Impact of Hearing Aids on Language Outcomes in Preschool Children With Mild Bilateral Hearing Loss.

This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3-5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.

Sound source localization patterns and bilateral cochlear implants: Age at onset of deafness effects.

The ability to determine a sound's location is critical in everyday life. However, sound source localization is severely compromised for patients with hearing loss who receive bilateral cochlear implants (BiCIs). Several patient factors relate to poorer performance in listeners with BiCIs, associated with auditory deprivation, experience, and age. Critically, characteristic errors are made by patients with BiCIs (e.g., medial responses at lateral target locations), and the relationship between patient factors and the type of errors made by patients has seldom been investigated across individuals. In the present study, several different types of analysis were used to understand localization errors and their relationship with patient-dependent factors (selected based on their robustness of prediction). Binaural hearing experience is required for developing accurate localization skills, auditory deprivation is associated with degradation of the auditory periphery, and aging leads to poorer temporal resolution. Therefore, it was hypothesized that earlier onsets of deafness would be associated with poorer localization acuity and longer periods without BiCI stimulation or older age would lead to greater amounts of variability in localization responses. A novel machine learning approach was introduced to characterize the types of errors made by listeners with BiCIs, making them simple to interpret and generalizable to everyday experience. Sound localization performance was measured in 48 listeners with BiCIs using pink noise trains presented in free-field. Our results suggest that older age at testing and earlier onset of deafness are associated with greater average error, particularly for sound sources near the center of the head, consistent with previous research. The machine learning analysis revealed that variability of localization responses tended to be greater for individuals with earlier compared to later onsets of deafness. These results suggest that early bilateral hearing is essential for best sound source localization outcomes in listeners with BiCIs.

Intravenous methadone causes acute toxic and delayed inflammatory encephalopathy with persistent neurocognitive impairments.

BACKGROUND: The mu-opioid agonist methadone is administered orally and used in opioid detoxification and in the treatment of moderate-to-severe pain. Acute oral methadone-use and -abuse have been associated with inflammatory and toxic central nervous system (CNS) damage in some cases and cognitive deficits can develop in long-term methadone users. In contrast, reports of intravenous methadone adverse effects are rare. CASE PRESENTATION: Here, we report a patient who developed acute bilateral hearing loss, ataxia and paraparesis subsequently to intravenous methadone-abuse. While the patient gradually recovered from these deficits, widespread magnetic resonance imaging changes progressed and delayed-onset encephalopathy with signs of cortical dysfunction persisted. This was associated with changes in the composition of monocyte and natural killer cell subsets in the cerebrospinal fluid. CONCLUSION: This case suggests a potential bi-phasic primary toxic and secondary inflammatory CNS damage induced by intravenous methadone.

Individual Differences in Speech Recognition Changes After Cochlear Implantation.

Importance: Cochlear implantation is highly effective at improving hearing outcomes, but results have been limited to groupwise analysis. That is, limited data are available for individual patients that report comparisons of preoperative aided speech recognition and postimplantation speech recognition. Objective: To assess changes in preoperative aided vs postoperative speech recognition scores for individual patients receiving cochlear implants when considering the measurement error for each speech recognition test. Design, Setting, and Participants: This cross-sectional study used a prospectively maintained database of patients who received cochlear implants between January 1, 2012, and December 31, 2017, at a tertiary, university-based referral center. Adults with bilateral sensorineural hearing loss undergoing cochlear implantation with 6- or 12-month postoperative measures using 1 or more speech recognition tests were studied. Exposures: Cochlear implantation. Main Outcomes and Measures: Postoperative word recognition (consonant-nucleus-consonant word test), sentence recognition (AzBio sentences in quiet), and sentence recognition in noise (AzBio sentences in +10-dB signal-to-noise ratio) scores, and association of each speech recognition score change with aided preoperative score to each test's measurement error. Results: Analysis of data from a total of 470 implants from 323 patients included 253 male (53.8%) patients; the mean (SD) age was 61.2 (18.3) years. Most patients had statistically significant improvement in all speech recognition tests postoperatively beyond measurement error, including 262 (84.8%) for word recognition, 226 (87.6%) for sentence recognition, and 33 (78.6%) for sentence recognition in noise. A small number of patients had equivalent preoperative and postoperative scores, including 45 (14.5%) for word recognition, 28 (10.9%) for sentence recognition, and 9 (21.4%) for sentence recognition in noise. Four patients (1.6%) had significantly poorer scores in sentence recognition after implantation. The associations between age at implantation and change in speech recognition scores were -0.12 (95% CI, -0.23 to -0.01) for word recognition, -0.22 (95% CI, -0.34 to -0.10) for sentence recognition, and -0.10 (95% CI, -0.39 to 0.21) for sentence recognition in noise. Patients with no significant improvement were similarly distributed between all preoperative aided speech scores for word recognition (range, 0%-58%) and sentence recognition (range, 0%-56%) testing. Conclusions and Relevance: In this cross-sectional study, with respect to preoperative aided speech recognition, postoperative cochlear implant outcomes for individual patients were largely encouraging. However, improvements in scores for individual patients remained highly variable, which may not be adequately represented in groupwise analyses and reporting of mean scores. Presenting individual patient data from a large sample of individuals with cochlear implants provides a better understanding of individual differences in speech recognition outcomes and contributes to more complete interpretations of successful outcomes after cochlear implantation.

The Influence of Forced Social Isolation on the Auditory Ecology and Psychosocial Functions of Listeners With Cochlear Implants During COVID-19 Mitigation Efforts.

OBJECTIVES: The impact of social distancing on communication and psychosocial variables among individuals with hearing impairment during COVID-19 pandemic. It was our concern that patients who already found themselves socially isolated (Wie et al. 2010) as a result of their hearing loss would be perhaps more susceptible to changes in their communication habits resulting in further social isolation, anxiety, and depression. We wanted to better understand how forced social isolation (as part of COVID-19 mitigation) effected a group of individuals with hearing impairment from an auditory ecology and psychosocial perspective. We hypothesized that the listening environments would be different as a result of social isolation when comparing subject's responses regarding activities and participation before COVID-19 and during the COVID-19 pandemic. This change would lead to an increase in experienced and perceived social isolation, anxiety, and depression. DESIGN: A total of 48 adults with at least 12 months of cochlear implant (CI) experience reported their listening contexts and experiences pre-COVID and during-COVID using Ecological Momentary Assessment (EMA; methodology collecting a respondent's self-reports in their natural environments) through a smartphone-based app, and six paper and pencil questionnaires. The Smartphone app and paper-pencil questionnaires address topics related to their listening environment, social isolation, depression, anxiety, lifestyle and demand, loneliness, and satisfaction with amplification. Data from these two-time points were compared to better understand the effects of social distancing on the CI recipients' communication abilities. RESULTS: EMA demonstrated that during-COVID CI recipients were more likely to stay home or be outdoors. CI recipients reported that they were less likely to stay indoors outside of their home relative to the pre-COVID condition. Social distancing also had a significant effect on the overall signal-to-noise ratio of the environments indicating that the listening environments had better signal-to-noise ratios. CI recipients also reported better speech understanding, less listening effort, less activity limitation due to hearing loss, less social isolation due to hearing loss, and less anxiety due to hearing loss. Retrospective questionnaires indicated that social distancing had a significant effect on the social network size, participant's personal image of themselves, and overall loneliness. CONCLUSIONS: Overall, EMA provided us with a glimpse of the effect that forced social isolation has had on the listening environments and psychosocial perspectives of a select number of CI listeners. CI participants in this study reported that they were spending more time at home in a quieter environments during-COVID. Contrary to our hypothesis, CI recipients overall felt less socially isolated and reported less anxiety resulting from their hearing difficulties during-COVID in comparison to pre-COVID. This, perhaps, implies that having a more controlled environment with fewer speakers provided a more relaxing listening experience.

Scalar Translocation Comparison Between Lateral Wall and Perimodiolar Cochlear Implant Arrays - A Meta-Analysis.

OBJECTIVES/HYPOTHESIS: Two types of electrode arrays for cochlear implants (CIs) are distinguished: lateral wall and perimodiolar. Scalar translocation of the array can lead to intracochlear trauma by penetrating from the scala tympani into the scala vestibuli or scala media, potentially negatively affecting hearing performance of CI users. This systematic review compares the lateral wall and perimodiolar arrays with respect to scalar translocation. STUDY DESIGN: Systematic review. METHODS: PubMed, Embase, and Cochrane databases were reviewed for studies published within the last 11 years. No other limitations were set. All studies with original data that evaluated the occurrence of scalar translocation or tip fold-over (TF) with postoperative computed tomography (CT) following primary cochlear implantation in bilateral sensorineuronal hearing loss patients were considered to be eligible. Data were extracted independently by two reviewers. RESULTS: We included 33 studies, of which none were randomized controlled trials. Meta-analysis of five cohort studies comparing scalar translocation between lateral wall and perimodiolar arrays showed that lateral wall arrays have significantly lower translocation rates (7% vs. 43%; pooled odds ratio = 0.12). Translocation was negatively associated with speech perception scores (weighted mean 41% vs. 55%). Tip fold-over of the array was more frequent with perimodiolar arrays (X2  = 6.8, P < .01). CONCLUSIONS: Scalar translocation and tip fold-overs occurred more frequently with perimodiolar arrays than with lateral wall arrays. In addition, translocation of the array negatively affects hearing with the cochlear implant. Therefore, if one aims to minimize clinically relevant intracochlear trauma, lateral wall arrays would be the preferred option for cochlear implantation. Laryngoscope, 131:1358-1368, 2021.

Changes in Tinnitus by Cochlear Implantation: A Parametric Study of the Effect of Single-Electrode Stimulation.

INTRODUCTION: While cochlear implantation may have a positive effect on tinnitus, it is not effective in reducing tinnitus in all patients. This may be due to different patients requiring different strategies of electrical stimulation in order to obtain a positive effect on tinnitus. It is, therefore, important to identify the most effective stimulation strategies to reduce tinnitus. The simplest possible strategy is stimulation by only one electrode. In this study, we investigated tinnitus suppression by electrical stimulation via a single electrode of the cochlear implant. METHODS: We performed a listening experiment in 19 adult participants, who had received a unilateral cochlear implant (CI) because of severe bilateral hearing loss. All of these patients had indicated that they suffered from tinnitus. During a 300-s interval, patients listened to blocks of single-electrode stimulation and rated the loudness of the stimulus and any effects on their tinnitus. The 300-s interval included a block of single-electrode stimulation (duration 120 s). In consecutive intervals, the stimulus differed in its cochlear location (basal or apical), its pulse rate (720 or 725 Hz, 1,200 Hz, and 2,400 or 2,320 Hz), and amplitude (just above threshold or equivalent to moderate loudness). Thus, 2 × 3 × 2 = 12 stimulus conditions were tested in each participant, and each condition was presented only once. During the experiment, the participants promptly rated the loudness of the stimuli and the loudness of their tinnitus on a Visual Analogue Scale (10-point VAS). RESULTS: Significantly more tinnitus reduction was observed with stimuli at a moderate intensity level (30%) compared to stimuli at near-threshold level (18%) (χ2 [1, N = 222] = 14.115, p < 0.01). No significant differences in tinnitus levels resulted from the different pulse rates and stimulation sites. Eight participants reported an increase of tinnitus loudness under at least one stimulus condition. Changes in tinnitus loudness were generally minor, and never exceeded 3 points on the VAS. The overall effect of cochlear implantation on tinnitus, that is, the effect with full-array stimulation, was not correlated with the effectiveness of the single-electrode stimulation on tinnitus. CONCLUSION: In conclusion, the effect of single-electrode stimulation on tinnitus is relatively insignificant in comparison to the effect of full-array stimulation. However, in some individual cases, sustained single-electrode stimulation may be beneficial for tinnitus management.

Haptic sound-localisation for use in cochlear implant and hearing-aid users.

Users of hearing-assistive devices often struggle to locate and segregate sounds, which can make listening in schools, cafes, and busy workplaces extremely challenging. A recent study in unilaterally implanted CI users showed that sound-localisation was improved when the audio received by behind-the-ear devices was converted to haptic stimulation on each wrist. We built on this work, using a new signal-processing approach to improve localisation accuracy and increase generalisability to a wide range of stimuli. We aimed to: (1) improve haptic sound-localisation accuracy using a varied stimulus set and (2) assess whether accuracy improved with prolonged training. Thirty-two adults with normal touch perception were randomly assigned to an experimental or control group. The experimental group completed a 5-h training regime and the control group were not trained. Without training, haptic sound-localisation was substantially better than in previous work on haptic sound-localisation. It was also markedly better than sound-localisation by either unilaterally or bilaterally implanted CI users. After training, accuracy improved, becoming better than for sound-localisation by bilateral hearing-aid users. These findings suggest that a wrist-worn haptic device could be effective for improving spatial hearing for a range of hearing-impaired listeners.

Assessment of Cochlear Implants for Adult Medicare Beneficiaries Aged 65 Years or Older Who Meet Expanded Indications of Open-Set Sentence Recognition: A Multicenter Nonrandomized Clinical Trial.

Importance: Current indications for Medicare beneficiaries to receive a cochlear implant are outdated. Multichannel cochlear implant systems may be effective when provided to Medicare beneficiaries using expanded indications. Objective: To examine the effectiveness of cochlear implants, as measured by improvement on the AzBio Sentence Test, for newly implanted Medicare beneficiaries who meet the expanded indications of an AzBio Sentence Test score of 41% to 60% in their best-aided condition. Design, Setting, and Participants: A multicenter nonrandomized trial examined preoperative and postoperative speech recognition, telephone communication, hearing device benefit, health utility, and quality of life for 34 participants enrolled at 8 different centers who received a cochlear implant between September 17, 2014, and July 10, 2018. All participants were 65 years or older, had bilateral moderate to profound hearing loss, and had a best-aided preoperative AzBio Sentence Test score in quiet of 41% to 60%. Analysis was performed on an intention-to-treat basis. Statistical analysis of final results took place from July 29 to October 1, 2019. Intervention: Multichannel cochlear implants. Main Outcomes and Measures: The study examined the a priori hypothesis that the cochlear implant would improve the AzBio Sentence Test score in the best-aided condition by 25% or more and in the implanted ear-alone condition by 30% or more. The study additionally examined word and telephone recognition and examined device benefit, health utility, and quality of life. Results: A total of 34 participants received a cochlear implant; 31 (23 men [74%]; median age, 73.6 years [range, 65.7-85.1 years]) completed testing through the 6-month evaluation, and 29 completed testing through the 12-month evaluation. Median preoperative AzBio Sentence Test scores were 53% (range, 26%-60%) for the best-aided condition and 24% (range, 0%-53%) for the cochlear implant-alone condition; median scores 12 months after implantation improved to 89% (range, 36%-100%) for the best-aided condition and 77% (range, 13%-100%) for the cochlear implant-alone condition. This outcome represents a median change of 36% (range, -22% to 75%) for the best-aided condition (lower bound of 1-sided 95% CI, 31%) and a median change of 53% (range, -15% to 93%) for the cochlear implant-alone condition (lower bound of 1-sided 95% CI, 45%). Conclusions and Relevance: Intervention with a cochlear implant was associated with improved sentence, word, and telephone recognition in adult Medicare beneficiaries whose preoperative AzBio Sentence Test scores were between 41% and 60%. These findings support expansion of the Center for Medicare & Medicaid current indications for cochlear implants. Trial Registration: ClinicalTrials.gov Identifier: NCT02075229.

Unilateral Cochlear Implants for Severe, Profound, or Moderate Sloping to Profound Bilateral Sensorineural Hearing Loss: A Systematic Review and Consensus Statements.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine. Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL. Design, Setting, and Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019. Main Outcomes and Measures: A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting. Results: In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement). Conclusions and Relevance: These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

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.

Using Contextual and Visual Cues to Improve Sung Word Recognition in Hearing Aid Users.

Older adults commonly experience hearing loss that negatively affects the quality of life and creates barriers to effective therapeutic interactions as well as music listening. Music therapists have the potential to address some needs of older adults, but the effectiveness of music interventions is dependent on the perception of spoken and musical stimuli. Nonauditory information, such as contextual (e.g., keywords, picture related to song) and visual cues (e.g., clear view of singer's face), can improve speech perception. The purpose of this study was to examine the benefit of contextual and visual cues on sung word recognition in the presence of guitar accompaniment. The researcher tested 24 community-dwelling older adult hearing aid (HA) users recruited through a university HA clinic and laboratory under 3 study conditions: (a) auditory stimuli only, (b) auditory stimuli with contextual cues, and (c) auditory stimuli with visual cues. Both visual and contextual nonauditory cues benefited participants on sung word recognition. Participants' music background and training were predictive of success without nonauditory cues, and visual cues provided greater benefit than contextual cues. Based on the results of this study, it is recommended that music therapists increase the accessibility of music interventions reliant upon lyric recognition through the incorporation of clear visual and contextual cues.

Cortical deafness of following bilateral temporal lobe stroke.

Cortical deafness is an extremely rare clinical manifestation that originates mainly from bilateral cortical lesions in the primary auditory cortex. Its main clinical manifestation is the bilateral sudden loss of hearing. Diagnosis is difficulty due to its rarity and similarity with other language and communication disorders, such as Wernicke's aphasia, auditory agnosia or verbal deafness. Herein, we present a case report of a young woman with a sudden bilateral loss of auditory comprehension. Initially, a psychiatric nature of the disorder was considered, but the persistence of the symptoms, lead to the diagnosis of cortical deafness secondary to bilateral ischemic lesions in both temporal lobes. Progressive improvement occurred and three months after the initial manifestations she manifested pure verbal deafness. Cortical deafness usually has a poor functional prognosis, with limited therapeutic options. Rehabilitation and speech therapy is recommended to improve the chance of patients achieving communication skills.

Cochlear implantation after traumatic brain injury without otic capsule fracture: A case report and literature review.

OBJECTIVE: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature. METHODS: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases. RESULTS: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL. CONCLUSIONS: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.

Association Between Hearing Loss and Postural Instability in Older Korean Adults.

Importance: Hearing loss is one of the most frequently occurring sensory disabilities worldwide. The association between hearing loss and postural instability in an older population remains to be studied. Objective: To determine whether the odds of postural instability increase with the degree of hearing loss and to suggest criteria for hearing rehabilitation for postural instability. Design, Setting, and Participants: This cross-sectional population-based study of 3864 participants 40 years and older with hearing loss used data from 2010 through 2012 in the fifth edition of the Korea National Health and Nutrition Examination Survey, a nationwide survey of South Korea. Pure tone audiometry was performed on both the left and right sides and each hearing grade of each side was classified into 1 of 3 subcategories: normal hearing, mild hearing loss, or moderate hearing loss (moderate hearing loss or worse). Postural instability was defined as the failure to remain standing on a foam pad surface with feet 10 cm apart with arms folded and hands cupping the elbows and eyes closed. A binomial logistic regression model was used to assess the association between hearing loss and postural instability. Data were analyzed from August 18, 2019, to September 2, 2019. Exposures: Age-related hearing loss defined as a threshold of 26 dB or more. Main Outcomes and Measures: The degree of hearing loss and postural instability. Results: Of the 3864 participants included in the study, the mean (SD) age was 57.8 (11.3) years and 2135 (55.2%) were women. Female sex was associated with higher odds of postural instability compared with male sex (odds ratio [OR], 1.65; 95% CI, 1.12-2.42, adjusted for age and hearing status), and the odds of postural instability increased with every 1 year increase in age (OR, 1.13; 95% CI, 1.10-1.16, adjusted for sex and hearing status). Unilateral or bilateral mild hearing loss was not associated with an increase in the odds of postural instability, but moderate hearing loss present on at least 1 side was associated with an increase in the odds of postural instability: unilateral moderate hearing loss group (OR, 2.71; 95% CI, 1.12-6.10, adjusted for age and sex), one mild and the other moderate hearing loss group (OR, 2.18; 95% CI, 1.16-4.09, adjusted for age and sex), and bilateral moderate hearing loss group (OR, 2.34; 95% CI, 1.27-4.33, adjusted for age and sex). Conclusions and Relevance: Aging, female sex, and having hearing loss were associated with postural instability in this analysis. Moderate or worse hearing loss present on at least 1 side was associated with increased odds of postural instability. Future research in this area is warranted to identify more precise interrelationships and preventive measures.

Insights into the molecular pathogenesis of cardiospondylocarpofacial syndrome: MAP3K7 c.737-7A > G variant alters the TGFß-mediated α-SMA cytoskeleton assembly and autophagy.

Transforming growth factor beta-activated kinase 1 (TAK1) is a highly conserved kinase protein encoded by MAP3K7, and activated by multiple extracellular stimuli, growth factors and cytokines. Heterozygous variants in MAP3K7 cause the cardiospondylocarpofacial syndrome (CSCFS) which is characterized by short stature, dysmorphic facial features, cardiac septal defects with valve dysplasia, and skeletal anomalies. CSCFS has been described in seven patients to date and its molecular pathogenesis is only partially understood. Here, the functional effects of the MAP3K7 c.737-7A > G variant, previously identified in a girl with CSCFS and additional soft connective tissue features, were explored. This splice variant generates an in-frame insertion of 2 amino acid residues in the kinase domain of TAK1. Computational analysis revealed that this in-frame insertion alters protein dynamics in the kinase activation loop responsible for TAK1 autophosphorylation after binding with its interactor TAB1. Co-immunoprecipitation studies demonstrate that the ectopic expression of TAK1-mutated protein impairs its ability to physically bind TAB1. In patient's fibroblasts, MAP3K7 c.737-7A > G variant results in reduced TAK1 autophosphorylation and dysregulation of the downstream TAK1-dependent signaling pathway. TAK1 loss-of-function is associated with an impaired TGFß-mediated α-SMA cytoskeleton assembly and cell migration, and defective autophagy process. These findings contribute to our understanding of the molecular pathogenesis of CSCFS and might offer the rationale for the design of novel therapeutic targets.

Associations of Retinal Vessel Caliber With Hearing Status in Childhood and Midlife: A Cross-Generational Population-Based Study.

Importance: Microvascular phenotypes, which can be assessed using retinal imaging, may be informative about the life course pathogenesis of hearing loss. Objective: To investigate whether differences in retinal vessel caliber (specifically wider venules and narrower arterioles) are associated with hearing threshold and hearing loss in mid-childhood and midlife. Design, Setting, and Participants: A population-based cross-sectional study (Child Health CheckPoint) was nested within the Longitudinal Study of Australian Children. A total of 1281 children and 1255 attending parents were assessed using retinal microvasculature and air conduction audiometry data at a main assessment center in 7 large cities in Australia. Main Outcomes and Measures: Air conduction audiometry was used to calculate the high Fletcher index (mean threshold of 1, 2, and 4 kHz), and bilateral hearing loss was defined as a high Fletcher index greater than 15 dB hearing level in the better-hearing ear. Retinal arteriolar and venular caliber were measured from fundus photographs using validated computer-based software. Linear and logistic regression quantified the associations of retinal vessel caliber with hearing threshold and hearing loss, respectively. Results: Of the 1281 included children (mean age, 11.4 years; 49.1% boys), the mean (SD) high Fletcher index was 7.9 (5.8) dB hearing level. Of the 1255 included adults (mean age, 43.8 years; 86.6% women), the mean (SD) high Fletcher index was 13.0 (6.8) dB hearing level; 109 of 1281 children (8.5%) and 328 of 1255 adults (26.1%) had hearing loss. In adults, each 1-SD (18.6-µm) wider retinal venular caliber (worse) was associated with higher (worse) hearing threshold at lower individual frequencies (eg, 2 kHz: ß = 0.63; 95% CI, 0.10-1.17) and overall high Fletcher index (eg, 2 kHz: ß = 0.52; 95% CI, 0.07-0.96), as well as a 1.20-fold (95% CI, 1.03-1.40) higher odds of hearing loss. In children, patterns of venular associations were similar but smaller and less certain. Narrower retinal arteriolar caliber (worse) was associated with a 1.16-fold (95% CI, 1.00-1.37) higher odds of hearing loss in adults (per 1-SD [14.0-µm] narrower arteriolar caliber) but not in children. Conclusions and Relevance: Adverse retinal microvascular characteristics are associated with hearing loss by midlife, with venular associations possibly emerging by age 11 to 12 years. Microvascular health may contribute to the pathogenesis of hearing loss across the life course, warranting replication and mechanistic studies to inform causal inference and prevention efforts.

Bilateral bone conduction stimulation provides reliable binaural cues for localization.

This study aimed to characterize binaural hearing abilities with bone conduction stimulation in simulated conductive hearing loss. Bone conduction hearing devices (BCDs) are a common method of rehabilitating conductive hearing loss. However, little is known about the access these devices provide to binaural cues. To study the ability of BCDs to restore access to binaural cues in conductive loss, normal hearing listeners were plugged unilaterally and bilaterally and localization ability was assessed using a non-surgical BCD attached to the mastoid/s via an adhesive (MED-EL, Corp). The results demonstrate that 1) application of the BCD in simulated unilateral conductive hearing loss does not restore access to binaural cues, evidenced by poor localization abilities. 2) bilateral application of BCDs in simulated bilateral conductive hearing loss provides access to binaural cues, 2) unilateral application of BCDs in simulated bilateral conductive hearing loss disrupts these cues and impairs localization performance, The transcutaneous stimulation of the adhesive BCD resulted in decreased access to sound compared to the normal open ear, resulting in asymmetries in aided versus non-aided hearing thresholds. Symmetrical hearing results in improved localization abilities, while asymmetric hearing disrupts sound localization abilities.

Listening-Related Fatigue in Children With Unilateral Hearing Loss.

Purpose Listening-related fatigue is an understudied construct that may contribute to the auditory, educational, and psychosocial problems experienced by children with unilateral hearing loss (UHL). Herein, we present an overview of listening-related fatigue in school-age children with hearing loss (CHL), with a focus on children with UHL. Method Following a review of research examining listening-related fatigue in adults and CHL, we present preliminary findings exploring the effects of unilateral and bilateral hearing loss on listening-related fatigue in children. For these exploratory analyses, we used data collected from our ongoing work developing and validating a tool, the Vanderbilt Fatigue Scale, for measuring listening-related fatigue in children. Presently, we are assessing 3 versions of the fatigue scale-child self-report, parent proxy, and teacher proxy. Using these scales, data have been collected from more than 900 participants. Data from children with unilateral and bilateral hearing loss and for children with no hearing loss are compared with adult Vanderbilt Fatigue Scale data. Results Results of our literature review and exploratory analyses suggest that adults and CHL are at increased risk for listening-related fatigue. Importantly, this increased risk was similar in magnitude regardless of whether the loss was unilateral or bilateral. Subjective ratings, based on child self-report and parent proxy report, were consistent, suggesting that children with unilateral and bilateral hearing loss experienced greater listening-related fatigue than children with no hearing loss. In contrast, results based on teacher proxy report were not sensitive to the effects of hearing loss. Conclusions Children with UHL are at increased risk for listening-related fatigue, and the magnitude of fatigue is similar to that experienced by children with bilateral hearing loss. Problems of listening-related fatigue in school-age CHL may be better identified by CHL themselves and their parents than by teachers and specialists working with the children.

Sound Localization in Toddlers with Normal Hearing and with Bilateral Cochlear Implants Revealed Through a Novel "Reaching for Sound" Task.

BACKGROUND: Spatial hearing abilities in children with bilateral cochlear implants (BiCIs) are typically improved when two implants are used compared with a single implant. However, even with BiCIs, spatial hearing is still worse compared to normal-hearing (NH) age-matched children. Here, we focused on children who were younger than three years, hence in their toddler years. Prior research with this age focused on measuring discrimination of sounds from the right versus left. PURPOSE: This study measured both discrimination and sound location identification in a nine-alternative forced-choice paradigm using the "reaching for sound" method, whereby children reached for sounding objects as a means of capturing their spatial hearing abilities. RESEARCH DESIGN: Discrimination was measured with sounds randomly presented to the left versus right, and loudspeakers at fixed angles ranging from ±60° to ±15°. On a separate task, sound location identification was measured for locations ranging from ±60° in 15° increments. STUDY SAMPLE: Thirteen children with BiCIs (27-42 months old) and fifteen age-matched (NH). DATA COLLECTION AND ANALYSIS: Discrimination and sound localization were completed for all subjects. For the left-right discrimination task, participants were required to reach a criterion of 4/5 correct trials (80%) at each angular separation prior to beginning the localization task. For sound localization, data was analyzed in two ways. First, percent correct scores were tallied for each participant. Second, for each participant, the root-mean-square-error was calculated to determine the average distance between the response and stimulus, indicative of localization accuracy. RESULTS: All BiCI users were able to discriminate left versus right at angles as small as ±15° when listening with two implants; however, performance was significantly worse when listening with a single implant. All NH toddlers also had >80% correct at ±15°. Sound localization results revealed root-mean-square errors averaging 11.15° in NH toddlers. Children in the BiCI group were generally unable to identify source location on this complex task (average error 37.03°). CONCLUSIONS: Although some toddlers with BiCIs are able to localize sound in a manner consistent with NH toddlers, for the majority of toddlers with BiCIs, sound localization abilities are still emerging.