Levels of evidence: universal newborn hearing screening (UNHS) and early hearing detection and intervention systems

Abstract Levels of evidence differ according to the audience addressed. Implementation of universal newborn hearing screening requires responses to a complex myriad of diverse groups: the general public, families with children who are deaf or hard of hearing, the deaf and hard of hearing communities, hospital administrators, physicians (pediatricians, general practitioners, ear nose and throat physicians, geneticists), managed care, Medicaid, insurance agencies, and politicians. The level of evidence required by medical/health agencies and task forces may differ from the levels of evidence available in education and intervention. Issues related to the low incidence of the disability, the lack of a normal distribution within the disability study, the obstacles to random assignment to treatment, and designs that include a control group with ''no treatment'' have implications legally and ethically for the professional providing services to families and children who are deaf or hard of hearing. This session will discuss issues related to ''convenience samples,'' number of subjects included in research studies, and the population required to obtain a large enough sample of children with low-incidence disabilities. The level of evidence required to demonstrate sensitive periods of development, which are a critical element for justification of implementing a universal newborn hearing screening includes both behavioral and neurological information. Sensitive periods may have different duration for different aspects of development, such as social-emotional development, auditory and speech development, or language development. Further complicating the question of sensitive periods of development are the distinct possibility that different sensitive periods exist for development of age-level vocabulary, for establishing English phonology, or for mastering English syntax. Research outcomes provide evidence that age of identification of hearing loss is reduced, that age of intervention initiation is lowered, and that the outcomes of intervention are better because of the establishment of a screening program. Most professionals in communication disorders believe that screening is not the actual cause of better developmental outcomes but that the age when children begin to have access to language and communication and the characteristics of th

Temporal and causal reasoning in deaf and hearing novice readers

Temporal and causal information in text are crucial in helping the reader form a coherent representation of a narrative. Deaf novice readers are generally poor at processing linguistic markers of causal/temporal information (i.e., connectives), but what is unclear is whether this is indicative of a more general deficit in reasoning about temporal/causal information. In Study 1, 10 deaf and 63 hearing children, matched for comprehension ability and age, were compared on a range of tasks tapping temporal/causal reasoning skills. In Study 2, 20 deaf and 32 hearing children, matched for age but not reading comprehension ability, were compared on revised versions of the tasks. The pattern of performance of the deaf was different from that of the hearing; they had difficulties when temporal and causal reasoning was text-based, but not when it was nonverbal, indicating that their global temporal/causal reasoning skills are comparable with those of their hearing counterparts

The Influence of Word Characteristics on the Vocabulary of Children with Cochlear Implants

This is the author's accepted manuscript. The original publication is available at http://jdsde.oxfordjournals.org/search?fulltext=The+Influence+of+Word+Characteristics+on+the+Vocabulary+or+Children+With+Cochlear+Implants&submit=yes&x=9&y=4The goal of this study was to explore the effects of phonotactic probability, word length, word frequency, and neighborhood density on the words known by children with cochlear implants (CIs) varying in vocabulary outcomes in a retrospective analysis of a subset of data from a longitudinal study of hearing loss. Generalized linear mixed modeling was used to examine the effects of these word characteristics at three time points: pre-implant, post-implant, and longitudinal follow-up. Results showed a robust effect of neighborhood density across group and time, whereas the effect of frequency varied by time. Significant effects of phonotactic probability or word length were not detected. Taken together, these findings suggest that children with CIs may be able to use spoken language structure in a manner similar to their normal hearing counterparts, despite the differences in the quality of the input. The differences in the effects of phonotactic probability and word length imply a difficulty in initiating word learning and limited working memory ability in children with CIs

Dissemination of information to General Practitioners: a questionnaire survey

BACKGROUND: Early identification of permanent hearing impairment in children enables appropriate intervention which reduces adverse developmental outcomes. The UK Government has introduced a universal hearing screening programme for neonates. All involved health professionals, including those in Primary Care, need to be aware of the service to enable them to offer appropriate support to their patients. A programme of information dissemination within Primary Care was therefore undertaken. The aim of the current study was to determine the extent to which the information had reached General Practitioners (GPs), the GPs' preferred mode of dissemination and the sources from which GPs accessed information METHODS: Postal questionnaire survey of a randomised sample of 1000 GPs in the Phase I pilot sites of the Neonatal Hearing Screening Programme (NHSP). RESULTS: Responses were received from 54.2% of the sample. Just under 50% of those responding had received information, 62.2% of respondents said they would like to receive more information and the preferred methods of dissemination were the written word and web-sites to allow access when needed. Few GPs perceive themselves to have a core role in the delivery of the NHSP and thence a need for knowledge in the subject. Many are keen to delegate detail to a third party, usually the health visitor, who has traditionally had responsibility for hearing screening. CONCLUSIONS: Dissemination efforts for service developments of relevance to GPs should concentrate on advertising a website address via brief but memorable posted literature and/or articles in relevant journals and magazines. The website should be GP-friendly, and have a dedicated area for GPs including information of specific relevance and downloadable information sheets

Effectiveness of universal newborn hearing screening: A systematic review and meta-analysis

Background: Permanent bilateral hearing loss (PBHL) is a serious condition in newborns, with a prevalence of at least one per 1000 live births. However, there has been no recent systematic review and meta-analysis of the effectiveness of universal newborn hearing screening programs (UNHS). Methods: We registered our study protocol on PROSPERO CRD42020175451. Primary outcomes were any identification of PBHL (ie, PBHL diagnosed at any time), age of identification of PBHL, and neurodevelopment. Two reviewers searched standard databases to March 2022 and extracted data. We used fixed and random effects meta-analysis to pool data and graded the certainty of evidence using standard methods. Results: The search retrieved 2834 records. We identified five studies reporting on the effects of UNHS vs no UNHS in 1 023 610 newborns. The relative risk of being identified with PBHL before nine months in infants with UNHS compared to infants without UNHS was 3.28 (95 % confidence interval (95 % CI) = 1.84, 5.85, one study, 1 023 497 newborns, low certainty evidence). The mean difference in the age of identification of PBHL in infants with UNHS compared to infants without UNHS was 13.2 months earlier (95 % CI = -26.3, -0.01, two studies, 197 newborns, very low certainty evidence). The relative risk of infants eventually being identified with PBHL in infants with UNHS compared to infants without UNHS was 1.01 (95 % CI = 0.89, 1.14, three studies, 1 023 497 newborns, low certainty evidence). At the latest follow-up at 3-8 years, the standardised mean difference (SMD) in receptive language development between infants with UNHS compared to infants without UNHS was 0.60 z scores (95 % CI = 0.07, 1.13, one study, 101 children, low certainty evidence) and the mean difference in developmental quotients was 7.72 (95 % CI = -0.03, 15.47, three studies, 334 children, very low certainty evidence). The SMD in expressive language development was 0.39 z scores (95 % CI = -0.20, 0.97, one study, 87 children, low certainty evidence) and the mean difference in developmental quotients was 10.10 scores (95 % CI = 1.47, 18.73, 3 studies, 334 children, very low certainty evidence). Conclusions: UNHS programs result in earlier identification of PBHL and may improve neurodevelopment. UNHS should be implemented across high-, middle-, and low-income countrie

Hearing Loss in Perinatally Human Immunodeficiency Virus- Infected and Human Immunodeficiency Virus -Exposed but Uninfected Children and Adolescents

This is not the published version.Background Little is known about hearing loss in children with HIV infection (HIV+). We examined the prevalence of hearing loss in perinatally HIV+ and HIV-exposed but uninfected (HEU) children, compared these to the percentage with hearing loss in the general population, and evaluated possible risk factors for hearing loss in HIV+ and HEU children. Methods Audiometric examinations were completed in children who met any pre-specified criteria for possible hearing loss. The hearing examination consisted of a tympanogram in each ear and pure-tone air-conduction threshold testing from 500 through 4000 Hz. Hearing loss was defined as the pure-tone average over these frequencies ≥20 dB hearing level (HL). The associations of demographic, parent/caregiver, HIV disease, and HIV treatment with hearing loss were evaluated with univariate and multivarible logistic regression models. Results Hearing testing was completed in 231 children (145 HIV+ and 86 HEU). Hearing loss occurred in 20.0% of HIV+ children and 10.5% of HEU children. After adjusting for caregiver education level, HIV infection was associated with increased odds of hearing loss [adjusted odds ratio (aOR)=2.13, 95% confidence interval (CI): 0.95–4.76, p=0.07]. Among HIV+ children, those with a CDC Class C diagnosis had over twice the odds of hearing loss (aOR=2.47, 95% CI: 1.04–5.87, p=0.04). The prevalence of hearing loss was higher in both HIV+ and HEU children compared with NHANES III children. Conclusions Hearing loss was more common in both HIV+ and HEU children than in healthy children. More advanced HIV illness increased the risk of hearing loss in HIV+ children

Neonatal hearing screening: modelling cost and effectiveness of hospital- and community-based screening

BACKGROUND: Children with congenital hearing impairment benefit from early detection and management of their hearing loss. These and related considerations led to the recommendation of universal newborn hearing screening. In 2001 the first phase of a national Newborn Hearing Screening Programme (NHSP) was implemented in England. Objective of this study was to assess costs and effectiveness for hospital and community-based newborn hearing screening systems in England based on data from this first phase with regard to the effects of alterations to parameter values. METHODS: Design: Clinical effectiveness analysis using a Markov Model. Outcome measure: quality weighted detected child months (QCM). RESULTS: Both hospital and community programmes yielded 794 QCM at the age of 6 months with total costs of £3,690,000 per 100,000 screened children in hospital and £3,340,000 in community. Simulated costs would be lower in hospital in 48% of the trials. Any statistically significant difference between hospital and community in prevalence, test sensitivity, test specificity and costs would result in significant differences in cost-effectiveness between hospital and community. CONCLUSION: This modelling exercise informs decision makers by a quantitative projection of available data and the explicit and transparent statements about assumptions and the degree of uncertainty. Further evaluation of the cost-effectiveness should focus on the potential differences in test parameters and prevalence in these two settings

Comparing the clinical effectiveness of different new-born hearing screening strategies. A decision analysis

BACKGROUND: Children with congenital hearing impairment benefit from early detection and treatment. At present, no model exists which explicitly quantifies the effectiveness of universal newborn hearing screening (UNHS) versus other programme alternatives in terms of early diagnosis. It has yet to be considered whether early diagnosis (within the first few months) of hearing impairment is of importance with regard to the further development of the child compared with effects resulting from a later diagnosis. The objective was to systematically compare two screening strategies for the early detection of new-born hearing disorders, UNHS and risk factor screening, with no systematic screening regarding their influence on early diagnosis. METHODS: Design: Clinical effectiveness analysis using a Markov Model. Data Sources: Systematic literature review, empirical data survey, and expert opinion. Target Population: All newborn babies. Time scale: 6, 12 and 120 months. Perspective: Health care system. Compared Strategies: UNHS, Risk factor screening (RS), no systematic screening (NS). Outcome Measures: Quality weighted detected child months (QCM). RESULTS: UNHS detected 644 QCM up until the age of 6 months (72,2%). RS detected 393 child months (44,1%) and no systematic screening 152 child months (17,0%). UNHS detected 74,3% and 86,7% weighted child months at 12 and 120 months, RS 48,4% and 73,3%, NS 23,7% and 60,6%. At the age of 6 months UNHS identified approximately 75% of all children born with hearing impairment, RS 50% and NS 25%. At the time of screening UNHS marked 10% of screened healthy children for further testing (false positives), RS 2%. UNHS demonstrated higher effectiveness even under a wide range of relevant parameters. The model was insensitive to test parameters within the assumed range but results varied along the prevalence of hearing impairment. CONCLUSION: We have shown that UNHS is able to detect hearing impairment at an earlier age and more accurately than selective RS. Further research should be carried out to establish the effects of hearing loss on the quality of life of an individual, its influence on school performance and career achievement and the differences made by early fitting of a hearing aid on these factors