Frequency and natural course of congenital cytomegalovirus-associated hearing loss in children.

BACKGROUND: Congenital cytomegalovirus-associated hearing loss (cCMV-associated HL) is a common cause of congenital or early-onset deafness. Although cCMV infection has been reported to lead to various types of HL, the natural course of cCMV-associated HL over a long period is not yet known. OBJECTIVES: To investigate the clinical phenotype of cCMV-associated HL in the largest study to date. METHODS: Thirty-one CMV-positive children, diagnosed by examining CMV DNA extracted from their dried umbilical cords retrospectively, were divided into unilateral and bilateral HL groups, and their hearing ability was evaluated using pure-tone audiometry and auditory steady-state response over time. RESULTS: Thirteen patients (41.9%) had unilateral HL and 18 (58.1%) had bilateral HL. In most cases of unilateral cCMV-associated HL, the ear with better hearing maintained a normal hearing threshold. Notably, in most cases of both unilateral and bilateral HL, the ear with worse hearing ultimately showed severe to profound HL. CONCLUSION: Our findings revealed that the natural course of cCMV-associated HL was different between the cases of unilateral and bilateral HL, as well as between the ears with better or worse hearing in all cases. These findings indicate that accurate diagnosis could enable proper follow-up and management of cCMV-associated HL in children.

Prevalence of Jervell-Lange Nielsen syndrome in children with congenital bilateral sensorineural hearing loss.

OBJECTIVE: Long QT syndrome (LQTS) is an inherited cardiac ion channel disorder (channelopathy) that is characterized by prolonged QT intervals on the electrocardiography (ECG) and possess the risk of sudden cardiac death (SCD). Jervell-Lange Nielsen syndrome (JLNS) is a specific subtype of LQTS that is accompanied by congenital sensorineural hearing loss, inherited autosomal recessively, and higher risk of SCD. In this study, we aimed to investigate JLNS prevalence in deaf children attending special schools for hearing loss, located in our province. METHODS: An ECG screening program was conducted in 6 special schools for children with hearing loss in Istanbul and a total of 440 students between 6 and 18 years old were included. Corrected QT interval (QTc) was calculated using the Bazett formula. Notably, 51 students, detected with any abnormal finding on ECG, were invited to our center for a comprehensive examination. RESULTS: A total of 8 patients were found with a prolonged QT interval. JLNS was diagnosed in 4 (0.9%) patients. In addition, 2 students had already been diagnosed with JLNS at another center earlier. The other 2 students, being siblings, were newly diagnosed with JLNS; and appropriate treatment was initiated. Genetic testing revealed a pathological homozygous mutation in KCNQ1 gene. The younger sibling (Case 1), who possessed a QTc of greater than 500 ms and a history of syncope, which was very suspicious for SCD, was implanted an implantable cardioverter-defibrillator. Propranolol treatment was initiated for both siblings. CONCLUSION: JLNS should be carefully considered and screened, especially in patients with a history of congenital deafness.

Hearing Aid Use, Auditory Development, and Auditory Functional Performance in Swedish Children With Moderate Hearing Loss During the First 3 Years.

Purpose This study investigated longitudinal hearing aid (HA) use in a cohort of children with moderate hearing loss (CHL), fitted with amplification before the age of 6 months. Additionally, the relationship of HA use and aided audibility on outcomes of parental questionnaires of auditory skills was examined, and these outcomes were compared to a group of children with normal hearing (CNH). Method Nine CHL and 29 CNH and their parents participated in the study. Measures were collected at initial fitting, 10, 18, 24, 30, and 36 months of age. Parents reported hours of HA use and situations the HAs were used. Datalogging and speech intelligibility index were also collected. Auditory skills were measured through parental questionnaires. Results The mean hours of HA use/day for this cohort increased from 7.55 at the ages of 10 months to 10.15 at 36 months according to datalogging. Parental estimations of hours of HA use and in which situations varied between subjects. Correlations between HA use from datalogging and speech intelligibility index to measures of auditory skills were weak. CHL showed similar results to CNH on auditory development at the ages of 10, 18, and 24 months but presented with significantly lower scores on auditory functional performance in noise at 30 and 36 months of age. Conclusions Longitudinal monitoring of HA use from fitting of amplification with the combination of objective and subjective tools may have a positive impact on HA use in CHL. The lower scores on listening in noise compared to CNH call for further attention.

Genetic Testing for Congenital Bilateral Hearing Loss in the Context of Targeted Cytomegalovirus Screening.

OBJECTIVES/HYPOTHESIS: To determine the prevalence of children with genetic hearing loss who are cytomegalovirus (CMV) positive at birth and the relative proportion of genetic and CMV etiology among children with congenital bilateral hearing loss. STUDY DESIGN: Database review. METHODS: We performed a review of clinical test results for patients undergoing comprehensive genetic testing for all known hearing loss-associated genes from January 2012 to January 2019. This population was reviewed for reported CMV status and genetic causes of congenital bilateral hearing loss. RESULTS: In the OtoSCOPE database, 61/4,282 patients were found to have a documented CMV status, and 661/4282 had documented bilateral congenital hearing loss. Two patients were identified who had both a positive CMV result and a genetic cause for their hearing loss. Forty-eight percent of patients with bilateral congenital hearing loss (320/661) were found to have a genetic etiology. In 62% (198/320), the hearing loss was associated with pathogenic variants in GJB2, STRC, SLC26A4 or an Usher syndrome-associated gene. CONCLUSIONS: We estimate that ~2% of CMV-positive newborns with hearing loss have a known genetic variant as a cause. The subcohort of CMV-positive newborns with symmetric mild-to-moderate bilateral hearing loss will have at least a 7% chance of having pathogenic gene variants associated with hearing loss. In a CMV-positive neonate who failed their newborn hearing screen bilaterally, genetic screening needs to be considered for accurate diagnosis and possible deferment of antiviral treatment. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2714-2718, 2020.

Outcomes of regional-based newborn hearing screening for 35,461 newborns for 5 years in Akita, Japan.

OBJECTIVES: Newborn hearing screening (NHS) has been actively performed since 2001 in Akita, Japan. The NHS coverage rate has increased yearly, and performance has been consistently >90% since 2012. The purpose of this study was to summarize NHS outcomes in the Akita prefecture of Japan and to obtain new insights for from our summarized data for the future. METHODS: A total of 35,461 newborns in hospitals and clinics where hearing screening was performed in Akita from 2012 to 2016 were included. The outcome data of NHS were collected for analysis. RESULTS: The overall screening coverage rate for hearing loss was 94.7%. Of the screened infants, 0.53% received a referral on the 2-stage automated auditory brainstem response (ABR), and 80.4% of referred infants had a check-up at the hospital to receive a diagnostic hearing examination. Finally, the prevalence of bilateral congenital hearing loss was 0.14%, that of bilateral moderate to profound hearing loss was 0.12%, and that of unilateral congenital hearing loss was 0.10%. Furthermore, the average consultation period in infants with risk factors was significantly later than that in infants without risk factors (p = 0.0015). Follow-up for infants diagnosed with normal hearing after diagnostic hearing examination revealed that 4.7% suffered bilateral moderate to profound hearing loss later. This percentage is significantly higher than that of the general group (p < 0.001). CONCLUSION: The prevalence of bilateral congenital hearing loss was 0.14% in Akita and 0.12% of infants were diagnosed with bilateral moderate to severe hearing loss. Medical personnel should be enlightened regarding the importance of performing hearing diagnostic examinations until 3 months of age. Even if infants were diagnosed with normal hearing after a diagnostic examination, we strongly suggest continuing follow-up until they are able to perform pure tone audiometry with accuracy.

Case study: cochlear implantation in cochlear otospongiosis.

Objective and importance: Cochlear implantation can be performed successfully in patients with otospongiosis of the temporal bone with the potential for excellent audiological outcomes and high patient satisfaction. The purpose of this case report is to highlight the clinical considerations for implantation in cochlear otospongiosis including the need for careful pre-operative implant device selection, intra-operative surgical challenges such as the presence of hypervascularity and possible cochlear ossification resulting in difficulty in placing the electrode array and the possibility of postoperative facial nerve stimulation.Clinical presentation: A 14-year-old girl with cochlear otospongiosis likely due to osteogenesis imperfecta presented with progressive bilateral profound sensorineural hearing loss underwent successful cochlear implantation despite several challenges.Conclusion: Cochlear implantation in patients with cochlear otospongiosis with profound sensorineural hearing loss potentially may be very successful. Thorough pre-operative radiological evaluation is necessary. Possible intra-operative and post-operative challenges unique to these patients must be kept in mind. Adequate precautions should be taken to optimize the likelihood of complete electrode insertion such as using a depth gauge prior to inserting the electrode array and performing an intra-operative x-ray and / or neural response telemetry to confirm correct electrode placement.

Comparison of the prevalence and features of inner ear malformations in congenital unilateral and bilateral hearing loss.

OBJECTIVES: The aim of the study was to clarify differences in the prevalence and features of bony malformations in inner ear between congenital unilateral sensorineural hearing loss (USNHL) and congenital bilateral sensorineural hearing loss (BSNHL). METHODS: We conducted a retrospective study of 378 consecutive infants referred from routine newborn hearing screening in the past 18 years. Clinical background, audiological data, and temporal bone computed tomography (CT) findings were analyzed. The prevalence of malformations between USNHL and BSNHL groups were compared using the Chi-square test. RESULTS: The proportion of family history of hearing loss was significantly higher in infants with BSNHL than in those with USNHL (26/107 [24.3%] vs. 4/105 [3.7%]; p = 0.0001). Temporal bone CT scanning revealed significantly a higher prevalence of inner ear malformations in infants with USNHL than in those with BSNHL (93/109 [85.3%] vs. 4/107 [3.7%]; p < 0.0001). The most frequent anomaly in USNHL was cochlear nerve canal stenosis (69.7%), followed by cochlear malformations (20.2%), and narrow internal auditory canal (17.4%). Four infants with BSNHL accompanied by inner ear anomaly had complications such as Down's syndrome, developmental delay, or epilepsy. CONCLUSIONS: The prevalence of bony malformations in inner ear and/or IAC was markedly higher in infants with congenital USNHL than in infants with BSNHL. Temporal bone CT scanning may help to clarify the etiology of congenital hearing loss, especially in USNHL.

An Analysis of Risk Factors in Unilateral Versus Bilateral Hearing Loss.

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P < .001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.

The common cavity vs incomplete partition type I conundrum: Decision making and management with the cochlear implant form electrode.

It has been seen that 20% of children with deafness have inner ear malformations. Studies demonstrate that Cerebrospinal Fluid (CSF) leakage occurs in 40-50% of patients with inner ear malformations. Differentiating between the various malformations pre-operatively allows the cochlear implant surgeon to plan and prepare for various difficulties associated with the management of such cases. In case of a CSF gusher, a choice of an electrode with a stopper helps in effectively closing off the cochleostomy, thus reducing the chances of post-operative infections. Case description: SM, a 10-year-old girl with the diagnosis of bilateral progressive profound hearing loss showed cochlear malformations on high resolution computed tomography (HRCT) scans of the temporal bones. A detailed study and discussion with the neuro-radiologist helped in identifying the cochleo-vestibular malformations (CVM) on both sides, thus making the choice of the better ear for electrode insertion and the type of electrode. She was subjected to left ear cochlear implantation using a lateral canal labyrinthotomy approach and a Form electrode (Med El®) with a special conical stopper was used to effectively seal the labyrinthostomy. Conclusion: Common cavity (CC) and Incomplete Partition (IP) Type I present as diagnostic challenges. Getting to the right diagnosis is of paramount importance as it helps in deciding the correct ear, choosing the correct electrode, and preparing for the surgical situations the surgeon might encounter. The Form electrode shows promise due to its unique conical seal which helps in preventing CSF leakage, thus reducing the chances of post-operative infections in cases of deformed cochlea.

Factors Affecting Sound-Source Localization in Children With Simultaneous or Sequential Bilateral Cochlear Implants.

OBJECTIVES: The study aimed to determine the effect of interimplant interval and onset of profound deafness on sound localization in children with bilateral cochlear implants, controlling for cochlear implant manufacturer, age, and time since second implant. DESIGN: The authors conducted a retrospective, observational study using routinely collected clinical data. Participants were 127 bilaterally implanted children aged 4 years or older, tested at least 12 mo post- second implant. Children used implants made by one of three manufacturers. Sixty-five children were simultaneously implanted, of whom 43% were congenitally, bilaterally profoundly deaf at 2 and 4 kHz and 57% had acquired or progressive hearing loss. Sixty-two were implanted sequentially (median interimplant interval = 58 mo, range 3-143 mo) of whom 77% had congenital and 23% acquired or progressive bilateral profound deafness at 2 and 4 kHz. Children participated in a sound-source localization test with stimuli presented in a random order from five loudspeakers at -60, -30, 0, +30, and +60 degrees azimuth. Stimuli were prerecorded female voices at randomly roved levels from 65 to 75 dB(A). Root mean square (RMS) errors were calculated. Localization data were analyzed via multivariable linear regression models, one applied to the whole group and the other to just the simultaneously implanted children. RESULTS: Mean RMS error was 25.4 degrees (SD = 12.5 degrees) with results ranging from perfect accuracy to chance level (0-62.7 degrees RMS error). Compared with simultaneous implantation, an interimplant interval was associated with worse localization by 1.7 degrees RMS error per year (p < 0.001). Compared with congenital deafness, each year with hearing thresholds better than 90 dB HL at 2 and 4 kHz bilaterally before implantation led to more accurate localization by 1.3 degrees RMS error (p < 0.005). Every year post-second implant led to better accuracy by 1.6 degrees RMS error (p < 0.05). Med-El was associated with more accurate localization than Cochlear by 5.8 degrees RMS error (p < 0.01) and with more accurate localization than Advanced Bionics by 9.2 degrees RMS error (p < 0.05). CONCLUSIONS: Interimplant interval and congenital profound hearing loss both led to worse accuracy in sound-source localization for children using bilateral cochlear implants. Interimplant delay should therefore be minimized for children with bilateral profound hearing loss. Children presenting with acquired or progressive hearing loss can be expected to localize better via bilateral cochlear implants than their congenitally deaf peers.

Assessment of balance skills and falling risk in children with congenital bilateral profound sensorineural hearing loss.

OBJECTIVE: To evaluate the balance skills and falling risk in children with a congenital bilateral profound sensorineural hearing loss (CBPSNHL). METHODS: 25 children with CBPSNHL and healthy 25 children with similar age and gender were included in the study. The flamingo balance test, the tandem stance test, and the one-leg standing test were performed to assess the patients' static balance skills. The pediatric balance scale (PBS) was used to evaluate the dynamic balance. Visual analog scale (VAS) was applied to the patients assess the frequency of falls. RESULTS: The flamingo balance test, the tandem stance test, and the one-leg standing test in the children with CBPSNHL were all significantly worse than the control group. Although the scores of PBS in patients with CBPSNHL were significantly lower than the control group (p < 0.001), the results of both groups were consistent with a low risk of falls. There was no significant difference between the VAS scores indicating the frequency of falls among the groups (p = 0.552). CONCLUSION: Static and dynamic balance skills of the children with CBPSNHL are significantly impaired compared to their healthy peers. Children with CBPSNHL also have a lower risk of falling just like their healthy peers and there is no significant difference between their falling frequencies. Balance skills of children with CBPSNHL can be assessed quickly and effectively on a hard floor (eyes closed), with a tandem standing test or a one-leg standing test.

Duplicated internal auditory canal with inner ear malformation: Case report and literature review.

Internal auditory canal anomalies are rare. Narrow internal auditory canal is believed to occur as a result of aplasia or hypoplasia of the vestibulocochlear nerve. Narrow duplication of the internal auditory canal is considered to be very rare. Narrow duplication of the internal auditory canal with inner ear malformation has been reported in only 3 cases. We present 2 cases of narrow duplication of the internal auditory canal with inner ear malformation. The first case had inner ear malformation on only one side and the second case had inner ear malformation on both sides. The embryogenesis may be different between internal auditory canal and inner ear.

What Is the Sensitive Period to Initiate Auditory Stimulation for the Second Ear in Sequential Cochlear Implantation?

OBJECTIVES: Bilateral cochlear implants (CI) are the standard treatment for bilaterally deaf children, but it is unclear how much the second CI can be delayed in sequential bilateral CI. We investigated the performances of sequential CI to answer this question. STUDY DESIGN: Retrospective case series review. SETTING: Tertiary referral center. METHODS: We studied a cohort of congenitally deaf children (n = 73) who underwent sequential CI without any inner ear anomaly or combined disabilities. Hearing threshold levels and speech perception were evaluated by aided pure tone audiometry and Asan-Samsung Korean word recognition test. The scores were analyzed by the ages at surgery and compared among the different age groups. RESULTS: When the second CI was performed before 3.5 years (the optimal period for the first CI), the second CI scores (96.9%) were comparable to the first CI scores. Although the first CI scores were more than or equal to 80% when the first CI was implanted before the age of 7 years, the second CI scores were more than or equal to 80% when the second CI was implanted before the age of 12 to 13 years. The hearing threshold levels were not different regardless of the ages and between the first and second CIs. CONCLUSION: Our cohort demonstrated that the second CI showed comparable results to the first CI when implanted before 3.5 years, suggesting that optimal periods for the first CI and the second CI are same. However, the sensitive period (12-13 yr) for the second CI with good scores (≥80%) was much longer than that (7 yr) of the first CI, suggesting that the first CI prolongs the sensitive period for the second CI. The second CI should be implanted early, but considered even at a later age.

Vocabulary development in Mandarin-speaking children with cochlear implants and its relationship with speech perception abilities.

BACKGROUND: China has the largest population of children with hearing impairments and cochlear implantation is gaining popularity there. However, the vocabulary development in this population is largely unexplored. AIMS: This study examined early vocabulary outcomes, factors influencing early vocabulary development and the relationship between speech perception and vocabulary development in Mandarin-speaking children during the first year of cochlear implant use. METHODS AND PROCEDURES: A battery of vocabulary tests was administered to 80 children before implantation and 3, 6, and 12 months after implantation. Demographic information was obtained to evaluate their relationships with vocabulary outcomes. OUTCOMES AND RESULTS: The Mandarin-speaking children, who received their cochlear implants before 3 years of age, developed vocabulary at a rate faster than that of their same-aged peers with normal hearing. Better pre-implant hearing levels, younger age at implantation, and higher maternal education level contributed to the early vocabulary development. The trajectories of speech perception development highly correlated with those of vocabulary development during 3 to 12 months of CI use. CONCLUSIONS: and Implications: These findings imply that the vocabulary development of children implanted before 3 years of age may catch up with that of their hearing peers.

Malformation of the eighth cranial nerve in children. / Malformaciones del octavo par en niños.

INTRODUCTION AND OBJECTIVES: Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5-6 in every 1,000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%-10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. METHODOLOGY: We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis (P<.05). RESULTS: Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. CONCLUSIONS: Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation.

Early speech perception in Mandarin-speaking children at one-year post cochlear implantation.

PURPOSE: The aim in this study was to examine early speech perception outcomes in Mandarin-speaking children during the first year of cochlear implant (CI) use. METHOD: A hierarchical early speech perception battery was administered to 80 children before and 3, 6, and 12 months after implantation. Demographic information was obtained to evaluate its relationship with these outcomes. RESULTS: Regardless of dialect exposure and whether a hearing aid was trialed before implantation, implant recipients were able to attain similar pre-lingual auditory skills after 12 months of CI use. Children speaking Mandarin developed early Mandarin speech perception faster than those with greater exposure to other Chinese dialects. In addition, children with better pre-implant hearing levels and younger age at implantation attained significantly better speech perception scores after 12 months of CI use. Better pre-implant hearing levels and higher maternal education level were also associated with a significantly steeper growth in early speech perception ability. CONCLUSIONS: Mandarin-speaking children with CIs are able to attain early speech perception results comparable to those of their English-speaking counterparts. In addition, consistent single language input via CI probably enhances early speech perception development at least during the first-year of CI use.

Schooling of hearing-impaired children and benefit of early diagnosis.

OBJECTIVE: To assess the impact of moderate-to-severe bilateral hearing loss on schooling and the factors influencing this impact, and to evaluate special schooling needs in addition to speech therapy. MATERIAL AND METHODS: Retrospective study including children with moderate-to-severe bilateral hearing loss, born between 1992 and 2006, diagnosed and managed in our institution. The age and degree of hearing loss in the better ear, the type of schooling and the level of schooling at the time of the last visit were recorded for each patient. RESULTS: Two hundred and twenty-five hearing-impaired children were included: 161 attended a regular school (58% of the 55 children with severe hearing loss and 76% of the 170 children with moderate hearing loss). The percentage of children with moderate hearing loss attending a regular school increased over time. This study did not demonstrate any difference in terms of grade retention according to the age at diagnosis for children with moderate hearing loss. No child with comorbidity affecting intellectual capacities attended a regular school. CONCLUSION: This study confirms that moderate-to-severe congenital bilateral hearing loss has an impact on the child's schooling, with grade retention that depends, but not exclusively, on the degree of hearing loss. A growing number of children with moderate bilateral hearing loss fitted with a hearing aid now attend a regular school.

A child with severe hearing loss associated with maternal cisplatin treatment during pregnancy.

BACKGROUND: Cisplatin is considered safe to use during the second and third trimesters of pregnancy in patients with cancer. CASE: A 34-year-old pregnant woman was diagnosed with cervical cancer. She received five weekly dosages of cisplatin and paclitaxel, starting at 26 5/7 weeks of gestation. An elective cesarean delivery was performed at 34 4/7 weeks of gestation. After birth, the neonate was diagnosed with severe bilateral perceptive hearing loss. CONCLUSION: Cisplatin during the second and third trimesters of pregnancy may lead to fetal ototoxicity.

The universal newborn hearing screening program of Taipei City.

OBJECTIVES: To establish a hearing screening program with high coverage, low referral rate, high follow-up rate, and early intervention in Taipei City. METHODS: From September 2009 to December 2010, 85% delivery units in Taipei City, which includes 20 hospitals and 14 obstetrics clinics, were recruited into the screening program in two stages. A total of 15,930 babies were born in these participating hospitals and clinics during the program period. Among these neonates, 15,790 underwent hearing screening test with automatic auditory brainstem response (AABR). The screening was free of charge to the parents. The hearing screening examination was performed 24-36 h after birth. The same test was repeated between 36 and 60 h of age if the baby failed the first hearing test. The neonate was referred to the diagnostic hospitals for further investigations if he failed the second test. RESULTS: The screening coverage rate was 99.1% (15,790/15,930). The incidence of bilateral moderate to severe and unilateral hearing loss was 1.4 per 1000 (22/15,790) and 1.5 per 1000 (24/15,790), respectively. Four percent (626/15,790) of newborns failed to pass the initial screening test and 1.0% of newborns failed to pass the second screening test. Therefore, 1.0% newborns were referred for diagnostic assessments. The follow-up rate was 94.4% (151/160). Sixty-four percent (14/22) of babies with bilateral hearing loss completed the full diagnostic hearing tests within 3 months of birth. CONCLUSIONS: The universal newborn hearing screening program is an adequate program for Taipei City with high coverage, low referral rate, and good follow-up rate. Screening fees covered by third parties, two-stage screening steps with AABR strategy, and the stringent monitoring system proved to be effective. LEVEL OF EVIDENCE: 2b, individual cohort study.