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Objective: To characterize transimpedance matrix (TIM) heatmap patterns in patients at risk of labyrinthine abnormality to better understand accuracy and possible TIM limitations., Study Design: Retrospective review of TIM patterns, preoperative, and postoperative imaging., Setting: Tertiary referral center., Patients: Patients undergoing cochlear implantation with risk of labyrinthine abnormality., Intervention: None., Results: Seventy-seven patients were evaluated. Twenty-five percent (n = 19) of patients had a TIM pattern variant identified. These variants were separated into 10 novel categories. Overall, 9% (n = 6) of electrodes were malpositioned on intraoperative x-ray, of which 50% (n = 3) were underinserted, 17% (n = 1) were overinserted, 17% (n = 1) had a tip foldover, and 17% (n = 1) had a coiled electrode. The number of patients with a variant TIM pattern and normal x-ray was 18% (n = 14), and the number of patients with normal TIM pattern and malposition noted on x-ray was 3% (n = 2; both were electrode underinsertions that were recognized due to open circuits and surgical visualization).A newly defined skip heat pattern was identified in patients with IP2/Mondini malformation and interscalar septum width <0.5 mm at the cochlear pars ascendens of the basal turn., Conclusions: This study defines novel patterns for TIM heatmap characterization to facilitate collaborative and comparative research moving forward. In doing so, it highlights a new pattern termed skip heat, which corresponds with a deficient interscalar septum of the cochlea pars ascendens of the basal turn in patients with IP2 malformation. Overall, the data assist the surgeon in better understanding the implications and limitations of TIM patterns within groups of patients with risk of labyrinthine abnormalities., Competing Interests: Conflicts of interest and source of funding: Dr. J Thomas Roland Jr. is a consultant for Cochlear Americas and received research funding for CI-related projects, which are not affiliated with this research., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: Children with high-frequency severe-to-profound hearing loss and low-frequency residual hearing who do not derive significant benefit from hearing aids are now being considered for cochlear implantation. Previous research shows that hearing preservation is possible and may be desirable for the use of electroacoustic stimulation (EAS) in adults, but this topic remains underexplored in children. The goal of this study was to explore factors relating to hearing preservation, acceptance, and benefits of EAS for children., Study Design: Retrospective review., Setting: Tertiary academic medical center., Patients: Forty children (48 ears) with preoperative low-frequency pure-tone averages of 75 dB HL or less at 250 and 500 Hz (n = 48)., Intervention: All patients underwent cochlear implantation with a standard-length electrode., Main Outcome Measure: Low-frequency audiometric thresholds, speech perception, and EAS usage were measured at initial stimulation, and 3 and 12 months postoperatively. Outcomes were compared between children with and without hearing preservation, and between EAS users and nonusers., Results: Hearing was preserved at similar rates as adults but worse for children with an enlarged vestibular aqueduct. Fewer than half of children who qualified to use EAS chose to do so, citing a variety of audiologic and nonaudiologic reasons. No differences were detected in speech perception scores across the groups for words, sentences, or sentences in noise tests., Conclusions: Neither hearing preservation nor EAS use resulted in superior speech perception in children with preoperative residual hearing; rather, all children performed well after implantation., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: To prospectively evaluate the frailty phenotype in a population of older adults and determine its association with 1) perioperative complications, 2) need for vestibular rehabilitation after surgery, and 3) early speech perception outcomes., Study Design: Prospective cohort study., Setting: Tertiary care hospital., Patients: Adults older than 65 years undergoing cochlear implantation., Interventions: The Fried Frailty Index was used to classify patients as frail, prefrail, or not frail based on five criteria: 1) gait speed, 2) grip strength, 3) unintentional weight loss, 4) weekly physical activity, and 5) self-reported exhaustion., Main Outcomes Measures: Rates of intraoperative and postoperative complications, postoperative falls, need for vestibular rehabilitation, and early speech perception outcomes., Results: Forty-six patients were enrolled in this study. Five patients (10.8%) were categorized as frail and 10 (21.7%) as prefrail. The mean ages of frail, prefrail, and not frail patients were 80.9, 78.8, and 77.5, respectively. There were no intraoperative complications among all groups. Three patients required postoperative vestibular rehabilitation; all were not frail. One postoperative fall occurred in a nonfrail individual. Mean (standard deviation) device use times at 3 months in frail, prefrail, and not frail patients were 7.6 (3.5), 11.1 (3.6), and 11.6 (2.9) hours per day, respectively. Consonant-nucleus-consonant word scores 3 months after surgery in frail, prefrail, and not frail patients were 13% (12.2), 44% (19.7), and 51% (22.4), respectively. The median (range) number of missed follow-up visits (surgeon, audiologist, speech language pathologist combined) was 7 (1-10) in frail patients, compared with a median of 3 (0-4) and 2 (0-5) in prefrail and not frail patients., Conclusions: Frail patients did not have increased rates of surgical complications, need for vestibular rehabilitation, or postoperative falls. However, frail patients experienced challenges in accessing postoperative care, which may be addressed by using remote programming and rehabilitation., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To evaluate the ability of the transimpedance matrix (TIM) measurement to detect cochlear implant electrode tip foldover by comparing results to a "gold standard," the intraoperative plain film radiograph., Study Design: Retrospective case series., Setting: Tertiary referral hospital., Patients: One hundred three patients who underwent cochlear implantation between June 2020 and August 2021., Interventions: Intraoperative electrophysiologic monitoring (electrode impedances, neural response telemetry, and TIM measurement) and modified Stenver's view plain film radiographs., Main Outcome Measures: Identification of tip foldover on both TIM and plain films., Results: In total, 103 patients (117 ears) had both a TIM measurement and intraoperative X-ray available for review, including 68 adults and 35 children. One hundred patients (85%) received the Cochlear Slim Modiolar electrode. Tip foldovers were noted in three of 117 implants (2.5%). In all cases, TIM was able to detect the foldover, and the electrode arrays were reinserted with the patients still under anesthesia, with repeat X-ray demonstrating a normal configuration. Two other abnormal TIM patterns were identified. One was in a patient with an obstructed cochlea in whom only 10 electrodes could be inserted, the other was in a patient with a common cavity abnormality. One additional patient underwent electrode repositioning intraoperatively because of overinsertion. In this patient, the TIM appeared to be within normal limits, but the over-insertion was apparent on X-ray. Overall, the sensitivity and specificity of TIM measurements in detecting electrode tip foldover were both 100%., Conclusion: TIM measurements were able to accurately identify tip foldovers. More research is needed to define the adjunctive role of TIM as an intraoperative measure., Competing Interests: J.T.R. is a consultant for Cochlear Americas and receives research grant money. S.O.M. is a consultant for Advanced Bionics. D.J. received grant money from Advanced Bionics and consulted for Cochlear Corp. The remaining authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To evaluate outcomes of auditory implants in children with CHARGE syndrome and describe the evolution in management of hearing loss in this complex population., Methods: A retrospective case review was performed at a tertiary referral center. Children with CHARGE syndrome who received either a cochlear implant (CI) or auditory brainstem implant (ABI) were included. Clinical records, demographic information, CHARGE features, neuroimaging, audiology, hearing rehabilitation interventions, operative notes, and outcomes were reviewed., Results: Thirteen children with CHARGE syndrome underwent a total of 19 cochlear implants between 2008 and 2020. Among the congenitally deafened children (n = 9), six underwent bilateral implantation (five simultaneous and one sequential). Bilateral implantation was performed even in the presence of diminutive-appearing cochlear nerves. The average age of implantation was 1.1 years, and the mean device use time was 9.4 hours per day. Patients showed improvements in subjective family assessment related to hearing. In this group, two patients use oral communication, five use total communication, and two use sign language exclusively. Among the children with progressive hearing loss, the mean age of hearing deterioration was 4.4 years of age, and the device use time on average was 9.8 hours per day. The highest performer in the cohort was a child who lost hearing in their only hearing ear at age 4 and had normal cochleovestibular anatomy on that side. One child received an auditory brainstem implant at age two after deriving no benefit from a CI and can detect environmental sounds but is currently a nonuser. Over time, we noted that implantation occurred earlier in life and that practice has shifted toward bilateral implantation., Conclusions: Compared to a previous institutional cohort, children evaluated in this study were often implanted at a younger age and bilaterally with significantly improved outcomes. A CI evaluation should be considered in children with CHARGE syndrome to maximize sensory input and auditory ability., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To investigate a new surgical and signal processing technique that provides apical stimulation of the cochlea using a cochlear implant without extending the length of the electrode array., Patients: Three adult patients who underwent cochlear implantation using this new technique., Interventions: The patients received a cochlear implant. The surgery differed from the standard approach in that a ground electrode was placed in the cochlear helicotrema via an apical cochleostomy rather than in its typical location underneath the temporalis muscle. Clinical fitting was modified such that low frequencies were represented using the apically placed electrode as a ground., Main Outcome Measures: Pitch scaling and speech recognition., Results: All surgeries were successful with no complications. Pitch scaling demonstrated that use of the apically placed electrode as a ground lowered the perceived pitch of electric stimulation relative to monopolar stimulation. Speech understanding was improved compared with preoperative scores., Conclusions: The new surgical approach and clinical fitting are feasible. A lower pitch is perceived when using the apically placed electrode as a ground relative to stimulation using an extracochlear ground (i.e., monopolar mode), suggesting that stimulation can be provided more apically without the use of a longer electrode array. Further work is required to determine potential improvements in outcomes and optimal signal processing for the new approach., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: To describe our institutional experience with cochlear implantation (CI) for rehabilitation of hearing loss in Neurofibromatosis type 2 (NF2) patients., Study Design: Retrospective review between 1989 and 2019., Setting: Tertiary-care center., Patients: Twenty-four patients (67% female, mean age 45.6years) with NF2. Management of their ipsilateral vestibular schwannoma included microsurgery (n=12), stereotactic radiation (n=5), and observation (n=7)., Interventions: Cochlear implantation., Main Outcome Measures: Ability to obtain open-set speech, daily device usage and long-term device benefit., Results: All patients achieved some degree of sound awareness with CI. Nineteen patients (79%) achieved open-set speech understanding with a mean word-recognition score of 43% (range 0-88%). Patients with tumors 1.5 cm or less demonstrated the better speech understanding, without significant differences among treatment modalities. For tumors greater than 1.5 cm, patients who underwent microsurgery had a lower rate of open-set speech understanding compared to those treated with radiation or observation. Regular daily device use in 83% of patients was found. Long-term use (>10years) was observed in several patients, though some ultimately required reimplantation with an auditory brainstem implant due to progressive tumor growth. Mean follow-up duration was 4.1 years (range 0.4-15)., Conclusions: Cochlear implantation can be an effective treatment for hearing loss in NF2 patients provided the cochlear nerve is intact, regardless of prior management for the ipsilateral tumor. The degree of benefit varies and is influenced by tumor size. Management strategies that preserve the cochlear nerve maximize the interval during which a CI could be of benefit to NF2 patients., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: To describe our experience with adults undergoing cochlear implantation (CI) for treatment of single-sided deafness (SSD)., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Fifty-three adults with SSD., Interventions: Unilateral CI., Main Outcome Measures: Speech perception testing in quiet and noise, tinnitus suppression, and device usage from datalogs., Results: The mean age at CI was 53.2 years (SD 11.9). The mean duration of deafness was 4.0 years (SD 7.8). The most common etiology was idiopathic sudden SNHL (50%). Word recognition improved from 8.7% (SD 15) preoperatively to 61.8% (SD 20) at a mean follow-up of 3.3 years (SD 1.8) (p < 0.0001). Adaptive speech recognition testing in the "binaural with CI" condition (speech directed toward the front and noise toward the normal hearing ear) revealed a significant improvement by 2.6-dB SNR compared to the preoperative unaided condition (p = 0.0002) and by 3.6-dB SNR compared to when a device to route sound to the contralateral side was used (p < 0.0001). Tinnitus suppression was reported to be complete in 23 patients (43%) and improved in 20 patients (38%) while the device was on. The addition of the CI did not lead to a decrement in hearing performance in any spatial configuration. Device usage averaged 8.7 (SD 3.7) hours/day., Conclusions: Cochlear implantation in adult SSD patients can suppress tinnitus and achieve speech perception outcomes comparable with CI in conventional candidates. Modest improvements in spatial hearing were also observed and primarily attributable to the head shadow effect. Careful patient selection and counseling regarding potential benefits are important to optimize outcomes., (Copyright © 2020, Otology & Neurotology, Inc.)

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: Retrospective case review, within-subject comparison., Setting: Tertiary referral center., Patients: Thirty-one pediatric (<18 yr) bimodal CI users who underwent sequential bilateral CI., Interventions: Sequential bilateral CI., Main Outcome Measures: 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.

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: Prospective, nonrandomized, multicenter study using within-subject experimental design SETTING:: Tertiary referral centers., Patients: Fifty-five adult patients with ≥ 3 months experience with a Nucleus 5, 6, Kanso, or 7 series sound processor., Intervention: 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: 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 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.

Objectives: Although there are various available treatment options for unilateral severe-to-profound hearing loss, these options do not provide the benefits of binaural hearing since sound is directed from the poorer ear to the better ear. The purpose of this investigation was to review our center's experience with cochlear implantation in such patients in providing improved auditory benefits and useful binaural hearing., Study Design: Retrospective chart review., Methods: Twelve adult patients and four pediatric patients with unilateral severe-to-profound hearing loss received an implant in the poorer ear. Outcome measures performed preoperatively on each ear and binaurally included consonant-nucleus-consonant (CNC) monosyllabic words and sentences in noise. The mean pure-tune average in the better ear was within normal range., Results: Test scores revealed a significant improvement in CNC and sentence in noise test scores from the preoperative to most recent postoperative evaluation in the isolated implant ear. All adult subjects use the device full-time., Conclusions: The data reveal significant improvement in speech perception performance in quiet and in noise in patients with single-sided deafness after implantation. Performance might depend on factors including length of hearing loss, age at implantation, and device usage.

Objective: To assess whether recombinant growth factor (hGH) therapy has an effect on cochlear implant (CI) performance., Patients: Two pediatric CI recipients (S1, S2) who underwent treatment with hGH for short stature were identified for review. S1 has bilateral labyrinthine dysplasia and received implants at ages 10 months (right) and 4 years 3 months (left). S2 was diagnosed with severe to progressive sensorineural hearing loss bilaterally and received a CI at age 9 years 10 months (left)., Intervention(s): Case series., Main Outcome Measure(s): Cochlear implant, hGH, and speech perception data were collected. Phonetically Balanced Kindergarten (PBK) and Consonant Nucleus Consonant (CNC) word recognition scores were reviewed to assess auditory perception. Electrode impedances, threshold levels, and comfort levels were also reviewed., Results: After 4 months of hGH, word recognition scores for S1 were observed to decrease from 90 to 72% (right) and were stable at 40% (left). Despite troubleshooting, performance continued to decline bilaterally to 52% (right) and 28% (left), and the decision was made to discontinue hGH. One month after cessation of hGH, word recognition scores began improving to 74% (right) and 68% (left). Word recognition scores for S2 were observed to have decreased from 92% the previous year to 82% after taking hGH for 2 months. Given both our previous experience with S1 and discussions with S2's parents, hGH was discontinued after 10 months of therapy. Two months after cessation of hGH, S2's word recognition had improved to 86% (left)., Conclusions: Our case studies illustrate that implanted children undergoing treatment with hGH may experience a decrease in speech perception, which recovers after the cessation of treatment. Since hGH use has become more prevalent in recent years, it is important to inquire whether children undergoing, or who have undergone, implantation are receiving hGH so that they may be appropriately monitored.

Objective: To analyze the effects of residual hearing on postoperative speech performance in traditional cochlear implant (CI) patients implanted with a conventional electrode., Study Design: Retrospective review., Setting: Academic tertiary referral center., Patients: A total of 129 adults implanted by a single surgeon at a tertiary care facility between June 2005 and November 2010 with measurable preoperative pure tone thresholds at any frequency were included., Intervention: Cochlear implantation with a conventional electrode via an anterior inferior cochleostomy., Main Outcome Measure: Speech perception using monosyllabic word scores in quiet and sentences in quiet and noise in the electric (CI-only) condition of the implanted ear. Preservation of hearing was defined as complete for postoperative thresholds within 10 dB of preimplant values and partial if greater than 11 dB. Pure tone audiometry and speech perception testing were performed preoperatively and at regular intervals postoperatively, with the 1-year evaluation being the final outcome period., Results: Preservation at any frequency or level was not a factor in speech perception outcome, although preservation was more common in low frequencies. Hearing preservation was correlated with younger age at implantation, but was not related to length of hearing loss, cause of deafness, device type, sex, preoperative speech performance, or low-frequency pure-tone average., Conclusion: Hearing can be preserved in traditional CI patients implanted with a conventional electrode. Although preservation of hearing may have implications for future technology, it is not currently correlated with speech performance in the CI-only condition.

Objective: This pilot study examined speech recognition, localization, temporal and spectral discrimination, and subjective reports of cochlear implant (CI) recipients with unilateral deafness., Study Design: Three adult male participants with short-term unilateral deafness (<5 yr) participated. All had sudden onset of severe-to-profound hearing loss in 1 ear, which then received a CI, and normal or near normal hearing in the other ear. Speech recognition in quiet and noise, localization, discrimination of temporal and spectral cues, and a subjective questionnaire were obtained over several days. Listening conditions were CI, normal hearing (NH) ear, and bilaterally (CI and NH)., Results: All participants had open-set speech recognition and excellent audibility (250-6,000 Hz) with the CI. Localization improved bilaterally compared with the NH ear alone. Word recognition in noise was significantly better bilaterally than with the NH ear for 2 participants. Sentence recognition in various noise conditions did not show significant bilateral improvement; however, the CI did not hinder performance in noise even when noise was toward the CI side. The addition of the CI improved temporal difference discrimination for 2 participants and spectral difference discrimination for all participants. Participants wore the CI full time, and subjective reports were positive., Conclusion: Overall, the CI recipients with unilateral deafness obtained open-set speech recognition, improved localization, improved word recognition in noise, and improved perception of their ability to hear in everyday life. A larger study is warranted to further quantify the benefits and limitations of cochlear implantation in individuals with unilateral deafness.

Objective: To generate an evidence-based algorithm for the use of intraoperative testing during cochlear implantation (CI)., Study Design: Retrospective review., Setting: Tertiary referral center., Patients: A total of 277 children (aged 6 mo to 17 yr) and adults 18 years and older with normal cochlear anatomy who underwent primary and revision cochlear implantation at a single center between 2005 and 2010 were included., Intervention: Intraoperative electrophysiologic monitoring and intraoperative Stenver's view plain film radiography., Main Outcome Measure: Intraoperative testing included the following: 1) individual electrode impedance measurements; 2) neural response telemetry (tNRT) levels for electrodes E20, E15, E10, and E5; and 3) plain film radiograph assessment of electrode position., Results: No patient demonstrated abnormalities on all 3 modalities. Open or short electrodes on impedance testing were found in 6% of patients; half of these normalized when remeasured. Absent tNRT responses on 1 or more electrodes occurred in 14% of patients, although complete lack of response was rare (1.4%) and did not correlate with a dysfunctional device. Spread of excitation was performed in 1 patient and was consistent with a tip rollover. Intraoperative radiography identified tip-rollover and extracochlear electrode placement in all cases (n = 5, 1.8%) and prompted the use of the backup device., Conclusion: Immediate intraoperative determination of device functionality and optimal electrode placement is advantageous. Of the modalities tested, including electrode impedance, tNRT, and plain radiograph, only the radiographic results impacted intraoperative surgical decision making and led to the use of the backup device.

Objective: To investigate whether children implanted in the first year of life show higher levels of speech perception than later-implanted children, when compared at the same ages and to investigate the time course of sensitive periods for developing speech perception skills. More specifically, to determine whether faster gains in speech perception are made by children implanted before 1 year old relative to those implanted at 2 or 3 years., Study Design: Retrospective cohort study., Setting: Tertiary academic referral center., Patients: 117 children with congenital profound bilateral sensorineural hearing loss, with no additional identified disabilities., Intervention: Cochlear implantation in the first, second, or third year of life., Main Outcome Measure: Development curves showing Lexical Neighborhood Test (LNT) word identification scores as a function of age., Results: Children implanted within the first year of life have a mean advantage of 8.2% LNT-easy word scores over those implanted in the second year (p < 0.001) and a 16.8% advantage in LNT-easy word scores over those implanted in the third year of life (p < 0.001). These advantages remained statistically significant after accounting for sex, residual hearing, and bilateral cochlear implant use. When speech perception scores were expressed as a function of "hearing age" rather than chronological age, however, there were no significant differences among the 3 groups., Conclusion: There is a clear speech perception advantage for earlier-implanted children over later-implanted children when compared at the same age but not when compared at the same time after implantation. Thus, the sensitive period for developing word identification seems to extend at least until age 3 years.

Objective: To determine whether intraoperative neural response telemetry (tNRT) is predictive of postoperative speech perception., Study Design: Retrospective review., Setting: Tertiary referral center., Patients: Children (n = 24) aged between 5 and 17 years and adults 18 years and older (n = 73) with severe-to-profound hearing loss and implanted with the Nucleus Freedom device between 2005 and 2008 and observed at least 1 year were included., Intervention: Intraoperative neural response telemetry after insertion of the electrode array., Main Outcome Measure: Measures included 1) intraoperative tNRT measurements and 2) preoperative and 1-year postoperative open-set word recognition scores using age-appropriate open-set tests for children and adults. Intraoperative neural response telemetry levels for electrodes E20, E15, E10, and E5 in each patient were correlated to performance at the 1-year evaluation interval., Results: No correlation existed between tNRT responses and open-set speech performance at the 1-year evaluation. Several patients had absent tNRT in the OR but developed speech recognition abilities, whereas the remaining patients had intraoperative responses with levels of postoperative performance ranging from 0% to 100%., Conclusion: This study suggests that there is no significant correlation between intraoperative tNRT and speech perception performance at 1 year. At the time of surgery, tNRT provides valuable information regarding the electrical output of the implant and the response of the auditory system to electrical stimulation and preliminary device programming data; however, it is not a valuable predictor of postoperative performance. Furthermore, the absence of tNRT does not necessarily indicate a lack of stimulation.

Objective: Bilateral sequential cochlear implantation outcomes are dependent on many different factors. Newer technology in the second implanted ear may also contribute to outcome. This study examines the effect of cochlear implant technology on speech recognition outcomes in a population of adult patients who have undergone bilateral sequential implantation using different technologies in each ear., Study Design: Retrospective chart review., Setting: Tertiary referral center., Patients: Twenty adults who underwent bilateral sequential cochlear implantation with different technologies and processing strategies in each ear were patients for this study. Control Group A included patients (n = 8) who were simultaneously implanted, and Control Group B (n = 3) were patients who were sequentially implanted with the same technology., Intervention: Bilateral sequential cochlear implantation., Outcome Measures: The outcome measure was the Consonant-Nucleus-Consonant monosyllabic word test administered in each implanted ear and in the binaural condition before and 1 year after operation. A multivariate analysis was performed to account for factors including duration of deafness, length of device usage, and severity of deafness., Results: There was significant improvement from before to 1 year after the operation in word scores for the individual ears and in the binaural condition for all groups. All patients were consistent users of both devices, and the use of different technology in the second implanted ear did not affect the patients' ability to benefit from bilateral implantation despite the use of different devices and processing strategies., Conclusion: Bilateral sequential implantation with newer and/or differing technology in the second implanted ear did not reduce the benefits of bilateral stimulation and should not be considered a deterrent to second-sided implantation.

Objective: To examine speech perception outcomes as related to a reduction in the number of functional electrodes postimplantation and to determine the effect of electrode reduction on subsequent device failure., Study Design: Retrospective review., Setting: Tertiary academic referral center., Patients: Of 1,520 children and adults with full insertions of the Advanced Bionics, Med El, and Nucleus devices, 15 (1%) were patients. Patients were included in the study if all electrodes were functional at initial stimulation, but the number of electrodes in use was subsequently reduced at follow-up programming sessions. Exclusion criteria included partial and split-array electrode insertions., Intervention(s): Patients with bilateral severe to profound sensorineural hearing loss underwent either unilateral or bilateral cochlear implantation., Main Outcome Measure(s): Postimplantation speech perception tests obtained with a full complement of functional electrodes were performed and the results compared to those obtained with 1 or more electrodes removed from the user program. Electrode deactivation was also correlated with device failure., Results and Conclusion: The results of this study indicate that deactivation of cochlear implant electrodes is relatively uncommon, and although the deactivation does not have a direct influence on speech performance outcomes, the loss of 5 or more electrodes can suggest impending device failure. Additionally, those patients with electrode deactivation coupled with a decline in speech perception scores should also be considered at risk for device failure.

Objective: To review our experience with partial standard and double-array cochlear implantation in the ossified cochlea., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Twenty cochleae in 19 adult and pediatric patients with ossified cochleae., Intervention(s): Patients underwent either partial insertion of a standard electrode array or double-array electrode insertion for their cochlear implantation., Main Outcome Measure(s): Number of electrodes inserted, number of active electrodes at follow-up, and open-set speech performance., Results: Twelve patients (four children and eight adults) had partial insertion of standard array electrode. The mean number of electrodes inserted and active at follow-up was 14.8 and 11.3, respectively. Eight patients (four children and four adults) had a double-array electrode implanted. The mean number of electrodes inserted and active at follow-up was 18.1 and 16.3, respectively. Open-set speech recognition was better in children regardless of duration of deafness. There were no complications., Conclusion: These findings suggest that the double array allows for more usable electrodes than in partially inserted cochlear implant. Compared with adults, children with ossified cochlea do well both in partial standard and double-array insertion. Both insertion techniques are safe, with no complications occurring in our patients. Straight and double arrays should be available in the operating suite for any patient in whom suspected cochlear ossification is confronted.

Objective: To describe a safe and effective technique for the implantation of common cavity cochleae., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Fifteen cochleae in 9 adults and children with common cavity deformities implanted with and without the use of intraoperative fluoroscopy., Intervention(s): : Fluoroscopically assisted cochlear implantation., Main Outcome Measure(s): Rates of extracochlear placement, bending, kinking, intrameatal placement, and complications were analyzed. Numbers of electrodes inserted, number of active electrodes, and speech performance were compared between the 2 groups., Results: Half of the nonfluoroscopy group had suboptimal placement of their electrode arrays. This group also had a higher rate of complications, lower numbers of electrodes implanted, and lower number of active electrodes at follow-up. Results of speech perception testing suggest that fluoroscopically assisted implantation of the common cavity results in improved performance., Conclusion: Intraoperative fluoroscopy is an easy, safe, and effective technique that results in improved outcomes for cochlear implant patients with common cavity deformities.

Objective: To examine speech perception outcomes and determine the impact of length of deafness and time between implants on performance in the sequentially bilateral implanted population., Study Design: Retrospective review., Setting: Tertiary academic referral center., Patients: Forty-three children (age, <18 yr) and 22 adults underwent sequential bilateral implantation with at least 6 months between surgeries. The mean age at the time of the second implant in children was 7.83 years, and mean time between implants was 5.16 years. Five children received the first side implant (C1) below 12 months of age; 16, at 12 to 23 months; 9, between the ages of 24 and 35 months; and 11, at 36 to 59 months; 2 were implanted above the age of 5 years. In adults, mean age at second implant was 46.6 years, and mean time between implants was 5.6 years., Intervention: Sequential implantation with 6 months or more between implantations., Main Outcome Measures: Speech perception tests were performed preoperatively before the second implantation and at 3 months postoperatively., Results: Results revealed significant improvement in the second implanted ear and in the bilateral condition, despite time between implantations or length of deafness; however, age of first-side implantation was a contributing factor to second ear outcome in the pediatric population., Conclusion: Sequential bilateral implantation leads to significantly better speech understanding. On average, patients improved, despite length of deafness, time between implants, or age at implantation.

Objective: To assess word recognition and pitch-scaling abilities of cochlear implant users first implanted with a Nucleus 10-mm Hybrid electrode array and then reimplanted with a full length Nucleus Freedom array after loss of residual hearing., Background: Although electroacoustic stimulation is a promising treatment for patients with residual low-frequency hearing,a small subset of them lose that residual hearing. It is not clear whether these patients would be better served by leaving in the 10-mm array and providing electric stimulation through it, or by replacing it with a standard full-length array., Methods: Word recognition and pitch-scaling abilities were measured in 2 users of hybrid cochlear implants who lost their residual hearing in the implanted ear after a few months. Tests were repeated over several months, first with a 10-mm array, and after, these patients were reimplanted with a full array. The word recognition task consisted of 2 50-word consonant nucleus consonant (CNC) lists. In the pitch-scaling task, 6 electrodes were stimulated in pseudorandom order, and patients assigned a pitch value to the sensation elicited by each electrode., Results: Shortly after reimplantation with the full electrode array, speech understanding was much better than with the 10-mm array. Patients improved their ability to perform the pitch-scaling task over time with the full array, although their performance on that task was variable, and the improvements were often small., Conclusion: 1) Short electrode arrays may help preserve residual hearing but may also provide less benefit than traditional cochlear implants for some patients. 2) Pitch percepts in response to electric stimulation may be modified by experience.

Objective: Emerging evidence in auditory neuroscience suggests that central auditory pathways process speech asymmetrically. In concert with left cortical specialization for speech, a "right-ear advantage" in speech perception has been identified. The purpose of this study is to determine if this central asymmetry in speech processing has implications for selecting the ear for cochlear implantation., Study Design: Retrospective cohort study., Setting: Academic university medical center, Patients: One hundred one adults with bilateral severe-to-profound sensorineural hearing loss, Intervention: Cochlear implantation with the Nucleus 24 Contour device., Main Outcome Measurements: Patients were divided into two groups according to the ear implanted. Results were compared between left-ear- and right-ear-implanted patients. Further subgroup analysis was undertaken, limited to right-handed patients. Postoperative improvement on audiograms and scores on speech perception tests (Hearing in Noise test, City University of New York in quiet and in noise test, Consonant-Vowel Nucleus- Consonant words, and phonemes) at 1 year was compared between groups. Analysis of covariance was used to control for any intergroup differences in preoperative characteristics., Results: The groups were matched in age, duration of hearing loss, duration of hearing aid use, percentage implanted in the better hearing ear, and preoperative audiologic testing. Postoperatively, there were no differences between left-ear- and right-ear-implanted patients in improvement on speech recognition tests., Conclusion: Despite central asymmetry in speech processing, our data do not support a right-ear advantage in speech perception outcomes with cochlear implantation. Therefore, among the many factors in choosing the ear for cochlear implantation, central asymmetry in speech processing does not seem to be a contributor to postoperative speech recognition outcomes.

Objective: Sensorineural hearing loss resulting from otosclerosis, meningitis, chronic otitis media, autoimmune ear disease, and trauma can be associated with partial or total obstruction of the cochlear scalae. Multichannel cochlear implantation may be difficult in a cochlea with an obstructed scala tympani. The purpose of this study is to determine the safety and efficacy of scala tympani electrode insertion., Study Design: Retrospective chart review., Setting: Academic medical center., Patients: Eight children and adults with profound sensorineural hearing loss who underwent cochlear implantation with known scala vestibuli electrode array insertion were subjects for this study., Interventions: Eight study subjects underwent implantation: five with the Nucleus 24RCS (Contour) device and three with the Nucleus 24M device., Outcome Measures: Imaging findings, operative findings, and age-appropriate speech perception testing., Results: All patients had full electrode insertion. Various obstructive patterns on computed tomography and magnetic resonance imaging were found, and there was a range of speech perception results. All but one patient improved based on age-appropriate monosyllabic word and sentence tests., Conclusion: Scala vestibuli multielectrode insertion is a viable alternative when scala tympani insertion is not possible because of abnormal anatomy or anatomical changes secondary to disease or previous implantation. We will also present an algorithm of options for decision making for implantation when encountering cochlear obstruction and difficult electrode insertion.

Objective: Perimodiolar electrode arrays were developed in an attempt to improve stimulation of specific neural populations and to decrease electrical thresholds, thereby decreasing power consumption. Postoperative radiographs show that coiling of the arrays is variable. Our previous study explored the relationship between the angle of coiling, threshold levels, and functional outcomes using the Nucleus Contour electrode array. This study compares coiling angle, electrical threshold levels, and speech perception measures with the Nucleus Contour Advance electrode array implanted using the new advance off stylet technique versus the Nucleus Contour electrode array implanted using the standard technique., Study Design: Retrospective review., Setting: University medical center., Patients: Forty-two adults and children with normal cochlear anatomy implanted with the Nucleus CI24RCA electrode using the advance off stylet technique with at least 1-year follow-up., Intervention: Therapeutic., Main Outcome Measures: Computer-aided radiographic analysis of perimodiolar electrode placement, electrical threshold measurements, and speech perception outcome measures at 1 year postimplantation., Results: The degree of modiolar coiling was tighter using the new electrode and technique in comparison with standard insertion technique using the Nucleus Contour electrode array. The tighter coiling tended to result in higher electrical thresholds. Lower speech perception outcome measures tended to correlate with a higher degree of coiling., Conclusion: The Nucleus Contour Advance electrode array combined with the advance off stylet technique resulted in a more consistent perimodiolar position. However, the tighter coiling resulted in statistically significant increased electrical thresholds and decreased speech perception outcomes. This finding may be secondary to multiple factors, not just coiling angle.

Objective: Perimodiolar electrode arrays were developed to improve stimulation of specific neural populations and to decrease power consumption. Postoperative radiographs suggest that some arrays are more tightly coiled than others. The purpose of this study was to evaluate whether the degree of modiolar coil correlates with electrical threshold and/or performance measures postimplantation with the Nucleus CI24RCS (Contour) device., Study Design: Retrospective review., Setting: Cochlear implant center and a tertiary care hospital., Patients: Twenty-eight adult cochlear implant recipients with normal cochlear anatomy who underwent implantation with the Nucleus C124RCS perimodiolar electrode and are at least 1 year postimplantation., Interventions: Therapeutic., Main Outcome Measures: Radiologic degree of perimodiolar electrode placement (determined by a computer algorithmic analysis) compared with electrical thresholds and standard speech perception outcome measures at 1 year postcochlear implantation., Results: No significant correlations were found between the degree of modiolar coiling of the electrode array and electrical thresholds and speech perception outcome measures at 1 year postcochlear implantation., Conclusions: The degree of coiling of a modiolar hugging electrode array was not directly correlated with the level of electrical thresholds or postoperative speech perception outcome measures. Appearance of coil tightness on postoperative radiographs could reflect either differences in array placement or intrinsic variations in cochlear anatomy, and variations in speech perception performance can be influenced by other factors, including length of deafness.

Objective: The development of oral language in children with a cochlear implant is dependent on numerous factors. Although baseline achievements have been established, ceiling attainment levels have yet to be explored. One indicator of a higher capability level is the ability of children with implants to learn to communicate orally using a second language. The purpose of this research was to explore 1) the feasibility of children with cochlear implants developing oral fluency in a second language and 2) the factors that affect the development., Study Design: Retrospective study of children fulfilling the criteria., Setting: University medical center., Patients: Eighteen profoundly hearing-impaired children who were reported to be bilingual., Intervention: Cochlear implantation at age 5 or younger., Main Outcome Measures: The subjects were evaluated using standard speech perception and receptive and expressive language measures., Results: The data revealed the ability of some pediatric cochlear implant recipients to develop competency in a second spoken language in addition to their primary language. Equally as important is the fact that the majority showed age-appropriate receptive and/or expressive language abilities in their primary language commensurate with normal-hearing children., Conclusion: High levels of achievement including the learning of a second spoken language are possible after implantation in the pediatric population. Variables include speech perception postimplantation, the linguistic environment, type of intervention, and educational placement.

Objective: To determine the efficacy of cochlear implants with current processing strategies in children and adults with long-term congenital deafness, and to determine whether the growth of auditory perceptual skills in these patients is similar to the data reported for patients who have undergone implantation with earlier devices and coding strategies., Study Design: A prospective study of children and adults fulfilling the study inclusion criteria., Setting: University medical center., Patients: Thirty-five congenitally deaf children who received implants after the age of 8 years and 14 congenitally deaf adults who received implants as adults participated in this study. Length of device use ranged from 6 months to 3 years., Main Outcome Measures: Open set phoneme, word recognition tests, and sentence recognition tests were administered in quiet and noise auditory only conditions preoperatively and postoperatively. Changes in test scores were examined by paired t tests, and differences between groups were compared by Student's t tests. Spearman correlation coefficients were calculated to identify associations between scores and subject characteristics. A two-sided alpha of less than 0.05 was considered statistically significant., Results: The results indicated significant improvement in open set speech perception skills in the children after implantation; the adults demonstrated improved mean scores on both word and sentence recognition. A shorter length of deafness correlated with better postoperative performance, but all subjects continued to improve over time. The improvement in test scores was similar between the devices, and no significant differences were detected between the different processing strategies., Conclusion: Children and adults with long-term congenital deafness can obtain considerable open set speech understanding after implantation. Length of deafness (age at implantation), length of device use, and mode of communication contribute to outcome.