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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.

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Middle-ear salivary gland choristoma (SGCh) is a rare, benign tumor that causes conductive hearing loss owing to middle-ear morphological abnormalities. Early diagnosis is challenging, and surgical resection is indispensable for a definitive diagnosis. We report the case of a 3-year-old boy diagnosed with middle-ear SGCh during the follow-up period for left-sided hearing loss discovered at newborn hearing screening (NHS). Long-term follow-up after the NHS result, subsequent computed tomography/magnetic resonance imaging, and surgical resection led to its relatively early diagnosis and treatment.

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Congenital or early-onset unilateral hearing loss (UHL) can disrupt the normal development of the auditory system. In extreme cases of UHL (i.e., single sided deafness), consistent cochlear implant use during sensitive periods resulted in cortical reorganization that partially reversed the detrimental effects of unilateral sensory deprivation. There is a gap in knowledge, however, regarding cortical plasticity i.e. the brain's capacity to adapt, reorganize, and develop binaural pathways in milder degrees of UHL rehabilitated by a hearing aid (HA). The current study was set to investigate early-stage cortical processing and electrophysiological manifestations of binaural processing by means of cortical auditory evoked potentials (CAEPs) to speech sounds, in children with moderate to severe-to-profound UHL using a HA. Fourteen children with UHL (CHwUHL), 6-14 years old consistently using a HA for 3.5 (±2.3) years participated in the study. CAEPs were elicited to the speech sounds /m/, /g/, and /t/ in three listening conditions: monaural [Normal hearing (NH), HA], and bilateral [BI (NH + HA)]. Results indicated age-appropriate CAEP morphology in the NH and BI listening conditions in all children. In the HA listening condition: (1) CAEPs showed similar morphology to that found in the NH listening condition, however, the mature morphology observed in older children in the NH listening condition was not evident; (2) P1 was elicited in all but two children with severe-to-profound hearing loss, to at least one speech stimuli, indicating effective audibility; (3) A significant mismatch in timing and synchrony between the NH and HA ear was found; (4) P1 was sensitive to the acoustic features of the eliciting stimulus and to the amplification characteristics of the HA. Finally, a cortical binaural interaction component (BIC) was derived in most children. In conclusion, the current study provides first-time evidence for cortical plasticity and partial reversal of the detrimental effects of moderate to severe-to-profound UHL rehabilitated by a HA. The derivation of a cortical biomarker of binaural processing implies that functional binaural pathways can develop when sufficient auditory input is provided to the affected ear. CAEPs may thus serve as a clinical tool for assessing, monitoring, and managing CHwUHL using a HA.

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OBJECTIVE(S): FDA-approved indications for cochlear implantation include patients with severe-to-profound unilateral hearing loss (UHL) or asymmetric hearing loss (AHL); however, these indications are not covered for Medicare beneficiaries. We assessed the outcomes of cochlear implant (CI) use for older adults with UHL or AHL. METHODS: Eighteen older adults (≥65 years of age at surgery) with UHL/AHL participated in a prospective, longitudinal investigation evaluating outcomes of CI use. Speech recognition for the affected ear was evaluated with consonant-nucleus-consonant (CNC) words. Spatial hearing was assessed with measures of sound source localization and sentence recognition in noise. The target sentence was presented from the front and the masker was either co-located with the target (SoNo), presented toward the affected ear (SoNci) or contralateral ear (SoNcontra). Perceived benefit was assessed with the Speech, Spatial, and Qualities of Hearing scale (SSQ) and the Tinnitus Handicap Inventory (THI). RESULTS: Participants experienced significant improvements with CI use for CNC words (mean [SD]; preop: 8% [10%], 1 yr: 51% [22%], 5 yr: 50% [19%]), masked sentence recognition (SoNcontra preop: 5% [6%], 1 yr: 22% [15%], 5 yr: 41% [14%]), and localization (preop: 76° [18°], 1 yr: 40° [11°], 5 yr: 41° [14°]), and reported significant improvements in hearing abilities (SSQ Spatial Hearing preop: 3 [1], 1 yr: 6 [2], 5 yr: 6 [2]) and tinnitus severity (THI preop: 16 [18], 1 yr: 4 [14], 5 yr: 6 [12]). CONCLUSION: Older adults with UHL/AHL experience significant improvements in speech recognition, spatial hearing, and subjective perceptions (e.g., hearing abilities and tinnitus severity) with a CI as compared to pre-operative abilities. LEVEL OF EVIDENCE: IV Laryngoscope, 2024.

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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.

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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.

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BACKGROUND: This study compared hearing outcomes with use of personal sound amplification products (PSAPs) and hearing aids (HAs) in patients with moderate to moderately severe unilateral hearing loss. METHODS: Thirty-nine participants were prospectively enrolled, and randomly assigned to use either one HA (basic or premium type) or one PSAP (basic or high-end type) for the first 8 weeks and then the other device for the following 8 weeks. Participants underwent a battery of examinations at three visits, including sound-field audiometry, word recognition score (WRS), speech perception in quiet and in noise, real-ear measurement, and self-report questionnaires. RESULTS: Functional gain was significantly higher with HAs across all frequencies (P < 0.001). While both PSAPs and HAs improved WRS from the unaided condition, HAs were superior to PSAPs. The speech recognition threshold in quiet conditions and signal-to-noise ratio in noisy conditions were significantly lower in the HA-aided condition than in the PSAP-aided condition, and in the PSAP-aided condition than in the unaided condition. Subjective satisfaction also favored HAs than PSAPs in questionnaires, Abbreviated Profile of Hearing Aid Benefit, International Outcome Inventory for Hearing Aids, and Host Institutional Questionnaire. CONCLUSION: While PSAPs provide some benefit for moderate to moderately severe unilateral hearing loss, HAs are more effective. This underscores the potential role of PSAPs as an accessible, affordable first-line intervention in hearing rehabilitation, particularly for individuals facing challenges in accessing conventional HAs.

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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.

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BACKGROUND: Many studies have shown increased academic problems in children with unilateral hearing loss (UHL). However, whether hearing devices can ameliorate the educational difficulties associated with UHL is not well studied. Therefore, the objective of the current systematic review was to answer the question: do nonsurgical amplification devices, bone-anchored hearing aids, and/or cochlear implants improve academic outcomes in school-aged children and adolescents with UHL? METHODS: Embase, MEDLINE, Scopus, CINAHL, APA PsycInfo, ClinicalTrials.gov, and Cochrane databases were searched from inception to December 21, 2022. Published, peer-reviewed studies comparing academic outcomes in patients with UHL aged ≥5 and ≤19 years with and without hearing devices (nonsurgical amplification devices, bone-anchored hearing aids, or cochlear implants) were included. Results of studies were qualitatively synthesized, and the risk of bias was evaluated with the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool. RESULTS: A total of 5,644 non-duplicate publications were identified by the search, and four studies were included for synthesis, every one of which was investigating nonsurgical amplification. One small, single-arm study demonstrated significant improvement in subjective classroom listening difficulties after a 3- to 4-month trial with a behind-the-ear hearing aid. The other three studies of nonsurgical amplification devices showed no benefit across multiple academic outcomes with FM systems and conventional and CROS-style hearing aids. DISCUSSION: The small sample sizes, heterogeneous and/or ill-defined study samples, and overall low quality of the available literature ultimately make it hard to draw definitive conclusions regarding nonsurgical amplification devices' effectiveness in improving academic outcomes in children with UHL. No articles were identified that studied cochlear implants or bone-anchored hearing aids. Further studies with high-quality study design, large sample sizes, and long-term follow-up are needed to answer this clinically important question.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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