Faculty Bibliography

OBJECTIVE:To evaluate the performance changes after sequential bilateral cochlear implantation in a pediatric population of bimodal cochlear implant (CI) users. To evaluate the factors which influence the parental and recipient decision to discontinue hearing aid use and seek a second implant. STUDY DESIGN/METHODS:Retrospective case review, within-subject comparison. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Thirty-one pediatric (<18 yr) bimodal CI users who underwent sequential bilateral CI. INTERVENTIONS/METHODS:Sequential bilateral CI. MAIN OUTCOME MEASURES/METHODS:Parental and/or recipient's reasons for discontinuing their hearing aid and pursuing a second implant, device usage from datalogs, speech understanding in the bimodal and bilateral CI condition. RESULTS:Parents/patients were motivated to pursue sequential bilateral CI based on their positive performance with CI1, the expectation of further improvement with a second CI, and the prospect of having a second independently functional ear. In the bimodal condition, mean word recognition score (WRS), sentence recognition in quiet (SIQ), and sentence recognition in noise (SIN) scores were 87.4, 97.3, and 92.9% respectively. At 1-year post-sequential bilateral CI, the mean WRS, SIQ, and SIN score were 92.7, 98.7, and 97.7%, respectively. The improvement in bilateral CI speech scores compared with bimodal scores was statistically significant for WRS (p = 0.015). A ceiling effect limited the ability to detect further meaningful differences on speech perception testing. CONCLUSIONS:The bilateral CI condition demonstrates equivalent or slightly superior performance compared with the bimodal condition. Several non-speech benefits were elicited from parents as reasons for pursuing a second implant. Close monitoring of the residual acoustic hearing, inquiring about the perceived benefits provided by the HA, and early counseling regarding the potential for sequential bilateral CI are important aspects in determining if and when a second implant is indicated.

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.

OBJECTIVE:Cochlear implant (CI) technology and techniques have advanced over the years. There has not been the same degree of change in programming and there remains a lack of standardization techniques. The purpose of this study is to compare performance in cochlear implant subjects using experienced clinician (EC) standard programming methods versus an Artificial Intelligence, FOX based algorithm for programming. STUDY DESIGN/METHODS:Prospective, nonrandomized, multicenter study using within-subject experimental design SETTING:: Tertiary referral centers. PATIENTS/METHODS:Fifty-five adult patients with ≥ 3 months experience with a Nucleus 5, 6, Kanso, or 7 series sound processor. INTERVENTION/METHODS:Therapeutic Main Outcome Measures: CNC words and AzBio sentences in noise (+10 dB SNR) tests were administered in a soundproof booth followed by a direct connect psychoacoustic battery using the EC program. Tests were repeated 1 month later using the optimized FOX program. Subjective measures of patient satisfaction were also measured. RESULTS:Performance for the EC program was compared to the FOX program for both measures. Group mean results revealed equivalent performance (Kruskal-Wallis ANOVA p = 0.934) with both programming methods. While some patients had better performance with the FOX method and some performed more poorly, the majority had equivalent performance and preferred the FOX system. CONCLUSION/CONCLUSIONS:The study demonstrated that on average, FOX outcomes are equivalent to those using traditional programming techniques. In addition, the FOX programming method can effect standardization across centers and increase access for many individuals who could benefit.

Objective: To describe cochlear implantation (CI) outcomes for rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment.
Method(s): Retrospective review between 1995 and 2019 from a single tertiary care center of all patients with retrocochlear pathology who underwent CI. Demographics, clinical history, and audiometric data were reviewed. Study endpoints include (1) logged device use, (2) ability to achieve auditory perception, and (3) word recognition score (WRS) in the CI-only condition.
Result(s): Thirty-two patients (63% of females) with retrocochlear pathology were implanted at our center. The average age at implantation was 46.9 years (SD: 19, range: 13-80). Mean duration of deafness was 4.5 years (SD: 5.0, range: 0.4-19.0). Etiology of hearing loss included VS in 24 (75%), CNS malignancy treated with radiation in 4 (13%), intralabyrinthine schwannoma in 2 (6%), head and neck malignancy treated with radiation in 1 (3%), and superficial siderosis in 1 (3%). The mean preoperative PTA was 95.8 dBHL (SD 24.7) and WRS was 7.2% (SD 13.1). Of the 24 VSs, 21 were NF2-associated and 3 were sporadic. The mean tumor size was 1.64 cm (SD: 0.6, range: 0.5-2.6 cm). At the time of CI, 11 patients had prior microsurgery, 6 patients had prior radiation to the ipsilateral tumor, and 7 patients had stable tumors without prior surgery or radiation. Device use was classified as regular (>7 hours/day) in 15 (47%), limited (<7 hours/day) in 12 (38%), and nonuse is 5 (16%). The audiometric outcomes of 26 patients are reported, as the other 6 patients have been implanted too recently for review. Auditory perception was achieved in 24/26 patients. The two patients who failed to achieve auditory perception underwent prior surgery. Open-set speech recognition (WRS > 20%) was achieved in 18 patients. Meaningful sound perception but without significant open-set speech (WRS < 20%) was seen in six patients. Altogether, the mean WRS at most recent follow-up (mean: 3.4 years, SD: 1.8) for the observation, microsurgery, and radiation cohorts was 51% (SD: 15), 36% (SD: 28), and 39% (SD: 26), respectively. Over long-term follow-up, two patients experienced decline in CI performance associated with tumor regrowth and necessitated additional surgery; both underwent explantation of the CI and successful auditory brainstem implantation. The remaining patients have demonstrated durable benefit. A multivariate analysis is presented to evaluate the effects of the following variables: duration of deafness, time interval between treatment and CI, diagnosis of NF2, treatment cohort, pathology, and status of hearing in the contralateral ear.
Conclusion(s): In appropriately selected patients, cochlear implantation is feasible for the rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment. Given the heterogeneity inherent to this population, outcomes are variable. In most cases, auditory percept was achieved and over half of the patients obtained open-set speech perception, irrespective of prior management and treatment

OBJECTIVES/OBJECTIVE:The auditory experience of early deafened pediatric cochlear implant (CI) users is different from that of postlingually deafened adult CI users due to disparities in the developing auditory system. It is therefore expected that the auditory psychophysical capabilities between these two groups would differ. In this study, temporal resolving ability was investigated using a temporal modulation detection task to compare the performance outcomes between these two groups. DESIGN/METHODS:The minimum detectable modulation depth of amplitude modulated broadband noise at 100 Hz was measured for 11 early deafened children with a CI and 16 postlingually deafened adult CI users. RESULTS:Amplitude modulation detection thresholds were significantly lower (i.e., better) for the pediatric CI users than for the adult CI users. Within each group, modulation detection thresholds were not significantly associated with chronologic age, age at implantation, or years of CI experience. CONCLUSIONS:Early implanted children whose auditory systems develop in response to electric stimulation demonstrate better temporal resolving abilities than postlingually deafened adult CI users. This finding provides evidence to suggest that early implanted children might benefit from sound coding strategies emphasizing temporal information.

OBJECTIVE:To assess auditory brainstem implant (ABI) outcomes in children with a prospective study. METHODS:Twelve patients with cochlear nerve deficiency received an auditory brainstem implant. Patients were evaluated with age appropriate speech perception and production assessments, and health related quality of life (HRQoL) surveys for parents of subjects and for subjects if age appropriate. RESULTS:Twelve patients received an ABI without major complications. Eleven out of twelve received some auditory benefit from their ABI. Parental HRQoL ratings were positive for all domains with the exception of communication. Self reported overall HRQoL metrics from two subjects were also positive. CONCLUSIONS:ABI is a good option for patients who are not eligible for or fail CI. Our findings show that despite varying degrees of postoperative performance, HRQoL ratings were positive. The presence of additional disabilities and health problems resulted in less positive HRQoL outcomes. Our results emphasize the need to assess outcomes in these patients beyond speech perception and communication.

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/CONCLUSIONS: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/METHODS:2b. Laryngoscope, 2018.

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.

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.

OBJECTIVE: To characterize the performance plateau after unilateral cochlear implantation (CI) in prelingually and postlingually deafened adults and to compare their relative progress. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Prelingually and postlingually deaf adults who received a unilateral CI and completed a minimum of 2 years of follow-up at our center. INTERVENTION: Unilateral CI. MAIN OUTCOME MEASURES:: Standard speech perception testing (consonant-nucleus-consonant [CNC] monosyllabic word test and hearing in noise test [HINT] or AzBio sentence test) were performed preoperatively and 3 and 12 months postoperatively, and annually thereafter. RESULTS: In postlingually deaf patients (n = 102), there was a significant improvement in word scores for 3 years postimplantation (p < 0.01). Beyond the 3 years postoperative time point, word scores continued to improve, albeit at a flatter rate. In prelingually deaf patients (n = 16) word scores improved significantly for 5 years postimplantation (p = 0.03). CONCLUSIONS: Adults with postlingual deafness undergoing unilateral CI show significant improvement in speech perception for 3 years postimplantation, at which point their performance continues to improve, albeit at a flatter rate. The performance of adults with prelingual deafness improves significantly as late as 5 years postimplantation. These time intervals reflect a change to the currently reported 6 to 12 months period and should impact on counseling, especially in the prelingual CI candidate.