Bilateral Profound Hearing Loss Research Articles

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

Objectives. 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.Methods. 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.Results. 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 technologyadopters, 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.Conclusion. 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.&#x0D;

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.

Ambulatory Phonation Monitoring in Prelingual and Postlingual Deaf Patients after Cochlear Implantation

Introduction: Hearing loss is known to play a fundamental role in voice production due to a lack of auditory feedback. In this study, we evaluated both fundamental frequency (F₀) and loudness of voice on adult deaf patients subjected to cochlear implantation, and we analyzed these results according to the prelingual or postlingual onset of the deafness. Methods: The study population, balanced in terms of sex, consisted of 32 adults who had undergone cochlear implantation due to severe or profound bilateral hearing loss (16 with prelingual deafness and 16 with postlingual deafness) and their outcomes were compared with a control group of 32 normal hearing (NH) subjects. All subjects were asked to utter the sustained vowel /a/ for at least 5 s and then to read an Italian phonetically balanced text. Voice recordings were performed by means of an ambulatory phonation monitoring (APM 3200). Measurements were performed without cochlear implant (CI), then with CI switched on, both in quiet condition and with background noise. Results: Compared to NH subjects, deaf individuals were overall characterized by higher F₀ and loudness values, especially in the vowel task than the reading. In the sustained vowel task, no patients demonstrated significant voice changes after switching on the CI; contrarily, in the reading task, the use of the CI reduced both loudness and F₀ up to values comparable to NH subjects, although only in males. There was no significant difference in speech parameters between prelingual and postlingual deafness, although overall lower values were evident in case of postlingual deafness. The use of the CI showed a significant reduction of F₀ in males with postlingual deafness and of loudness, both for patients with prelingual and postlingual deafness. Finally, there was a positive correlation between postoperative hearing thresholds and overall speech loudness, highlighting how subjects with better hearing outcomes after CI positioning generally speak with a lower loudness and therefore a reduced vocal effort and load. Discussion/Conclusion: We found similar speech performances between prelingual and postlingual deafness, both in the vowel /a/ phonation and in the reading, providing a further suggestion that prelingual adult patients may benefit from cochlear implantation in phonation as well, in addition to the known excellent hearing outcomes. Overall, these results highlight the ability of the CI to adjust in everyday speech certain phonatory aspects such as F₀ and loudness by restoring the auditory feedback.

The Effect of Age, Type of Noise, and Cochlear Implants on Adaptive Sentence-in-Noise Task.

Adaptive tests of sentences in noise mimic the challenge of daily listening situations. The aims of the present study were to validate an adaptive version of the HeBio sentence test on normal hearing (NH) adults; to evaluate the effect of age and type of noise on speech reception threshold in noise (SRTn); and to test it on prelingual adults with cochlear implants (CI). In Experiment 1, 45 NH young adults listened to two lists accompanied by four-talker babble noise (4TBN). Experiment 2 presented the sentences amidst 4TBN or speech-shaped noise (SSN) to 80 participants in four age groups. In Experiment 3, 18 CI adult users with prelingual bilateral profound hearing loss performed the test amidst SSN, along with HeBio sentences and monosyllabic words in quiet and forward digits span. The main findings were as follows: SRTn for NH participants was normally distributed and had high test–retest reliability; SRTn was lower among adolescents and young adults than middle-aged and older adults, and were better for SSN than 4TBN; SRTn for CI users was higher and more variant than for NH and correlated with speech perception tests in quiet, digits span, and age at first CI. This suggests that the adaptive HeBio can be implemented in clinical and research settings with various populations.

Parents’ experiences of remote microphone systems for children with hearing loss

Purpose Remote microphone systems improve intelligibility in difficult conditions when the performance of hearing aids/cochlear implants is insufficient. The purpose of this study was to explore parents’ experiences with remote microphone systems for their children with hearing loss and to determine the advantages and disadvantages as perceived by parents. Materials and methods Data were collected by means of semi-structured interviews with parents of children with moderate to profound bilateral hearing loss (2–19 years old). The open coding method and thematic analysis were used. The final sample consisted of 19 mothers and 9 fathers who had experience with remote microphone systems. Results Parents listed the advantages of remote microphone systems for their child, for themselves and for other carers, such as better hearing and understanding, a life more similar to that of their peers without hearing loss, safety in road traffic, lower fatigue, vocabulary acquisition, better school results. Some limitations were identified, namely low benefits, technical issues and a reluctance to use the device by children or teachers. Conclusions It is crucial to provide parents with information about assistive devices and the consequences of limited access to hearing speech. Professionals should motivate parents, children and teachers to use remote microphone systems even in situations when the benefit may not be obvious if there is a potential benefit for the child. IMPLICATIONS FOR REHABILITATION The main advantages of remote microphone systems perceived by parents are better hearing and understanding, a life more similar to peers without hearing loss, safety in traffic, lower fatigue, vocabulary acquisition and better school results. The main disadvantages are for parents: persisting hearing problems, low benefits, technical issues and a reluctance to use the device by children or teachers. Sufficient and accurate information is needed about remote microphone systems, but also about the consequences of hearing loss in general. The possibility to try wireless devices before buying and troubleshooting help is appreciated.

Cochlear implants evaluation for congenital rubella syndrome patients in Dr. Soetomo Academic Medical Center

Congenital rubella syndrome (CRS) is an infection caused by rubella virus that transmitted to the fetus during pregnancy which can cause congenital hearing loss. Cochlear implant is an effective therapy in children with severe to profound bilateral hearing loss. Categories of Auditory Performance (CAP) method is a receptive scale assessment of auditory stimulation that can be applied to all children regardless of age, before and after cochlear implant. Evaluating the results of cochlear implants using CAP-II in patients with CRS. A retrospective descriptive study were conducted to samples taken from medical records of CRS patients who had cochlear implants in 2011-2018 at ORL-HNS department of Dr. Soetomo Academic Medical Center. Evaluation of CAP-II results was performed 6 months, 12 months and 24 months after cochlear implant. Eleven CRS patient who underwent cochlear implant during 2011-2018. Six months after cochlear implant showed the highest scale was 6. At 12-months evaluation showed the highest scale was 7, while at 24-months evaluation showed the highest scale was 8. Cochlear implants provide good auditory performance based on CAP-II scale in sensorineural hearing loss of CRS patients.

Younger Age at Cochlear Implant Activation Results in Improved Auditory Skill Development for Children With Congenital Deafness.

The U.S. Food and Drug Administration indications for cochlear implantation in children is currently 9 months of age and older for children with bilateral profound sensorineural hearing loss (SNHL). Studies have shown that earlier activation of a cochlear implant (CI) can lead to better spoken language outcomes. As auditory skills are a precursor to the development of spoken language, this study was developed to investigate the influence of age at CI activation on auditory skill acquisition in young children. A secondary aim was to describe the auditory skills of children implanted prior to 9 months of age as compared to children with older ages of activation. Functional Listening Index (FLI) scores obtained during routine clinical visits were reviewed for 78 pediatric CI recipients with congenital bilateral profound hearing loss who were activated before 2 years of age. A linear mixed-effects model assessed the effect of age at CI activation on cumulative FLI scores over time. There was a significant interaction between age at activation and chronological age at the time of evaluation, indicating that children with earlier access to sound achieved a greater number of auditory skills than those with later CI activations when measured at the same chronological age. Children activated before the age of 9 months approximated scores expected of children with typical hearing, whereas children activated between 9 and 24 months of age did not. Younger age at CI activation is associated with increased auditory skills over time. Children who undergo cochlear implantation and CI activation before 9 months achieve more auditory skills by 4 years of age than children who are activated at later ages. These data suggest that reducing the approved age at cochlear implantation for children with congenital bilateral profound SNHL may support optimal auditory skill acquisition.