicate that the highest proportions of SL users detected in some Siberian regions are consistent with the reported accumulation of specific hereditary HL forms in indigenous Yakut, Tuvinian and Altaian populations.

AB - The absence of comparable epidemiological data challenges the correct estimation of the prevalence of congenital hearing loss (HL) around the world. Sign language (SL) is known as the main type of communication of deaf people. We suggest that the distribution of SL can be interpreted as an indirect indicator of the prevalence of congenital HL. Since a significant part of congenital HL is due to genetic causes, an assessment of the distribution of SL users can reveal regions with an extensive accumulation of hereditary HL. For the first time, we analyzed the data on the distribution of SL users that became available for the total population of Russia by the 2010 census. Seventy-three out of 85 federal regions of Russia were ranked into three groups by the 25th and 75th percentiles of the proportion of SL users: 14 regions-"low proportion"; 48 regions-"average proportion"; and 11 regions-"high proportion". We consider that the observed uneven prevalence of SL users can reflect underlying hereditary forms of congenital HL accumulated in certain populations by specific genetic background and population structure. At least, the data from this study indicate that the highest proportions of SL users detected in some Siberian regions are consistent with the reported accumulation of specific hereditary HL forms in indigenous Yakut, Tuvinian and Altaian populations.

UR - http://www.scopus.com/inward/record.url?scp=85097035834&partnerID=8YFLogxK

U2 - 10.1371/journal.pone.0242219

DO - 10.1371/journal.pone.0242219

M3 - Article

C2 - 33253245

AN - SCOPUS:85097035834

VL - 15

SP - e0242219

JO - PLoS ONE

JF - PLoS ONE

SN - 1932-6203

IS - 11 November

M1 - e0242219

ER -