Duration Of Deafness Research Articles

Cochlear Implantation and Electric Acoustic Stimulation in Children With TMPRSS3 Genetic Mutation.

Mutations in the TMPRSS3 gene, although rare, can cause high frequency hearing loss with residual hearing at low frequencies. Several previous studies have reported cochlear implant (CI) outcomes for adults with TMPRSS3 mutation with mixed results. Although some studies have suggested that TMPRSS3 is expressed in spiral ganglion cells, it remains unclear if previously reported poor CI outcomes in this population were secondary to long durations of deafness or to the effects of the TMPRSS3 mutation. To date, no studies in the literature have reported CI outcomes for children with TMPRSS3 mutation treated with CI. The current case series aimed to describe outcomes for three children with sloping hearing loss caused by TMPRSS3 mutation who underwent bilateral CI. Case series. Academic medical center. Three children (3-4 yr) with TMPRSS3 mutation and normal sloping to profound high frequency hearing loss. CI and electric acoustic stimulation (EAS). Outcome measures were residual hearing thresholds, speech recognition scores, and electrode placement determined via intraoperative CT imaging. All three children maintained residual acoustic hearing and received benefit from EAS. Mean change in low-frequency pure-tone average was 17 dB. Mean postoperative word and sentence recognition scores in the bilateral EAS condition were 80 and 75%, respectively. Results indicate that CI with EAS is an appropriate treatment for children with TMPRSS3 genetic mutation. Pediatric results from this case series show more favorable CI outcomes than are currently reported for adults with TMPRSS3 mutation suggesting that the intervention may be time sensitive.

Cochlear Implantation in Children With Single-Sided Deafness

In 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling. To evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates. MEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020. Inclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria. The Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes. Outcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD. Twelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16° to -15.40°; I2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than the duration of deafness among regular device users (median difference, 6.84; 95% CI, 4.02-9.58). This systematic review and meta-analysis found that cochlear implantation for children with SSD was associated with clinically meaningful improvements in audiological and patient-reported outcomes; shorter duration of deafness may lead to better outcomes. These findings can guide future research efforts, refine cochlear implantation candidacy criteria, and aid in family counseling and shared decision-making.

Outcomes of Cochlear Implantation in Patients with Post-Meningitis Deafness: A Systematic Review and Narrative Synthesis.

Summarise outcomes following cochlear implantation (CI) in patients with post-meningitis deafness. Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Studies with a minimum of 20 individuals with post-meningitis deafness were included. Review conducted in accordance with the PRISMA statement. Searches identified 906 abstracts and 291 full texts. Of these, 19 studies met the inclusion criteria, reporting outcomes in 610 patients with 650 implants. Audiological outcomes improved across all studies following cochlear implantation. 7 studies demonstrated a statistically significant difference between pre and post-CI outcomes. Patients with no cochlear ossification, full electrode insertion, shorter duration of deafness and no neurological sequelae generally appeared to perform best. A total of 31 minor and 19 major complications were reported, with 15 cases of reimplantation. The methodological quality of the included studies was sufficient, predominantly consisting of cohort studies. 15 studies were OCEBM grade III and 4 studies were OCEBM grade IV. All studies had a minimum of 20 individuals with post-meningitic deafness and used multi-channel cochlear implant devices. Audiological outcomes following cochlear implantation in meningitis are satisfactory, providing functional levels of speech perception and intelligibility. Improvement in hearing is dependent on the amount of cochlear ossification, duration of deafness prior to implantation, electrode insertion depth and presence of neurological sequalae. Cochlear implantation in meningitis patients can be challenging due to the presence of ossification and inaccuracies of pre-operative imaging. Therefore, early and bilateral implantation is recommended in all patients with post-meningitis hearing loss to improve the likelihood of full electrode insertion.

Long-term auditory processing outcomes in early implanted young adults with cochlear implants: The mismatch negativity vs. P300 response

ObjectiveLong-term outcomes of early implanted, young adult cochlear implant (CI) users remain variable. We measured auditory discrimination by means of event-related potentials in this population to examine whether variability at the level of cortical auditory processing helps to explain speech abilities. MethodsUsing an auditory oddball paradigm, the P300 and Mismatch Negativity (MMN) were measured in 8 young adult CI users and 14 normal-hearing peers. We related P300 amplitude and latency to clinical speech perception scores in quiet and to duration of deafness. ResultsAll individuals showed P300 responses. The MMN response was less robust in both groups. There was no evidence for differences in P300 responses between CI users and controls. P300 amplitude was associated with speech perception scores (r = 0.70, p = .05) and duration of deafness (r = −0.83, p = .009). ConclusionsEarly CI implantation yields good auditory processing outcomes at young adult age and, in contrast to MMN, the P300 provides a robust measure for auditory processing on an individual level. SignificanceAt the cortical level, early implanted, long-term CI users have good auditory discrimination, leaving variability in implantation outcomes unexplained. This group provides unique insight into the long-term neurophysiological underpinnings of early implantation.

Cochlear Implantation.

Hearing impairment that is too severe to be adequately treated with conventional hearing aids can lead, in children, to severe developmental disturbances of hearing and language, and, in adults, to communicative and social deprivation. Recent advances in medical device technology and in microsurgical techniques have led to an expansion of the indications for cochlear implantation (CI) for adults with progressive hearing loss in older age, and to a restructuring of the process of care for these patients in Germany. This review is based on pertinent publications retrieved by a selective search in PubMed, as well as on the CI guidelines and CI "white book" of the German Society of Otolaryngology and Head and Neck Surgery. Early and accurate diagnosis is crucial for the successful auditory rehabilitation of high-grade hearing impairment. In children, a key role is played by newborn auditory screening, which is mandatory in Germany and enables the provision of a CI in the first year of life when necessary. 86% of the children receiving a CI achieve linguistic comprehension of fluently spoken sentences. For adults, positive prognostic factors for hearing after the provision of a CI include a highly motivated patient, "postlingual" onset of the hearing impairment (i.e., after the acquisition of language), and a brief duration of deafness. Auditory rehabilitation is associated with significant improvement, not just of hearing and of the comprehension of spoken language, but also of quality of life, particularly in elderly patients. For patients of any age with bilateral hearing loss, CIs should be provided on both sides, if possible. The more common complications of the procedure, with a probability of 2-4% each, are technical implant defects, dizziness, and wound-healing disturbances. Cochlear implantation, performed in specialized centers, is a safe and reliable technique and regularly enables the successful rehabilitation of hearing in both children and adults.

Using cluster analysis and hearing histories to predict performance outcomes among bilateral cochlear-implant users

Cochlear-implant researchers are often interested in how participants' hearing histories impact performance. For example, it is common to include duration of deafness, age at onset of deafness, and age at implantation as covariates in statistical analyses. Unfortunately, multi-collinearity often exists among hearing history variables, which can affect the convergence of statistical models. This problem could be alleviated by using cluster analysis, a statistical method that can sort participants into groups based on their similarity on a set of variables. Cluster membership, rather than collinear hearing history variables, can then be entered into statistical models. To measure the efficacy of this method in cochlear-implant research, we performed a cluster analysis on the bilateral cochlear-implant users in our lab's database with complete hearing history information (n = 53). Bilateral cochlear-implant users were chosen for this initial analysis to measure whether across-ear differences affect outcomes. Four clusters emerged based on demographic and hearing history variables. Preliminary analyses showed that cluster membership could serve as a useful predictor for performance outcomes in bilateral cochlear-implant users. Validating clusters in the greater cochlear-implant user population could result in useful information for audiologists counseling potential implant candidates about expected outcomes.

Anamnesis as a Prognostic Factor in Cochlear Implantation in Adults

Introduction: This study aims to analyze possible preoperative factors taken from the medical history that may assist the otolaryngologist in counseling an adult patient before cochlear implantation (CI). Objective: Analysis of preoperative factors taken during the initial patient presentation for a possible prognostic role in the auditory rehabilitation outcome. Methods: A cohort of 232 (272 CI implantations) postlingually deafened adults was evaluated in this study. Hearing results at 1, 2, and up to 3 years postoperatively were compared with various preoperative factors: living status, cause of deafness, gender, side of implantation, residual hearing, and duration of deafness. Postoperative hearing performance was measured based on the German Freiburg monosyllabic word test and the Oldenburg sentence test. Results: Duration of deafness showed a negative correlation to word recognition and a positive correlation to increased speech reception threshold in sentence testing. A significant decline in hearing outcome was shown starting around the second decade of deafness. Residual hearing as defined in our cohort and side of implantation showed limited benefit in speech understanding. Living status, gender, and cause of deafness did not show any prognostic value. Conclusion: In this retrospective review it could be shown that simple case history information can only provide limited prognostic insight before CI. The duration of deafness is the most reliable anamnestic factor present on initial patient evaluation.

Prologue to the Forum: Literacy and Language Issues In Children With Hearing Loss

Purpose The purpose of this forum is to provide an update on reading outcomes in preschool- and school-age children with hearing loss and to provide guidance on intervention strategies to improve reading skills. Conclusions Preschool- and school-age children with hearing loss may show deficits in various reading and reading-related skills depending on their age, duration of deafness, severity of hearing loss, age at which they were fitted with hearing technologies, speech recognition skills, communication mode, and quality of intervention. Several linguistic and cognitive factors also are associated with reading outcomes. Thus, intervention programs must be multicomponent in nature and designed to fit the unique needs of each child.

"Large and giant vestibular schwannomas: overall outcomes and the factors influencing facial nerve function".

(1) To study the overall outcomes of patients surgically treated for large/giant vestibular schwannomas (VSs) and (2) to identify and analyze preoperative and intraoperative prognostic factors influencing facial nerve (FN) outcome. A retrospective clinical study was conducted at a quaternary referral otology and skull-base center. A total of 389 cases were enrolled. The inclusion criteria were patients with tumor > 30mm undergoing surgery with a minimum follow-up of 12months. Neurofibromatosis-II, previous radiotherapy, revision surgeries, preoperative FN House-Brackmann (HB) grade > I, partial resections, incomplete records, or those lost to follow-up for a minimum period of 1year were excluded. In addition, partial resections and cases where FN was sacrificed intraoperatively were also excluded and were analyzed separately. The mean duration of symptoms was 35.4months, pronounced more in elderly (58.3months) than in younger individuals (28.4months). Mean tumor diameter was 36mm and 52.7% was cystic. Total resection (TR), near total resection (NTR), and subtotal resections (STR) were achieved in 77.4%, 9.5%, and 13.2% of cases, respectively. Regrowth was observed only after STR (19.6%). Good (HB I-II), moderate (HB III), and poor (HB IV-VI) FN functions were observed in 36.8%, 51.7%, and 11.6% cases, respectively. Younger individuals underwent TR in 259 (86.9%) cases against 42 (46.2%) in elderly individuals. Non-total resections (NTR/STR) were performed in 49 (53.8%) cases in elderly as against 39(13.1%) in younger individuals. Good FN outcome was observed in 28 (57.1%) cases of non-total resections in elderly as against 13 (33.3%) cases in younger individuals. On multiple logistic regression analysis, size of the tumor, preoperatively prolonged duration of symptoms, profound deafness, and antero-superiorly located FN with respect to the tumor played a detrimental role in the final facial nerve outcome postoperatively. On the contrary, in large tumors (3-3.9cm), presence of vertigo/disequilibrium had a relatively better impact on final FN outcome. Partial resections accounted for 41(7.8%) cases and FN was interrupted in 71(13.6%) cases in total. Factors detrimental to better FN outcome were giant VSs (> 4cm), antero-superiorly located FN intratumorally, preoperatively prolonged duration of symptoms, and profound deafness. In large tumors (3-3.9cm), presence of vertigo/disequilibrium had a better impact on FN outcome. Understandably, cases with TR in comparison with NTR/STR had worse FN outcomes. In comparison with younger patients, elderly patients underwent higher NTR/STR resulting in better FN outcomes. The above factors can be used as prognosticators for patient counseling and surgical decision making.

Reorganized Brain White Matter in Early- and Late-Onset Deafness With Diffusion Tensor Imaging.

Individuals with early- and late-onset deafness showed different functional and morphological brain changes, but white matter alterations in both deaf groups still need to be elucidated. This study aimed to investigate changes in white matter integrity and white matter anatomical connectivity in both early- and late-onset deaf groups compared with hearing group. Diffusion tensor imaging data from 7 early-onset deaf (50.7 ± 6.5 years), 11 late-onset deaf (50.9 ± 12.3 years), and 9 hearing adults (48.9 ± 9.5 years) were preprocessed using FSL software. To find changes in white matter integrity, tract-based spatial statistics was used, which implemented on FSL software. Fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD) were calculated and compared among the groups with age as a nuisance variable. To find out the effect of onset age or duration of deafness to the white matter integrity, onset-age or duration of deafness was treated as a variable of interest in the general linear model implemented on tract-based spatial statistics. White matter connectivity was constructed by a deterministic tractography and compared among the groups. In comparison to the hearing group, the early-onset deaf group did not show any significant changes but the late-onset deaf group showed decreased FA and increased RD in the several white matter areas. AD in the late-onset deaf group was not significantly different compared with the hearing group. The regions included the corpus callosum, posterior and superior corona radiata, internal capsule, posterior thalamic radiation, superior longitudinal fasciculus, and tapetum of the right hemisphere. Increased RD was also additionally observed in the right external capsule, fornix, and cerebral peduncle. The onset age or duration of deafness was not significantly correlated with the white matter integrity in the early-onset deaf group. In contrast, the onset age showed a significantly positive correlation with the RD, and a negative correlation with the FA, in the late-onset deaf group. The correlated white matter areas were also similar to the findings of comparison with the hearing group. In comparison to the hearing group, the early-onset deaf group did not show altered white matter connectivity, while the late-onset deaf group showed decreased white matter connectivity in between the right lingual and hippocampal areas. The present results suggest that late-onset deaf adults showed decreased FA and increased RD, and early-onset deaf adults showed no difference compared with the hearing group. In the late-onset deaf adults, onset-age showed a significantly positive correlation with RD and negative correlation with FA. Duration of deafness was not significantly correlated with the changes. Increased RD indicating demyelination occurred in the brain, and the changes were not limited to the auditory cortex but expanded to almost whole brain areas, suggesting significant effect of auditory deprivation on the brain later in life. The altered white matter connectivity in between the right limbic-occipital areas observed in the late-onset deaf group might be caused by altered language functions after auditory deprivation. Future studies are necessary incorporating functional and anatomical aspects of the brain changes in deaf group.

Place-Pitch Interval Perception With a Cochlear Implant.

Pitch is poorly perceived by cochlear implant (CI) users. However, as it is not well understood how pitch is encoded with electric stimulation, improving pitch representation with a CI is challenging. Changes in place of stimulation along the cochlea have been described as changes in pitch and can be accurately ranked by CI users. However, it remains unknown if place-pitch can be used to encode musical intervals, which are a necessary attribute of pitch. The objective of these experiments is to determine if place-pitch coding can be used to represent musical intervals with a CI. In the first experiment, 10 CI users and 10 normal hearing (NH) controls were tested on their sensitivity to changes in the semitone spacing between each of the notes in the melody "Happy Birthday." The changes were implemented by uniformly expanding or compressing the frequency differences between each note in the melody. The participant's task was to scale how "out-of-tune" the melody was for various semitone spacing distortions. The notes were represented by pure-tones ≥440 Hz to minimize potential useful temporal information from the stimuli. A second experiment replicated the first experiment using single-sided deafened CI users allowing for a within-subject control. A third experiment verified that the CI users who participated in Experiment 1 were each able to determine pitch direction reliably. Unlike NH listeners, CI listeners often ranked all distortions of interval spacing similarly in both the first and second experiment, and no effect of interval spacing was detected across CI users. Some participants found distorted interval spacings to be less out-of-tune than the nominally correct interval spacings. However, these patterns were inconsistent across listeners. Although performance was better for the NH listeners, the third experiment demonstrated that the CI listeners were able to reliably identify changes in pitch direction from place-pitch coding. The data suggest that place-pitch intervals are not properly represented through a CI sound processor. Some limited support is found for place-pitch being useful for interval encoding as some participants demonstrated improved ratings for certain interval distortions. Presumably the interval representation for these participants could be improved by a change to the frequencies represented by each electrode. However, as these patterns vary across listeners, there is not a universal correction to frequency representation that will solve this issue. As results are similar for single-sided deafened CI users, the limitations in ratings are likely not limited by an eroded representation of the melody caused by an extended duration of deafness.

Electrophysiological Estimates of the Electrode-Neuron Interface Differ Between Younger and Older Listeners With Cochlear Implants.

The primary objective of this study was to quantify differences in evoked potential correlates of spiral ganglion neuron (SGN) density between younger and older individuals with cochlear implants (CIs) using the electrically evoked compound action potential (ECAP). In human temporal bone studies and in animal models, SGN density is the lowest in older subjects and in those who experienced long durations of deafness during life. SGN density also varies as a function of age at implantation and hearing loss etiology. Taken together, it is likely that younger listeners who were deafened and implanted during childhood have denser populations of SGNs than older individuals who were deafened and implanted later in life. In animals, ECAP amplitudes, amplitude growth function (AGF) slopes, and their sensitivity to stimulus interphase gap (IPG) are predictive of SGN density. The authors hypothesized that younger listeners who were deafened and implanted as children would demonstrate larger ECAP amplitudes, steeper AGF slopes, and greater IPG sensitivity than older, adult-deafened and implanted listeners. Data were obtained from 22 implanted ears (18 individuals). Thirteen ears (9 individuals) were deafened and implanted as children (child-implanted group), and nine ears (9 individuals) were deafened and implanted as adults (adult-implanted group). The groups differed significantly on a number of demographic variables that are implicitly related to SGN density: (1) chronological age; (2) age at implantation; and (3) duration of preimplantation hearing loss. ECAP amplitudes, AGF linear slopes, and thresholds were assessed on a subset of electrodes in each ear in response to two IPGs (7 and 30 µsec). Speech recognition was assessed using a medial vowel identification task. Compared with the adult-implanted listeners, individuals in the child-implanted group demonstrated larger changes in ECAP amplitude when the IPG of the stimulus was increased from 7 to 30 µsec (i.e., greater IPG sensitivity). On average, child-implanted participants also had larger ECAP amplitudes and steeper AGF linear slopes than the adult-implanted participants, irrespective of IPG. IPG sensitivity for AGF linear slope and ECAP threshold did not differ between age groups. Vowel recognition performance was not correlated with any of the ECAP measures assessed in this study. The results of this study support the theory that young CI listeners who were deafened and implanted during childhood may have denser neural populations than older listeners who were deafened and implanted as adults. Potential between-group differences in SGN integrity emphasize a need to investigate optimized CI programming parameters for younger and older listeners.

Acoustic Hearing Can Interfere With Single-Sided Deafness Cochlear-Implant Speech Perception.

Cochlear implants (CIs) restore some spatial advantages for speech understanding in noise to individuals with single-sided deafness (SSD). In addition to a head-shadow advantage when the CI ear has a better signal-to-noise ratio, a CI can also provide a binaural advantage in certain situations, facilitating the perceptual separation of spatially separated concurrent voices. While some bilateral-CI listeners show a similar binaural advantage, bilateral-CI listeners with relatively large asymmetries in monaural speech understanding can instead experience contralateral speech interference. Based on the interference previously observed for asymmetric bilateral-CI listeners, this study tested the hypothesis that in a multiple-talker situation, the acoustic ear would interfere with rather than improve CI speech understanding for SSD-CI listeners. Experiment 1 measured CI-ear speech understanding in the presence of competing speech or noise for 13 SSD-CI listeners. Target speech from the closed-set coordinate response-measure corpus was presented to the CI ear along with one same-gender competing talker or stationary noise at target-to-masker ratios between -8 and 20 dB. The acoustic ear was presented with silence (monaural condition) or with a copy of the competing speech or noise (bilateral condition). Experiment 2 tested a subset of 6 listeners in the reverse configuration for which SSD-CI listeners have previously shown a binaural benefit (target and competing speech presented to the acoustic ear; silence or competing speech presented to the CI ear). Experiment 3 examined the possible influence of a methodological difference between experiments 1 and 2: whether the competing talker spoke keywords that were inside or outside the response set. For each experiment, the data were analyzed using repeated-measures logistic regression. For experiment 1, a correlation analysis compared the difference between bilateral and monaural speech-understanding scores to several listener-specific factors: speech understanding in the CI ear, preimplantation duration of deafness, duration of CI experience, ear of deafness (left/right), acoustic-ear audiometric thresholds, and listener age. In experiment 1, presenting a copy of the competing speech to the acoustic ear reduced CI speech-understanding scores for target-to-masker ratios ≥4 dB. This interference effect was limited to competing-speech conditions and was not observed for a noise masker. There was dramatic intersubject variability in the magnitude of the interference (range: 1 to 43 rationalized arcsine units), which was found to be significantly correlated with listener age. The interference effect contrasted sharply with the reverse configuration (experiment 2), whereby presenting a copy of the competing speech to the contralateral CI ear significantly improved performance relative to monaural acoustic-ear performance. Keyword condition (experiment 3) did not influence the observed pattern of interference. Most SSD-CI listeners experienced interference when they attended to the CI ear and competing speech was added to the acoustic ear, although there was a large amount of intersubject variability in the magnitude of the effect, with older listeners particularly susceptible to interference. While further research is needed to investigate these effects under free-field listening conditions, these results suggest that for certain spatial configurations in a multiple-talker situation, contralateral speech interference could reduce the benefit that an SSD-CI otherwise provides.

A behavioral method to estimate charge integration efficiency in cochlear implant users

BackgroundIn cochlear implants, pulse amplitude (PA) or pulse phase duration (PPD) can be used to increase loudness. Loudness grows more slowly with increasing PPD, resulting in a larger dynamic range (DR), possibly reflecting “leaky” charge integration associated with neural degeneration due to hearing loss. Here, we propose a method to estimate charge integration efficiency for CI users. New methodThe DR was measured with increasing PA or PPD, relative to a common threshold anchor with a short PPD (25μs/ph); DRs were converted to the common unit of charge (nC). Charge integration efficiency was calculated as the dB difference in DR with increasing PPD or PA. Loudness growth functions were also compared as PA or PPD was increased relative to the common threshold. ResultsTen CI ears were tested; all participants were adult users of Cochlear© devices. DR was significantly larger when PPD was increased, requiring (on average) 70 % more charge than when PA was increased. A significant correlation (p = 0.007) was observed between duration of deafness and charge integration efficiency, largely driven by a participant with long auditory deprivation in both ears. Loudness growth was slower when PPD was increased, consistent with previous studies.Comparison to Existing Methods. The present method offers a quick behavioral test with which to measure charge integration efficiency, which may be a useful measure of neural health. DiscussionCharge integration efficiency may be used to probe neural health independent of absolute detection thresholds, which mostly reflect the proximity of electrodes to neural populations.

Cochlear Implantation for Treatment of Tinnitus in Single-sided Deafness: A Systematic Review and Meta-analysis

Quantify the benefit of cochlear implantation (CI) for tinnitus relief among individuals with single-sided deafness (SSD). PubMed, Scopus, and Cochrane databases were searched through July 10, 2019. Search strategies used a combination of subject headings (e.g., MeSH in PubMed) and keywords for the following three concepts: single-sided deafness, cochlear implantation, and tinnitus. English articles that reported the preintervention (baseline) tinnitus-related patient-reported outcome measures (e.g., Tinnitus Handicap Inventory [THI] and Visual Analog Scale [VAS] for loudness) in patients with SSD that underwent CI were included. Number of patients, mean age, etiology of hearing loss, duration of deafness, baseline and follow-up THI and VAS scores. A total of 17 studies met inclusion criteria encompassing 247 patients with SSD receiving a cochlear implant (mean age 50.2 yr, range 23-71). For THI, CI resulted in a mean difference of -35.4 points [95% CI -55.8 to -15.0, p < 0.001]. VAS decreased by -4.6 points [CI -6.0 to -3.3, p < 0.001]. A weighted proportion of 14.9% [CI 6.4-26.1] of patients experienced complete resolution of tinnitus, while 74.5% [CI 63.1-84.5] experienced partial improvement; 7.6% [CI 4.1-12.6] of patients had no change in severity, and 3.0% [CI 1.0-6.7] experienced worsening of their tinnitus. On both THI and VAS, patients reported significant reduction in their scores, representing an overall improvement in tinnitus severity while wearing the cochlear implant. Most patients with SSD will experience partial improvement or complete resolution of tinnitus with a cochlear implant.

Long-term cochlear implantation outcomes in patients following head injury.

ObjectiveIn cases of a severe to profound sensorineural hearing loss following head injury, the cochlear implant (CI) is the primary option for auditory rehabilitation. Few studies, however, have investigated long‐term CI outcomes in patients following head trauma, including those without temporal bone fracture (TBF). Herein, the aim of this study is to examine CI outcomes following cases of head injury with and without TBF.MethodsAudiometric outcomes of patients who received a CI due to a head injury resulting in severe to profound hearing loss at two tertiary care hospitals were analyzed. Patients were divided into those who received a CI in a fractured temporal bone (group A, n = 11 patients corresponding to 15 ears) and those who received a CI in a non‐fractured temporal bone (group B, n = 8 patients corresponding to nine ears). Primary outcomes included duration of deafness prior to CI and postoperative consonant‐nucleus‐constant whole word (CNC) scores.ResultsNineteen patients (84% male), corresponding to 24 CIs, were identified. Fifteen CI were performed on ears with TBF (group A), and nine CI were performed on ears without TBF (group B). No patients had an enlarged vestibular aqueduct (EVA). The mean duration of deafness was 5.7 and 11.3 years in group A and group B, respectively. The mean duration of CI follow‐up (CI experience) was 6.5 years in group A and 2.1 years in group B. The overall mean postoperative CNC score for all subjects was 68.6% (±21.2%, n = 19 with CNC testing). There was no difference in CNC score between group A and group B (69.8% and 66% respectively, P = .639).ConclusionThe study is among the largest series examining long‐term outcomes of CI after head injury. CI is an effective method for auditory rehabilitation in patients after head injury.Level of evidenceIV.

The molecular etiology of deafness and auditory performance in the postlingually deafened cochlear implantees

Recent advances in molecular genetic testing (MGT) have improved identification of genetic aetiology of candidates for cochlear implantation (CI). However, whether genetic information increases CI outcome predictability in post-lingual deafness remains unclear. Therefore, we evaluated the outcomes of CI with respect to genetic aetiology and clinical predictors by comparing the data of study subjects; those with an identified genetic aetiology (GD group), and those without identifiable variants (GUD group). First, we identified the genetic aetiology in 21 of 40 subjects and also observed genetic etiologic heterogeneity. The GD group demonstrated significantly greater improvement in speech perception scores over a 1-year period than did the GUD group. Further, inverse correlation between deafness duration and the 1-year improvement in speech perception scores was tighter in the GD group than in the GUD group. The weak correlation between deafness duration and CI outcomes in the GUD group might suggest the pathophysiology underlying GUD already significantly involves the cortex, leading to lesser sensitivity to further cortex issues such as deafness duration. Under our MGT protocol, the correlation between deafness duration and CI outcomes were found to rely on the presence of identifiable genetic aetiology, strongly advocating early CI in individual with proven genetic aetiologies.

What can we learn from adult cochlear implant recipients with single-sided deafness who became elective non-users?

Objective: This retrospective study investigates the incidence of elective cochlear implant (CI) non-use amongst a cohort of adult CI recipients with single-sided deafness (SSD), identifies the causes that led to non-use, and assesses how non-use could be prevented. Methods: All adults with SSD who received a CI between 2008 and 2018 and who became elective CI non-users were included. Elective non-users were defined as CI recipients who decided to stop using their CI or, if explantation was necessary, refused reimplantation. Results: 5/114 (4.4%) adults with SSD who received a CI became elective non-users. The 5 non-users were a mean 44.2 years old (range 33–70 years) at implantation, had a mean duration of deafness of 7.1 years (range 0.5–20 years) at implantation, and used their CI for a mean 11.5 months (range 1.5–60 months) before (fully) discontinuing use. The primary cause of elective non-use was postoperative discouragement due to unrealistic expectations (4 participants) regarding sound perception with the CI or about the greater than expected level of commitment necessary for rehabilitation. Conclusions: Elective non-use among adult CI recipients with single-sided deafness was very rare and could be further prevented by comprehensive counselling to ensure that candidates have realistic expectations about the rehabilitation requirements and the outcomes with the CI.

Are Cochlear Implants a Viable Option Following Temporal Bone Fracture?

Are Cochlear Implants a Viable Option Following Temporal Bone Fracture?

Prognostic factors in cochlear implantation in adults: Determining central process integrity

The purpose of this study is to examine various preoperative factors that can play a role in the auditory rehabilitation outcome of cochlear implant (CI) recipients. In order to determine the level of integrity of central processing preoperatively, special attention was given to residual hearing, duration of deafness, and cochlear nerve diameter as prognostic factors. A cohort of 232 (272 CI implantations) postlingually deafened adults was evaluated in this study. Hearing results at 1, 2 and up to 3years postoperatively were compared with various preoperative factors: promontory stimulation testing, residual hearing, duration of deafness, and magnetic resonance imaging of the cochlear nerve. Postoperative hearing performance was measured based on the German Freiburg monosyllabic word test and the Oldenburg sentence test. Postoperative hearing performance showed a significant improvement in each consecutive year after implantation. Duration of deafness showed a negative correlation to word recognition and a positive correlation to increased signal-to-noise-ratio in sentence testing. A significant decline in hearing outcome was shown starting around the second decade of deafness corresponding to 66% of life spent in deafness. MR imaging of cochlear nerve diameter shows a positive correlation of larger nerve diameter to better speech understanding. Promontory stimulation testing did not show any prognostic value. In this retrospective review it could be shown that there is an intricate interaction in the preoperative variables: duration of deafness - as well as the ratio of life spent in deafness; residual hearing; and cochlear nerve diameter.