Review
Societal impact of bilirubin-induced hearing impairment in resource-limited nations

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Summary

Infants with bilirubin-induced neurologic dysfunction (BIND) are characterized by several developmental disabilities including auditory impairments. This paper explores the societal impact of bilirubin-induced auditory impairments, inclusive of hearing impairments and auditory neuropathy spectrum disorders, on these infants, their families, and on the community in resource-limited countries (per capita income of US$6,000 or less). Auditory impairments have substantial emotional, social, and economic impact on the affected infants, their families and communities. The burden is exacerbated by widespread poverty, unfavorable community attitudes towards disabilities, and lack of requisite health, educational, and social services. Curtailing the incidence of avoidable severe hyperbilirubinemia through proactive and effective management of infants at risk or with severe hyperbilirubinemia is necessary at all levels of healthcare delivery. Early detection and intervention for unavoidable auditory impairments should be widely promoted to provide improved developmental trajectories for the affected infants.

Introduction

Jaundice-related mortality and disabilities, rarely reported in the developed world, persist in disturbing proportions in many resource-limited countries [1; T.M. Slusher et al., unpublished data]. For example, in one recent review of the global burden of hyperbilirubinemia, Sub-Saharan Africa and South Asia were reported to be the leading contributors to an estimated 1.1 million babies who yearly would develop severe hyperbilirubinemia [total serum/plasma bilirubin level (TB) >20 mg/dL (342 μmol/L)] worldwide [1]. Another systematic review found that low- and middle-income countries (LMICs) consistently reported substantially higher rates of exchange transfusions and bilirubin-induced neurologic dysfunction (BIND), such as acute bilirubin encephalopathy (ABE) and chronic bilirubin encephalopathy or kernicterus, than in high-income countries [T.M. Slusher et al., unpublished data]. This is principally due to three levels of delay in providing care for infants with or at risk of severe hyperbilirubinemia, namely: making the decision to seek appropriate care, reaching an appropriate health facility, and receiving adequate/appropriate care [2].

The burden of this high prevalence of severe hyperbilirubinemia or kernicterus is perhaps best characterized by the associated adverse neurodevelopmental outcomes from early infancy [3], [4], [5], [6], [7]. For example, several studies have demonstrated significant etiological or statistical associations between auditory impairments in children and severe hyperbilirubinemia, with or without evidence of ABE or kernicterus [6], [7]. Like many disabilities, significant auditory impairments from early infancy have profound impacts on the affected child, their family, and society over their lifetime [8], [9]. This review therefore sets out to explore the societal impact of infants with auditory impairments secondary to BIND in resource-limited countries.

Section snippets

Definitions and terminologies

There are presently no consistent definitions of “resource-poor,” “resource-constrained,” or “resource-limited” countries in the literature. Although LMICs are frequently described as “resource-poor,” the term “LMICs” based on the World Bank classification broadly covers ∼140 countries with per capita gross national incomes (GNI) ranging from US$150 to 12,615 [10]. In view of the wide variations in income distribution and developmental status among these countries, we considered the 91

Spectrum of bilirubin-induced auditory impairments

Auditory dysfunctions typically associated with severe hyperbilirubinemia include mild-to-profound hearing loss [7], [14], [15], including high-frequency sensorineural hearing loss [16], [17] and auditory neuropathy spectrum disorders (ANSD) [18], [19], [20]. Affected infants may also have additional disabilities such as cerebral palsy, gross motor deficits, epilepsy, and behavioral problems [3], but rarely bilirubin-induced intellectual deficits [4], [21]. The threshold at which unbound

Impact of hearing impairment on the child

Early detection of infants with hearing impairments has been shown to be beneficial to optimal communication and developmental outcomes [39], [40]. However, unlike the practice in many developed countries, routine screening of newborns for congenital and early-onset hearing impairment is rare in LMICs [41]. Infants with hearing impairments are therefore not detected until a lack of response to a loud sound or a delay in speech prompts parental concern to seek medical intervention. Depending on

Impact of hearing impairment on the family

Early hospital discharge and the high proportion of infants delivered outside hospitals in LMICs result in the onset of severe hyperbilirubinemia occurring more frequently at home. The immediate impact on the family is that of concern for the ensuing severe illness in an otherwise healthy newborn. The prevailing maternal sequential health-behavior ‒ starting with self-medication, then a visit to traditional healers, and finally to a health facility ‒ results in considerable delays in receiving

Impact of hearing impairment on the community

While disabilities including auditory impairments are established as either causes or consequences of poverty, studies specifically exploring the societal or economic costs of auditory impairments in LMICs at the community level are rare [70], [71]. This information is necessary in considering possible governmental or community intervention for the affected children. Studies have also been limited in developed countries. It is, however, difficult from the available studies to disaggregate the

Addressing the burden of bilirubin-induced auditory impairments

Given the potential and substantial burden of bilirubin-induced auditory impairments in LMICs, primary prevention of BIND is of the highest priority. The low incidence of BIND in developed countries made possible by timely recognition and treatment of infants with or at risk of clinically significant hyperbilirubinemia is attainable in LMICs if the various constraints in the effective management of the affected infants are addressed at all levels of healthcare delivery [2]. The promotion of

Conclusion

Bilirubin-induced auditory impairments with or without co-morbidities with other developmental disabilities have substantial emotional, social, and economic impacts on the affected infants, their families, and communities in LMICs. The burden is exacerbated by the widespread poverty, unfavorable community disposition to disabilities, and lack of requisite health, educational, and social support services for these infants. Curtailing the incidence of avoidable severe hyperbilirubinemia through

Conflict of interest statement

None declared.

Funding sources

None.

Acknowledgements

The author would like to thank Professor Valerie E. Newton and Professor Steven M. Shapiro for helpful comments on an earlier draft of this manuscript.

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    This work was presented in part by the author at the World Health Summit, October 19–22, 2014, Berlin, Germany.

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