Does COVID-19 have an impact on hearing?

BACKGROUNDS: SARS-CoV-2 is known to be a neurotrophic virus. However, the effect of this virus on the hearing system is still uncertain. OBJECTIVES: We aimed to investigate the possible effect of COVID-19 on hearing. MATERIALS AND METHODS: Thirty healthcare workers who had COVID-19 after hearing evaluation with pure tone audiometry (PTA) for any reason in the last 1 year were included in the study. PTA and transient evoked otoacoustic emissions (TEOAE) tests were performed in 15 of 30 patients during the active infection period. For all 30 patients, otoscopic examination plus PTA and TEOAE tests were performed at the end of the first month after their treatment. RESULTS: When the PTA results of 30 patients (60 ears) before and after COVID-19 were compared, a significant decrease in hearing level was found only at 1000 Hz (p < .05). There were no significant differences at other frequencies. When the PTA and TEAOE test results of 15 patients (30 ears) that were performed during and after COVID-19 were compared, no significant differences were found. CONCLUSION AND SIGNIFICANCE: We conclude that COVID-19 may cause hearing loss. However, this result needs to be confirmed with comprehensive studies to be conducted in larger patient groups.

Embryonic Origins of Virus-Induced Hearing Loss: Overview of Molecular Etiology.

Hearing loss, one of the most prevalent chronic health conditions, affects around half a billion people worldwide, including 34 million children. The World Health Organization estimates that the prevalence of disabling hearing loss will increase to over 900 million people by 2050. Many cases of congenital hearing loss are triggered by viral infections during different stages of pregnancy. However, the molecular mechanisms by which viruses induce hearing loss are not sufficiently explored, especially cases that are of embryonic origins. The present review first describes the cellular and molecular characteristics of the auditory system development at early stages of embryogenesis. These developmental hallmarks, which initiate upon axial specification of the otic placode as the primary root of the inner ear morphogenesis, involve the stage-specific regulation of several molecules and pathways, such as retinoic acid signaling, Sonic hedgehog, and Wnt. Different RNA and DNA viruses contributing to congenital and acquired hearing loss are then discussed in terms of their potential effects on the expression of molecules that control the formation of the auditory and vestibular compartments following otic vesicle differentiation. Among these viruses, cytomegalovirus and herpes simplex virus appear to have the most effect upon initial molecular determinants of inner ear development. Moreover, of the molecules governing the inner ear development at initial stages, SOX2, FGFR3, and CDKN1B are more affected by viruses causing either congenital or acquired hearing loss. Abnormalities in the function or expression of these molecules influence processes like cochlear development and production of inner ear hair and supporting cells. Nevertheless, because most of such virus-host interactions were studied in unrelated tissues, further validations are needed to confirm whether these viruses can mediate the same effects in physiologically relevant models simulating otic vesicle specification and growth.

Late-onset Hearing Loss From Congenital Cytomegalovirus Infection After Newborn Period in a Highly Immune Population in China.

After following 141 children with likely asymptomatic congenital cytomegalovirus infection in a highly immune population in China, four children (2.8%) were found to have late-onset hearing loss. No maternal or childhood factors, except higher saliva cytomegalovirus viral load at birth (P = 0.03), were associated with increased risk of developing a hearing loss.

Estimated Cost-effectiveness of Newborn Screening for Congenital Cytomegalovirus Infection in China Using a Markov Model.

Importance: Congenital cytomegalovirus infection (cCMVi) is one of the most common infections associated with childhood hearing loss. Prevention and mitigation of cCMVi-related hearing loss will require an increase in newborn screening, which is not yet available in China. Objective: To estimate the cost-effectiveness of newborn screening strategies for cCMVi from the perspective of the Chinese health care system. Design, Setting, and Participants: A decision tree for a simulated cohort population of 15 000 000 live births was developed to compare the costs and health effects of 3 mutually exclusive interventions: (1) no screening, (2) targeted screening using CMV polymerase chain reaction assay for newborns who fail a universal hearing screening, and (3) universal screening for CMV among all newborns. Markov diagrams were used to evaluate the lifetime horizon (76 years). Main Outcomes and Measures: Cost, hearing-related health outcomes, and incremental cost-effectiveness ratios (ICERs) were estimated based on a direct medical costs perspective. Costs and ICERs were reported in 2018 US dollars. Results: Incidence of cCMVi among newborns was reported to be approximately 0.7% in China. Targeted screening was less costly but also less effective than universal screening, identifying 41% of cases needing antiviral treatment and preventing nearly half of less severe or profound hearing loss. To avoid 1 CMV-related severe or profound hearing loss, 13 and 16 newborns need to be treated by targeted and universal screening, respectively. The ICERs of targeted and universal screening vs no screening were $79 and $2087 per quality-adjusted life-year gained, respectively, at the discounted rate of 3.5%. Both screening options were cost-effective for the Chinese health care system based on the willingness-to-pay threshold of 3 × gross domestic product per capita. The sensitivity analysis showed that the prevalence of cCMVi, as well as diagnosis and treatment costs, were key factors that may be associated with decision-making. Conclusions and Relevance: To achieve cost-effectiveness and best health outcomes, universal screening could be considered for the Chinese population. While the results are specific to China, the model may easily be adapted for other countries.

Lassa Fever Induced Hearing Loss: The Neglected Disability of Hemorrhagic Fever.

OBJECTIVE: Lassa fever (LF) a hemorrhagic fever endemic to Western has an incidence of approximately 500,000 cases per year. Here, we evaluate hearing loss and other sequelae following LF. METHODS: This case-control study enrolled laboratory confirmed LF survivors, non-LF Febrile controls and Matched Community controls with no history of LF or recent hospitalization for a febrile illness. Study participants completed a symptom questionnaire. Pure-tone audiometry was completed by a subset of participants. RESULTS: One hundred forty-seven subjects were enrolled aged from 3-66 years (mean = 23.3). LF survivors were significantly more likely to report balance difficulties (55% vs 20%, p < 0.001), hair loss (32% vs 7%, p < 0.001), difficulty speaking (19% vs 1%, p < 0.001), social isolation (50% vs 0%, p < 0.001), and hearing loss (17% vs 1%, p = 0.002) in comparison to Matched-Community Controls. Similar trends were noted in comparison to Febrile Controls, although these findings were non-significant. Fifty subjects completed audiometry. Audiometry found that LF survivors had significantly more bilateral hearing loss in comparison to Matched-Community Controls (30% vs 4%, p = 0.029). CONCLUSION: This study characterizes the sequelae of LF and highlights the need for increased access to hearing care in West Africa.

Nature and extent of hearing loss in HIV-infected children: A scoping review.

INTRODUCTION: Antiretroviral therapy (ART) has had a major impact on life expectancy from HIV as many people now live with it as a chronic disease. Chronic HIV has been associated with a range of comorbid disabilities and health conditions, one of which is hearing loss. Undiagnosed and untreated hearing loss, particularly in children, has been linked to poorer spoken language skills, with subsequent effects on academic performance. METHODS: This systematic scoping review aimed to summarize the available peer-reviewed literature on hearing loss in HIV-infected children, specifically to describe its extent and nature. The review followed the framework proposed by Arksey and O'Malley. Key search terms included hearing loss (and synonyms), child (and synonyms), and HIV. Electronic databases (EBSCOhost Research Platform, PubMed, Web of Science and Scopus databases) were searched for any relevant articles published from January 1, 2000 to June 30, 2019. Reference lists of included articles were pearled for additional relevant articles not already identified. Each stage of the selection process was conducted independently by two authors. The results were then collated by a third author who also resolved any discrepancies. Extracted data included sample descriptors, audiologic tests, hearing loss prevalence, hearing loss descripts, and factors associated with hearing loss. RESULTS: Seventeen articles were included; 10 from Africa, four from South America, two from North America and the remaining article from Asia. Although most of the articles reported on pure tone audiometry, the samples as well as the cut-off criteria for normal hearing were heterogenous. Prevalence of hearing loss varied across articles (from 6% to 84%). Conductive hearing loss occurred more frequently than sensorineural or mixed hearing loss. ART use and ear infection were reported as significant in three of five articles that reported on significant associates of HIV-related hearing loss. CONCLUSION: There was a modest volume of research from a limited number of countries. Heterogeneity in sampling and audiometric methods precluded a clear understanding of potential associations between chronic HIV-related hearing loss and contributing factors.

Outcome and management of newborns with congenital cytomegalovirus infection.

Congenital cytomegalovirus (CMV) infection is the most common non-genetic cause of hearing loss and neurological disorder in children. Its overall prevalence is approximately 0.5% in Europe. In France, systematic screening during pregnancy is not recommended; screening is performed only if there are maternal or fetal symptoms suggestive of this infection. Approximately 90% of infected newborns are asymptomatic at birth, and among them the risk of neurosensory sequelae is 5-15%. By contrast, the prevalence of neurosensory impairment in symptomatic newborns at birth varies from 17% to 60%. Congenital CMV infection must be confirmed at birth before the 21st day of life by polymerase chain reaction (PCR) on saliva or urine samples. A complete clinical examination, blood tests (blood count, liver function test, CMV PCR), hearing tests, brain ultrasound and eye fundus examination should be performed. Neurological and auditory follow-up must be extended well beyond the neonatal period because the occurrence of neurosensory sequelae may be delayed. Oral valganciclovir is the recommended treatment in moderate or severe congenital CMV infections for a period of 6 weeks to 6 months; such treatment requires regular monitoring because of its possible side effects.

Auditory findings associated with Zika virus infection: an integrative review / Achados auditivos associados à infecção pelo Zika vírus: uma revisão integrativa

Abstract Introduction: Possible associations between Zika virus infection and hearing loss were observed during the epidemic in the Americas. Objective: To describe the auditory alterations, pathogenesis and recommendations for follow-up in individuals with prenatal or acquired Zika virus infection. Methods: Bibliographic research conducted in March/2018-April/2019 at the main available databases. Article selection, data extraction and quality evaluation were carried out by two independent reviewers. Studies containing auditory evaluation of patients with congenital or acquired Zika virus infection; and/or hypotheses or evidences on the pathophysiology of auditory impairment associated with Zika virus; and/or recommendations on screening and follow-up of patients with auditory impairment by Zika virus were included. Results: A total of 27 articles were selected. Sensorineural and transient hearing loss were reported in six adults with acquired Zika virus infection. Of the 962 studied children, 482 had microcephaly and 145 had diagnostic confirmation of Zika virus; 515 of the 624 children with auditory evaluation performed only screening tests with otoacoustic emissions testing and/or automated click-stimuli auditory brainstem response testing. Studies in prenatally exposed children were very heterogeneous and great variations in the frequency of altered otoacoustic emissions and automated click-stimuli auditory brainstem response occurred across the studies. Altered otoacoustic emissions varied from 0% to 75%, while altered automated click-stimuli auditory brainstem response varied from 0% to 29.2%. Sensorineural, retrocochlear or central origin impairment could not be ruled out. One study with infected mice found no microscopic damage to cochlear hair cells. Studies on the pathogenesis of auditory changes in humans are limited to hypotheses and recommendations still include points of controversy. Conclusion: The available data are still insufficient to understand the full spectrum of the involvement of the auditory organs by Zika virus, the pathogenesis of this involvement or even to confirm the causal association between auditory involvement and virus infection. The screening and follow-up recommendations still present points of controversy.

Resumo Introdução: Possíveis associações entre a infecção pelo Zika vírus e perda auditiva foram observadas durante a epidemia nas Américas. Objetivo: Descrever as alterações auditivas, a patogênese e as recomendações de seguimento em indivíduos com infecção por Zika vírus pré-natal ou adquirida. Método: Uma pesquisa bibliográfica foi realizada em março/2018 a abril/2019 nas principais bases de dados disponíveis. A seleção dos artigos, extração de dados e avaliação de qualidade foram realizadas por dois revisores independentes. Estudos com avaliação auditiva de pacientes com infecção por Zika vírus congênita ou adquirida; e/ou hipóteses ou evidências sobre a fisiopatologia do comprometimento auditivo associado ao Zika vírus; e/ou recomendações sobre triagem e seguimento de pacientes com comprometimento auditivo pelo Zika vírus foram incluídos na pesquisa. Resultados: Um total de 27 artigos foram selecionados. Perdas auditivas neurossensorial e transitória foram relatadas em seis adultos com infecção pelo Zika vírus adquirida. Das 962 crianças estudadas, 482 apresentavam microcefalia e 145 tinham confirmação diagnóstica do Zika vírus; 515 das 624 crianças com avaliação auditiva haviam realizado apenas testes de triagem com teste de emissões otoacústicas e/ou teste de potencial evocado auditivo de tronco encefálico automático com estímulo clique. Estudos em crianças expostas no período pré-natal foram muito heterogêneos e grandes variações na frequência de emissões otoacústicas e potencial evocado auditivo de tronco encefálico automático alterados ocorreram ao longo dos estudos; alterações nas emissões otoacústicas variaram de 0% a 75%, enquanto as alterações no potencial evocado auditivo de tronco encefálico automático variaram de 0% a 29,2%. Não foi possível descartar comprometimento neurossensorial, retrococlear ou de origem central. Um estudo com camundongos infectados não encontrou dano microscópico nas células ciliadas da cóclea. Estudos sobre a patogênese das alterações auditivas em humanos estão limitados a hipóteses e recomendações ainda apresentam pontos de controvérsia. Conclusão: Os dados disponíveis ainda são insuficientes para compreender todo o espectro do envolvimento dos órgãos auditivos pelo Zika vírus, a patogênese desse envolvimento ou até mesmo para confirmar a associação causal entre o envolvimento auditivo e a infecção pelo vírus. As recomendações de triagem e seguimento ainda apresentam pontos de controvérsia.

Current small animal models for LASV hearing loss.

Lassa virus (LASV) is endemic in West Africa, causing an estimated 100000-300000 new infections and up to 5000-10000 deaths yearly. There are no vaccines and therapeutics are extremely limited. Typical case fatality rates are ∼1%, although a recent 2018 Nigerian outbreak featured an unprecedented 25.4% case fatality rate. Survivors of infection suffer a lifetime of sequelae with sudden onset sensorineural hearing loss (SNHL) being the most prevalent. The cause of this hearing loss remains unknown, and there is a critical need for further research on its mechanisms and potential therapeutics. The objective of this review is to outline the only currently available small animal model for LASV-induced hearing loss and to identify potential surrogate models.

A Review of Hearing Loss Associated with Zika, Ebola, and Lassa Fever.

The neglected tropical diseases Zika, Ebola, and Lassa fever (LF) have all been noted to cause some degree of hearing loss (HL). Hearing loss is a chronic disability that can lead to a variety of detrimental effects, including speech and language delays in children, decreased economic productivity in adults, and accelerated cognitive decline in older adults. The objective of this review is to summarize what is known regarding HL secondary to these viruses. Literature for this review was gathered using the PubMed database. Articles were excluded if there were no data of the respective viruses, postinfectious complications, or conditions related to survivorship. A total of 50 articles were included in this review. Fourteen articles discussing Zika virus and subsequent complications were included. Across these studies, 56 (21.2%) of 264 Zika-infected individuals were found to have HL. Twenty-one articles discussing Ebola virus and subsequent complications were included, with 190 (5.7%) of 3,350 Ebola survivors found to have HL. Fifteen additional articles discussing LF and subsequent complications were included. Of 926 individuals with LF, 79 (8.5%) were found to have HL. These results demonstrate a relationship between HL and infection. The true prevalence is likely underestimated, however, because of lack of standardization of reporting and measurement. Future studies of viral sequelae would benefit from including audiometric evaluation. This information is critical to understanding pathophysiology, preventing future cases of this disability, and improving quality of life after survival of infection.

Diagnosis of congenital CMV infection via DBS samples testing and neonatal hearing screening: an observational study in Italy.

BACKGROUND: Congenital Cytomegalovirus (cCMV) is the most common cause of non-genetic hearing loss in childhood. A newborn hearing screening program (NHSP) is currently running in Italy, but no universal cCMV nor statewide hearing-targeted CMV screening programs have been implemented yet. This observational monocentric study was aimed at estimating the rate of cCMV infections identified by CMV-DNA analysis on Dried Blood Spots (DBS) samples in deaf children identified via NHSP in Northern Italy in the period spanning from 2014 to 2018. METHODS: Children with a confirmed diagnosis of deafness and investigated for CMV-DNA by nucleic acid extraction and in-house polymerase-chain reaction (PCR) on stored newborns screening cards (DBS-test) were included in this study. Deafness was defined by a hearing threshold ≥20 decibel (dB HL) by Auditory Brainstem Responses (ABR); all investigated DBS samples were collected within 3 days of life. RESULTS: Overall, 82 children were included (median age: 3.4 months; lower-upper quartiles: 2-5.3 months; males: 60.9%). Most of them (70.7%) presented bilateral hearing loss with a symmetrical pattern in 79.3% of the cases. ABR thresholds were ≥ 70 dB HL (severe/profound deafness) in 46.5% of children. Among all tested children, 6.1% resulted positive for cCMV. The rate of severe/profound deafness was statistically higher in children with cCMV infection. CONCLUSIONS: The addition of DBS-test to the NHSP allowed the identification, in their first months of life, of a cCMV infection in 6.1% of children who had failed NHS. The introduction of a targeted CMV screening strategy could help clinicians in the differential diagnosis and in the babies' management. DBS samples can be considered a "universal newborns biobank": their storage site and duration should be the subject of political decision-making.

Auditory findings associated with Zika virus infection: an integrative review.

INTRODUCTION: Possible associations between Zika virus infection and hearing loss were observed during the epidemic in the Americas. OBJECTIVE: To describe the auditory alterations, pathogenesis and recommendations for follow-up in individuals with prenatal or acquired Zika virus infection. METHODS: Bibliographic research conducted in March/2018-April/2019 at the main available databases. Article selection, data extraction and quality evaluation were carried out by two independent reviewers. Studies containing auditory evaluation of patients with congenital or acquired Zika virus infection; and/or hypotheses or evidences on the pathophysiology of auditory impairment associated with Zika virus; and/or recommendations on screening and follow-up of patients with auditory impairment by Zika virus were included. RESULTS: A total of 27 articles were selected. Sensorineural and transient hearing loss were reported in six adults with acquired Zika virus infection. Of the 962 studied children, 482 had microcephaly and 145 had diagnostic confirmation of Zika virus; 515 of the 624 children with auditory evaluation performed only screening tests with otoacoustic emissions testing and/or automated click-stimuli auditory brainstem response testing. Studies in prenatally exposed children were very heterogeneous and great variations in the frequency of altered otoacoustic emissions and automated click-stimuli auditory brainstem response occurred across the studies. Altered otoacoustic emissions varied from 0% to 75%, while altered automated click-stimuli auditory brainstem response varied from 0% to 29.2%. Sensorineural, retrocochlear or central origin impairment could not be ruled out. One study with infected mice found no microscopic damage to cochlear hair cells. Studies on the pathogenesis of auditory changes in humans are limited to hypotheses and recommendations still include points of controversy. CONCLUSION: The available data are still insufficient to understand the full spectrum of the involvement of the auditory organs by Zika virus, the pathogenesis of this involvement or even to confirm the causal association between auditory involvement and virus infection. The screening and follow-up recommendations still present points of controversy.

Association of Ebola Virus Infection With Hearing Loss in Regions Where Ebola Virus Infection Is Endemic: A Systematic Review.

Importance: Many survivors of Ebola virus infection describe new-onset hearing loss after infection. The prevalence, severity, and pathophysiologic features of hearing loss in this population have not been well characterized. Objective: To perform a systematic review of the current literature to characterize hearing loss in survivors of Ebola virus infection. Evidence Review: This study adhered to the relevant sections of the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Searches through PubMed, Embase, and Google Scholar were performed to include publications written in English from January 1, 1965, to October 1, 2018. Relevant vocabulary terms and key terms related to Ebola and hearing loss were used. Two investigators independently screened the eligible studies, extracted data, and assessed quality and risk of bias. Findings: Of 127 publications reviewed, 15 met the criteria for inclusion; 3 were retrospective case-control studies (level of evidence, 3), and 12 were cross-sectional studies or case reports (level of evidence, 4). Studies included 1775 survivors of Ebola virus infection (993 female [55.9%]) and 363 uninfected controls (186 female [51.2%]) from the Democratic Republic of the Congo, Uganda, Guinea, Liberia, and Sierra Leone. The duration of follow-up ranged from 0 to 29 months (median, 5 months). Hearing loss was reported in 147 survivors of Ebola virus infection (8.3%). Among studies that compared survivors with controls, the reported odds ratios for hearing loss in survivors was 7.50 (95% CI, 3.91-14.39; range, 1.4-12.1). Including all studies, the odds ratio of hearing loss in survivors vs controls in countries where Ebola virus infection is endemic was 1.84 (95% CI, 1.10-3.08). Conclusions and Relevance: Survivors of Ebola virus infection had higher rates of hearing loss than uninfected controls in regions where the infection is endemic. Further research with consistent objective methods and pure-tone audiometry may be needed to better characterize the hearing loss, understand its pathophysiologic features, and develop treatments.

Results of a Targeted Screening Program for Congenital Cytomegalovirus Infection in Infants Who Fail Newborn Hearing Screening.

BACKGROUND: Congenital cytomegalovirus (CMV) infection is a major cause of sensorineural hearing loss. By law, newborns in Connecticut who fail newborn hearing screening are tested for infection with CMV. This targeted screening is controversial, because most children with congenital CMV infection are asymptomatic, and CMV-related hearing loss can have a delayed onset. Our hospital uses a saliva polymerase chain reaction (PCR) assay (confirmed by a urine PCR assay) to detect CMV. Here, we report the results of the first year of our screening program. METHODS: We reviewed the medical records of newborns in the Yale New Haven Health System who failed the newborn hearing screening test between January 1 and December 31, 2016. RESULTS: Of 10964 newborns, 171 failed newborn hearing screening, and 3 of these newborns had positive saliva CMV PCR test results. Of these 3 newborns, 2 had positive results on the confirmatory test (for 1 of them the confirmatory test was not performed until the infant was 10 weeks old), and 1 had a negative result on the confirmatory test. Three additional newborns with congenital CMV infection were tested because of clinical indications (1 for ventriculomegaly on prenatal ultrasound and 2 for CMV infection of the mother). Results of audiology follow-up were available for 149 (87.1%) of the 171 newborns who failed newborn hearing screening; 127 (85.2%) had normal results. CONCLUSION: Our targeted screening program for congenital CMV infection had a low yield. Consideration should be given to other strategies for identifying children at risk of hearing loss as a result of congenital CMV infection.

Blood Viral Load in Symptomatic Congenital Cytomegalovirus Infection.

BACKGROUND: Viral loads (VLs) frequently are followed during treatment of symptomatic congenital cytomegalovirus disease, but their predictive value is unclear. METHODS: Post hoc analysis of 2 antiviral studies was performed. Seventy-three subjects were treated for 6 weeks and 47 subjects were treated for 6 months. Whole blood VL was determined by real-time polymerase chain reaction before and during therapy. RESULTS: Higher baseline VL was associated with central nervous system involvement (3.82 log, range 1-5.65 vs 3.32 log, range 1-5.36; P = .001), thrombocytopenia (3.68 log, range 1-5.65 vs 3.43 log, range 1-5.36; P = .03), and transaminitis at presentation (3.73 log, range 1-5.60 vs 3.39 log, range 1-5.65; P = .009), but with overlap in the amount of virus detected between groups. In subjects treated for 6 months, lower VL at presentation correlated with better hearing outcomes at 12 months, but VL breakpoints predictive of hearing loss were not identified. Sustained viral suppression during 6 months of therapy correlated with better hearing outcomes at 6, 12, and 24 months (P = .01, P = .0007, P = .04), but a majority without viral suppression still had improved hearing. CONCLUSIONS: In infants with symptomatic congenital cytomegalovirus disease, higher whole blood VL before initiation of antiviral therapy has no clinically meaningful predictive value for long-term outcomes.

Hearing Screening in children with Congenital Zika Virus Syndrome in Fortaleza, Ceará, Brazil, 2016. / Triagem auditiva de crianças com síndrome congênita pelo vírus Zika atendidas em Fortaleza, Ceará, 2016.

OBJECTIVE: to describe the results of hearing screening performed in children with Congenital Zika Virus Syndrome (CZS) in Fortaleza, Ceará, Brazil. METHODS: this was a descriptive cross-sectional study involving children with CZS receiving health care in Fortaleza, 2016; the hearing screening tests performed were immittance audiometry, transient otoacoustic emissions (TOAE), acoustic reflexes, and cochleopalpebral reflex (CPR). RESULTS: The study included 45 children with an average age of 10 months. 44 of them underwent tympanometric screening, with 16 of these having the right ear within the normal range and 22 having the left ear within the normal range. Among the 43 children evaluated by TOAE, 30 "passed" in both ears, nine "refered" in both ears and four "refered" just in ear; 13/43 "refered" and needed to repeat screening. 43 children evaluated by CPR, 37 showed responses. CONCLUSION: most of the children evaluated had completed cochlear function and middle ear results refer in compatible with their age range.

Recognition, treatment, and sequelae of congenital cytomegalovirus in Australia: An observational study.

BACKGROUND AND OBJECTIVES: Australian national surveillance data was used to assess recognition, sequelae, and antiviral therapy for congenital cytomegalovirus (CMV) cases. STUDY DESIGN: Data from congenital CMV cases reported through the Australian Paediatric Surveillance Unit born January 1999 to December 2016 were described and Chi-square tests used to characterise trends and associations in case reporting, maternal CMV serology testing, and antiviral therapy. Descriptive analyses for hearing loss and developmental delay were reported for cases born ≥2004, following introduction of universal neonatal hearing screening. RESULTS: There were 302 congenital CMV cases (214 symptomatic, 88 asymptomatic). Congenital CMV was suspected in 70.6% by 30 days of age, with no differences across birth cohorts. Maternal CMV serology testing was associated with maternal illness during pregnancy but not birth cohort. There was increasing antiviral use for symptomatic cases, being used in 14% born 1999-2004, 19.6% born 2005-2010, and 44.4% born 2011-2016 (p < 0.001). For those born ≥2004, hearing loss was reported in 42.1% of symptomatic and 26.6% of asymptomatic cases; while developmental delay was reported in 16.9% of symptomatic and 1.3% of asymptomatic cases. CONCLUSION: There appears to be under-reporting and under-recognition of congenital CMV despite increasing use of antiviral therapy. Universal newborn CMV screening should be considered to facilitate follow-up of affected children and targeted linkage into hearing and developmental services, and to provide population-level infant CMV epidemiology to support research and evaluation of antiviral and adjunctive therapies.

A rational approach to identifying newborns with hearing loss caused by congenital cytomegalovirus infection by dried blood spot screening.

OBJECTIVE: Congenital cytomegalovirus (cCMV) infection is the most common congenital infection, with the majority of infected newborns having no detectable signs. The aim of this study was to examine the accuracy of our newly developed DBS-based assay as an appropriate mass screening method for cCMV infection. METHODS: Between May 2011 and October 2016, newborns delivered at six hospitals in Nagano Prefecture, Japan were enrolled prospectively. We employed dried blood spot (DBS)-based assays with real-time quantitative PCR (qPCR). RESULTS: Prior to the clinical study, confirmation analysis was carried out using positive and negative controls. The sensitivity and specificity of this DBS-based qPCR assay for the detection of CMV DNA were 83 and 97%, respectively. During the study period, 9675 newborns were enrolled. The total recovery rate of DBS was 99.92% (9,667/9,675). From our analysis of the 9,667 samples, 47 DBS samples were found positive by the qPCR test (0.48%), and 9620 (99.5%) DBS samples were CMV-negative. CONCLUSIONS: The risk of neural disorders associated with cCMV infection is thought likely to increase with CMV viral load in the blood. DBS screening for cCMV may be sufficient in a clinical setting, and offers a realistic and feasible option for universal mass screening.