Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment.

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL.

Speech, language, functional communication, psychosocial outcomes and QOL in school-age children with congenital unilateral hearing loss.

Introduction: Children with early-identified unilateral hearing loss (UHL) might be at risk for delays in early speech and language, functional communication, psychosocial skills, and quality of life (QOL). However, a paucity of relevant research prohibits strong conclusions. This study aimed to provide new evidence relevant to this issue. Methods: Participants were 34 children, ages 9;0 to 12;7 (years;months), who were identified with UHL via newborn hearing screening. Nineteen children had been fitted with hearing devices, whereas 15 had not. Assessments included measures of speech perception and intelligibility; language and cognition; functional communication; psychosocial abilities; and QOL. Results and discussion: As a group, the children scored significantly below the normative mean and more than one standard deviation below the typical range on speech perception in spatially separated noise, and significantly below the normative mean on written passage comprehension. Outcomes in other aspects appear typical. There was however considerable within participant variation in the children's degree of hearing loss over time, raising the possibility that this pattern of results might change as children get older. The current study also revealed that participants with higher levels of nonverbal ability demonstrated better general language skills and better ability to comprehend written passages. By contrast, neither perception of speech in collocated noise nor fitting with a hearing device accounted for unique variance in outcome measures. Future research should, however, evaluate the fitting of hearing devices using random assignment of participants to groups in order to avoid any confounding influence of degree of hearing loss or children's past/current level of progress.

A Comparative Study of Hearing Handicap Inventory and Pure-Tone Audiometry Scores in Unilateral Hearing Impaired Patients.

Objective: To investigate the consistency between the hearing handicap inventory (HHI) and pure-tone audiometry (PTA) scores in assessing hearing status to provide valuable insights for clinical application. Methods: Retrospective analysis of clinical data and the HHI reporting status of 6540 patients admitted between April 2020 and July 2022 for self-reported unilateral hearing loss who met the study inclusion and exclusion criteria. The kappa coefficient was used to evaluate the consistency of HHI and PTA in assessing the hearing status of the participants. Results: The PTA results showed that among the 6540 participants, 3895 exhibited normal hearing, 1434 showed mild hearing loss, 809 presented with moderate hearing loss, and 402 showed severe hearing loss. The mean hearing thresholds from 0.5 to 4 kHz in healthy ears ranged from 3.65 to 18.45 dB HL, with a mean of 10.83 ± 5.29 dB HL; in ears affected by hearing loss, this ranged from 35 to 125 dB HL, with a mean of 69.63 ± 28.45 dB HL. The HHI scores showed that 4820 people had normal hearing, 1245 had mild-to-moderate hearing loss, and 475 had severe hearing loss. The kappa coefficients of normal, mild-to-moderate, and severe hearing loss were 0.312, 0.223, and 0.716, respectively (P = .001). The consistency between the 2 groups was particularly significant in the assessment of severe hearing loss. Using the PTA results as a benchmark, the sensitivity, specificity, positive predictive value, and negative predictive value of the HHI were found to be 73.08%, 87.83%, 95.60%, and 70.98%, respectively. Conclusion: The HHI and PTA results were consistent in the assessment of hearing status, particularly in the assessment of severe hearing loss, and the level of consistency between the 2 methods was high. The combined use of these tools can facilitate a comprehensive assessment of the auditory status of patients with hearing loss.

Cochlear Implantation Outcomes in Pediatric Unilateral Hearing Loss: Impact of Device Use and Direct Input.

OBJECTIVE: To investigate the impact of daily cochlear implant (CI) use on speech perception outcomes among children with unilateral hearing loss (UHL). STUDY DESIGN: Multi-institutional retrospective case series of pediatric patients with UHL who underwent CI between 2018 to 2022. SETTING: Three tertiary children's hospitals. METHODS: Demographics were obtained including duration of deafness and age at CI. Best consonant-nucleus-consonant (CNC) word scores and data logs describing hours of CI usage were assessed postimplantation. Use of direct audio input (DAI) during rehabilitation was recorded. RESULTS: Twenty-seven children were included, with a mean age at CI of 7.8 years. Mean datalogging time was 7.8 ± 3.0 hours/day. 40.7% of children utilized daily DAI. The mean CNC score using the best score during the study period was 34.9%. There was no significant correlation between hours of CI usage and CNC score. There was a significant improvement in CNC score associated with whether the child used DAI during rehabilitation (CNC 50.91% [yes] vs 23.81% [no]), which remained significant when adjusting for age at CI, duration of deafness, and data log hours. CONCLUSION: Unlike children with bilateral hearing loss and CI, children with UHL and CI demonstrate no significant correlation between hours of daily CI usage and CNC scores. However, children who used DAI during postoperative rehabilitation achieved significantly higher CNC scores than those who did not. This suggests that rehabilitation focused on isolated listening with the implanted ear maybe critical in optimizing outcomes with CI in UHL patients.

Auditory Cortical Plasticity in Patients with Single-Sided Deafness Before and After Cochlear Implantation.

PURPOSE: This study investigated neuroplastic changes induced by postlingual single-sided deafness (SSD) and the effects of a cochlear implantation for the deaf ear. Neural processing of acoustic signals from the normal hearing ear to the brain was studied before and after implantation using a positron emission tomography (PET)/CT scanner. METHODS: Eight patients with postlingual SSD received a cochlear implant (CI) in a prospective clinical trial. Dynamic imaging was performed in a PET/CT scanner using radioactively labeled water ([15O]H2O) to localize changes in the regional cerebral blood flow (rCBF) with and without an auditory task of logatomes containing speech-like elements without meaningful context. The normal hearing ear was stimulated before implantation and after the use of the cochlear implant for at least 8 months (mean 13.5, range 8.1-26.6). Eight age- and gender-matched subjects with normal hearing on both sides served as healthy control subjects (HCS). RESULTS: When the normal hearing ear of SSD patients was stimulated before CI implantation, the [15O]H2O-PET showed a more symmetrical rCBF in the auditory regions of both hemispheres in comparison to the HCS. The use of CI increased the asymmetry index (AI) in six of eight patients indicating an increase of activity of the contralateral hemisphere. Non-parametric statistics revealed a significant difference in the AI between patients before CI implantation and HCS (p < .01), which disappeared after CI implantation (p = .195). CONCLUSION: The functional neuroimaging data showed a tendency towards normalization of neuronal activity after CI implantation, which supports the effectiveness of CI in SSD patients. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01749592, December 13, 2012.

Objective and subjective efficacy of hearing aids in patients with mild-to-moderate unilateral hearing loss: a prospective study.

PURPOSE: In patients with unilateral sensorineural hearing loss (USNHL), we explored both objective functional audiological gains and subjective satisfaction, indicating when a unilateral hearing aid is valuable. METHODS: Thirty-seven patients with mild-to-moderate USNHL (mean pure-tone thresholds between 25 and 70 dB) were prescribed unilateral hearing aids. Functional gain, the aided speech discrimination score (SDS), the Hearing in Noise Test (HINT) score, and the sound localization test score were collected, and a questionnaire (the Hearing Handicap Inventory for the Elderly, HHIE) completed after 1, 2, and 3 months of hearing aid use. We classified the participants as having 'no handicap' (HHIE < 17), 'mild-to-moderate handicap' (17-42), and 'significant handicap' (> 42). RESULTS: The decrease in handicap afforded by unilateral hearing aids was largest in the 'significant handicap' group (the HHIE total score fell from 59.1 to 37.2; P = 0.007). There were no between-group differences in either functional gain or the aided SDS. Only the 'significant handicap' group evidenced an improved HINT score; the composite signal-to-noise ratio (SNR) fell from - 1.5 to - 2.2 dB [S/N] (P = 0.023). The HHIE usefully indicated when a hearing aid alleviated the discomfort of USNHL; patients with unaided HHIE scores ≥ 20 evidenced significant decreases in the composite SNR (- 1.7 to - 2.0 dB [S/N]; P = 0.045). CONCLUSIONS: When considering whether to prescribe a unilateral hearing aid for patients with mild-to-moderate USNHL, it is helpful to use the HHIE to evaluate discomfort. If the total score is ≥ 20, a hearing aid is appropriate.

Auditory capacity of the better-hearing ear in asymmetric hearing loss.

PURPOSE: Our aim was to investigate the course of the hearing capacity of the better-hearing ear in single-sided deafness (SSD) and asymmetric hearing loss (AHL) over time, in a multicenter study. METHODS: We included 2086 pure-tone audiograms from 323 patients with SSD and AHL from four hospitals and 156 private practice otorhinolaryngologists. We collected: age, gender, etiology, duration of deafness, treatment with CI, number and monosyllabic speech recognition, numerical rating scale (NRS) of tinnitus intensity, and the tinnitus questionnaire according to Goebel and Hiller. We compared the pure tone audiogram of the better-hearing ear in patients with SSD with age- and gender-controlled hearing thresholds from ISO 7029:2017. RESULTS: First, individuals with SSD showed a significantly higher hearing threshold from 0.125 to 8 kHz in the better-hearing ear compared to the ISO 7029:2017. The duration of deafness of the poorer-hearing ear showed no relationship with the hearing threshold of the better-hearing ear. The hearing threshold was significantly higher in typically bilaterally presenting etiologies (chronic otitis media, otosclerosis, and congenital hearing loss), except for Menière's disease. Second, subjects that developed AHL did so in 5.19 ± 5.91 years and showed significant reduction in monosyllabic word and number recognition. CONCLUSIONS: Individuals with SSD show significantly poorer hearing in the better-hearing ear than individuals with NH from the ISO 7029:2017. In clinical practice, we should, therefore, inform our SSD patients that their disease is accompanied by a reduced hearing capacity on the contralateral side, especially in certain etiologies.

Benefits of unilateral cochlear implantation in adults with asymmetric hearing loss: Audiologic and patient-related outcome measures.

PURPOSE: To investigate the benefits of cochlear implantation in adults with single-sided deafness (SSD) and asymmetric hearing loss (AHL). STUDY DESIGN: Prospective within-subjects repeated-measures. SETTING: Two tertiary cochlear implant centers. PATIENTS: Fourteen adults with severe-to-profound sensorineural hearing loss in the worse hearing ear and up to moderate SNHL in the better hearing ear. INTERVENTION: Cochlear implantation in the worse hearing ear. MAIN OUTCOME MEASURES: Consonant-nucleus-consonant (CNC) test, AzBio sentence test in noise, and lateralization testing were conducted preoperatively and at 3-, 6-, and 12-months post-activation. Patient-related outcomes were measured using the Speech, Spatial, and Qualities of Hearing Scale and Glasgow Benefit Inventory. Tinnitus Handicap Inventory was administered to subjects with tinnitus. RESULTS: Mean length of hearing loss in the worse hearing ear was 3.5 years. The mean CNC change scores from baseline were 54.8, 55.9, and 58.9 percentage points at 3-, 6-, and 12-months (p < 0.001). AzBio sentence test in noise demonstrated improved scores in all spatial configurations, although statistically significant in S0N0 (speech front, noise front) only. Lateralization testing showed significant improvement of 22.9, 24.5, and 24.0 percentage points at 3-, 6-, and 12 months post-activation (p = 0.002). All patient-related outcome measures revealed significant improvement. CONCLUSION: This study demonstrates improved speech perception in noise, sound lateralization, quality of life, and reduction in tinnitus perception in adults with SSD/AHL who undergo cochlear implantation. Our results add to the growing body of evidence that cochlear implant should be offered to this population.

Impact of room acoustics and visual cues on speech perception and talker localization by children with mild bilateral or unilateral hearing loss.

Introduction: This study evaluated the ability of children (8-12 years) with mild bilateral or unilateral hearing loss (MBHL/UHL) listening unaided, or normal hearing (NH) to locate and understand talkers in varying auditory/visual acoustic environments. Potential differences across hearing status were examined. Methods: Participants heard sentences presented by female talkers from five surrounding locations in varying acoustic environments. A localization-only task included two conditions (auditory only, visually guided auditory) in three acoustic environments (favorable, typical, poor). Participants were asked to locate each talker. A speech perception task included four conditions [auditory-only, visually guided auditory, audiovisual, auditory-only from 0° azimuth (baseline)] in a single acoustic environment. Participants were asked to locate talkers, then repeat what was said. Results: In the localization-only task, participants were better able to locate talkers and looking times were shorter with visual guidance to talker location. Correct looking was poorest and looking times longest in the poor acoustic environment. There were no significant effects of hearing status/age. In the speech perception task, performance was highest in the audiovisual condition and was better in the visually guided and auditory-only conditions than in the baseline condition. Although audiovisual performance was best overall, children with MBHL or UHL performed more poorly than peers with NH. Better-ear pure-tone averages for children with MBHL had a greater effect on keyword understanding than did poorer-ear pure-tone averages for children with UHL. Conclusion: Although children could locate talkers more easily and quickly with visual information, finding locations alone did not improve speech perception. Best speech perception occurred in the audiovisual condition; however, poorer performance by children with MBHL or UHL suggested that being able to see talkers did not overcome reduced auditory access. Children with UHL exhibited better speech perception than children with MBHL, supporting benefits of NH in at least one ear.

Audiological characteristics of children with congenital unilateral hearing loss: insights into Age of reliable behavioural audiogram acquisition and change of hearing loss.

Objectives: The aims of this study were to report the audiological characteristics of children with congenital unilateral hearing loss (UHL), examine the age at which the first reliable behavioural audiograms can be obtained, and investigate hearing changes from diagnosis at birth to the first reliable behavioural audiogram. Method: This study included a sample of 91 children who were diagnosed with UHL via newborn hearing screening and had reliable behavioural audiograms before 7 years of age. Information about diagnosis, audiological characteristics and etiology were extracted from clinical reports. Regression analysis was used to explore the potential reasons influencing the age at which first reliable behavioural audiograms were obtained. Correlation and ANOVA analyses were conducted to examine changes in hearing at octave frequencies between 0.5 and 4 kHz. The proportions of hearing loss change, as well as the clinical characteristics of children with and without progressive hearing loss, were described according to two adopted definitions: Definition 1: criterion (1): a decrease in 10 dB or greater at two or more adjacent frequencies between 0.5 and 4 kHz, or criterion (2): a decrease in 15 dB or greater at one octave frequency in the same frequency range. Definition 2: a change of ≥20 dB in the average of pure-tone thresholds at 0.5, 1, and 2 kHz. Results: The study revealed that 48 children (52.7% of the sample of 91 children) had their first reliable behavioural audiogram by 3 years of age. The mean age at the first reliable behavioural audiogram was 3.0 years (SD 1.4; IQR: 1.8, 4.1). We found a significant association between children's behaviour and the presence or absence of ongoing middle ear issues in relation to the delay in obtaining a reliable behavioural audiogram. When comparing the hearing thresholds at diagnosis with the first reliable behavioural audiogram across different frequencies, it was observed that the majority of children experienced deterioration rather than improvement in the initial impaired ear at each frequency. Notably, there were more instances of hearing changes (either deterioration or improvement), in the 500 Hz and 1,000 Hz frequency ranges compared to the 2,000 Hz and 4,000 Hz ranges. Seventy-eight percent (n = 71) of children had hearing deterioration between the diagnosis and the first behavioural audiogram at one or more frequencies between 0.5 and 4 kHz, with a high proportion of them (52 out of the 71, 73.2%) developing severe to profound hearing loss. When using the averaged three frequency thresholds (i.e., definition 2), only 26.4% of children (n = 24) in the sample were identified as having hearing deterioration. Applying definition 2 therefore underestimates the proportion of children that experienced hearing changes. The study also reported diverse characteristics of children with or without hearing deterioration. Conclusion: The finding that 78% of children diagnosed with UHL at birth had a decrease in hearing loss between the hearing levels at first diagnosis and their first behavioural audiogram highlights the importance of monitoring hearing threshold levels after diagnosis, so that appropriate intervention can be implemented in a timely manner. For clinical management, deterioration of 15 dB at one or more frequencies that does not recover warrants action.

[Spontaneous otoliquorrhea, complicated by recurrent secondary meningitis, in a 6-year-old child with an abnormality of the inner ear]. / Spontannaya otolikvoreya, oslozhnennaya retsidiviruyushchim vtorichnym meningitom, u rebenka 6 let s anomaliei razvitiya vnutrennego ukha.

Congenital anomalies in the inner ear structures development are one of the reasons for unilateral hearing loss in children. Unilateral hearing loss is predominantly congenital, thus children with this pathology are initially deprived of the ability to hear with both ears, which leads to the formation of a specific «individual hearing norm¼. Due to this, children do not complain of hearing loss, and unsuspecting parents can only guess why their child is poorly studying and lagging behind his peers. Unfortunately, in some situations the detection of malformations of inner ear structures occurs only after the development of complications, namely spontaneous otoliquorrea, leading to bacterial meningitis, and exactly episodes of meningitis (sometimes recurrent) become the cause for examination of patient and diagnosis of this pathology. We present one of this clinical observations.

Middle Ear Cholesteatoma and Vestibular Schwannoma Resection Followed by Cochlear Implant: Surgical Challenges and Audiological Outcomes.

(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such cases. Moreover, the presence of middle and inner ear pathologies combined may represent a surgical challenge. No studies have described a combined surgical approach for these coexisting conditions (VS and cholesteatoma), nor the hearing rehabilitation outcomes of using cochlear implants for these patients. (2) Case Report: This paper is on a female patient who underwent simultaneous surgical treatments for VS and middle ear cholesteatoma in the right ear followed by a cochlear implant, describing the technique and the audiological results. (3) Conclusions: The surgical approach was successful and enabled the resection of lesions with the auditory nerve and cochlea preservation. Cochlear implantation in the right ear showed positive postoperative results, with an improvement in the results with the CI in silent and noisy environments.

The Role of Bone-Anchored Hearing Devices and Remote Microphones in Children with Congenital Unilateral Hearing Loss.

Congenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA). This was a retrospective observational study on a group of children with UHL. The Simplified Italian Matrix Sentence Test was used for the assessment of speech reception threshold (SRT) in different conditions: speech and noise from the front (S0N0), speech at 45° from the side of the better ear and noise at 45° from the opposite side (SbNw), and vice versa (SwNb). Each test was conducted unaided, with a bone-anchored hearing device (BAHD), and with a remote microphone (RM) system. The use of a BAHD and RM led to an improvement in SRT in S0N0 and SwNb conditions. The SSD subgroup demonstrated significant benefits with both devices in SwNb, and the UAA subgroup from the use of BAHD in S0N0. In conclusion, the study underscores the potential benefits of both devices in enhancing speech perception for UHL children, providing insights into effective intervention strategies for these challenging cases.

[Cochlear implant and tinnitus]. / Cochleaimplantat und Tinnitus.

Cochlear implant (CI) treatment is now established as a successful 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).

Management of Bevacizumab-Induced Proteinuria Using an Angiotensin Receptor Blocker (ARB) in a Neurofibromatosis Type 2 (NF-2) Patient With Vestibular Schwannoma.

Neurofibromatosis type 2 (NF-2) is a genetic condition that by definition includes bilateral vestibular schwannoma, a non-malignant lesion also known as acoustic neuroma. Patients often develop hearing impairment and hearing loss as a result of the involvement of the vestibulocochlear nerve bilaterally as well as attempts at surgical repair. A common treatment for NF-2-mediated schwannoma is the antiangiogenic agent, bevacizumab. In many cases, patients require prolonged and even lifelong treatment with bevacizumab to control schwannoma growth. However, long-term use of bevacizumab can be associated with multiple side effects including hypertension and proteinuria including nephrotic syndrome (>3g of protein excreted in the urine in 24 hours). In these situations, the challenge with discontinuing prolonged bevacizumab can be rapid tumor growth and worsening hearing loss. Pre-clinical data suggests that hearing loss can be prevented following treatment with the angiotensin receptor blocker (ARB), losartan, in an animal model of NF-2. ARBs are already established in nephrology guidelines to treat proteinuria and hypertension. Here, we present a patient with NF-2 who developed nephrotic syndrome while on bevacizumab. Attempts to discontinue bevacizumab resulted in near-immediate hearing loss. Treatment with the ARB telmisartan together with bevacizumab resulted in improved hearing, reduced proteinuria, and controlled hypertension.

Language and health-related quality of life outcomes of children early-detected with unilateral and mild bilateral hearing loss.

Introduction: We aimed to describe the language and health-related quality of life (HRQoL) outcomes of children early-identified with unilateral or mild bilateral permanent hearing loss. This was a cross-sectional community-based study of children with mild bilateral or unilateral permanent hearing loss (including unilateral auditory neuropathy spectrum disorder (ANSD)), drawn from a population-based databank in Victoria, Australia. Methods: Enrolment in this databank is independent of early intervention and amplification approaches. Language and caregiver-reported HRQoL outcomes are described by type and degree of loss at three timepoints across child development: at age 2 years (n = 255), 5-7 years (n = 173) and 9-12 years (n = 45). Results: Across all age groups, average language outcomes were poorer than population normative scores by between a half to two thirds of a standard deviation. Children with mild bilateral hearing loss demonstrated poorer average language outcomes than children with unilateral hearing loss, particularly at younger ages. Children with unilateral ANSD showed language outcomes comparable to their peers with unilateral profound hearing loss. Children had poorer HRQoL psychosocial scores compared to physical scores, without obvious patterns of outcomes linked to degree or type of hearing loss. Discussion: This study demonstrates children with early-identified unilateral or mild bilateral hearing loss have average language and HRQoL outcomes poorer than population normative expectations from an early age. These outcomes are observed at later ages across childhood. These findings provide a contemporary description of language and quality of life outcomes for children identified but not targeted by universal newborn hearing screening and raise questions of how to provide better support for these populations of children and their families.

Characterisation of the treatment provided for children with unilateral hearing loss.

Background: Children with permanent unilateral hearing loss (UHL) are an understudied population, with limited data to inform the guidelines on clinical management. There is a funding gap in healthcare provision for the children with UHL in the United Kingdom, where genetic screening, support services, and devices are not consistently provided or fully funded in all areas. They are a disparate population with regard to aetiology and their degree of hearing loss, and hence their device choice and use. Despite having one "good ear", some children with UHL can have similar outcomes, socially, behaviourally, and academically, to children with bilateral hearing loss, highlighting the importance of understanding this population. In this longitudinal cohort study, we aimed to characterise the management of the children with UHL and the gaps in the support services that are provided for the children in Nottingham, United Kingdom. Methods: A cohort study was conducted collecting longitudinal data over 17 years (2002-2019) for 63 children with permanent congenital confirmed UHL in a large tertiary regional referral centre for hearing loss in Nottingham, United Kingdom. The cases of UHL include permanent congenital, conductive, mixed, or sensorineural hearing loss, and the degree of hearing loss ranges from mild to profound. The data were taken from their diagnostic auditory brainstem responses and their two most recent hearing assessments. Descriptors were recorded of the devices trialled and used and the diagnoses including aetiology of UHL, age of first fit, degree of hearing loss, when and which type of device was used, why a device was not used, the support services provided, concerns raised, and who raised them. Results: Most children (45/63; 71%) trialled a device, and the remaining 18 children had no device trial on record. Most children (20/45; 44%) trialled a bone-conduction device, followed by contralateral routing of signal aid (15/45; 33%) and conventional hearing aids (9/45; 20%). Most children (36/45; 80%) who had a device indicated that they wore their device "all day" or every day in school. Few children (8/45; 18%) reported that they wore their device rarely, and the reasons for this included bullying (3/8), feedback from the device (2/8), and discomfort from the device (2/8). Only one child reported that the device was not helping with their hearing. The age that the children were first fitted with their hearing device varied a median of 2.5 years for hearing aids and bone-conduction devices and 7 years for a contralateral routing of signal aid. The length of time that the children had the device also varied widely (median of 26 months, range 3-135 months); the children had their bone-conduction hearing aid for the longest period of time (median of 32.5 months). There was a significant trend where more recent device fittings were happening for children at a younger age. Fifty-one children were referred by the paediatric audiologist to a support service, 72.5% (37/51) were subsequently followed up by the referred service with no issue, whilst the remaining 27.5% (14/51) encountered an issue leading to an unsuccessful provision of support. Overall, most children (65%, 41/63) had no reported concerns, and 28.5% (18/63) of the children went on to have a documented concern at some point during their audiological care: five with hearing aid difficulties, five with speech issues, four with no improvement in hearing, three facing self-image or bullying issues, and one case of a child struggling to interact socially with friends. Three of these children had not trialled a device. We documented every concern reported from the parents, clinicians, teachers of the deaf, and from the children themselves. Where concerns were raised, more than half (58.6%, 10/18) were by schools and teachers, the remaining four concerns were raised by the family, and further four concerns were raised by the children themselves. Conclusion: To discover what management will most benefit which children with permanent UHL, we first must characterise their treatment, their concerns, and the support services available for them. Despite the children with UHL being a highly disparate population-in terms of their aetiology, their device use, the degree of hearing loss, and the age at which they trial a device-the majority report they use their device mostly in school. In lieu of available data and in consideration of the devices that are available to them, it could be useful to support families and clinicians in understanding the devices which are most used and where they are used. Considering the reasons for cessation of regular device use counselling and support services would be vital to support the children with UHL.

Fitting a Hearing Aid on the Better Ear, Worse Ear, or Both: Associations of Hearing-aid Fitting Laterality with Outcomes in a Large Sample of US Veterans.

Longitudinal electronic health records from a large sample of new hearing-aid (HA) recipients in the US Veterans Affairs healthcare system were used to evaluate associations of fitting laterality with long-term HA use persistence as measured by battery order records, as well as with short-term HA use and satisfaction as assessed using the International Outcome Inventory for Hearing Aids (IOI-HA), completed within 180 days of HA fitting. The large size of our dataset allowed us to address two aspects of fitting laterality that have not received much attention, namely the degree of hearing asymmetry and the question of which ear to fit if fitting unilaterally. The key findings were that long-term HA use persistence was considerably lower for unilateral fittings for symmetric hearing loss (HL) and for unilateral worse-ear fittings for asymmetric HL, as compared to bilateral and unilateral better-ear fittings. In contrast, no differences across laterality categories were observed for short-term self-reported HA usage. Total IOI-HA score was poorer for unilateral fittings of symmetric HL and for unilateral better-ear fittings compared to bilateral for asymmetric HL. We thus conclude that bilateral fittings yield the best short- and long-term outcomes, and while unilateral and bilateral fittings can result in similar outcomes on some measures, we did not identify any HL configuration for which a bilateral fitting would lead to poorer outcomes. However, if a single HA is to be fitted, then our results indicate that a better-ear fitting has a higher probability of long-term HA use persistence than a worse-ear fitting.

Capturing Visual Attention With Perturbed Auditory Spatial Cues.

Lateralized sounds can orient visual attention, with benefits for audio-visual processing. Here, we asked to what extent perturbed auditory spatial cues-resulting from cochlear implants (CI) or unilateral hearing loss (uHL)-allow this automatic mechanism of information selection from the audio-visual environment. We used a classic paradigm from experimental psychology (capture of visual attention with sounds) to probe the integrity of audio-visual attentional orienting in 60 adults with hearing loss: bilateral CI users (N = 20), unilateral CI users (N = 20), and individuals with uHL (N = 20). For comparison, we also included a group of normal-hearing (NH, N = 20) participants, tested in binaural and monaural listening conditions (i.e., with one ear plugged). All participants also completed a sound localization task to assess spatial hearing skills. Comparable audio-visual orienting was observed in bilateral CI, uHL, and binaural NH participants. By contrast, audio-visual orienting was, on average, absent in unilateral CI users and reduced in NH listening with one ear plugged. Spatial hearing skills were better in bilateral CI, uHL, and binaural NH participants than in unilateral CI users and monaurally plugged NH listeners. In unilateral CI users, spatial hearing skills correlated with audio-visual-orienting abilities. These novel results show that audio-visual-attention orienting can be preserved in bilateral CI users and in uHL patients to a greater extent than unilateral CI users. This highlights the importance of assessing the impact of hearing loss beyond auditory difficulties alone: to capture to what extent it may enable or impede typical interactions with the multisensory environment.