Detecting Hearing Loss in Infants With a Syndrome or Craniofacial Abnormalities Following the Newborn Hearing Screen

Author(s):  
Philippa Horn ◽  
Carlie Driscoll ◽  
Jane Fitzgibbons ◽  
Rachael Beswick

Purpose The current Joint Committee on Infant Hearing guidelines recommend that infants with syndromes or craniofacial abnormalities (CFAs) who pass the universal newborn hearing screening (UNHS) undergo audiological assessment by 9 months of age. However, emerging research suggests that children with these risk factors are at increased risk of early hearing loss despite passing UNHS. To establish whether earlier diagnostic audiological assessment is warranted for all infants with a syndrome or CFA, regardless of screening outcome, this study compared audiological outcomes of those who passed UNHS and those who referred. Method A retrospective analysis was performed on infants with a syndrome or CFA born between July 1, 2012, and June 30, 2017 who participated in Queensland, Australia's state-wide UNHS program. Results Permanent childhood hearing loss (PCHL) yield was higher among infants who referred on newborn hearing screening (51.20%) than in those who passed. Nonetheless, 27.47% of infants who passed were subsequently diagnosed with hearing loss (4.45% PCHL, 23.02% transient conductive), but PCHL was generally milder in this cohort. After microtia/atresia, the most common PCHL etiologies were Trisomy 21, other syndromes, and cleft palate. Of the other syndromes, Pierre Robin sequence featured prominently among infants who passed the hearing screen and were subsequently diagnosed with PCHL, whereas there was a broader mix of other syndromes that caused PCHL in infants who referred on screening. Conclusion Children identified with a syndrome or CFA benefit from early diagnostic audiological assessment, regardless of their newborn hearing screening outcome.

2019 ◽  
Vol 29 (Supplement_4) ◽  
Author(s):  
A Doncarli ◽  
H Tillaut ◽  
V Goulet

Abstract Background The French newborn hearing screening programme set up in 2014 aims to identify affected children as early as possible to allow appropriate care. Our goal was to evaluate the implementation of this programme at the national level. Methods The programme consist in a screening using a test (T1) and a retest (T2) in the maternity hospital in all newborns. In some region, a delayed test (T3) is performed afterwards in children for whom it was not possible to confirm normal hearing. Children suspected of deafness subsequently enter the diagnosis phase. We defined evaluation indicators after stakeholders consultation. We developed a web application to collect aggregated data on live births. We estimated the rate of coverage, refusal, children suspected of hearing loss at the end of the screening phase and the prevalence of bilateral deafness. Results In 2015, one year after the initiation of the programme, the coverage rate was already very high (88%) and even more so in 2016 (96%). Parental acceptance was very good (refusal: 0.1%). By the end of 2016, 19 out 27 regions had added a T3. The rate of suspicion of bilateral hearing loss was decreased by using a T3 (1.4% vs 0.9%). Bilateral deafness rate, estimated after a 2-years follow up, was 1.3‰ although it was estimated only on 51.5% of suspected children for whom diagnosis data had been transmitted. Conclusions The objective of a 90% coverage set by the French ministry of health has been met after 2 years of operation of the program. T3 appears useful in relieving diagnostic structures from false positives. Our estimated prevalence of bilateral deafness could be more accurate if data collection was improved but is consistent with prevalence reported in North America. Key messages The universal newborn hearing screening program has been successfully set up in France in terms of coverage. Future work should focus on improving the collection of follow up data to better characterise diagnosed children.


2014 ◽  
Vol 101 (1) ◽  
pp. 9-15 ◽  
Author(s):  
Hannah Pimperton ◽  
Hazel Blythe ◽  
Jana Kreppner ◽  
Merle Mahon ◽  
Janet L Peacock ◽  
...  

ObjectiveTo determine whether the benefits of universal newborn hearing screening (UNHS) seen at age 8 years persist through the second decade.DesignProspective cohort study of a population sample of children with permanent childhood hearing impairment (PCHI) followed up for 17 years since birth in periods with (or without) UNHS.SettingBirth cohort of 100 000 in southern England.Participants114 teenagers aged 13–19 years, 76 with PCHI and 38 with normal hearing. All had previously their reading assessed aged 6–10 years.InterventionsBirth in periods with and without UNHS; confirmation of PCHI before and after age 9 months.Main outcome measureReading comprehension ability. Regression modelling took account of severity of hearing loss, non-verbal ability, maternal education and main language.ResultsConfirmation of PCHI by age 9 months was associated with significantly higher mean z-scores for reading comprehension (adjusted mean difference 1.17, 95% CI 0.36 to 1.97) although birth during periods with UNHS was not (adjusted mean difference 0.15, 95% CI −0.75 to 1.06). The gap between the reading comprehension z-scores of teenagers with early compared with late confirmed PCHI had widened at an adjusted mean rate of 0.06 per year (95% CI −0.02 to 0.13) during the 9.2-year mean interval since the previous assessment.ConclusionsThe benefit to reading comprehension of confirmation of PCHI by age 9 months increases during the teenage years. This strengthens the case for UNHS programmes that lead to early confirmation of permanent hearing loss.Trial registration numberISRCTN03307358.


2018 ◽  
Vol 105 ◽  
pp. 181-186 ◽  
Author(s):  
Katarzyna Wroblewska-Seniuk ◽  
Piotr Dabrowski ◽  
Grazyna Greczka ◽  
Katarzyna Szabatowska ◽  
Agata Glowacka ◽  
...  

2021 ◽  
Vol 104 (1) ◽  
pp. 95-99

Objective: 1) To share the experience in establishing the first province to set up the universal newborn hearing screening (UNHS) in Thailand. 2) To report the results of four consecutive years of UNHS in Trang. Materials and Methods: All newborns in Trang province, between October 2013 and September 2017, who received UNHS, were included in this study. The present study was a descriptive study. Results: There were three main obstacles to be solved to establish the program. There was no supported budget from the central government to run the UNHS. There was a lack of audiologists or trained personnel to run the UNHS. Finally, there was no province in Thailand that has done the project before. A budget for the Trang’s UNHS project was allocated, which was 3,100,000 baht from the Trang provincial administrative organization, to purchase hearing screening machines for every community hospital. The personnel to run the UNHS, which were nurses, were trained in every hospital. The protocols, referral, and follow-up programs were newly designed to establish the hospital network for the program. There were 28,254 newborns in Trang and 27,983 (99.04%) were screened. The high-risk newborns were 1,415 (5.1%). The low-risk group was 26,568 (94.9%). The referral rate of transient evoked otoacoustic emission (TEOAE) was 5.9%. In the low-risk past screening group, there was one newborn (0.005%) that presented later with delayed speech and profound hearing loss after 1½ years and the MRI showed bilateral IAC stenosis. There were two newborns with severe hearing loss, one was Mondini dysplasia, and the other was normal on imaging, in 169 unpassed low-risk newborns. In the high-risk group, 73 (5.2%) were unpassed. After diagnostic tests, 71 (97.2%) were normal, one had severe hearing loss with normal imaging, and the other had bilateral microtia. The incidence of bilateral severe SNHL in high-risk newborn was (1/1,415) 0.71:1,000 births. The incidence of bilateral severe SNHL in low-risk newborn was (3/26,568) 0.11:1,000 births. After two years of follow-up, there was no delayed speech due to hearing loss in all the present study newborns. Conclusion: The rate of congenital hearing loss is not as high as in the literature, but the UNHS is still important to the newborns and their parents. Keywords: UNHS in Trang, Province-based UNHS, Incidence of hearing loss in newborn


2017 ◽  
Vol 33 (2) ◽  
pp. 206-214 ◽  
Author(s):  
Xiuzhi Chen ◽  
Min Yuan ◽  
Jun Lu ◽  
Qi Zhang ◽  
Mei Sun ◽  
...  

Objectives:The aim of this study was to evaluate the universal newborn hearing screening (UNHS) and intervention program in Shanghai, China.Methods:This study included the quantitative analyses of the UNHS-Shanghai database in 2002–12 and qualitative assessment of the program. The Otoacoustic Emissions and the Automated Auditory Brainstem Evoked Responses tests were conducted in screening. The costs and benefits were calculated based on the number of participants in each stage. The short-term and long-term periods were defined as from birth to 15 years of age or to death (82-year-olds), respectively. Sensitivity analyses were conducted.Results:A total 1,574,380 newborns were included, representing 93.6 percent of all eligible babies in Shanghai during the study period. The prevalence of newborn hearing loss was 1.66‰. The short-term/long-term program costs were ¥488.5 million (US$75.52 million)/¥1.08 billion (US$167.12 million), and the short-term/long-term program benefit was ¥980.1 million (US$151.53 million)/¥8.13 billion (US$1.26 billion). The program benefit was greater than its cost if the proportion of hearing-loss children enrolled in regular schools was no less than 41.4 percent of all hearing impaired children, as well as if the wage growth rate ranged from 3 percent to 8 percent. Qualitative results also suggested that stakeholders strongly supported this program.Conclusions:The universal newborn hearing screening and intervention program in Shanghai is justified in terms of the resource input in the long run, although there is still room for further improvement with respect to educational rehabilitation and a better infrastructure system.


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