Dissociated Representation of Binaural Cues in Single-Sided Deafness: Implications for Cochlear Implantation.

Congenital single-sided deafness (SSD) leads to an aural preference syndrome that is characterized by overrepresentation of the hearing ear in the auditory system. Cochlear implantation (CI) of the deaf ear is an effective treatment for SSD. However, the newly introduced auditory input in congenital SSD often does not reach expectations in late-implanted CI recipients with respect to binaural hearing and speech perception. In a previous study, a reduction of the interaural time difference (ITD) sensitivity has been shown in unilaterally congenitally deaf cats (uCDCs). In the present study, we focused on the interaural level difference (ILD) processing in the primary auditory cortex. The uCDC group was compared with hearing cats (HCs) and bilaterally congenitally deaf cats (CDCs). The ILD representation was reorganized, replacing the preference for the contralateral ear with a preference for the hearing ear, regardless of the cortical hemisphere. In accordance with the previous study, uCDCs were less sensitive to interaural time differences than HCs, resulting in unmodulated ITD responses, thus lacking directional information. Such incongruent ITDs and ILDs cannot be integrated for binaural sound source localization. In normal hearing, the predominant effect of each ear is excitation of the auditory cortex in the contralateral cortical hemisphere and inhibition in the ipsilateral hemisphere. In SSD, however, auditory pathways reorganized such that the hearing ear produced greater excitation in both cortical hemispheres and the deaf ear produced weaker excitation and preserved inhibition in both cortical hemispheres.

Structural connectivity changes in unilateral hearing loss.

Network connectivity, as mapped by the whole brain connectome, plays a crucial role in regulating auditory function. Auditory deprivation such as unilateral hearing loss might alter structural network connectivity; however, these potential alterations are poorly understood. Thirty-seven acoustic neuroma patients with unilateral hearing loss (19 left-sided and 18 right-sided) and 19 healthy controls underwent diffusion-weighted and T1-weighted imaging to assess edge strength, node strength, and global efficiency of the structural connectome. Edge strength was estimated by pair-wise normalized streamline density from tractography and connectomics. Node strength and global efficiency were calculated through graph theory analysis of the connectome. Pure-tone audiometry and word recognition scores were used to correlate the degree and duration of unilateral hearing loss with node strength and global efficiency. We demonstrate significantly stronger edge strength and node strength through the visual network, weaker edge strength and node strength in the somatomotor network, and stronger global efficiency in the unilateral hearing loss patients. No discernible correlations were observed between the degree and duration of unilateral hearing loss and the measures of node strength or global efficiency. These findings contribute to our understanding of the role of structural connectivity in hearing by facilitating visual network upregulation and somatomotor network downregulation after unilateral hearing loss.

Factors Predictive of Binaural Hearing Restoration by Cochlear Implant in Single-Sided Deafness.

INTRODUCTION: Cochlear implants (CIs) can restore binaural hearing in cases of single-sided deafness (SSD). However, studies with a high level of evidence in support of this phenomenon are lacking. The aim of this study is to analyze the effectiveness of CIs using several spatialized speech-in-noise tests and to identify potential predictors of successful surgery. METHODS: Ten cases underwent standard CI surgery (MEDEL-Flex24). The speech-in-noise test was used in three different spatial configurations. The noise was presented from the front (N0), toward the CI (NCI), and toward the ear (Near), while the speech was always from the front (S0). For each test, the speech-to-noise ratio at 50% intelligibility (SNR50) was evaluated. Seven different effects were assessed (summation, head shadow [HS], speech released of masking [SRM], and squelch for the CI and for the ear). RESULTS: A significant summation effect of 1.5 dB was observed. Contralateral PTA was positively correlated with S0N0-B and S0NCI-B (CIon and unplugged ear). S0N0-B results were positively correlated with S0N0-CIoff (p < 0.0001) and with S0Near-CIoff results (p = 0.004). A significant positive correlation was found between delay post-activation and HS gain for the CI (p = 0.005). Finally, the HS was negatively correlated with the squelch effect for the ear. CONCLUSION: CI benefits patients with SSD in noise and can improve the threshold for detecting low-level noise. Contralateral PTA could predict good postoperative results. Simple tests performed preoperatively can predict the likelihood of surgical success in reversing SSD.

Masked Speech Perception with Bone Conduction Device, Contralateral Routing of Signals Hearing Aid, and Cochlear Implant Use in Adults with Single-Sided Deafness: A Prospective Hearing Device Comparison using a Unified Testing Framework.

INTRODUCTION: For the treatment of single-sided deafness (SSD), common treatment choices include a contralateral routing of signals (CROS) hearing aid, a bone conduction device (BCD), and a cochlear implant (CI). The primary aim of this study was to compare speech understanding in noise and binaural benefits in adults with postlingual SSD between preoperative unaided baseline, preoperative CROS and BCD trial devices, and CI, following recommendations from a consensus protocol. In addition, we investigated the effect of masker type on speech understanding. METHODS: This was a prospective study with twelve participants. Binaural effects of head shadow, squelch, summation, and spatial release from masking were assessed by measuring speech reception thresholds (SRTs) in five different spatial target-masker configurations using two different maskers: two-talker babble (TTB), and speech-shaped noise (SSN). Preoperatively, participants were assessed unaided and with CROS and BCD trial devices. After cochlear implantation, participants were assessed at 1, 3, and 6 months post-activation. RESULTS: For TTB, significant improvements in SRT with a CI relative to preoperatively unaided were found in all spatial configurations. With CI at 6 months, median benefits were 7.8 dB in SSSDNAH and 5.1 dB in S0NAH (head shadow), 3.4 dB in S0N0 (summation), and 4.6 dB in S0NSSD and 5.1 dB in SAHNSSD (squelch). CROS yielded a significant head shadow benefit of 2.4 dB in SSSDNAH and a significant deterioration in squelch of 2.5 dB in S0NSSD and SAHNSSD, but no summation effect. With BCD, there was a significant summation benefit of 1.5 dB, but no head shadow nor squelch effect. For SSN, significant improvements in SRT with CI compared to preoperatively unaided were found in three spatial configurations. Median benefits with CI at 6 months were: 8.5 dB in SSSDNAH and 4.6 dB in S0NAH (head shadow), 1.4 dB in S0N0 (summation), but no squelch. CROS showed a significant head shadow benefit of 1.7 dB in SSSDNAH, but no summation effect, and a significant deterioration in squelch of 2.9 dB in S0NSSD and 3.2 dB in SAHNSSD. With BCD, no binaural effect was obtained. Longitudinally, we found significant head shadow benefits with a CI in SSSDNAH in both maskers at all postoperative intervals and in S0NAH at 3 and 6 months post-activation. CONCLUSION: With a CI, a clear benefit for masked speech perception was observed for all binaural effects. Benefits with CROS and BCD were more limited. CROS usage was detrimental to the squelch effect.

Association Between Hearing Loss, Asymmetric Hearing, and Postural Instability.

OBJECTIVES: Recent studies have suggested that older adults with hearing loss (HL) are at a greater risk of postural instability than those with normal hearing. However, little is known regarding this association in middle-aged individuals. The relationships between HL laterality, asymmetric hearing, and posture control are similarly unclear. The purpose of this study was to investigate the effects of hearing status on postural control and to explore the dose-response relationship between the hearing threshold and postural instability risk in middle-aged adults. DESIGN: This cross-sectional study included 1308 participants aged 40 to 69 years with complete audiometric and standing balance function data from the 2001-2004 National Health and Nutrition Examination Survey. Speech-frequency HL was defined as a pure-tone average at 0.5, 1, 2, and 4 kHz of >25 dB in the better-hearing ear; high-frequency HL was defined as a pure-tone average at 3, 4, and 6 kHz of >25 dB. Asymmetric hearing was defined as a difference in the pure-tone average >15 dB between ears. Postural instability was defined as participants ending the modified Romberg test in condition 4. RESULTS: After adjustment for sociodemographic variables, lifestyle, and comorbidities, speech-frequency HL, except for unilateral HL, was associated with increased postural instability (mild HL: odds ratio [OR], 2.33; 95% confidence interval [CI], 1.25-4.35; moderate-to-severe HL: OR, 3.59; 95% CI, 1.61-8.03). Compared with individuals with normal bilateral hearing, participants with bilateral HL also showed a higher risk of postural instability (OR, 2.88; 95% CI, 1.61-5.14). The OR for postural instability among participants with asymmetric hearing compared with those with symmetric hearing was 2.75 (95% CI, 1.37-5.52). Furthermore, each 10 dB increase in the speech-frequency hearing threshold was associated with a 44% higher risk of postural instability. CONCLUSIONS: Hearing loss is associated with poorer postural control. Individuals with asymmetric hearing have a higher postural instability risk compared with those with symmetric hearing. Further studies are needed to confirm these findings and the causality. Moreover, future studies are warranted to assess whether hearing aids are beneficial for the restoration of impaired balance functions.

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

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

Expanding the indications for the bone anchored hearing system (BAHS) in patients with single sided deafness.

PURPOSE: Provide data to support expansion of FDA indications for the Bone anchored hearing system (BAHS). MATERIALS AND METHODS: This retrospective study in a tertiary otologic referral center included106 consecutive subjects who were implanted with a Bone Anchored Hearing System (BAHS) between January 2009 and January 2015 for single sided deafness. Subjects were divided into three groups by bone conduction pure tone average (PTA) of the better hearing ear: 0-20 dB (group 1), 21-40 dB (group 2) and 41-55 dB (group 3). All patients underwent BAHS implantation. Speech perception data (Hearing In Noise Test and Consonant-Nucleus-Consonant testing) was collected before and after surgical intervention. Patient-reported quality of life measures were obtained at least 6 months after activation. These included the Abbreviated Profile of Hearing Aid Benefit and Glasgow Benefit Inventory. RESULTS: All three groups of subjects demonstrated statistically significant improvement in outcome measures following BAHS. Subject reported quality of life outcome measures demonstrated significant improvement in disability from hearing loss and in quality of life. CONCLUSIONS: Patients with single sided deafness who have bone conduction thresholds worse than 20 dB in their contralateral ear are still able to benefit significantly from BAHS.

Rehabilitation for unilateral deafness - Narrative review comparing a novel bone conduction solution with existing options.

Patients with single sided deafness (SSD) struggle with sound localization and speech in noise. Existing treatment options include contralateral routing of signal (CROS) systems, percutaneous bone conduction hearing devices (BCHDs), passive transcutaneous BCHDs, active BCHDs, and cochlear implants. Implanted devices provide benefits in speech in noise compared to CROS devices. Percutaneous BCHDs transmit sound efficiently but have aesthetic drawbacks and skin complications. Scalp attenuation impacts passive transcutaneous BCHD performance. Active BCHDs overcome these issues and provide benefits for speech in noise. Cochlear implantation is the only existing option that restores binaural input but introduces electrical rather than acoustic stimuli to the deaf ear. Active BCHDs have been designed to maintain efficient sound transmission and avoid chronic skin irritation and cosmetic concerns that may occur with percutaneous BCHDs. Cochlear implantation may be a superior option for recently deafened SSD patients, though this requires further study. The duration of deafness, patient age and comorbidities, and a shared decision-making model among patients, surgeons, and audiologists should be considered in device selection. The aim of this manuscript is to review available devices, discuss surgical considerations for implantable devices, review available published results for speech in noise and sound quality with each device, and provide an overview to guide shared decision making for patients and providers. This review consolidates available literature and reviews experience with a newer active transcutaneous active BCHD available for use in the SSD population.

Another 'BEE'? - Brain-Eye-Ear (BEE) Disease Secondary to HbSC Disease Masquerading as Multiple Sclerosis.

Recurrent episodes of neurological dysfunction and white matter lesions in a young adult raise suspicion for multiple sclerosis (MS). However, occlusive retinopathy, hearing loss and absence of CSF oligoclonal bands are atypical for MS and should make the clinician consider an alternative diagnosis. We describe a man with hearing loss, visual signs and symptoms, and an accumulating burden of brain lesions, who was treated for a clinical diagnosis of MS for nearly two decades. Genetic testing revealed a unifying diagnosis.

Minimal invasive pocket technique for magnet bone implant hearing aid without fixation.

INTRODUCTION: The BAHA (bone-anchored hearing aid) Attract is a magnetic transcutaneous bone conduction device anchored into the temporal bone. The standard surgical technique for BAHA Attract is a multi-tools time-consuming process, which requires a large cutaneous incision. The objective of this study is to describe and test the feasibility of a minimally invasive pocket (MIP) technique for Magnet Bone Implant Hearing Aid (MBIHA) with a modified magnet of BAHA Attract without fixation and without any tissue reduction. We use a 3-cm vertical skin incision and a subperiosteal pocket. METHOD: A study of 10 patients with conductive or mixed hearing loss who benefited from a MBIHA using the MIP technique is presented. The pure tone average (PTA) (dB) for air-conduction thresholds and the speech recognition threshold (SRT) (dB) in speech audiometry in quiet are calculated. The Entific Medical Systems (EMS) questionnaire and the postoperative clinical outcomes are realized. RESULTS: We found a significant improvement of 33.8 dB on average for the PTA and 44.8 dB for the SRT with MBIHA at 3 months, compared with unaided situation. No implant was removed or displaced after 2 years of follow-up. The skin condition remains intact in all the cases. CONCLUSION: The minimally subperiosteal pocket surgical technique MIP without fixation and with tissue preservation for the MBIHA is safe, rapid and effective for patients with conductive or mixed hearing loss. It opens new perspectives of development and modify conventional concept in magnetic coupling of bone-conducted device.

The effect of an active transcutaneous bone conduction device on spatial release from masking.

Objective: The aim was to quantify the effect of the experimental active transcutaneous Bone Conduction Implant (BCI) on spatial release from masking (SRM) in subjects with bilateral or unilateral conductive and mixed hearing loss.Design: Measurements were performed in a sound booth with five loudspeakers at 0°, +/-30° and +/-150° azimuth. Target speech was presented frontally, and interfering speech from either the front (co-located) or surrounding (separated) loudspeakers. SRM was calculated as the difference between the separated and the co-located speech recognition threshold (SRT).Study Sample: Twelve patients (aged 22-76 years) unilaterally implanted with the BCI were included.Results: A positive SRM, reflecting a benefit of spatially separating interferers from target speech, existed for all subjects in unaided condition, and for nine subjects (75%) in aided condition. Aided SRM was lower compared to unaided in nine of the subjects. There was no difference in SRM between patients with bilateral and unilateral hearing loss. In aided situation, SRT improved only for patients with bilateral hearing loss.Conclusions: The BCI fitted unilaterally in patients with bilateral or unilateral conductive/mixed hearing loss seems to reduce SRM. However, data indicates that SRT is improved or maintained for patients with bilateral and unilateral hearing loss, respectively.

Auditory, Social, and Behavioral Skills of Children With Unilateral/Mild Hearing Loss.

This study explored the impact of mild bilateral or unilateral hearing loss on auditory, social, and behavior skills in early school-aged children. Thirty-two children (aged 5-9 years) were evaluated with parent and teacher questionnaires. Most outcomes were within the range of expected scores. However, functional auditory skills were below published results for children with typical hearing. On the social skills scale, about 21.4% (parent-reported) and 20.0% (teacher-reported) of children were below one standard deviation (SD) of the normative mean (i.e., a standard score below 85). On the parent-reported behavior test, over a quarter of children scored beyond 1 SD on some subscales. Laterality of hearing loss had no effect on outcomes (p > .05). Agreement between parents and teachers varied from poor (intraclass correlation coefficient [ICC]: .162) to moderate (ICC: .448). Results indicate that these children are functioning in most areas like their peers with typical hearing. Additional research on this population of children who may benefit from early identification and amplification is warranted.

Downward cross-modal plasticity in single-sided deafness.

The auditory cortex has been shown to participate in visual processing in individuals with complete auditory deprivation. However, it remains unclear whether partial hearing deprivation like single-sided deafness (SSD) leads to similar cross-modal plasticity. To investigate this, we enrolled individuals with long-term SSD, into functional MRI scans under resting-state and a visuo-spatial working memory task. Contrary to previous findings in bilateral deafness, our study revealed decreased activation in the auditory cortex in both left (LSSD) and right (RSSD) single-sided deafness compared to normal hearing controls, with statistical significance in RSSD. The degree of involvement was correlated with residual hearing ability in RSSD. These observations suggest that SSD can lead to a downward cross-modal plasticity: the more hearing ability lost, the fewer brain resources in the auditory cortex can be applied to visual tasks. In addition, the fronto-parietal cortex was observed to be less activated during the visual task in RSSD while the resting-state fMRI revealed increased functional connectivity between the fronto-parietal cortex and the auditory cortex, suggesting fronto-parietal resources may be recruited less by vision but more by hearing. The LSSD showed a similar alteration trend with RSSD, but without statistical significance. Together these findings may indicate that when hearing is partially deprived in SSD, there may be redistribution for brain resources between hearing and vision, and vision tends to allocate less resources. Our findings in this pilot study of unilateral auditory-deprived individuals enrich the understanding of cross-modal plasticity in the brain.

Data Logging Evidence of Cochlear Implant Use in Single-Sided and Bilateral Deafness.

PURPOSE: This study analyses data logs in order to investigate the usage pattern of cochlear implant (CI) recipients with single-sided deafness (SSD-CI) and bilaterally deaf, uni- or bilaterally implanted CI recipients (Uni-CI and Bil-CI). Data logging is available from SCAN, an automated auditory scene classifier which categorizes auditory input into 6 listening environments. METHODS: CI usage data were retrospectively available from data logs of 206 CI recipients using the Nucleus 6 system obtained between January 2013 and June 2015. For all recipients, we analysed time on air and time spent in the listening environments. For statistical analysis, we matched the CI recipients according to age and duration of CI experience and classified them into 4 age groups. RESULTS: SSD-CI showed a similar time on air compared to Uni- and Bil-CI. Usage behaviour of SSD-CI was comparable to Uni- and Bil-CI regarding exposure to music, speech in quiet and speech in noise. With increasing age, exposure to quiet increased and exposure to music decreased across all CI recipient groups in relation to time on air. CONCLUSION: In total, the CI usage pattern of SSD-CI is comparable for the majority of listening environments and age groups to that of Uni- and Bil-CI. The results of our study show that SSD-CI benefit equally from CI implantation.

Mechanisms of Localization and Speech Perception with Colocated and Spatially Separated Noise and Speech Maskers Under Single-Sided Deafness with a Cochlear Implant.

OBJECTIVES: This study tested listeners with a cochlear implant (CI) in one ear and acoustic hearing in the other ear, to assess their ability to localize sound and to understand speech in collocated or spatially separated noise or speech maskers. DESIGN: Eight CI listeners with contralateral acoustic hearing ranging from normal hearing to moderate sensorineural hearing loss were tested. Localization accuracy was measured in five of the listeners using stimuli that emphasized the separate contributions of interaural level differences (ILDs) and interaural time differences (ITD) in the temporal envelope and/or fine structure. Sentence recognition was tested in all eight CI listeners, using collocated and spatially separated speech-shaped Gaussian noise and two-talker babble. Performance was compared with that of age-matched normal-hearing listeners via loudspeakers or via headphones with vocoder simulations of CI processing. RESULTS: Localization improved with the CI but only when high-frequency ILDs were available. Listeners experienced no additional benefit via ITDs in the stimulus envelope or fine structure using real or vocoder-simulated CIs. Speech recognition in two-talker babble improved with a CI in seven of the eight listeners when the target was located at the front and the babble was presented on the side of the acoustic-hearing ear, but otherwise showed little or no benefit of a CI. CONCLUSION: Sound localization can be improved with a CI in cases of significant residual hearing in the contralateral ear, but only for sounds with high-frequency content, and only based on ILDs. In speech understanding, the CI contributed most when it was in the ear with the better signal to noise ratio with a speech masker.

Effect of Audibility and Suprathreshold Deficits on Speech Recognition for Listeners With Unilateral Hearing Loss.

OBJECTIVES: We examined the influence of impaired processing (audibility and suprathreshold processes) on speech recognition in cases of sensorineural hearing loss. The influence of differences in central, or top-down, processing was reduced by comparing the performance of both ears in participants with a unilateral hearing loss (UHL). We examined the influence of reduced audibility and suprathreshold deficits on speech recognition in quiet and in noise. DESIGN: We measured speech recognition in quiet and stationary speech-shaped noise with consonant-vowel-consonant words and digital triplets in groups of adults with UHL (n = 19), normal hearing (n = 15), and bilateral hearing loss (n = 9). By comparing the scores of the unaffected ear (UHL+) and the affected ear (UHL-) in the UHL group, we were able to isolate the influence of peripheral hearing loss from individual top-down factors such as cognition, linguistic skills, age, and sex. RESULTS: Audibility is a very strong predictor for speech recognition in quiet. Audibility has a less pronounced influence on speech recognition in noise. We found that, for the current sample of listeners, more speech information is required for UHL- than for UHL+ to achieve the same performance. For digit triplets at 80 dBA, the speech recognition threshold in noise (SRT) for UHL- is on average 5.2 dB signal to noise ratio (SNR) poorer than UHL+. Analysis using the speech intelligibility index (SII) indicates that on average 2.1 dB SNR of this decrease can be attributed to suprathreshold deficits and 3.1 dB SNR to audibility. Furthermore, scores for speech recognition in quiet and in noise for UHL+ are comparable to those of normal-hearing listeners. CONCLUSIONS: Our data showed that suprathreshold deficits in addition to audibility play a considerable role in speech recognition in noise even at intensities well above hearing threshold.

Benefits of Cochlear Implantation for Single-Sided Deafness: Data From the House Clinic-University of Southern California-University of California, Los Angeles Clinical Trial.

OBJECTIVES: Cochlear implants (CIs) have been shown to benefit patients with single-sided deafness (SSD) in terms of tinnitus reduction, localization, speech understanding, and quality of life (QoL). While previous studies have shown cochlear implantation may benefit SSD patients, it is unclear which point of comparison is most relevant: baseline performance before implantation versus performance with normal-hearing (NH) ear after implantation. In this study, CI outcomes were assessed in SSD patients before and up to 6 mo postactivation. Benefits of cochlear implantation were assessed relative to binaural performance before implantation or relative to performance with the NH ear alone after implantation. DESIGN: Here, we report data for 10 patients who completed a longitudinal, prospective, Food and Drug Administration-approved study of cochlear implantation for SSD patients. All subjects had severe to profound unilateral hearing loss in one ear and normal hearing in the other ear. All patients were implanted with the MED-EL CONCERTO Flex 28 device. Speech understanding in quiet and in noise, localization, and tinnitus severity (with the CI on or off) were measured before implantation (baseline) and at 1, 3, 6 mo postactivation of the CI processor. Performance was measured with both ears (binaural), the CI ear alone, and the NH ear alone (the CI ear was plugged and muffed). Tinnitus severity, dizziness severity, and QoL were measured using questionnaires administered before implantation and 6 mo postactivation. RESULTS: Significant CI benefits were observed for tinnitus severity, localization, speech understanding, and QoL. The degree and time course of CI benefit depended on the outcome measure and the reference point. Relative to binaural baseline performance, significant and immediate (1 mo postactivation) CI benefits were observed for tinnitus severity and speech performance in noise, but localization did not significantly improve until 6 mo postactivation; questionnaire data showed significant improvement in QoL 6 mo postactivation. Relative to NH-only performance after implantation, significant and immediate benefits were observed for tinnitus severity and localization; binaural speech understanding in noise did not significantly improve during the 6-mo study period, due to variability in NH-only performance. There were no correlations between behavioral and questionnaire data, except between tinnitus visual analog scale scores at 6 mo postactivation and Tinnitus Functional Index scores at 6 mo postactivation. CONCLUSIONS: The present behavioral and subjective data suggest that SSD patients greatly benefit from cochlear implantation. However, to fully understand the degree and time course of CI benefit, the outcome measure and point of comparison should be considered. From a clinical perspective, binaural baseline performance is a relevant point of comparison. The lack of correlation between behavioral and questionnaire data suggest that represent independent measures of CI benefit for SSD patients.

Speech-in-Noise and Quality-of-Life Measures in School-Aged Children With Normal Hearing and With Unilateral Hearing Loss.

OBJECTIVES: (1) Measure sentence recognition in co-located and spatially separated target and masker configurations in school-aged children with unilateral hearing loss (UHL) and with normal hearing (NH). (2) Compare self-reported hearing-related quality-of-life (QoL) scores in school-aged children with UHL and NH. DESIGN: Listeners were school-aged children (6 to 12 yrs) with permanent UHL (n = 41) or NH (n = 35) and adults with NH (n = 23). Sentence reception thresholds (SRTs) were measured using Hearing In Noise Test-Children sentences in quiet and in the presence of 2-talker child babble or a speech-shaped noise masker in target/masker spatial configurations: 0/0, 0/-60, 0/+60, or 0/±60 degrees azimuth. Maskers were presented at a fixed level of 55 dBA, while the level of the target sentences varied adaptively to estimate the SRT. Hearing-related QoL was measured using the Hearing Environments and Reflection on Quality of Life (HEAR-QL-26) questionnaire for child subjects. RESULTS: As a group, subjects with unaided UHL had higher (poorer) SRTs than age-matched peers with NH in all listening conditions. Effects of age, masker type, and spatial configuration of target and masker signals were found. Spatial release from masking was significantly reduced in conditions where the masker was directed toward UHL subjects' normal-hearing ear. Hearing-related QoL scores were significantly poorer in subjects with UHL compared to those with NH. Degree of UHL, as measured by four-frequency pure-tone average, was significantly correlated with SRTs only in the two conditions where the masker was directed towards subjects' normal-hearing ear, although the unaided Speech Intelligibility Index at 65 dB SPL was significantly correlated with SRTs in four conditions, some of which directed the masker to the impaired ear or both ears. Neither pure-tone average nor unaided Speech Intelligibility Index was correlated with QoL scores. CONCLUSIONS: As a group, school-aged children with UHL showed substantial reductions in masked speech perception and hearing-related QoL, irrespective of sex, laterality of hearing loss, and degree of hearing loss. While some children demonstrated normal or near-normal performance in certain listening conditions, a disproportionate number of thresholds fell in the poorest decile of the NH data. These findings add to the growing literature challenging the past assumption that one ear is "good enough."

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

[Influence of single-sided deafness on the auditory capacity of the better ear. German version]. / Einfluss einseitiger Taubheit auf das Hörvermögen des besseren Ohrs.

BACKGROUND: Patients with single-sided deafness (SSD) are limited by their asymmetric hearing in various areas of everyday life. OBJECTIVE: The aim of this investigation was to perform an age-correlated comparison of the hearing threshold of the better ear of SSD patients with a normal-hearing (NH) reference cohort. In addition, the potential influence of etiology, duration of deafness, and cochlear implantation (CI) of the poorer ear on the peripheral hearing ability of the better ear was investigated. MATERIALS AND METHODS: In a multicenter study, the mean bone conduction hearing threshold of the better ear of 413 adult SSD patients was compared with that of an NH cohort drawn from ISO 7029:2017 for the frequencies 0.5, 1, 2, and 4 kHz. RESULTS: SSD patients showed significantly poorer hearing in the better ear compared to the age-correlated group of NH subjects. CI, duration of deafness, and etiology had no significant effect on the hearing ability of the better ear. CONCLUSION: The origin of the poorer hearing of the better-hearing ear of SSD patients compared to an age-correlated NH cohort is still unclear. It is most likely a combination of different anatomical, immunological, etiological, and microcirculatory causes, which lead to poorer hearing of the better-hearing ear in SSD patients.