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Cochlear implants (CIs) provide access to sound for children and adults who do not receive adequate benefit from hearing aids. Age at implantation is known to affect outcomes across the lifespan. The effects of age on CI outcomes are examined for infants, children, adolescents, and older adults. A variety of outcome measures are considered, including speech perception, language, cognition, and quality of life measures. For those meeting candidacy criteria, CIs are beneficial at any age. In general, younger age is related to greater benefit when considering pre-lingual deafness. Other factors such as additional disabilities, may mitigate this effect. Post-lingually deafened adults demonstrate similar benefit regardless of age, though the oldest individuals (80+) may see smaller degrees of improvement from preoperative scores. Benefit can be measured in many ways, and the areas of greatest benefit may vary based on age: young children appear to see the greatest effects of age at implantation on language measures, whereas scores on cognitive measures appear to be most impacted for the oldest population. Future research should consider implantation at extreme ages (5–9 months or > 90 years), unconventional measures of CI benefit including qualitative assessments, and longitudinal designs. [ABSTRACT FROM AUTHOR]

Objective: To assess bimodal auditory performance in children with a cochlear implant (CI) and contralateral auditory brainstem implant (ABI).Methods: This is a retrospective case review performed at a tertiary referral center. Four patients with cochlear nerve deficiency initially underwent cochlear implantation but were not benefiting from their devices and underwent ABI in the contralateral ear. The main outcome measures included age-appropriate speech perception and production assessments.Results: Three subjects performed better on their auditory perception assessments using both of their devices than with either device alone. One subject had only preliminary outcomes, but subjectively performed best with both devices.Conclusions: We observed continued improvement in CI performance over time, even if no benefit was evident before the decision for ABI. This could suggest that ABI and CI have a synergistic effect, or it could simply be the adaptive ability of the developing brain to utilize the signals coming from these devices. There is preliminary evidence to support choosing the ear contralateral to the CI for an ABI in a pediatric patient with bilateral cochlear nerve deficiency. [ABSTRACT FROM AUTHOR]

Introduction: While cochlear implants (CI) prior to 12 months of age have become common, the prevalence and impact of issues that either arise or were not evident prior to implantation is unknown. Methods: Retrospective chart review of children implanted under 12 months of age with minimum 3 years follow up. The children were divided into three groups: those with no identified additional disabilities, those with no known disabilities at time of implantation but diagnosed with additional disabilities following implantation, and those that had known anticipated additional disabilities at time of implantation. Results: 108 children under the age of 12 months were implanted at our Center between 2000 and 2013 with an average age of 9 months at time of implantation and n = 93 met inclusion criteria. In 79.6% (74/93) of children, there were no additional issues detected. In 11.8% (11/93), additional issues were known at the time of implantation while in 8.6% (8/93) of the children were diagnosed with additional issues that were not evident prior to implantation. The auditory and linguistic benefits vary commensurate with the severity of their disabilities. Those with anticipated issues preoperatively did not perform as well. Conclusions: Children implanted below one year of age but diagnosed with additional disabilities following implantation obtained substantial though varying degrees of benefit. In none of these cases would knowledge of the disability have altered the decision to offer early CI. It is important to address these potential issues when counseling families about outcomes. [ABSTRACT FROM AUTHOR]

Objective: To demonstrate the long-term benefits of implantation in patients with high-frequency sensorineural hearing loss, this report provides 5-year follow-up on a group of implant recipients who were subjects of the Cochlear™ Nucleus® Hybrid™ L24 Implant System pivotal clinical study.Methods: The results of three related clinical studies were compiled to provide outcome data after 1, 3, and 5 years of implant use in a group of subjects who presented with preoperative high-frequency hearing loss and were implanted with a Nucleus Hybrid L24 (Cochlear Ltd., Sydney, Australia) cochlear implant. A subset of the 50 adult subjects (N = 32) who participated in the Hybrid L24 pivotal Investigational Device Exemption (IDE) completed comprehensive evaluations at 12 months postactivation, 3 years postactivation, and then as part of a postapproval study at 5 years postactivation. Testing included audiometric, speech perception, and subjective satisfaction measures.Results: Mean unilateral speech perception performance was significantly improved at all postoperative intervals compared to preoperative best-aided results and has remained stable to 5 years postactivation. Ninety-four percent of subjects had measurable hearing, and 72% continued to use electric-acoustic stimulation in the implanted ear after 5 years of implant use. Subjective satisfaction results support objective performance improvements.Conclusion: Results demonstrate long-term success of patients with high-frequency hearing loss following Hybrid L24 (Cochlear) cochlear implantation. Benefits include speech perception abilities significantly better than those in the preoperative best-aided condition, with additional benefit in those using electric-acoustic stimulation in the implanted ear.Level Of Evidence: 2b. Laryngoscope, 1939-1945, 2018. [ABSTRACT FROM AUTHOR]

Ninety-four unilateral CI patients with bimodal listening experience (CI plus HA in contralateral ear) completed a questionnaire that focused on attitudes toward hearing aid use postimplantation, patterns of usage, and perceived bimodal benefits in daily life. Eighty participants continued HA use and 14 discontinued HA use at the time of the questionnaire. Participant responses provided useful information for counseling patients both before and after implantation. The majority of continuing bimodal (CI plus HA) participants reported adapting to using both devices within 3 months and also reported that they heard better bimodally in quiet, noisy, and reverberant conditions. They also perceived benefits including improved sound quality, better music enjoyment, and sometimes a perceived sense of acoustic balance. Those who discontinued HA use found either that using the HA did not provide additional benefit over the CI alone or that using the HA degraded the signal from the CI. Because there was considerable overlap in the audiograms and in speech recognition performance in the unimplanted ear between the two groups, we recommend that unilateral CI recipients are counseled to continue to use the HA in the contralateral ear postimplantation in order to determine whether or not they receive functional or perceived benefit from using both devices together. [ABSTRACT FROM AUTHOR]

Objective: The aim of this case series was to assess the impact of auditory rehabilitation with cochlear implantation on the cognitive function of elderly patients over time. Design: This is a longitudinal case series of prospective data assessing neurocognitive function and speech perception in an elderly cohort pre- and post-implantation. Setting: University cochlear implant center. Participants: The patients were post-lingually deafened elderly female (mean, 73.6 years; SD, 5.82; range, 67-81 years) cochlear implant recipients (n=7). Measurements: A neurocognitive battery of 20 tests assessing intellectual function, learning, short- and long-term memory, verbal fluency, attention, mental flexibility, and processing speed was performed prior to and 2-4.1 years (mean, 3.7) after cochlear implant (CI). Speech perception testing using Consonant-Nucleus-Consonant words was performed prior to implantation and at regular intervals postoperatively. Individual and aggregate differences in cognitive function pre- and post-CI were estimated. Logistic regression with cluster adjustment was used to estimate the association (%improvement or %decline) between speech understanding and years from implantation at 1 year, 2 years, and 3 years post-CI. Results: Improvements after CI were observed in 14 (70%) of all subtests administered. Declines occurred in five (25%) subtests. In 55 individual tests (43%), post-CI performance improved compared to a patient's own performance before implantation. Of these, nine (45%) showed moderate or pronounced improvement. Overall, improvements were largest in the verbal and memory domains. Logistic regression demonstrated a significant relationship between speech perception and cognitive function over time. Five neurocognitive tests were predictive of improved speech perception following implantation. Conclusion: Comprehensive neurocognitive testing of elderly women demonstrated areas of improvement in cognitive function and auditory perception following cochlear implantation. Multiple neurocognitive tests were strongly associated with current speech perception measures. While these data shed light on the complex relationship between hearing and cognition by showing that CI may slow the expected age-related cognitive decline, further research is needed to examine the impact of hearing rehabilitation on cognitive decline. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To evaluate the safety and efficacy of acoustic and electric sound processing for individuals with significant residual low-frequency hearing and severe-to-profound high-frequency sensorineural hearing loss.Study Design: Prospective, single-arm repeated measures, single-subject design.Methods: Fifty individuals, ≥ 18 years old, with low-frequency hearing and severe high-frequency loss were implanted with the Cochlear Nucleus Hybrid L24 implant at 10 investigational sites. Preoperatively, subjects demonstrated consonant-nucleus-consonant word scores of 10% through 60% in the ear to be implanted. Subjects were assessed prospectively, preoperatively, and postoperatively on coprimary endpoints of consonant-nucleus-consonant words, AzBio sentences in noise, and self-assessment measures.Results: Significant mean improvements were observed for coprimary endpoints: consonant-nucleus-consonant words (35.8 percentage points) and AzBio sentences in noise (32.0 percentage points), both at P < 0.001. Ninety-six percent of subjects performed equal or better on speech in quiet and 90% in noise. Eighty-two percent of subjects showed improved performance on speech in quiet and 74% in noise. Self-assessments were positive, corroborating speech perception results.Conclusion: The Nucleus Hybrid System provides significant improvements in speech intelligibility in quiet and noise for individuals with severe high-frequency loss and some low-frequency hearing. This device expands indications to hearing-impaired individuals who perform poorly with amplification due to bilateral high-frequency hearing loss and who previously were not implant candidates. [ABSTRACT FROM AUTHOR]

Objective: Preservation of residual low-frequency hearing has become a priority in cochlear implantation. The purpose of this study was to compare rates of hearing preservation and effects on performance of loss of low-frequency acoustic hearing with two different length electrodes.Study Design: Retrospective chart review.Setting: Tertiary Care Hospital.Patients: Twelve patients were implanted with the CI422 a slim-straight electrode; the second group consisted of 10 patients implanted with the Hybrid-L, a shorter hearing preservation electrode.Main Outcome Measure: Audiometric thresholds and speech perception measures.Results: At 1 year, 3/10 (30%) patients with the Hybrid-L and 7/12 (58%) patients with the CI422 lost residual acoustic hearing resulting in a profound hearing loss in the implanted ear. In comparing these patients in particular, mean CNC words in the implanted ear were 72% in the CI422 electrode group and 15% in the Hybrid-L electrode group at 1 year (P = 0.03). While hearing preservation rates with the Hybrid-L tended to be better, among recipients who lost residual hearing, speech perception was better in those with the longer CI422 electrode.Conclusions: With emphasis on preservation of residual hearing, patients need to be counseled regarding possible outcomes and options should loss of residual hearing occur following implantation. While shorter electrodes may have better rates of hearing preservation, the patients with the longer straight electrode in our study had significantly better speech understanding following the loss of residual hearing. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis In pediatric patients with congenital malformations of the inner ear, anomalies within the anatomy may facilitate unintentional insertion of the cochlear implant electrode into the internal auditory canal. Revision procedures for removal and replacement of cochlear implant electrodes following internal auditory canal insertion are fraught with potential danger, including the theoretical risk of injury to vasculature within the internal auditory canal, repeat insertion within the internal auditory canal, and cerebrospinal fluid leak. The objective of this presentation is to describe a technique for revision cochlear implantation following internal auditory canal insertion to minimize the potential associated risks. Study Design Case series. Methods A retrospective chart review was performed on all patients at a tertiary care facility who underwent revision cochlear implantation for internal auditory canal insertion between January 1999 and July 2011. Results A total of four patients referred from outside institutions have undergone revision cochlear implantation for internal auditory canal insertion. The records from these patients were reviewed. Electrodes were safely removed in all cases without injury to the anterior inferior cerebellar artery or its branches (i.e., labyrinthine artery). Complete insertion was accomplished on reimplantation. Neural response telemetry was performed in all cases, and responses were noted. Fluoroscopy was utilized to visualize electrode progression during insertion. A detailed description of the operative technique is provided. Conclusions This case series describes a technique for revision cochlear implantation that appears to be safe and effective in preventing potential associated complications. Laryngoscope, 123:3141-3147, 2013 [ABSTRACT FROM AUTHOR]

Objectives To determine which demographic or performance variables are associated with inconsistent use of a second implant in pediatric recipients of sequential bilateral cochlear implants (CIs). Methods A retrospective chart review was conducted on pediatric recipients of sequential bilateral CIs. Children were divided into two age groups, 5-9 and 10-17 years of age. For each group, we examined whether inconsistent use of the second implant (CI-2) was associated with a variety of demographic variables, or speech-perception scores. Results In children aged 5-9 years, inconsistent use of CI-2 was not significantly associated with any demographic variable, but was related to both the word-recognition score with CI-2, and the difference in word-recognition scores between the first implant (CI-1) and CI-2. In children aged 10-17 years, these relationships were not significant due to smaller number of subjects. Finally, CI-2 word-recognition scores across all children were significantly correlated with the age of implantation for both CI-1 and CI-2, and the time between CI-1 and CI-2 surgeries. Discussion Speech-recognition scores obtained with CI-2, and the extent to which it differs from CI-1, are most closely related with inconsistent use of CI-2 in pediatric sequential implantees. These results are consistent with similar data previously reported by other investigators. While children implanted with CI-2 at a later age generally perform more poorly, most children still use both implants, and benefit from CI-2 even when receiving the implant as an adolescent. Conclusion In pediatric recipients of sequential bilateral CIs, inconsistent use of CI-2 is related to the speech recognition scores with CI-2, and the difference in speech-recognition scores between CI-1 and CI-2. In addition, speech-recognition scores with CI-2 are related to the amount of time between CI-1 and CI-2 surgeries, and the age of implantation for both CI-1 and CI-2. [ABSTRACT FROM AUTHOR]

Objective: To determine cochlear implant electrode stability in the young patient. Electrode migration due to future skull growth was a concern that led to prohibiting implantation in children less than 2 years of age. Recently, the high level of performance achieved by young implantees has led to a re-evaluation of this lower age limit, requiring an assessment of the effects of skull growth over time. Study Design: Prospective radiographic analysis of electrode position of cochlear implants in young children. Methods: Twenty-seven children implanted with the Nucleus (Cochlear Corp., Denver, CO) or Clarion (Advanced Bionics Inc., Sylmar, CA) multichannel cochlear prostheses were subjects for this study. Follow-up radiographic studies were obtained for a period of 1 month to 5 years after implantation. The age at time of implantation ranged from 14 months to 5 years. An intraoperative modified Stenver's view plain radiograph was obtained as a baseline. After implantation, on a yearly basis transorbital Stenver's and base views were obtained for comparative purposes. Additional radiographs were obtained whenever a change in performance or electrode map caused suspicion for extrusion. Electrode position was determined using a computer graphics enhancement technique whereby image contrast filters enhanced the visibility of the electrode array and surrounding bony structures. Results: An analysis of the data revealed no migration of the electrode array over time. Conclusions: The confirmation of the stability of the electrode array alleviates the concern of the effects of skull growth on cochlear implantation in young children. [ABSTRACT FROM AUTHOR]

Objective To compare the intraoperative electrically evoked auditory brainstem response (EABR) morphologies between neurofibromatosis II (NF2) adult auditory brainstem implant (ABI) recipients who had auditory percepts post-operatively and those who did not and between NF2 adult ABI recipients and non-NF2 pediatric ABI recipients. Methods This was a retrospective case series at a single tertiary academic referral center examining all ABI recipients from 1994 to 2016, which included 34 NF2 adults and 11 non-NF2 children. The morphologies of intraoperative EABRs were evaluated for the number of waveforms showing a response, the number of positive peaks in those responses, and the latencies of each of these peaks. Results 27/34 adult NF2 patients and 9/10 children had EABR waveforms. 20/27 (74.0%) of the adult patients and all of the children had ABI devices that stimulated post-operatively. When comparing the waveforms between adults who stimulated and those who did not stimulate, the proportion of total number of intraoperative EABR peaks to total possible peaks was significantly higher for the adults who stimulated than for those who did not (p < 0.05). Children had a significantly higher proportion of total number of peaks to total possible peaks when compared to adults who stimulated (p < 0.02). Additionally, there were more likely to be EABR responses at the initial stimulation than intraoperatively in the pediatric ABI population (p = 0.065). Conclusions The value of intraoperative EABR tracing may lie in its ability to predict post-operative auditory percepts based on the placement of the array providing the highest number of total peaks. [ABSTRACT FROM AUTHOR]