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Aim: To estimate the Single-sided deafness (SSD) among sensorineural hearing loss (SNHL) in Tertiary Care Rehabilitation Centre. To determine the audiological characteristics, associated and medical problems, and rehabilitative approaches among SSD. Method: A retrospective study was conducted to report on demographic details, audiological evaluation, and hearing aid management among SSD clients. A total of 11,534 cases of SNHL were reported to the Audiology department at the tertiary care rehabilitative center at Mysuru between January 2014 and December 2017. Results: A total of 225 were diagnosed as having SSD, accounting for approximately two cases of SSD in a cohort of 100 SNHL cases (225/11,534*100). In addition, males are affected more than females. SSD is found to have more in the right ear than the left ear. Their major complaints were tinnitus and vertigo, apart from reduced hearing sensitivity from the affected ear. In the side of the ear with profound hearing loss among SSD clients showed normal middle ear status with absent acoustic reflexes, otoacoustic emissions, and auditory brainstem response. Only 54 individuals (24%) out of 225 SSD clients underwent a hearing aid trial. Twenty-one (9%) SSD clients purchased a hearing aid, and a minuscule seven (3%) of 225 SSD clients are using a hearing aid. The preference for hearing aid uptake was low because of the experience of vibratory sensation and having near-normal hearing in one ear. Conclusion: Two cases of SSD were observed among SNHL and they are less likely to get benefits from the hearing aid. [ABSTRACT FROM AUTHOR]

Background/Objectives: Mitochondrial transfer RNA mutations are one of the most important causes of hereditary hearing loss in humans. In most cases, its presentation is bilateral and symmetrical; however, there are numerous cases of single-sided presentation or asymmetrical onset described in the literature that may represent a diagnostic challenge. The aim of this review is to present the evidence of auditory asymmetry in mitochondrial diseases, highlighting the possible presence of cases with atypical presentation. Methods: A review of the English literature to date on hearing loss and mitochondrial diseases was performed using PubMed, Scopus, and Google Scholar databases. The literature review was performed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines for scoping review. Results: A total of 10 full-text articles were included in this review, comprising 25 patients with single-sided or asymmetrical hearing loss associated with mitochondrial disease. Conclusions: Sensorineural hearing loss due to mitochondrial disease can represent a complex diagnostic challenge in cases of asymmetric or unilateral presentation. It is critical to recognize this clinical variant and to diagnose it in daily clinical practice. [ABSTRACT FROM AUTHOR]

Sudden Sensorineural Hearing Loss (SSNHL) is a distressing condition with limited treatment options. This prospective study aimed to investigate the effectiveness of intratympanic Platelet Rich Plasma (PRP) as a salvage treatment for SSNHL. 56 consecutive patients with unilateral SSNHL, unresponsive to conventional treatments, were administered two intratympanic injections of autologous PRP 10 days apart and hearing threshold evaluated after one month. Audiometric data before and after PRP injections revealed a significant mean hearing gain of 11.99 dB which was more in lower frequencies (250, 500, 1000 Hz) reaching upto 13.88dB. Notably, improvement was observed in patients with amino glycoside-induced hearing loss, even after prolonged symptom duration. Additionally, 8 out of 17 patients reported subjective improvement in tinnitus. No serious adverse events were noted, and patients tolerated the procedure well. Intratympanic PRP holds promise as a salvage treatment for SSNHL, particularly in cases where conventional treatments have failed. Further research with larger sample sizes and comparative studies is warranted to establish PRP's role as a primary therapy for SSNHL and explore its long-term benefits. Its role in treatment of tinnitus also needs to be explored. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to evaluate the efficacy of auditory training (AT) in patients with unilateral hearing loss (UHL) using hearing aids (HAs), comparing traditional methods with a new approach involving a wireless remote microphone. Methods: The study included 96 participants, divided into two groups, with ages ranging from 42 to 64 years, comprising both male and female subjects. A clinical trial including consecutive moderate UHL patients was performed at our institution. For the study group, a Roger Pen was used during AT with patients inside a sound-attenuating cabin. Controls followed conventional sessions. Professional speech and language pathologists performed the rehabilitation. Audiological outcomes were measured, including word recognition at signal-to-noise ratios (SNRs) of 0 dB, +5 dB, and +10 dB, to determine the effectiveness of the training. Measurements also included the Speech, Spatial, and Qualities of Hearing Scale to assess perceived auditory abilities. Results: A total of 46 and 50 UHL patients were randomly included in the study and control groups, respectively. No differences were found in terms of sex, age, presence of tinnitus, duration of hearing loss, pure tone average, and speech-in-noise perception without an HA. Following HA fitting and AT, a notable enhancement in the ability to identify speech in noisy environments was observed in the study group. This improvement was significant at SNRs of +5 and +10. When comparing the ability to identify speech in noise using HAs across both groups, it was observed that hearing capabilities post-wireless AT showed a significant improvement at an SNR of +5. Only the study group had a significant improvement in the total Speech, Spatial, and Qualities of Hearing Scale score after the training. Conclusions: In our group of UHL patients, we found significantly better speech-in-noise perception when HA fitting was followed by wireless AT. Wireless AT may facilitate usage of HAs, leading to binaural hearing in UHL patients. Our findings suggest that future interventions might benefit from incorporating wireless technology in AT programs.

Purpose: The purpose of this study was to evaluate the efficacy of auditory training (AT) in patients with unilateral hearing loss (UHL) using hearing aids (HAs), comparing traditional methods with a new approach involving a wireless remote microphone. Methods: The study included 96 participants, divided into two groups, with ages ranging from 42 to 64 years, comprising both male and female subjects. A clinical trial including consecutive moderate UHL patients was performed at our institution. For the study group, a Roger Pen was used during AT with patients inside a sound-attenuating cabin. Controls followed conventional sessions. Professional speech and language pathologists performed the rehabilitation. Audiological outcomes were measured, including word recognition at signal-to-noise ratios (SNRs) of 0 dB, +5 dB, and +10 dB, to determine the effectiveness of the training. Measurements also included the Speech, Spatial, and Qualities of Hearing Scale to assess perceived auditory abilities. Results: A total of 46 and 50 UHL patients were randomly included in the study and control groups, respectively. No differences were found in terms of sex, age, presence of tinnitus, duration of hearing loss, pure tone average, and speech-in-noise perception without an HA. Following HA fitting and AT, a notable enhancement in the ability to identify speech in noisy environments was observed in the study group. This improvement was significant at SNRs of +5 and +10. When comparing the ability to identify speech in noise using HAs across both groups, it was observed that hearing capabilities post-wireless AT showed a significant improvement at an SNR of +5. Only the study group had a significant improvement in the total Speech, Spatial, and Qualities of Hearing Scale score after the training. Conclusions: In our group of UHL patients, we found significantly better speech-in-noise perception when HA fitting was followed by wireless AT. Wireless AT may facilitate usage of HAs, leading to binaural hearing in UHL patients. Our findings suggest that future interventions might benefit from incorporating wireless technology in AT programs. [ABSTRACT FROM AUTHOR]

Copyright of Istanbul Gelisim University Journal of Health Sciences / İstanbul Gelişim Üniversitesi Sağlık Bilimleri Dergisi is the property of Istanbul Gelisim Universitesi Saglik Bilimleri Yuksekokulu and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The authors present the case of a young boy who experienced progressive unilateral hearing loss initially believed to be unrelated to any other medical condition. Methods: The patient received a thorough evaluation, which included a comprehensive battery of audiological tests, a CT scan, and a gadolinium-enhanced MRI. Results: A repeated imaging investigation revealed the presence of a mass that mimicked a vestibular schwannoma (VS), but despite this, the boy was ultimately diagnosed with cerebral manifestations of B-cell acute lymphoblastic leukemia (B-ALL). Conclusions: Cerebral lesions originating from the internal auditory canal are rare in cases of B-ALL. In this case, the initial signs and symptoms of the disease were solely related to the audiovestibular system, making the diagnostic process particularly complicated. Unilateral hearing loss cases may indicate the presence of potentially life-threatening conditions, even if the hearing loss appears to be clinically non-syndromic. For these reasons, unilateral hearing losses necessitate a comprehensive interdisciplinary diagnostic approach from the very start of auditory manifestation and, in particular, if the hearing impairment demonstrates threshold progression. [ABSTRACT FROM AUTHOR]

In acute aminoglycoside ototoxicity of the unilateral inner ear, physical abnormalities, such as nystagmus and postural alteration, are relieved within a few days by neural compensation. To examine exploratory behavior over an extended period, behaviors of freely moving mice after unilateral kanamycin injection into the inner ear were recorded in a home cage environment. The tail was excluded from deep learning-mediated object detection because of its delayed movement relative to the body. All detection results were confirmed using a convolutional neural network classification model. In kanamycin-injected mice, the total distance moved in 15 min increased on postoperative day 3. Furthermore, injured mice turned more frequently toward the healthy side up to 17 days after the surgery. This tendency resulted in increased clockwise movements in home cage recordings. Moreover, tail suspension and twisting toward the healthy side induced a physical sign for up to 14 days after the injury; the mice rapidly rotated with dorsal bending. Our analysis strategy employing deep learning helps to evaluate neuronal compensatory processes for an extended period and is useful for assessing the efficacy of therapeutic interventions.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A vestibular schwannoma is a benign tumor; however, the schwannoma itself and interventions can cause sensorineural hearing loss. Most vestibular schwannomas are unilateral tumors that affect hearing only on one side. Attention has focused on improving the quality of life for patients with unilateral hearing loss and therapeutic interventions to address this issue have been emphasized. Herein, we encountered a patient who was a candidate for hearing preservation surgery based on preoperative findings and had nonserviceable hearing after the surgery, according to the Gardner–Robertson classification. Postoperatively, the patient had decreased listening comprehension and ability to localize sound sources. He was fitted with bilateral hearing aids, and his ability to localize sound sources improved. Although the patient had postoperative nonserviceable hearing on the affected side and age-related hearing loss on the unaffected side, hearing aids in both ears were useful for his daily life. Therefore, the patient was able to maintain a binaural hearing effect and the ability to localize the sound source improved. This report emphasizes the importance of hearing preservation with vestibular schwannomas, and the demand for hearing loss rehabilitation as a postoperative complication can increase, even if hearing loss is nonserviceable. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: Subjects with mild to moderate hearing loss today often receive hearing aids (HA) with open-fitting (OF). In OF, direct sound reaches the eardrums with minimal damping. Due to the required processing delay in digital HA, the amplified HA sound follows some milliseconds later. This process occurs in both ears symmetrically in bilateral HA provision and is likely to have no or minor detrimental effect on binaural hearing. However, the delayed and amplified sound are only present in one ear in cases of unilateral hearing loss provided with one HA. This processing alters interaural timing differences in the resulting ear signals. Methods: In the present study, an experiment with normal-hearing subjects to investigate speech intelligibility in noise with direct and delayed sound was performed to mimic unilateral and bilateral HA provision with OF. Results: The outcomes reveal that these delays affect speech reception thresholds (SRT) in the unilateral OF simulation when presenting speech and noise from different spatial directions. A significant decrease in the median SRT from -18.1 to -14.7 dB SNR is observed when typical HA processing delays are applied. On the other hand, SRT was independent of the delay between direct and delayed sound in the bilateral OF simulation. Discussion: The significant effect emphasizes the development of rapid processing algorithms for unilateral HA provision. [ABSTRACT FROM AUTHOR]

Our study investigated the differences in speech performance and neurophysiological response in groups of school-age children with unilateral hearing loss (UHL) who were otherwise typically developing (TD). We recruited a total of 16 primary school-age children for our study (UHL = 9/TD = 7), who were screened by doctors at Shin Kong Wu-Ho-Su Memorial Hospital. We used the Peabody Picture Vocabulary Test–Revised (PPVT-R) to test word comprehension, and the PPVT-R percentile rank (PR) value was proportional to the auditory memory score (by The Children's Oral Comprehension Test) in both groups. Later, we assessed the latency and amplitude of auditory ERP P300 and found that the latency of auditory ERP P300 in the UHL group was prolonged compared with that in the TD group. Although students with UHL have typical hearing in one ear, based on our results, long-term UHL might be the cause of atypical organization of brain areas responsible for auditory processing or even visual perceptions attributed to speech delay and learning difficulties. [ABSTRACT FROM AUTHOR]

Objective: Cochlear implants (CIs) for single‐sided deafness (SSD) have only been approved for patients 5 years and older despite data supporting that younger children can also benefit from implantation. This study describes our institution's experience with CI for SSD in children 5 years and younger. Study Design: Case series with chart review. Setting: Tertiary referral center. Methods: A case series with chart review identified 19 patients up to age 5 years who underwent CI for SSD between 2014 and 2022. Baseline characteristics, perioperative complications, device usage, and speech outcomes were collected. Results: The median age at CI was 2.8 (range, 1.0‐5.4) years, with 15 (79%) patients being below age 5 at implantation. Etiologies of hearing loss were idiopathic (n = 8), cytomegalovirus (n = 4), enlarged vestibular aqueduct (n = 3), hypoplastic cochlear nerve (n = 3), and meningitis (n = 1). The median preoperative pure‐tone average was 90 (range, 75‐120) and 20 (range, 5‐35) dB eHL in the poor and better hearing ears, respectively. No patients had postoperative complications. Twelve patients achieved consistent device use (average, 9 h/d). Three of the seven who were not consistent users had hypoplastic cochlear nerves and/or developmental delays. The three patients with available preoperative and postoperative speech testing showed significant benefits, and five patients with available postoperative testing demonstrated speech recognition in the implanted ear when isolated from the better ear. Conclusion: CI can safely be performed in younger children with SSD. Patients and families accept early implantation, as evidenced by consistent device use, and derive notable benefits in speech recognition. Candidacy can be broadened to include SSD patients under age five years, particularly individuals without hypoplastic cochlear nerves or developmental delay. [ABSTRACT FROM AUTHOR]

IntroductionChildren 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.MethodsParticipants 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 discussionAs 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.

Objectives To train participants to localize sound using virtual reality (VR) technology, appropriate auditory stimuli that contain accurate spatial cues are essential. The generic head-related transfer function that grounds the programmed spatial audio in VR does not reflect individual variation in monaural spatial cues, which is critical for auditory spatial perception in patients with single-sided deafness (SSD). As binaural difference cues are unavailable, auditory spatial perception is a typical problem in the SSD population and warrants intervention. This study assessed the applicability of binaurally recorded auditory stimuli in VR-based training for sound localization in SSD patients. Methods Sixteen subjects with SSD and 38 normal-hearing (NH) controls underwent VR-based training for sound localization and were assessed 3 weeks after completing training. The VR program incorporated prerecorded auditory stimuli created individually in the SSD group and over an anthropometric model in the NH group. Results Sound localization performance revealed significant improvements in both groups after training, with retained benefits lasting for an additional 3 weeks. Subjective improvements in spatial hearing were confirmed in the SSD group. Conclusion By examining individuals with SSD and NH, VR-based training for sound localization that used binaurally recorded stimuli, measured individually, was found to be effective and beneficial. Furthermore, VR-based training does not require sophisticated instruments or setups. These results suggest that this technique represents a new therapeutic treatment for impaired sound localization.

IntroductionSubjects with mild to moderate hearing loss today often receive hearing aids (HA) with open-fitting (OF). In OF, direct sound reaches the eardrums with minimal damping. Due to the required processing delay in digital HA, the amplified HA sound follows some milliseconds later. This process occurs in both ears symmetrically in bilateral HA provision and is likely to have no or minor detrimental effect on binaural hearing. However, the delayed and amplified sound are only present in one ear in cases of unilateral hearing loss provided with one HA. This processing alters interaural timing differences in the resulting ear signals.MethodsIn the present study, an experiment with normal-hearing subjects to investigate speech intelligibility in noise with direct and delayed sound was performed to mimic unilateral and bilateral HA provision with OF.ResultsThe outcomes reveal that these delays affect speech reception thresholds (SRT) in the unilateral OF simulation when presenting speech and noise from different spatial directions. A significant decrease in the median SRT from –18.1 to –14.7 dB SNR is observed when typical HA processing delays are applied. On the other hand, SRT was independent of the delay between direct and delayed sound in the bilateral OF simulation.DiscussionThe significant effect emphasizes the development of rapid processing algorithms for unilateral HA provision.

Posterior circulation stroke is an uncommon cause of sudden-onset sensorineural hearing loss (SSNHL). Anterior inferior cerebellar artery occlusion results in ipsilateral peripheral audiovestibular dysfunction. Few reports describe posterior circulation stroke presenting with contralateral SSNHL and acute vestibular and focal neurological signs. We present a case of contralateral SSNHL and isolated acute central vestibular dysfunction in the absence of other central focal neurological deficits. To the best of our knowledge, this has not been described to date. The patient was identified to have asymmetrical SSNHL, subtle skew deviation with left head tilt, and significant refixation saccades on video head impulse test despite bilaterally normal vestibulo-ocular reflex gains. Left pontine infarct was suspected and confirmed on magnetic resonance imaging. The patient was treated with an appropriate antiplatelet regimen. We highlight the importance of a thorough clinical diagnostic work-up as posterior circulation strokes with isolated audiovestibular deficits can be easily missed if other significant neurological deficits are absent.

(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. [ABSTRACT FROM AUTHOR]

Identify and evaluate the effectiveness of methods for improving postoperative cochlear implant (CI) hearing performance in subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). Embase, PubMed, Scopus. Systematic review and narrative synthesis. English language studies of adult CI recipients with SSD and AHL reporting a postoperative intervention and comparative audiometric data pertaining to speech in noise, speech in quiet and sound localization were included. 32 studies met criteria for full text review and 6 (n = 81) met final inclusion criteria. Interventions were categorized as: formal auditory training, programming techniques, or hardware optimization. Formal auditory training (n = 10) found no objective improvement in hearing outcomes. Experimental CI maps did not improve audiologic outcomes (n = 9). Programed CI signal delays to improve synchronization demonstrated improved sound localization (n = 12). Hardware optimization, including multidirectional (n = 29) and remote (n = 11) microphones, improved sound localization and speech in noise, respectively. Few studies meeting inclusion criteria and small sample sizes highlight the need for further study. Formal auditory training did not appear to improve hearing outcomes. Programming techniques, such as CI signal delay, and hardware optimization, such as multidirectional and remote microphones, show promise to improve outcomes for SSD and AHL CI users. [ABSTRACT FROM AUTHOR]

To compare cochlear implant (CI) data logging of patients with single-sided deafness (SSD) and bilateral sensorineural hearing loss (biSNHL) in various acoustic environments and study the implications of data logging on auditory performance. Retrospective case control study. Adult CI patients with SSD or biSNHL from 2010 to 2021 with usage data collected at 3-, 6-, and 12-months following device activation were identified. The CI listening environment was defined as speech in noise, speech in quiet, quiet, music or noise. Auditory performance was measured using the CNC word, AzBio sentence tests and the Tinnitus Handicap Index (THI). 60 adults with SSD or biSNHL were included. CI patients with biSNHL wore their devices more than those with SSD at 3-months post-activation (11.18 versus 8.97 hours/day, p = 0.04), though there were no significant differences at 6-12 months. Device usage was highest in the speech in quiet environment. In SSD CI users, there was a positive correlation (p = 0.03) between device use and CNC scores at 12-months and an improvement in THI scores at 12-months (p = 0.0004). CI users with SSD and biSNHL have comparable duration of device usage at longer follow-up periods with greatest device usage recorded in speech in quiet environments. [ABSTRACT FROM AUTHOR]

Copyright of HNO is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

Sensorineural hearing loss (SNHL) is known to be associated with lesions at various sites, including outer hair cell (OHC) and inner hair cell (IHC) injury, as well as auditory dyssynchrony or vestibulocochlear nerve tumors. Therefore, it is important to establish the differential diagnosis of SNHL, which however is particularly challenging in patients with unilateral SNHL. The test battery approach is effective for accurate diagnosis in such cases. In this report, we discuss the usefulness of the test battery approach to accurately detect the site of the lesion in a patient with unilateral hearing loss. A 14-year-9-month-old adolescent who observed decreased hearing sensitivity in the left ear accompanied by difficulty with understanding speech at a distance was diagnosed with unilateral hearing loss in the left ear. In this report, we describe a rare and diagnostically challenging case of unilateral SNHL in a patient with normal OHC function and selective injury to IHCs, which was diagnosed using a test battery approach. [ABSTRACT FROM AUTHOR]

Objectives: Genetic testing is the standard‐of‐care for diagnostic evaluation of bilateral, symmetric, sensorineural hearing loss (HL). We sought to determine the efficacy of a comprehensive genetic testing method, exome sequencing (ES), in a heterogeneous pediatric patient population with bilateral symmetric, bilateral asymmetric, and unilateral HL. Methods: Trio‐based ES was performed for pediatric patients with confirmed HL including those with symmetric, asymmetric, and unilateral HL. Results: ES was completed for 218 probands. A genetic cause was identified for 31.2% of probands (n = 68). The diagnostic rate was 40.7% for bilateral HL, 23.1% for asymmetric HL, and 18.3% for unilateral HL, with syndromic diagnoses made in 20.8%, 33.3%, and 54.5% of cases in each group, respectively. Secondary or incidental findings were identified in 10 families (5.52%). Conclusion: ES is an effective method for genetic diagnosis for HL including phenotypically diverse patients and allows the identification of secondary findings, discovery of deafness‐causing genes, and the potential for efficient data re‐analysis. Level of Evidence: 4 Laryngoscope, 133:2417–2424, 2023 [ABSTRACT FROM AUTHOR]

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.

IntroductionThis 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.MethodsParticipants 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.ResultsIn 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.ConclusionAlthough 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.

ObjectivesThe 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.MethodThis 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.ResultsThe 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.ConclusionThe 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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Although existing studies of audiology first point of contact clinics which screen for retrocochlear pathology have demonstrated positive clinical outcomes, they have provided limited information regarding service impacts. Thus, this study aimed to evaluate both the clinical and health service outcomes of an audiology first point of contact (FPOC) clinic for adults referred to ENT services with suspected retrocochlear pathology. Retrospective cohort study. All 1123 patients referred to the clinic over a 6-year period (2013–2019). Most (73.7%) of the 1123 patients referred to the Retrocochlear Clinic were managed and discharged by the audiologist and did not require ENT appointment. Almost half (43.1%) were directly referred for MRI imaging, of which 4 (1.1%) were diagnosed with Vestibular Schwannoma. Waiting times for first appointments significantly (p < 0.001) reduced from a median of 748 days to 63.5 days over the 6-year period. Attendance rates also significantly (p < 0.001) improved over this time (from 52.2% to 90%). There were no adverse events reported. Of those discharged without attending an ENT appointment, 1.8% were rereferred to ENT within 12 months of discharge. This audiology FPOC Retrocochlear Clinic was shown to be a safe and effective alternative service model. [ABSTRACT FROM AUTHOR]

Background and Aim: Despite more affordable and advanced technologies for early detection of congenital hearing loss, unilateral hearing loss is the prevalent form of hearing loss affecting school-aged children. This study aimed to examine the impact of Words-in-Noise (WIN) training on speech perception of noise in children with unilateral hearing loss. Methods: Thirteen children aged 8 to 12 years with unilateral hearing loss underwent a WIN training program in noise. The participants were tested before and after training on word identification in noise and cortical auditory evoked potentials. Results: A comparison of the mean signal-to-noise ratio 50% between pre- and post-training indicated that signal-to-noise ratio 50% score decreased after training sessions. WIN training reduced the latency in N1 and P2 waves in the Fz electrode and the N1 wave in the Pz electrode and increased the amplitude of the waves in the Fz and Pz electrodes. The observed data suggest that all participants' performance improved on word identification in noise and some electrophysiological parameters. Cortical auditory evoked potentials components changes did not correlate with the WIN scores. Conclusion:The Persian version of the WIN training improved speech perception ability in the presence of competitive noise in children with unilateral hearing loss. Therefore, this software solution can partially solve speech comprehension problems with noise in these children. [ABSTRACT FROM AUTHOR]

Background and Objectives: The speech tests used to evaluate language performance in patients with bilateral deafness (BiD) and cochlear implant (CI) are problematic if applied to patients with single-sided deafness (SSD) because normal ear hearing should be excluded. Thus, we investigated the feasibility of using wireless connection to evaluate speech intelligibility of the CI ear in patients with SSD. Subjects and Methods: Patients with BiD and SSD were administered the word recognition scores (WRS) and speech intelligibility tests using an iPad-based wireless connection and conventional methods. To exclude normal side hearing in patients with SSD, masking noise and "plugged and muffed" method were used in the WRS and speech intelligibility tests, respectively. Results: In patients with BiD, the WRS and speech intelligibility tests results using wireless connection and conventional methods were similar. In patients with SSD, the WRS using masking noise in the normal hearing ear was similar to that of using wireless connection. However, 3 of 11 patients with SSD showed under-masked results if using the "plugged and muffed" method. Conclusions: Speech intelligibility testing using wireless connection is a convenient and reliable method for evaluating CI performance in patients with SSD. The "plugged and muffed" method is not recommended for evaluating CI performance in patients with SSD. [ABSTRACT FROM AUTHOR]

Through a cross-sectional community study of 2044 children aged 2 years, we (1) examine the impact of hearing loss on early spoken expressive vocabulary outcomes and (2) investigate how early intervention-related factors impact expressive vocabulary outcomes in children with hearing loss predominantly identified through universal newborn hearing screening. We used validated parent/caregiver-reported checklists from two longitudinal cohorts (302 children with unilateral or bilateral hearing loss, 1742 children without hearing loss) representing the same population in Victoria, Australia. The impact of hearing loss and amplification-related factors on vocabulary was estimated using g-computation and multivariable linear regression. Children with versus without hearing loss had poorer expressive vocabulary scores, with mean scores for bilateral loss 0.5 (mild loss) to 0.9 (profound loss) standard deviations lower and for unilateral loss marginally (0.1 to 0.3 standard deviations) lower. For children with hearing loss, early intervention and amplification by 3 months, rather than by 6 months or older, resulted in higher expressive vocabulary scores. Children with hearing loss demonstrated delayed spoken expressive vocabulary despite whole-state systems of early detection and intervention. Our findings align with calls to achieve a 1-2-3 month timeline for early hearing detection and intervention benchmarks for screening, identification, and intervention. [ABSTRACT FROM AUTHOR]

Objective: To assess long‐term binaural hearing abilities for cochlear implant (CI) users with unilateral hearing loss (UHL) or asymmetric hearing loss (AHL). Methods: A prospective, longitudinal, repeated measures study was completed at a tertiary referral center evaluating adults with UHL or AHL undergoing cochlear implantation. Binaural hearing abilities were assessed with masked speech recognition tasks using AzBio sentences in a 10‐talker masker. Performance was evaluated as the ability to benefit from spatial release from masking (SRM). SRM was calculated as the difference in scores when the masker was presented toward the CI‐ear (SRMci) or the contralateral ear (SRMcontra) relative to the co‐located condition (0°). Assessments were completed pre‐operatively and at annual intervals out to 5 years post‐activation. Results: Twenty UHL and 19 AHL participants were included in the study. Linear Mixed Models showed significant main effects of interval and group for SRMcontra. There was a significant interaction between interval and group, with UHL participants reaching asymptotic performance early and AHL participants demonstrating continued growth in binaural abilities to 5 years post‐activation. The improvement in SRM showed a significant positive correlation with contralateral unaided hearing thresholds (p = 0.050) as well as age at implantation (p = 0.031). Conclusions: CI recipients with UHL and AHL showed improved SRM with long‐term device use. The time course of improvement varied by cohort, with the UHL cohort reaching asymptotic performance early and the AHL cohort continuing to improve beyond 1 year. Differences between cohorts could be driven by differences in age at implantation as well as contralateral unaided hearing thresholds. Level of Evidence: 3 Laryngoscope, 133:1480–1485, 2023 [ABSTRACT FROM AUTHOR]

Abstract Background Waardenburg syndrome (WS) is the most common form of syndromic deafness with phenotypic and genetic heterogeneity in the Chinese population. This study aimed to clarify the clinical characteristics and the genetic cause in eight Chinese WS families (including three familial and five sporadic cases). Further genotype–phenotype relationships were also investigated. Methods All probands underwent screening for the known WS-related genes including PAX3, SOX10, MITF, EDNRB, EDN3, and SNAI2 using next-generation sequencing to identify disease-causing genes. Further validation using Sanger sequencing was performed. Relevant findings for the associated genotype–phenotype from previous literature were retrospectively analyzed. Result Disease-causing variants were detected in all eight probands by molecular genetic analysis of the WS genes (SOX10(NM_006941.4): c.544_557del, c.553 C > T, c.762delA, c.336G > A; MITF(NM_000248.3): c.626 A > T; PAX3(NM_181459.4): c.838delG, c.452-2 A > G, c.214 A > G). Six mutations (SOX10:c.553 C > T, c.544_557del, c.762delA; PAX3: c.838delG, c.214 A > G; MITF:c.626 A > T) were first reported. Clinical evaluation revealed prominent phenotypic variability in these WS patients. Twelve WS1 cases and five WS2 cases were diagnosed in total. Two probands with SOX10 mutations developed progressive changes in iris color with age, returning from pale blue at birth to normal tan. Additionally, one proband had a renal malformation (horseshoe kidneys).All cases were first described as WS cases. Congenital inner ear malformations were more common, and semicircular malformations were exclusively observed in probands with SOX10 mutations. Unilateral hearing loss occurred more often in cases with PAX3 mutations. Conclusion Our findings helped illuminate the phenotypic and genotypic spectrum of WS in Chinese populations and could contribute to better genetic counseling of WS.

Survivors of childhood bacterial meningitis (BM) often develop hearing impairment (HI). In low- and middle-income countries (LMICs), BM continues to be a significant cause of hearing disability. We assessed hearing among BM survivors using auditory steady-state responses (ASSR), providing frequency-specific estimated audiograms, and examined whether ASSR would provide a greater understanding of BM-induced HI. Survivors from two prospective BM trials (ISRCTN62824827; NCT01540838) from Luanda Children's Hospital were examined in a follow-up visit with a median duration of 26 months after BM. The hearing of 50 BM survivors and 19 control children was evaluated using ASSR and auditory brainstem response (ABR) after interview and neurological and otorhinolaryngological examinations. The median age of survivors was 80 (IQR 86) months. We diagnosed HI (better ear hearing ≥ 26 dB) in 9/50 (18%) children. Five of the fifty survivors (10%) and 14/100 ears (14%) had profound HI (>80 dB). Severe-to-profound HI affected all frequencies steadily, affecting only the ears of BM survivors (18/100 vs. 0/38, p = 0.003). When looking only at the severely or profoundly affected ears, young age, low Glascow coma score, pneumococcal aetiology, and ataxia were associated with a worse hearing outcome. [ABSTRACT FROM AUTHOR]

This study aimed to compare spatial hearing performance between adult individuals with the unilateral sensorineural hearing loss and unilateral loss of horizontal semicircular canal function (termed canal paresis/weakness) in the same ear and adults with normal hearing thresholds and normal vestibular function and to examine associated factors (duration of hearing loss and rate of canal paresis).The study participants consisted of 20 adults (aged 48±11 years) with unilateral sensorineural hearing loss and unilateral canal paresis (unilateral weakness≥25%) in the same ear. The control group comprised 25 adults (aged 45±13 years) with normal hearing and a unilateral weakness rate below 25%. Pure tone audiometry, bithermal binaural air caloric test, Turkish Spatial Hearing Questionnaire (T-SHQ), and Standardized Mini-Mental State Exam were applied to all the individuals. When the performance of the participants in T-SHQ was examined both in terms of the subscales and the total scale, there was a statistically significant difference between the two groups in relation to the scores. A statistically significant, high, negative correlation was detected between the duration of hearing loss, the rate of canal paresis and all the subscale scores and total score of T-SHQ. According to these results, as the duration of hearing loss increased, the scores obtained from the questionnaire decreased. As the rate of canal paresis increased, vestibular involvement increased, and the T-SHQ score decreased. This study showed that adults with unilateral hearing loss and unilateral canal paresis in the same ear had lower spatial hearing performance than those with normal hearing and balance. [ABSTRACT FROM AUTHOR]

Unilateral hearing loss (UHL) leads to an alteration of binaural cues resulting in a significant increment of spatial errors in the horizontal plane. In this study, nineteen patients with UHL were recruited and randomized in a cross-over design into two groups; a first group (n = 9) that received spatial audiovisual training in the first session and a non-spatial audiovisual training in the second session (2 to 4 weeks after the first session). A second group (n = 10) received the same training in the opposite order (non-spatial and then spatial). A sound localization test using head-pointing (LOCATEST) was completed prior to and following each training session. The results showed a significant decrease in head-pointing localization errors after spatial training for group 1 (24.85° ± 15.8° vs. 16.17° ± 11.28°; p < 0.001). The number of head movements during the spatial training for the 19 participants did not change (p = 0.79); nonetheless, the hand-pointing errors and reaction times significantly decreased at the end of the spatial training (p < 0.001). This study suggests that audiovisual spatial training can improve and induce spatial adaptation to a monaural deficit through the optimization of effective head movements. Virtual reality systems are relevant tools that can be used in clinics to develop training programs for patients with hearing impairments. [ABSTRACT FROM AUTHOR]

This work examined speech and language skills of a cohort of children with unilateral and bilateral mild/moderate hearing loss at time of discharge from preschool therapy services as they were transitioning to the school system. The study consisted of a retrospective chart review of children born between 2003 and 2011 and identified with a unilateral or a bilateral hearing loss up to 55 dB. Speech and language outcomes measures were obtained from three different tests commonly administered as part of the therapy protocol. Comparisons were made with normative data. A total of 238 children were diagnosed with a permanent unilateral or bilateral hearing loss by six years of age. Speech language standardised test scores were available for 74 children (65 English-speaking, 9 French-speaking). While overall results on all outcome measures remained within the expected range for children with typical hearing, of the 65 children with speech and language outcomes, 13 of 52 (25.0%) and 15 of 53 (28.3%) obtained lower scores on expressive communication and articulation, respectively, compared to children with typical hearing. These children were identified and fitted with hearing aids at a later age. Findings of this study suggest possible benefits of early identification and intervention and warrant additional research on early-identified children with unilateral or mild/moderate bilateral hearing loss. [ABSTRACT FROM AUTHOR]

IntroductionWe 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.MethodsEnrolment 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).ResultsAcross 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.DiscussionThis 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.

IntroductionThe aim of this study was to quantify the amount of deterioration in hearing and to document the trajectory of hearing loss in early identified children with unilateral hearing loss (UHL). We also examined whether clinical characteristics were associated with the likelihood of having progressive hearing loss.MethodsAs part of the Mild and Unilateral Hearing Loss Study, we followed a population-based cohort of 177 children diagnosed with UHL from 2003 to 2018. We applied linear mixed models to examine hearing trends over time including the average amount of change in hearing. Logistic regression models were used to examine the relationship between age and severity at diagnosis, etiology, and the likelihood of progressive loss and amount of deterioration in hearing.ResultsThe median age of the children at diagnosis was 4.1 months (IQR 2.1, 53.9) and follow-up time was 58.9 months (35.6, 92.0). Average hearing loss in the impaired ear was 58.8 dB HL (SD 28.5). Over the 16-year period, 47.5% (84/177) of children showed deterioration in hearing in one or both ears from their initial diagnostic assessment to most recent assessment including 21 (11.9%) who developed bilateral hearing loss. Average deterioration in the impaired ear ranged from 27 to 31 dB with little variation across frequencies. Deterioration resulted in a change in category of severity for 67.5% (52/77) of the children. Analysis for children who were followed for at least 8 years showed that most lost a significant amount of hearing rapidly in the first 4 years, with the decrease stabilizing and showing a plateau in the last 4 years. Age and severity at diagnosis were not significantly associated with progressive/stable loss after adjusting for time since diagnosis. Etiologic factors (ENT external/middle ear anomalies, inner ear anomalies, syndromic hearing loss, hereditary/genetic) were found to be positively associated with stable hearing loss.ConclusionAlmost half of children with UHL are at risk for deterioration in hearing in one or both ears. Most deterioration occurs within the first 4 years following diagnosis. Most children did not experience sudden “large” drops in hearing but more gradual decrease over time. These results suggest that careful monitoring of UHL especially in the early years is important to ensure optimal benefit from early hearing loss detection.

Numerous studies have shown that children with mild bilateral (MBHL) or unilateral hearing loss (UHL) experience speech perception difficulties in poor acoustics. Much of the research in this area has been conducted via laboratory studies using speech-recognition tasks with a single talker and presentation via earphones and/or from a loudspeaker located directly in front of the listener. Real-world speech understanding is more complex, however, and these children may need to exert greater effort than their peers with normal hearing to understand speech, potentially impacting progress in a number of developmental areas. This article discusses issues and research relative to speech understanding in complex environments for children with MBHL or UHL and implications for real-world listening and understanding. [ABSTRACT FROM AUTHOR]

Children with unilateral sensorineural hearing loss (uSNHL) have a high risk of speech-language delays and academic difficulties. Still, challenges remain in the diagnosis of uSNHL. With a prospective cross-sectional design, 20 infants were consecutively recruited from a universal newborn hearing screening program and invited to genetic testing. Eighteen of the subjects agreed to genetic testing, 15 subjects with OtoSCOPE® v.9 screening 224 genes, and four subjects underwent targeted testing, screening for chromosomal abnormalities or 105–137 gene mutations. The genetic results were described together with the 20 infants' previously published auditory profiles and imaging results. Genetic causes for the uSNHL were found in 28% of subjects (5/18) including CHARGE syndrome (CHD7), autosomal recessive non-syndromic hearing loss (GJB2), Townes–Brocks syndrome (SALL1), Pendred Syndrome (SLC26A4) and Chromosome 8P inverted duplication and deletion syndrome. In subjects with comorbidities (malformation of fingers, anus, brain, and heart), 100% were diagnosed with a genetic cause for uSNHL (3/3 subjects), while 13% (2/15 subjects) were diagnosed without comorbidities observed at birth (p = 0.002). Genetic testing for congenital uSNHL is currently efficient for alleged syndromes, whereas genetic variants for non-syndromic congenital uSNHL need further research. [ABSTRACT FROM AUTHOR]

Objectives: Fatigue is frequently observed in children with chronic diseases and can affect the quality of life (QoL). However, research in children with unilateral hearing loss (UHL) is scarce. Subsequently, no studies investigated the effects of hearing aids on fatigue in children. This study investigates subjective fatigue and hearing‐related QoL in children with UHL. Furthermore, it evaluates the influence of hearing aids, subject‐specific factors, and respondent‐type on subjective fatigue. Study Design: A cross‐sectional study was conducted from June 2020 until September 2020 at the department of otorhinolaryngology in a tertiary referral center. Methods: The primary outcome was the difference in subjective fatigue and hearing‐related QoL between children with unaided UHL, aided UHL, and normal hearing. Subjective fatigue and hearing‐related QoL were measured using the Pediatric Quality of Life Inventory™ Multidimensional Fatigue Scale (PedsQL™‐MFS) and Hearing Environments and Reflection on Quality of Life (HEAR‐QL™) questionnaires. Results: Along with 36 aided children with UHL, 34 unaided and 36 normal‐hearing children were included. Child reports revealed significantly more cognitive fatigue in children with aided UHL than children with normal hearing (median difference 12.5, P =.013). Parents reported more fatigue in children with UHL compared to normal‐hearing siblings. Especially children with aided UHL seemed at increased risk for fatigue. Children with UHL scored lower on hearing‐related QoL than children with normal hearing. No apparent differences were found in fatigue and QoL between children with unaided and aided UHL. Conclusion: Children with unaided and even aided UHL seem to experience more subjective fatigue and lower hearing‐related QoL than children with normal hearing. Prospective longitudinal studies are required to investigate the influence of hearing aids on fatigue and QoL in individual patients. Level of Evidence: 3 Laryngoscope, 2021 Laryngoscope, 133:189–198, 2023 [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]