Abstract

The incidence of hearing impairment (HI) is higher in low- and middle-income countries when compared to high-income countries. There is therefore a necessity to estimate the burden of this condition in developing world. The aim of our study was to use a systematic approach to provide summarized data on the prevalence, etiologies, clinical patterns and genetics of HI in Cameroon. We searched PubMed, Scopus, African Journals Online, AFROLIB and African Index Medicus to identify relevant studies on HI in Cameroon, published from inception to 31 October, 2019, with no language restrictions. Reference lists of included studies were also scrutinized, and data were summarized narratively. This study is registered with PROSPERO, number CRD42019142788. We screened 333 records, of which 17 studies were finally included in the review. The prevalence of HI in Cameroon ranges from 0.9% to 3.6% in population-based studies and increases with age. Environmental factors contribute to 52.6% to 62.2% of HI cases, with meningitis, impacted wax and age-related disorder being the most common ones. Hereditary HI comprises 0.8% to 14.8% of all cases. In 32.6% to 37% of HI cases, the origin remains unknown. Non-syndromic hearing impairment (NSHI) is the most frequent clinical entity and accounts for 86.1% to 92.5% of cases of HI of genetic origin. Waardenburg and Usher syndromes account for 50% to 57.14% and 8.9% to 42.9% of genetic syndromic cases, respectively. No pathogenic mutation was described in GJB6 gene, and the prevalence of pathogenic mutations in GJB2 gene ranged from 0% to 0.5%. The prevalence of pathogenic mutations in other known NSHI genes was <10% in Cameroonian probands. Environmental factors are the leading etiology of HI in Cameroon, and mutations in most important HI genes are infrequent in Cameroon. Whole genome sequencing therefore appears as the most effective way to identify variants associated with HI in Cameroon and sub-Saharan Africa in general.

Highlights

  • Hearing impairment (HI) is considered disabling when the loss of hearing is greater than 40 dB in the better-hearing ear in adults (15 years or older) or greater than 30 dB in the better-hearingGenes 2020, 11, 233; doi:10.3390/genes11020233 www.mdpi.com/journal/genesGenes 2020, 11, 233 ear in children (0 to 14 years) [1]

  • No pathogenic mutation was described in GJB6 gene, and the prevalence of pathogenic mutations in GJB2 gene ranged from 0% to 0.5%

  • When using a threshold of 40 dB in adults and 35 dB in children, the population-based prevalence of HI in Cameroon is about 3.6% and increases with age. It is low at 1.1% in children, is up to 6.5% in adults and rises to a level of 14.8% in participants aged of 50 years or more. This is consistent with report from the World Health Organization (WHO) which estimated that the prevalence of disabling HI in sub-Saharan Africa is about 4.5% in the general population, 1.9% in children and 6.4% in adults [1]

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Summary

Introduction

Hearing impairment (HI) is considered disabling when the loss of hearing is greater than 40 dB in the better-hearing ear in adults (15 years or older) or greater than 30 dB in the better-hearingGenes 2020, 11, 233; doi:10.3390/genes11020233 www.mdpi.com/journal/genesGenes 2020, 11, 233 ear in children (0 to 14 years) [1]. HI can be due to environmental or genetic causes, and in many cases it is not possible to establish a definite etiology [4,5] Environmental factors such as meningitis, measles or ototoxicity are the leading causes of HI in low- and middle-income countries, while their burden is lower in high income countries [5,6,7,8]. This is attributed to poor healthcare systems that are not always adequately equipped to prevent, screen and manage causes of HI [7]. Over 400 syndromes with HI have been described, including Waardenburg syndrome, branchiootorenal syndrome, Usher syndrome, Pendred syndrome, keratitis–ichthyosis–deafness syndrome and Alport syndrome [7,11,12]

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