Abstract
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.
Highlights
Hearing loss (HL) is one of the most common sensory disorders that makes it a serious public health problem
We present the estimation of the prevalence of hereditary severe or profound HL in Russia based on the analysis of the 2010 census data concerning the knowledge of sign language
Information on Sign language (SL) users reported by local Federal State Statistics Service (FSSS) offices was available for 73 out of 85 federal subjects of Russia, and in this case, the total number of SL users was 107,064 out of 122,527,891 respondents, which corresponds to the same proportion of 0.087%
Summary
Hearing loss (HL) is one of the most common sensory disorders that makes it a serious public health problem. According to the World Health Organization (WHO), the number of people with disabling HL is approximately 466 million worldwide, including 34 million children [1]. The WHO data are based on the Global Burden of Diseases (GBD) project, which has estimated the HL prevalence based on only 42 studies in 29 countries [2]. Among many studies on HL prevalence in the general population, only a small number of surveys are suitable for analysis, and more population-based surveys are needed in all regions of the world [2, 3]. Uniform description of HL prevalence is available for only particular populations or regions since different studies are based on highly specific data and resources
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