Newborn and Infant Hearing Screening Facing Globally Growing Numbers of People Suffering from Disabling Hearing Loss

Recent prevalence estimates indicate that in 2015 almost half a billion people—about 6.8% of the world’s population—had disabling hearing loss and that prevalence numbers will further increase. The World Health Organization (WHO) currently estimates that at least 34 million children under the age of 15 have disabling hearing loss. Based on a 2012 WHO report, approximately 7.5 million of these children were under the age of 5 years. This review article focuses on the importance of high-quality newborn and infant hearing screening (NIHS) programs as one strategy to ameliorate disabling hearing loss as a global health problem. Two WHO resolutions regarding the prevention of deafness and hearing loss have been adopted urging member states to implement screening programs for early identification of ear diseases and hearing loss in babies and young children. The effectiveness of these programs depends on factors such as governmental mandates and guidance; presence of a national committee with involvement of professionals, industries, and stakeholders; central oversight of hearing screening; clear definition of target parameters; presence of tracking systems with bi-directional data transfer from screening devices to screening centers; accessibility of pediatric audiological services and rehabilitation programs; using telemedicine where connectivity is available; and the opportunity for case discussions in professional excellence circles with boards of experts. There is a lack of such programs in middle- and low-income countries, but even in high-income countries there is potential for improvement. Facing the still growing burden of disabling hearing loss around the world, there is a need to invest in national, high-quality NIHS programs

A cyclic-AMP-gated conductance in cochlear hair cells

AbstractThe patch clamp technique was used to record cAMP-dependent currents of the guinea pig cochlear hair cell plasma membrane. Data obtained indicate that the channels passing this current are moderately selective for monovalent cations and are effectively blocked by L-cis-diltiazem and reversibly blocked by 1 mM Mg2+ or Ca2+. The single-channel unit conductance estimated in the absence of divalent cations is about 16 pS. The results demonstrate that cyclic nucleotide-dependent channels of cochlear hair cells are virtually identical to the photoreceptor and olfactory ones

Newborn and Infant Hearing Screening Facing Globally Growing Numbers of People Suffering from Disabling Hearing Loss

Recent prevalence estimates indicate that in 2015 almost half a billion people—about 6.8% of the world’s population—had disabling hearing loss and that prevalence numbers will further increase. The World Health Organization (WHO) currently estimates that at least 34 million children under the age of 15 have disabling hearing loss. Based on a 2012 WHO report, approximately 7.5 million of these children were under the age of 5 years. This review article focuses on the importance of high-quality newborn and infant hearing screening (NIHS) programs as one strategy to ameliorate disabling hearing loss as a global health problem. Two WHO resolutions regarding the prevention of deafness and hearing loss have been adopted urging member states to implement screening programs for early identification of ear diseases and hearing loss in babies and young children. The effectiveness of these programs depends on factors such as governmental mandates and guidance; presence of a national committee with involvement of professionals, industries, and stakeholders; central oversight of hearing screening; clear definition of target parameters; presence of tracking systems with bi-directional data transfer from screening devices to screening centers; accessibility of pediatric audiological services and rehabilitation programs; using telemedicine where connectivity is available; and the opportunity for case discussions in professional excellence circles with boards of experts. There is a lack of such programs in middle- and low-income countries, but even in high-income countries there is potential for improvement. Facing the still growing burden of disabling hearing loss around the world, there is a need to invest in national, high-quality NIHS programs

Evaluation of new technology for intraoperative evoked compound action potential threshold measurements

<div><p></p><p><i>Objective:</i> To determine whether new technology for intraoperative evoked compound action potential (ECAP) threshold measurements—the CR120 Intraoperative Remote Assistant device—is comparable to the existing Custom Sound^® clinical system, as well as assess test-retest accuracy of the systems. <i>Design:</i> Within subject, repeated measures comparative design. <i>Study sample:</i> ECAP data were collected from 81 pediatric subjects (41 females and 40 males). <i>Results:</i> The average ECAP threshold measurement time for the Custom Sound system was 6.2 minutes (SD = 1.0) versus 4.8 minutes (SD = 0.7) for the CR120 device. ECAP thresholds measured with Custom Sound and the CR120 device had an average Pearson product-moment correlation coefficient for all electrodes (N = 1724) of 0.92, p < 0.01; an average mean absolute difference of 6 CLs (SD = 6); and a 95% confidence interval of − 1 to 1 CLs. The test-retest results for Custom Sound and the CR120 device are also included. <i>Conclusion:</i> A significant reduction in the measurement time for ECAP thresholds was noted with the CR120 device. Furthermore, ECAP thresholds measured with the CR120 device are equivalent to Custom Sound within a clinically acceptable range.</p></div

Newborn and infant hearing screening facing globally growing numbers of people suffering from disabling hearing loss

Recent prevalence estimates indicate that in 2015 almost half a billion people - about 6.8% of the world’s population - had disabling hearing loss and that prevalence numbers will further increase. The World Health Organization (WHO) currently estimates that at least 34 million children under the age of 15 have disabling hearing loss. Based on a 2012 WHO report, approximately 7.5 million of these children were under the age of 5 years. This review article focuses on the importance of high-quality newborn and infant hearing screening (NIHS) programs as one strategy to ameliorate disabling hearing loss as a global health problem. Two WHO resolutions regarding the prevention of deafness and hearing loss have been adopted urging member states to implement screening programs for early identification of ear diseases and hearing loss in babies and young children. The effectiveness of these programs depends on factors such as governmental mandates and guidance; presence of a national committee with involvement of professionals, industries, and stakeholders; central oversight of hearing screening; clear definition of target parameters; presence of tracking systems with bi-directional data transfer from screening devices to screening centers; accessibility of pediatric audiological services and rehabilitation programs; using telemedicine where connectivity is available; and the opportunity for case discussions in professional excellence circles with boards of experts. There is a lack of such programs in middle- and low-income countries, but even in high-income countries there is potential for improvement. Facing the still growing burden of disabling hearing loss around the world, there is a need to invest in national, high-quality NIHS programs

Audiological Evidence of Frequent Hereditary Mild, Moderate and Moderate-to-Severe Hearing Loss

Congenital and early onset bilateral sensorineural hearing loss (SNHL) is mainly caused by mutations in numerous genes. The introduction of universal newborn hearing screening (UNHS) has increased the number of infants with mild, moderate, and moderate-to-severe sensorineural hearing loss (SNHL) detected in the first year of life. We aimed to evaluate the audiological features in patients with mild, moderate, and moderate-to-severe SNHL according to genotype. Audiological and genetic data were analyzed for 251 patients and their relatives with congenital bilateral mild, moderate, and moderate-to-severe SNHL. Hearing loss severity, audiogram profile, interaural symmetry, and dynamics of hearing thresholds were analyzed. In this case, 165 patients had GJB2 gene mutations, 30 patients were identified with STRC mutations, and 16 patients had pathogenic or likely pathogenic USH2A mutations. The presence of at least one GJB2 non-truncating variant in genotype led to less severe hearing impairment. The flat and gently sloping audiogram profiles were mostly revealed in all groups. The follow-up revealed the stability of hearing thresholds. GJB2, STRC, and USH2A pathogenic variants were detected in most patients in our cohort and were congenital in most cases