Diagnosis Of Congenital Hearing Loss Research Articles

Epidemiology, aetiology and diagnosis of congenital hearing loss via hearing screening of 153 913 newborns.

Congenital hearing loss (HL), one of the most common paediatric chronic conditions, significantly affects speech and language development. Its early diagnosis and medical intervention can be achieved via newborn hearing screening. However, data on the prevalence and aetiology of congenital HL in infants who fail newborn hearing screening are limited. The sample population included 153 913 infants who underwent newborn hearing screening, and the prevalence of congenital HL, defined as moderate to profound bilateral HL (BHL) or unilateral HL (UHL) (≥40 dB HL), in one prefecture of Japan was measured to minimize the loss-to-follow-up rate, a common factor affecting the screening procedure. Comprehensive aetiological investigation, including physiology, imaging, genetic tests, and congenital cytomegalovirus screening, was performed on children diagnosed with congenital HL. The calculated prevalence of congenital HL was 1.62 per 1000 newborns (bilateral, 0.84; unilateral, 0.77). More than half of the cases with congenital bilateral or severe to profound UHL showed genetic aetiology or cochlear nerve deficiency (CND), respectively. Approximately 4% and 6% of the cases of congenital BHL and UHL were associated with congenital cytomegalovirus infection and auditory neuropathy spectrum disorder, respectively. This is an epidemiological and comprehensive aetiological study of congenital HL, as determined via newborn hearing screening according to its severity and laterality, in a large-scale general population of a developed country. Our findings can serve as a reference for optimizing care and intervention options for children with HL and their families.

Analysis of the Results of Cytomegalovirus Testing Combined with Genetic Testing in Children with Congenital Hearing Loss.

To improve the etiological diagnosis of congenital hearing loss by combining whole-exome sequencing (WES) with cytomegalovirus (CMV) testing and to explore the potential benefits of adding CMV screening to newborn hearing screening, 80 children under 2 years of age with bilateral sensorineural hearing loss were recruited. Peripheral venous blood was extracted from the children for WES analysis. Saliva after mouthwash and the first urine in the morning were collected and used as samples to quantify CMV DNA copy number in urine and saliva by qPCR; among the 80 children with congenital deafness, 59 (74%) were found to have genetic variants that may cause congenital deafness, including 44 with GJB2 or SLC26A4 gene variant, 1 with STRC gene variant, and 14 with other genetic variants. A total of 12 children carried deafness gene variants associated with a syndrome; CMV test results showed that in two children, the CMV DNA copy number in saliva was >1000/mL, which indicates that they were CMV-positive, and their genetic test results were negative. A neonatal CMV test combined with genetic screening can improve the etiological diagnosis rate of congenital deafness, and the direct evidence of neonatal CMV infection deserves further verification.

Cochlear Implantation in Infants: Why and How.

In children with congenital deafness, cochlear implantation (CI) prior to 12 months of age offers the opportunity to foster more typical auditory development during late infancy and early childhood. Recent studies have found a positive association between early implantation and expressive and receptive language outcomes, with some children able to achieve normal language skills by the time of school entry. Universal newborn hearing screening improved early detection and diagnosis of congenital hearing loss, allowing for earlier intervention, including decision-making regarding cochlear implant (CI) candidacy. It can be more challenging to confirm CI candidacy in infants; therefore, a multidisciplinary approach, including objective audiometric testing, is recommended to not only confirm the diagnosis but also to counsel families regarding expectations and long-term management. Surgeons performing CI surgery in young children should consider both the anesthetic risks of surgery in infancy and the ways in which mastoid anatomy may differ between infants and older children or adults. Multiple studies have found CI surgery in infants can be performed safely and effectively. This article reviews current evidence regarding indications for implantation in children younger than 12 months of age and discusses perioperative considerations and surgical technique.

Application of next generation sequencing for the diagnosis of congenital hearing loss

To identify genetic mutations among patients with hearing loss but without common GJB2, SLC26A4, 12 SrRNA mutations. Thirty-three patients were subjected to next-generation sequencing (NGS). Suspected mutations were verified by Sanger sequencing. Four patients were found to harbor previously known pathogenic variations, and four were found to carry suspicious pathogenic variations, which yielded a detection rate of 24.2%. NGS can improve the detection rate for mutations underlying congenital hearing loss and improve the efficiency and accuracy of the diagnosis.

Circulating cell free DNA and miRNA Amount in Congenital Hearing Loss

Objective: Our aim is to detect the amount of miRNA and free DNA in the peripheral blood of young people with congenital hearing loss and compare this with control group.
 Materials and Methods: In our study, 16 patients who have congenital hearing loss and go to the private school for deaf children and 16 healthy individuals were selected in the same age group. 5 cc blood was taken from peripheral vessels of each individual. We compared the circulating cell-free DNA and miRNA amount with the results of the control group.
 Results: The ccfDNA amount of the patients with hearing loss was lower than the control group and It was statistically significant. On the contrary, we found the higher amount of ccfmiRNA in plasma samples of the patients with hearing loss. The statistical analysis showed that ccfmiRNA amount in congenital loss is consistently significantly higher than the control group.
 Conclusion: The miRNA and freeDNA can be used early in the diagnosis of congenital hearing loss.

Large scale newborn deafness genetic screening of 142,417 neonates in Wuhan, China.

Almost one third of the three million people in China suffering severe deafness are children, and 50% of these cases are believed to have genetic components to their etiology. Newborn hearing genetic screening can complement Universal Neonatal Hearing Screening for the diagnosis of congenital hearing loss as well as identifying children at risk for late-onset and progressive hearing impairment. The aim of this joint academic and Ministry of Health project was to prototype a cost effective newborn genetic screen in a community health setting on a city-wide level, and to ascertain the prevalence of variation at loci that have been associated with non-syndromic hearing loss. With the participation of 143 local hospitals in the city of Wuhan, China we screened 142,417 neonates born between May 2014 and Dec. 2015. The variants GJB2 c.235delC, SLC26A4 c.919-2A>G, and mitochondrial variants m.1555A>G and m.1494C>T were assayed using real time PCR. Newborns found to carry a variant were re-assayed by sequencing in duplicate. Within a subset of 707 newborns we assayed using real-time PCR and ARMS-PCR to compare cost, sensitivity and operating procedure. The most frequent hearing loss associated allele detected in this population was the 235delC variant in GJB2 gene. In total, 4289 (3.01%) newborns were found to carry at least one allele of either GJB2 c.235delC, SLC26A4 c.919-2A>G or two assayed MT-RNR1 variants. There was complete accordance between the real-time PCR and the ARMS PCR, though the real-time PCR had a much lower failure rate. Real-time PCR had a lower cost and operating time than ARMS PCR. Ongoing collaboration with the participating hospitals will determine the specificity and sensitivity of the association of the variants with hearing loss at birth and arising in early childhood, allowing an estimation of the benefits of newborn hearing genetic screening in a large-scale community setting.

Population-based cross-sectional study to assess newborn hearing screening program in central Germany

ObjectivesEarly diagnosis of congenital hearing loss is fundamental to minimize the negative consequences on the speech development. To lower the age at diagnosis and at intervention in hearing impaired children, not only universal newborn hearing screening (NHS) but also tracking is considered essential. The aim of the study was to evaluate the first six years after implementation of the population based newborn hearing screening program in Saxony-Anhalt, one German Federal State. MethodsThe cross-sectional cohort study consisted of three cohort samples. Overall 102,301 infants born between January 2010 and December 2015 were included. NHS protocol was developed as dual target group protocol with two sub-protocols. The screening technique included Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Response (AABR) test. Newborns were assigned to the sub-protocols according to their audiological risk factors. Additionally, to evaluate the quality of NHS and tracking (false-negative screening) we were analysing data from a cohort of hearing impaired children diagnosed up to the age of three years. We calculated quality indicators and compared them with international guidelines. Results101,102 (98.8%) infants were screened. The prevalence of bilateral neonatal hearing loss was 2.32 per 1,000 newborns. The median age was two days at first screening, three month at diagnostic testing, and four month at intervention onset. 2.6% infants were lost to follow-up. 56.3% had a final diagnosis of bilateral sensorineural hearing loss. The sensitivity of 0.85 (KI 95%: 0.76-0.91) and a specificity of 0.84 (KI 95%: 0.84–0.85) was calculated for the NHS program. ConclusionThe analysis of benchmarks and outcomes of NHS demonstrated that the program reaches its main goal to identify the hearing impaired newborns in a timely manner.

Hearing impairment caused by mutations in two different genes responsible for nonsyndromic and syndromic hearing loss within a single family.

Usher syndrome is rare genetic disorder impairing two human senses, hearing and vision, with the characteristic late onset of vision loss. This syndrome is divided into three types. In all cases, the vision loss is postlingual, while loss of hearing is usually prelingual. The vestibular functions may also be disturbed in Usher type 1 and sometimes in type 3. Vestibular areflexia is helpful in making a proper diagnosis of the syndrome, but, often, the syndrome is misdiagnosed as a nonsyndromic hearing loss. Here, we present a Polish family with hearing loss, which was clinically classified as nonsyndromic. After excluding mutations in the DFNB1 locus, we implemented the next-generation sequencing method and revealed that hearing loss was syndromic and mutations in the USH2A gene indicate Usher syndrome. This research highlights the importance of molecular analysis in establishing a clinical diagnosis of congenital hearing loss.

Diagnosis and Management of Congenital Sensorineural Hearing Loss.

Hearing loss is the most common sensory disorder in the USA. The diagnosis of congenital hearing loss starts with newborn hearing screening, which is best performed with auditory brainstem evoked responses in order to avoid the risk of missing auditory neuropathy spectrum disorder. A careful history and physical exam can occasionally help reveal the etiology for congenital hearing loss. Imaging studies, either CT temporal bones or MRI of the internal auditory canals without gadolinium, and genetic testing, in particular for connexin 26, connexin 30, and Pendred syndrome, are the most useful diagnostic tests. Management of congenital hearing loss involves early fitting of amplification. Early cochlear implantation, preferably before 2 years of age, should be strongly considered for children with bilateral severe hearing loss.

Age of diagnosis of congenital hearing loss: Private v. public healthcare sector.

The age of diagnosis of congenital hearing loss is one of the most important determinants of communication outcome. A previous study by the lead author had evaluated the performance of the public health services in Bloemfontein, South Africa (SA), in this regard. This study aimed to examine whether the private health services in the same city were any better. To determine whether the age of diagnosis of congenital hearing loss (CHL) in children seen in the private healthcare sector in Bloemfontein, Free State Province, SA, was lower than that in the public healthcare system in the same city. A comparative study design was utilised and a retrospective database review conducted. Data obtained from this study in the private healthcare sector were compared with data from a previous study in the public healthcare sector using the same study design. Forty-eight children aged <6 years with disabling hearing impairment (DHI) were identified in the private healthcare sector during the study period; 33/47 (70.2%) did not undergo hearing screening at birth. The median age of diagnosis of DHI in the private healthcare sector was 2.24 years, and this was statistically significantly lower than the median age of diagnosis of 3.71 years in the public healthcare sector (p<0.0001; 95% confidence interval (CI) 0.99-2.0). The median age of diagnosis of congenital hearing loss (CHL) in the private healthcare sector was 3.01 years in children who were not screened at birth, and 1.25 years in those who were screened at birth. This difference was statistically significant (p<0.01; 95% CI 0.72-2.47). We also compared the median age of diagnosis of CHL in children from the private healthcare sector who were not screened at birth (median 3.01 years) with that in children in the public healthcare sector (median 3.71 years). This difference was statistically significant (p<0.01; 95% CI 0.41-1.56). Children in the Free State are diagnosed with CHL at a younger age in the private healthcare sector than in the public healthcare sector. With the social and economic benefits of early intervention in cases of DHI well established internationally, SA healthcare providers in both the public and private sectors need to develop screening, diagnostic and (re)habilitation services for children with hearing impairment.

Delays in Diagnosis of Congenital Hearing Loss in Rural Children

To examine the incidence of pediatric congenital hearing loss and the timing of diagnosis in a rural region of hearing healthcare disparity. Data from the Kentucky newborn hearing-screening program was accessed to determine the incidence of congenital hearing loss in Kentucky, both in the extremely rural region of Appalachia and non-Appalachian region of Kentucky. We also performed a retrospective review of records of children with congenital hearing loss at our institution to determine the timing of diagnostic testing. In Kentucky, during 2009-2011, there were 6970 newborns who failed hearing screening; the incidence of newborn hearing loss was 1.71 per 1000 births (1.28/1000 in Appalachia and 1.87/1000 in non-Appalachia); 23.8% of Appalachian newborns compared with 17.3% of non-Appalachian children failed to obtain follow-up diagnostic testing. Children from Appalachia were significantly delayed in obtaining a final diagnosis of hearing loss compared with children from non-Appalachian regions (P = .04). Congenital hearing loss in children from rural regions with hearing healthcare disparities is a common problem, and these children are at risk for a delay in the timing of diagnosis, which has the potential to limit language and social development. It is important to further assess the causative factors and develop interventions that can address this hearing healthcare disparity issue.

Sonographic imaging of fetal tympanic rings

To examine the feasibility of ultrasonographic imaging of fetal tympanic rings. This was an observational cohort study of 80 healthy fetuses in low-risk pregnancies, divided into four gestational-age subgroups (12, 16, 23 and 32 weeks), each comprising 20 consecutive fetuses. Tympanic ring visualization was achieved by two-dimensional and three-dimensional (3D) sonography. A standard algorithm for tympanic ring examination was constructed using 3D multiplanar reconstruction. The volume acquisition plane was directed to the inferolateral aspect of the fetal temporal bone. Transvaginal scans were carried out in the 12-week and 16-week subgroups, and transabdominal scans in the 23-week and 32-week subgroups. Study parameters included the inferomedial inclination angle (IMIA) of the tympanic ring relative to the vertical skull axis, the anteromedial inclination angle (AMIA) of the tympanic ring relative to the anteroposterior skull axis and the longest (LTRD) and shortest (STRD) tympanic ring diameter, the latter measured perpendicular to the LTRD. The feasibility of tympanic ring demonstration was assessed in each gestational-age subgroup. Tympanic rings appeared as round-oval, thin, echogenic structures in a plane tangential to the inferolateral surface of the fetal skull below the inferior border of the squamous part of the temporal bone. Higher demonstration rates were achieved in the 16-week and 23-week subgroups (90% and 80%, respectively) than in the others. LTRD and STRD each showed a linear correlation with gestational age (r = 0.96 for both measurements; P < 0.01). Mean IMIA ranged from 41.0 to 60.4° and mean AMIA from 17.3 to 23.4° across the different gestational-age subgroups. The malleal manubrium was observed only in examinations in the second half of pregnancy, appearing as a bright echo within the upper area of the tympanic ring in 56% (9/16) and 82% (9/11) of cases with tympanic ring imaging appropriate for measurement of the study parameters in the 23-week and 32-week subgroups, respectively. This is the first report of sonographic imaging of fetal tympanic rings and shows that this is feasible in the second trimester. We discuss the possible implications of our findings for the prenatal diagnosis of congenital hearing loss.

13歳で診断された小児難聴の 1 症例

13歳で診断された小児難聴の 1 症例

The age of diagnosis of congenital hearing loss in Universitas Hospital

Congenital hearing loss affects 3 - 6/1 000 children worldwide. The benefits of early identification of hearing loss and early intervention have been clearly established. There are no previous studies reporting on the age of diagnosis of congenital hearing loss in the Free State province. To determine the age of diagnosis of congenital hearing loss in the Otorhinolaryngology Clinic at Universitas Hospital. Secondary aims included determining age at first visit, as well as the time delay between first visit and diagnosis, and documenting any interventions which took place. A retrospective, descriptive study was undertaken, analysing data from 2001 to 2010. A total of 260 cases of congenital hearing loss were analysed. The median age of diagnosis of hearing loss was 44.5 months. The median age of first visit was 40.9 months, and the median delay between first visit and diagnosis was 49 days. The median age of diagnosis far exceeds national and international benchmarks. This has a profoundly negative impact on the development and outcomes of children with hearing loss. These results have been used to motivate for the expansion of hearing screening and diagnostic services in the province.

High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss

ObjectiveRadiological and genetic examination has recently advanced for diagnosis of congenital hearing loss. The aim of this study was to elucidate the prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss (USNHL) for better management of hearing loss and genetic and lifestyle counseling. MethodsWe conducted a retrospective study of charts and temporal bone computed tomography (CT) findings of 69 consecutive patients 0–15 years old with USNHL. In two cases, genetic examination was conducted. ResultsOf these patients, 66.7% had inner-ear and/or internal auditory canal malformations. The prevalence of malformations in infants (age <1 year) was 84.6%, which was significantly higher than that in children 1–15 years old (55.8%; p<0.01). Almost half of the patients (32; 46.4%) had cochlear nerve canal stenosis; 13 of them had cochlear nerve canal stenosis alone, and in 19 it accompanied other malformations. Internal auditory canal malformations were observed in 22 subjects (31.8%), 14 (20.3%) had cochlear malformations, and 5 (7.2%) had vestibular/semicircular canal malformations. These anomalies were seen only in the affected ear, except in two of five patients with vestibular and/or semicircular canal malformations. Two patients (2.9%) had bilateral enlarged vestibular aqueducts. Mutations were found in SLC26A4 in one of the two patients with bilateral large vestibular aqueducts. The prevalence of a narrow internal auditory canal was significantly higher in subjects with cochlear nerve canal stenosis (50.0%) than in subjects with normal cochlear nerve canals (11.1%; p<0.01). There were no correlations between the type and number of malformations and hearing level. ConclusionsThe prevalence of inner-ear and/or internal auditory canal malformations detected by high-resolution temporal bone CT in children with USNHL was very high. Radiological and genetic examination provided important information to consider the pathogenesis and management of hearing loss. Temporal bone CT should be recommended to children with USNHL early in life. SLC26A4 mutation also should be examined in cases with bilateral enlarged vestibular aqueduct.

A case of delayed diagnosis of congenital hearing loss

A case of delayed diagnosis of congenital hearing loss

Considerations in early amplification: Selection, fitting, validation, counseling

The implementation of universal newborn hearing screening programs has increased the number of infants diagnosed with hearing loss and referred for amplification in the first few weeks of life. There is compelling evidence that children born with hearing loss who have been identified and begun intervention by 6 months of age will have significantly better language development than their later-identified peers. 1-3 The challenges of fitting appropriate amplification on this population include limited behavioral response to sound, possible complicating middle ear fluid, parental uncertainties, possible existence of additional handicaps, and limited resources to support and pay for hearing aids. Despite these issues, there is consensus that the diagnosis of congenital hearing loss should be completed in the first 3 months of life and be followed immediately with the consideration of hearing aid use. 4 As access to follow-up from newborn hearing screening improves, pediatric audiologists are faced with recommending technology for infants during their early weeks of life. The options available to them are expanding rapidly as amplification technology grows and diversifies. Outcome studies of children fitted with advanced amplification features are not common and cannot keep up with the features being introduced to the market. Most infants are fitted with the goal of providing audibility for environmental speech to enhance speech and language development and with the expectation that patients will use their hearing aids daily. The pediatric audiologist’s role is to share information on hearing loss and available amplification technology with the parents and their medical and support team. Parents depend on various professionals to acquire the information and tools they need to become empowered, informed consumers. Reaching decisions about the choice of technology for an infant includes examining available features and selecting what might be the best fit for the infant given all the available information and circumstances.

Implementation of a nation‐wide automated auditory brainstem response hearing screening programme in neonatal intensive care units

Aim: As part of a future national neonatal hearing screening programme in the Netherlands, automated auditory brainstem response (AABR) hearing screening was implemented in seven neonatal intensive care units (NICUs). The objective was to evaluate key outcomes of this programme: participation rate, first stage success rate, pass/referral rates, rescreening compliance, diagnostic referral rates, age of first diagnostic evaluation and prevalence of congenital hearing loss (CHL). Methods: This prospective cohort study collected data on 2513 survivors. NICU graduates with one or more risk factors according to the Joint Committee on Infant Hearing were included in a two‐stage AABR hearing screening programme. Conventional ABR was used to establish a diagnosis of CHL. Results: A total of 2513 newborns enrolled in the programme with a median gestational age of 31.6 (range 24–43) wk and a median birthweight of 1450 (range 510–4820) g. In 25 (1%) cases parents refused screening. Four out of 2513 newborns were initially lost; 2484 newborns have been tested initially. A final 98% participation rate (2465/2513) was obtained for the whole programme. After a median postmenstrual age at the first test of 33.7 (range 27–54) wk, a pass rate of 2284/2484 (92%) resulted at the first stage. The rescreening compliance after the first test was 92% (184/200). A referral rate for diagnostic ABR of 3.1% (77/2484) resulted. Of the 77 referrals 14 (18.2%) had normal screening thresholds, 15 (19.5%) had unilateral CHL and 48 (62.3%) had bilateral CHL. The prevalence of unilateral CHL was 0.6% (15/2484) and of bilateral CHL 1.9% (48/2484). Conclusion: A financially supported two‐stage AABR hearing screening programme can be successfully incorporated in NICU centres and detects a high prevalence of CHL in NICU graduates. Neonatal hearing screening should be part of standard clinical practice in all NICU infants.

Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss

The advent of hearing screening in newborns in many states has led to an increase in the use of genetic testing and related genetic services in the follow-up of infants with hearing loss. A significant proportion of those with congenital hearing loss have genetic etiologies underlying their hearing loss. To ensure that those identified with congenital hearing loss receive the genetic services appropriate to their conditions, the Maternal and Child Health Bureau of the Health Resources and Services Administration funded the American College of Medical Genetics to convene an expert panel to develop guidelines for the genetic evaluation of congential hearing loss. After a brief overview of the current knowledge of hearing loss, newborn screening, and newborn hearing screening, we provide an overview of genetic services and a guideline that describes how best to ensure that patients receive appropriate genetic services. The significant contribution of genetic factors to these conditions combined with the rapid evolution of knowledge about the genetics of these conditions overlaid with the inherently multidisciplinary nature of genetic services provides an example of a condition for which a well-integrated multidisciplinary approach to care is clearly needed.

The Colorado newborn hearing screening project, 1992-1999: on the threshold of effective population-based universal newborn hearing screening.

Objective. Although previous studies have documented the feasibility and benefits of universal newborn hearing screening in selected hospitals, none have reviewed the effectiveness of regionally mandated participation of large numbers of hospitals with variable levels of motivation to succeed. The purpose of this study was to measure hospital participation and overall screening success in a statewide program for universal newborn hearing screening and to track improvements in program establishment and outpatient follow-up over time. Methods. Four Colorado hospitals began voluntarily performing hearing screening before hospital discharge on all newborns in 1992. By 1996, 26 Colorado hospitals were participating in universal newborn hearing screening. The publication of screening results from these early years served as a catalyst for legislation requiring increased hospital participation in establishing universal screening programs. Data systems were subsequently developed to improve statistical tracking and follow-up. Eight years’ worth of cumulative study data as well as the results from calendar year 1999 (the year of greatest hospital participation) were reviewed for collective measures of successful screening and follow-up. Three hospitals did not initiate newborn hearing screening programs until after the study period ended in 1999. Of the 57 hospitals that were screening newborns in 1999, the chosen method of screening at 52 hospitals was automated auditory brainstem response testing; 3 hospitals used otoacoustic emission testing, and the remaining 2 hospitals used 2-stage screening. Hearing loss was defined as a threshold of 35 decibels or greater in 1 or both ears at the time of confirmatory testing. Results. During the full 8-year study period, 1992 to 1999, 148 240 newborns were screened. A total of 291 infants who were born during the study period received a diagnosis of congenital hearing loss. In this cohort of 291 children, the cumulative frequency of bilateral hearing loss was 71% (range: 48%–94% by calendar year), the frequency of sensorineural hearing loss was 82% (range: 67%–88%), and the frequency of 1 or more risk factors was 47% (range: 37%–61%). During calendar year 1999, a total of 63 590 births were recorded at 60 birthing hospitals in Colorado. The families of 263 (0.4%) of these newborns refused newborn hearing screening. Of the remaining 63 327 newborns, 87% (55 324 infants) were screened for hearing acuity before hospital discharge, a far greater percentage than the 19% of all newborns screened during the first 5 years of voluntary hospital participation, and approaching the American Academy of Pediatrics’s recommendation of 95% of newborns completing hospital-based testing in a successful screening program. As a result of this statewide hearing screening program, congenital hearing loss was diagnosed in 86 Colorado newborns during 1999, representing an occurrence rate of approximately 1 affected child in every 650 newborns. In this group of 86 infants, 59 had bilateral sensorineural hearing loss, 17 had unilateral sensorineural hearing loss, 4 had bilateral conductive hearing loss, and 6 had unilateral conductive hearing loss. Mild hearing loss was present in 6 infants, moderate hearing loss was present in 42 infants, severe hearing loss was present in 33 infants, and profound hearing loss was present in the remaining 5 infants. Only 32 of the 86 affected newborns in 1999 had 1 or more risk factors for hearing loss subsequently identified. After failing an initial hospital-based screening at 1 of the 57 participating hospitals in 1999, 2.3% of infants screened (1283 newborns) were referred for follow-up testing, easily exceeding the standard of Conclusions. Universal screening for congenital hearing loss is demonstrated to be feasible in a large regional effort of legislatively mandated participation. The success of such an endeavor is dependent on educational efforts for community professionals, commitment on the part of program planners, and data systems that more accurately track and recall infants who fail initial hospital-based screening.