Bilateral Profound Hearing Loss Research Articles

Cochlear implantation in syndromic patients: difficulties and lessons learnt.

Identify the prevalence of syndromes in a cohort of patients who underwent cochlear implantation, to report on the presence of inner and middle ear malformations and highlight the surgical difficulties encountered. Observational, retrospective study. Tertiary referral children's hospital pediatric cochlear implant program. An IRB-approved retrospective chart review of children undergoing cochlear implantation at a tertiary academic medical center, from 2018 to 2023. Preoperative imaging data of syndromic patients in that cohort with special attention to the presence of inner ear or middle ear malformations were collected. Abnormal intraoperative findings and difficulties reported by the surgeons were also noted. 1024 children were unilaterally implanted for bilateral profound hearing loss. There were 45 cases diagnosed with associated syndromes (4.3%). The commonest syndromes were Jervell and Lange Nielsen (JLN) syndrome followed by Waardenberg syndrome (WS), in a prevalence of 34% and 32% respectively. These syndromes had no associated inner ear malformations (IEM). Less common syndromes included Branchio-oto-renal (BOR) syndrome, CHARGE association and Treacher Collins syndrome, 3 cases each, and keratosis icthyosis deafness syndrome (KID), Usher syndrome and Albino, 2 cases each and an H syndrome case. There were 9 cases (20%) with IEM, with 6 cases of perilymph gusher. Two cases had middle ear anomalies and one case had a facial nerve course abnormality. The outcome of these cases was similar to non-syndromic cases. Children with syndromic HL should be dealt with on a case by case scenario to diagnose inner and middle ear malformations. Additional disabilities can affect the rehabilitation procedures. All children with congenital hearing loss should undergo pediatric, cardiologic, ophthalmologic and nephrologic examination in order to exclude the syndromic etiology of hearing loss.

Does cochlear implantation affect personality of hearing-impaired patients? A five-year follow-up study

Background Previous studies found that in patients with profound hearing loss the NEO- personality factor Openness-to-experience is lowered. Objective Assuming that lowered Openness-to-experience may be due to limited access to sounds, we hypothesized that levels of Openness-to-experience would increase in these patients after cochlear implantation. Material and methods Twenty adults (mean age: 61 years; active CI users) with bilateral profound hearing loss were assessed with the NEO-Five-Factor-Inventory before cochlear implantation (pre) and five years later (post). Results No significant pre-post changes in personality were seen. Both before and five years after cochlear implantation, the sample had normal age- and gender-specific mean values on the factors Extraversion, Neuroticism, Agreeableness, and Conscientiousness (T ≈ 50), but significantly lowered mean values on Openness-to-experience (T ≈ 42, p < 0.001). Conclusions and significance Cochlear implantation apparently has no (or at best very little) effect on Openness-to-experience in profoundly hearing impaired patients. While this study demonstrates once again, that high-grade hearing loss may be associated with less openness to new experiences, the reason for this association remains unclear.

The effect of cochlear implantation on tinnitus in patients with post lingual bilateral profound hearing loss.

In this study we investigated the effect of cochlear implantation on tinnitus in patients with post lingual bilateral profound hearing loss. In this retrospective study, 66 post-lingual deaf patients over 18 years old who referred for cochlear implantation and complained of tinnitus were included. Patients were divided into two groups with residual hearing (in frequency 250-500Hz) and without residual hearing. All data including age, gender, type of implants, type of surgery, cause of the disease, and information on the THI score before cochlear implantation, one month after cochlear implantation and one year after cochlear implantation was recorded and analyzed. Nine patients had residual hearing, while 57 patients did not. After one month and one year, in both groups, the mean THI score has decreased significantly (p-value = 0.001), but there was no significant difference in the mean THI score before surgery and one month and one year after surgery between two groups with and without residual hearing. There was no significant difference in tinnitus severity according to THI-grade between two groups. Also there was no significant difference in THI score and the cause of the hearing loss, type and insertion method of implantation, one month and one year after the surgery between two groups. According to our results, the was no significant correlation between residual hearing and tinnitus score before surgery, one month and one year after the surgery.

Economic Analysis of Cochlear Implants in Adults With Bilateral Severe-to-Profound Hearing Loss. A Systematic Review of the Literature.

To determine the cost-effectiveness or cost-utility of cochlear implants (CI) in adults with severe to profound bilateral hearing loss. PubMed (Medline), The Cochrane Library, Embase via Elsevier, EBSCOhost CINAHL, and Scopus. The study included adult participants with severe to profound bilateral neurosensory hearing loss. The analysis encompassed quality of life improvements, costs, cost-effectiveness, cost-utility, cost-benefit, quality-adjusted life year (QALY), incremental cost-effectiveness ratio (ICER), and incremental cost-utility ratio (ICUR). Systematic reviews, meta-analyses, case series, and prospective or retrospective cohort studies published in English between 2010 and 2023 were retrieved. Exclusion criteria included incomplete studies, abstracts, clinical cases, editorials, letters, studies involving pediatric populations, single-side deafness, methodology research, noneconomic aspects of CI, mixed child and adult data, and studies published before 2010. The risk of bias was assessed following the criteria outlined in Appendix I of the economic evaluation's quality assessment as per the NICE Guideline Development Method. Ten articles met the criteria and were included in the qualitative synthesis. One study conducted a prospective cost-utility analyses, one carried out a cost-benefit analyses, one was a randomized controlled clinical trial focusing on cost-utility, and another was a clinical trial addressing cost-effectiveness. Six studies employed Markov models, and one study utilized uniquely the Monte Carlo method. None quantified the economic impact of improved hearing on cognitive function. The heterogeneity of sources impacted data quality. Unilateral and sequential bilateral CI appeared to be cost-effective when compared with bilateral hearing aids or nontechnological support. When properly indicated, simultaneous bilateral CIs are cost-effective compared to no interventions and to unilateral cochlear implantation through differential discounting or variations from the base cases, especially with a life expectancy of 5-10 years or longer.

Amish Infantile Epilepsy in an Indian Child

AbstractConsanguineous marriages in India continue to give rise to a wide spectrum of recessively inherited disorders that require a broader base of knowledge. A 2-year-old boy presented with global development delay, persistent vomiting, drug refractory seizures, deafness, and central hypotonia. He had profound bilateral hearing loss, barium swallow showed severe reflux but magnetic resonance imaging brain was normal, leading to a diagnostic dilemma. A normal electromyogram with nerve conduction velocity ruled out disorders of muscle and nerve. Whole-exome sequencing showed salt and pepper development regression syndrome but phenotypically, he did not have the classic skin changes. He has shown mild improvement in cognition, mobility, and weight gain with citicoline, antireflux medications, antiseizure medications, and a protein diet. Accurate diagnosis based on cohort of symptoms and appropriate early intervention can help improve the quality of life in such children.

Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss

Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Objective and Subjective Measures of Spatial Hearing in Unilateral Cochlear Implant Users with Bilateral Profound Hearing Loss

Purpose: The ability to benefit from spatial separation between target and masker signals is important in multi-sound source listening environments. The goal of this study was to measure the spatial release from masking (SRM) in unilateral cochlear implant (CI) users with bilateral profound hearing loss. We also determined the relationships between the SRMs and the self-reported spatial hearing abilities.Methods: Fourteen unilateral CI users with bilateral profound hearing loss participated in this study. The target sentence was always presented to the front of the listener, and the nonfluctuating speech-shaped noise (SSN) or fluctuating speech noise was either co-located with the target (speech at 0°, noise at 0°, S0N0) or spatially separated at ± 90°. The SRM was quantified as the difference between speech recognition thresholds (SRTs) in the co-located and spatially separated conditions. The self-reported spatial hearing abilities were also measured using validated subjective questionnaires.Results: Overall, the SRTs were lower (better) with SSN than with fluctuating speech noise. When the noise was presented to the non-CI ear (speech at 0°, noise at non-CI ear, S0Nnonci), speech-in-noise recognition was the greatest due to head shadow or better-ear listening effect, resulting in the SRMs of approximately 5~6 dB regardless of noise type. When the noise was given to the CI ear (speech at 0°, noise at CI ear, S0Nci), some individuals exhibited positive SRMs (3~8 dB), while others showed negative SRMs, leading to little SRMs overall. When the SSN was given, subjects with less SRMs (less spatial separation benefits on the objective test) reported greater subjective spatial hearing difficulties.Conclusion: The spatial hearing of unilateral CI users varied by the position of the sound source. Listeners' spatial hearing abilities, which are unpredictable from clinical routine tests, need to be assessed by either objective or subjective measures.

Late-onset of Labyrinthine Dysfunction in Chronic Brucella-induced Encephalitis

Brucellosis, caused by Brucella, dominates in the Middle East. Acute or chronic brucellosis can involve any organ system, and the involvement of the central nervous system leads to encephalitis, called neurobrucellosis (NB). We report a rare case from north India where a variant of Brucella caused NB led to progressive labyrinthine dysfunction; who developed vertigo, disequilibrium, and gradual progressive sensorineural (SN) hearing loss. Audiological tests pure-tone audiometry (PTA), otoacoustic emissions (OAE), stapedial muscle reflexes, auditory evoked brainstem responses (ABR), and vestibular evoked myogenic potentials (VEMPs) were conducted on the patients. PTA showed a bilateral profound SN hearing loss, an absent OAE, and ABR suggestive of cochlear dysfunction. The cervical and ocular VEMP showed delayed n1 and p1 latencies, suggesting superior and inferior nerve branch involvement. Vertigo or disequilibrium and SN hearing loss can be the late-onset manifestations of labyrinthine dysfunction in chronic Brucella-induced encephalitis.

Screening of New-Born through Otoacoustic Emission Device in a Rural Setting for Early Detection of Hearing Impairment - An Observational Study

BACKGROUND&#x0D; Hearing is a special sense needed for proper speech, language, mental development and academic performance. The objectives of our study were to screen neonates at rural area with otoacoustic emission device for detecting hearing impairment and develop a comprehensive rehabilitation model for hearing impaired children in rural areas. Hearing loss initial signs are subtle and therefore for early detection routine neonatal hearing screening is the most effective tool. At first otoacoustic emissions are done, failing which neonates undergo BERA as final screening method to identify hearing impairment as a confirmatory test. In a country like India where neonatal hearing tests are not a part of the routine screening program, the chances of missing early detection and possible rehabilitation are large. In this context, the current project envisages to develop a practical and pragmatic screening model for detecting early neonatal hearing impairment using otoacoustic emission technique in a rural sector reaching every door step possible.&#x0D; METHODS&#x0D; From the government health records collected for a duration of one and half years, all babies delivered in PHC were included for the screening. The babies were screened every Thursday (Immunisation day) with OAE devices by an audiologist at the PHC. The total number of infants screened were 1737. The first screening test done was otoacoustic emission, failing which patient was referred for BERA as a confirmatory test.&#x0D; RESULTS&#x0D; The total number of babies screened by OAE in our study was 1737. Out of the total infants screened, 1st OAE referrals were 327 babies. All the 327 infants were screened by OAE for the 2nd time, out of which 10 infants were referred for the 2nd consecutive time. 317 infants were passed on 2nd OAE screening. All the 10 infants who were referred for the 2nd time were subjected to BERA at Ramaiah medical Hospital. Out of these 10 infants, BERA showed severe to profound bilateral hearing loss in 7 infants (Confirmed Hearing loss). Other 3 infants had normal BERA reading hence normal hearing. The statistics showed that the overall rate of hearing loss prevalence was 4.03 per thousand.&#x0D; CONCLUSIONS&#x0D; In order to diagnose hearing loss in neonates, it is critical to identify hearing loss at birth and provide a screening test for it. OAE screening of neonates should be done at the earliest to detect hearing impairment so that hearing and speech rehabilitation can be started at the earliest. It should be done at each &amp; every health care centre on a large-scale basis to cover all the babies.

Cochlear implant and tinnitus

Cochlear implant (CI) treatment is now established as asuccessful standard of care for auditory rehabilitation of profoundly deaf or severe hearing loss patients. CI candidates with tinnitus also benefit from improved health-related quality of life (HRQoL) and tinnitus burden. Current CI indications include bilateral (double-sided) profound hearing loss and deafness (DSD), unilateral (single-sided) deafness (SSD), and asymmetric hearing loss (AHL). The new and expanded indications for cochlear implants result in different healthcare situations, which may also be associated with differences in tinnitus burden before and after CI treatment. In this article, we discuss the prevalence of tinnitus in different patient groups and the influence of CI on tinnitus prevalence and severity in these groups. In addition, further therapeutic options for tinnitus suppression based on the CI principle are presented, including the development of an anti-tinnitus implant (proof of concept).

Pilot Implementation of a Community-based, eHealth-enabled Service Delivery Model for Newborn Hearing Screening and Intervention in the Philippines.

This study explores the potential of the HeLe Service Delivery Model, a community-based newborn hearing screening (NHS) program supported by a web-based referral system, in improving provision of hearing care services. This prospective observational study evaluated the HeLe Service Delivery Model based on records review and user perspectives. We collected system usage logs from July to October 2018 and data on patient outcomes. Semi-structured interviews and review of field reports were conducted to identify implementation challenges and facilitating factors. Descriptive statistics and content analysis were used to analyze quantitative and qualitative data, respectively. Six hundred ninety-two (692) babies were screened: 110 in the RHUs and 582 in the Category A NHS hospital. Mean age at screening was 1.4±1.05 months for those screened in the RHU and 0.46±0.74 month for those in the Category A site. 47.3% of babies screened at the RHU were ≤1 month old in contrast to 86.6% in the Category A hospital. A total of 10 babies (1.4%) received a positive NHS result. Eight of these ten patients were referred via the NHS Appointment and Referral System; seven were confirmed to have bilateral profound hearing loss, while one patient missed his confirmatory testing appointment. The average wait time between screening and confirmatory testing was 17.1±14.5 days. Facilitating factors for NHS implementation include the presence of champions, early technology adopters, legislations, and capacity-building programs. Challenges identified include perceived inconvenience in using information systems, cost concerns for the patients, costly hearing screening equipment, and unstable internet connectivity. The lack of nearby facilities providing NHS diagnostic and intervention services remains a major block in ensuring early diagnosis and management of hearing loss in the community. The eHealth-enabled HeLe Service Delivery Model for NHS is promising. It addresses the challenges and needs of community-based NHS by establishing a healthcare provider network for NHS in the locale, providing a capacity-building program to train NHS screeners, and deploying health information systems that allows for documentation, web-based referral and tracking of NHS patients. The model has the potential to be implemented on a larger scale - a deliberate step towards universal hearing health for all Filipinos.

Cochlear Implants for Children with Unilateral Hearing Loss: Debunking the Myths

Cochlear implants (CIs) have revolutionized the field of audiology, providing a life-changing solution for children with bilateral profound hearing loss. Expanding criteria has allowed children with significant unilateral hearing loss (UHL) to benefit from this technology as well. The practice is not without controversy, however. While we have known since 1984 that children with UHL face educational challenges (Bess &amp; Tharpe, 1984, 1986), watchful waiting until a child is old enough to be fitted with a Contralateral Routing of Signal (CROS) device has been the status quo (Bagatto, 2020; Bagatto et al., 2019). While research has continued to establish the benefits of cochlear implantation in this population, it has yet to become the standard of care. As is inherent in controversial practices, several myths and misconceptions surrounding the use of CIs for children with UHL pervade clinical hearing care systems. Here, we will debunk these myths by examining the evidence-based benefits and considerations associated with cochlear implantation in children with UHL. Through an analysis of current research and clinical evidence, we will highlight the potential advantages of CIs as a viable treatment option, promoting informed decision-making and improving the quality of life for children with UHL.

Congenital Cytomegalovirus and Hearing Loss: The State of the Art.

In developed countries, congenital cytomegalovirus (cCMV) infection is the most common congenital viral infection, representing the leading non-genetic cause of sensorineural hearing loss (HL). Diagnosis of cCMV infection can be performed by detection of CMV DNA in urine or saliva within 2-3 weeks after birth, or later in dried blood samples on the Guthrie card. Currently, there are many controversies regarding the preventive, diagnostic, and therapeutic approaches to cCMV infection. HL secondary to cCMV is highly variable in onset, side, degree, audiometric configuration, and threshold changes over time. Therefore, it is of paramount importance to perform a long and thorough audiological follow-up in children with cCMV infection to ensure early identification and prompt treatment of progressive and/or late-onset HL. Early cochlear implantation appears to be a valid solution not only for children with bilateral profound HL, but also for those with single-sided deafness, improving localization ability and understanding speech in noisy environments. Moreover, the decision to apply a unilateral cochlear implant in children with cCMV is strengthened by the non-negligible possibility of hearing deterioration of the contralateral ear over time.

Age and Incidence of Cochlear Implantation in the Pediatric Population With Congenital Bilateral Profound Hearing Loss.

The current study characterizes age and incidence of cochlear implantation among qualifying children with congenital bilateral profound hearing loss in the U.S. Deidentified cochlear implantation data were acquired from prospectively collected patient registries from two cochlear implant (CI) manufacturers (Cochlear Americas and Advanced Bionics). Children <36 months old were assumed to have congenital bilateral profound sensorineural hearing loss. U.S. CI centers. Children <36 months old who received CIs. Cochlear implantation. Age at implantation and incidence. A total of 4,236 children <36 months old underwent cochlear implantation from 2015 to 2019. The median age at implantation was 16 months (interquartile range, 12-24 mo) and did not change significantly during the 5-year study period ( p = 0.09). Patients residing closer to CI centers ( p = 0.03) and treated at higher-volume centers ( p = 0.008) underwent implantation at a younger age. Bilateral simultaneous implantation increased from 38% to 53% of CI surgeries in 2015 and 2019, respectively. Children who received bilateral simultaneous CIs were younger compared with those receiving unilateral or bilateral sequential CIs (median, 14 versus 18 mo; p < 0.001). The incidence of cochlear implantation increased from 7,648 per 100,000 person-years in 2015 to 9,344 in 2019 ( p < 0.001). Although the incidence of pediatric CI recipients and the frequency of bilateral simultaneous implantation increased over the study period, age at implantation did not change significantly and far exceeded current Food and Drug Administration (9 mo) and American Academy of Otolaryngology and Head and Neck Surgery position statement (6-12 mo) guidelines.

Bilateral simultaneous cochlear implantation is a safe method of hearing rehabilitation in adults.

Bilateral cochlear implantation is an effective treatment for patients with bilateral profound hearing loss. In contrast to children, adults mostly choose a sequential surgery. This study addresses whether simultaneous bilateral CI is associated with higher rates of complications compared to sequential implantation. 169 bilateral CI surgeries were analyzed retrospectively. 34 of the patients were implanted simultaneously (group 1), whereas 135 patients were implanted sequentially (group 2). The duration of surgery, the incidence of minor and major complications and the duration of hospitalization of both groups were compared. In group 1, the total operating room time was significantly shorter. The incidences of minor and major surgical complications showed no statistically significant differences. A fatal non-surgical complication in group 1 was particularly extensively reappraised without evidence of a causal relationship to the chosen mode of care. The duration of hospitalization was 0.7days longer than in unilateral implantation but 2.8days shorter than the combined two hospital stays in group 2. In the synopsis of all considered complications and complication-relevant factors, equivalence of simultaneous and sequential cochlear implantation in adults in terms of safety was found. However, potential side effects related to longer surgical time in simultaneous surgery must be considered individually. Careful patient selection with special consideration to existing comorbidities and preoperative anesthesiologic evaluation is essential.

Hearing rehabilitation in postmeningitis deafness in the context of the time factor

Postmeningitis deafness appears in 0-11% of the meningitis cases. Cochlear ossification can develop in these patients, which may make the hearing rehabilitation impossible with cochlear implantation. Due to ossification, it is critical to refer patients to the implant centre without any delay. The aim of this study was to examine the time factor between the appearance of deafness and the first examination in a cochlear implant centre, the possibilities and effectivity of hearing rehabilitation. In our tertial referral centre, postmeningitis deafened patients were examined between 2014 and 2022 retrospectively. Hearing results, imaging, possibilities of rehabilitation, complications of cochlear implantations and the hearing results were investigated. 8 patients (3 children, 5 adults) were investigated. The time between the start of deafness and the first appearance varied between 3 weeks to 9 years. Bilateral profound hearing loss was measured in all patients. In 6 cases, cochlear ossification was observed (4 patients bilateral). Cochlear implantation was conducted in 5 patients (4 bilateral, 1 unilateral). In 3 cases, implantation was impossible due to severe ossification. Hearing results showed good hearing levels with poor speech perception in all cases. The rehabilitation of severe hearing loss caused by meningitis can present many challenges to clinicians. A critical point in the care is the urgent referral of patients to a cochlear implantation centre as soon as possible after the life-threatening condition has passed. The implementation of further diagnostic and the earliest possible implantation is the responsibility of the implantation centre itself. It is recommended to develop a new protocol with the involvement of allied professions to clear patient pathways for an effective treatment strategy. Orv Hetil. 2023; 164(19): 729-738.

Enterovirus characterized from cerebrospinal fluid in a cohort from the Eastern United States.

Enteroviruses (EVs) are predominant causes of a spectrum of neurological diseases. To better understand the origins of the outbreaks of disease associated with EV, it is essential to develop an efficient surveillance system that identifies the circulating EVs and correlate their genomic evolution with the disease presentations. The clinical presentations of patients with positive EV from cerebrospinal fluid (CSF) between 2014 and 2022, diagnosed at the Johns Hopkins Medical Microbiology Laboratory, were compared from year to year. EV typing and whole genome sequencing were performed and correlated to the spectrum of disease. A total of 95 CSF specimens were positive for EV between 2014 and 2022. The percentage positivity ranged from the lowest of 1.1% in 2020 to the highest of 3.2% in 2015. The median ages declined from 22 years in 2014 to less than one year starting in 2016 to 34 in 2022. Typing using VP1 sequencing revealed that E30 and E6 were associated with meningitis in adults but coxsackieviruses (CVs-B3 and B5) were detected from pediatric patients with fever. Whole genome sequencing revealed multiple recombination events. In 2020, a recombinant CV-A9 was detected in a CSF sample associated with unusual presentation of sepsis, profound acute bilateral sensory neural hearing loss, and myofasciitis. EV genomic surveillance is needed for a better understanding of the genetic determinants of neurovirulence. Whole genome sequencing can reveal recombination events missed by traditional molecular surveillance methods.

The Effect of Universal Newborn Hearing Screening on Spoken Language after Cochlear Implantation.

The universal newborn hearing screening (UNHS) was fully implemented across New Zealand by 2010 to improve outcomes for children with prelingual deafness. A previous audit undertaken by our center, the Southern Cochlear Implant Programme (SCIP), demonstrated that UNHS has significantly reduced the time to referral and surgery for cochlear implants in these children. This study aims to evaluate the relationship between earlier implantation and language development, the time taken to achieve age-appropriate language, and the effect of socioeconomic status on language skills. This is a retrospective cohort study comparing prelingual children with severe to profound bilateral hearing loss who underwent cochlear implantation in SCIP before and after the introduction of the UNHS. The language outcomes were assessed using the Preschool Language Scale and/or the Peabody Picture Vocabulary Test. For the purpose of our study, the standard scores of these tests were expressed as global language scores (GLS). GLSs between 85 and 115 are considered within normal range for age. The socioeconomic status was categorized based on the New Zealand Index of Deprivation (NZDep). Children in the post-UNHS group (46/95 children) were referred to SCIP and received CI at a significantly earlier age (mean = 7 vs 20 mo, p = 8.95E-10, and mean = 13 vs. 24.7 mo, p = 1.43E-07). At 2 years postimplantation, the GLS was significantly higher in the post-UNHS group (mean scores = 93.3 vs. 79.1, p = 0.0213). The scores remained statistically higher in the post-UNHS group when assessed at 3 and 4 years postimplantation. At 2, 3, and 4 years postimplantation, there is a significant linear decrease in GLS with increasing age at cochlear implantation. We found no correlation between NZDep and GLS. Children identified through UNHS have the advantage of earlier diagnosis, earlier hearing intervention, and longer duration with the implants, and they can achieve age-appropriate spoken language after 2 years of implantation.

Modified T2 Statistics for Improved Detection of Aided Cortical Auditory Evoked Potentials in Hearing-Impaired Infants.

The cortical auditory evoked potential (CAEP) is a change in neural activity in response to sound, and is of interest for audiological assessment of infants, especially those who use hearing aids. Within this population, CAEP waveforms are known to vary substantially across individuals, which makes detecting the CAEP through visual inspection a challenging task. It also means that some of the best automated CAEP detection methods used in adults are probably not suitable for this population. This study therefore evaluates and optimizes the performance of new and existing methods for aided (i.e., the stimuli are presented through subjects' hearing aid(s)) CAEP detection in infants with hearing loss. Methods include the conventional Hotellings T2 test, various modified q-sample statistics, and two novel variants of T2 statistics, which were designed to exploit the correlation structure underlying the data. Various additional methods from the literature were also evaluated, including the previously best-performing methods for adult CAEP detection. Data for the assessment consisted of aided CAEPs recorded from 59 infant hearing aid users with mild to profound bilateral hearing loss, and simulated signals. The highest test sensitivities were observed for the modified T2 statistics, followed by the modified q-sample statistics, and lastly by the conventional Hotelling's T2 test, which showed low detection rates for ensemble sizes <80 epochs. The high test sensitivities at small ensemble sizes observed for the modified T2 and q-sample statistics are especially relevant for infant testing, as the time available for data collection tends to be limited in this population.

Phylogenetic analysis of congenital rubella virus from Indonesia: a case report

BackgroundRubella is a common inherited infection resulting in congenital cataracts and a significant cause of permanent vision loss in developing countries. In 2016, Indonesia had the highest number of congenital rubella syndrome (CRS) cases globally. Here, we report the first genotype of the rubella virus extracted from the eye lens from a child with congenital cataracts due to CRS.Case presentationA female neonate was delivered by an elective caesarean delivery with normal birth weight at term from a 24-year-old mother in the rural setting. The baby presented with bilateral congenital cataracts, small-moderate secundum atrial septal defect, severe supravalvular pulmonary stenosis, and profound bilateral hearing loss. She also had microcephaly and splenomegaly. The patient's serology showed persistent positive IgG for rubella virus at the age of four years and four months. Following extraction during cataract surgery, viral detection of the lenses identified the presence of rubella. Phylogenetic analysis confirmed that the virus was grouped into genotype 1E.ConclusionsOur study reports the first phylogenetic analysis of the rubella virus extracted from the eye lens of a child with CRS in Indonesia. The detection of the rubella virus from eye lenses is remarkably promising. Our findings also emphasize the importance of molecular epidemiology in tracking the origin of rubella infection toward achieving virus eradication.