Posture, Gait, Quality of Life, and Hearing with a Vestibular Implant.

BACKGROUND: Bilateral vestibular hypofunction is associated with chronic disequilibrium, postural instability, and unsteady gait owing to failure of vestibular reflexes that stabilize the eyes, head, and body. A vestibular implant may be effective in alleviating symptoms. METHODS: Persons who had had ototoxic (7 participants) or idiopathic (1 participant) bilateral vestibular hypofunction for 2 to 23 years underwent unilateral implantation of a prosthesis that electrically stimulates the three semicircular canal branches of the vestibular nerve. Clinical outcomes included the score on the Bruininks-Oseretsky Test of Motor Proficiency balance subtest (range, 0 to 36, with higher scores indicating better balance), time to failure on the modified Romberg test (range, 0 to 30 seconds), score on the Dynamic Gait Index (range, 0 to 24, with higher scores indicating better gait performance), time needed to complete the Timed Up and Go test, gait speed, pure-tone auditory detection thresholds, speech discrimination scores, and quality of life. We compared participants' results at baseline (before implantation) with those at 6 months (8 participants) and at 1 year (6 participants) with the device set in its usual treatment mode (varying stimulus pulse rate and amplitude to represent rotational head motion) and in a placebo mode (holding pulse rate and amplitude constant). RESULTS: The median scores at baseline and at 6 months on the Bruininks-Oseretsky test were 17.5 and 21.0, respectively (median within-participant difference, 5.5 points; 95% confidence interval [CI], 0 to 10.0); the median times on the modified Romberg test were 3.6 seconds and 8.3 seconds (difference, 5.1; 95% CI, 1.5 to 27.6); the median scores on the Dynamic Gait Index were 12.5 and 22.5 (difference, 10.5 points; 95% CI, 1.5 to 12.0); the median times on the Timed Up and Go test were 11.0 seconds and 8.7 seconds (difference, 2.3; 95% CI, -1.7 to 5.0); and the median speeds on the gait-speed test were 1.03 m per second and 1.10 m per second (difference, 0.13; 95% CI, -0.25 to 0.30). Placebo-mode testing confirmed that improvements were due to treatment-mode stimulation. Among the 6 participants who were also assessed at 1 year, the median within-participant changes from baseline to 1 year were generally consistent with results at 6 months. Implantation caused ipsilateral hearing loss, with the air-conducted pure-tone average detection threshold at 6 months increasing by 3 to 16 dB in 5 participants and by 74 to 104 dB in 3 participants. Changes in participant-reported disability and quality of life paralleled changes in posture and gait. CONCLUSIONS: Six months and 1 year after unilateral implantation of a vestibular prosthesis for bilateral vestibular hypofunction, measures of posture, gait, and quality of life were generally in the direction of improvement from baseline, but hearing was reduced in the ear with the implant in all but 1 participant. (Funded by the National Institutes of Health and others; ClinicalTrials.gov number, NCT02725463.).

Mitigation of informational masking in individuals with single-sided deafness by integrated bone conduction hearing aids.

OBJECTIVES: To confirm an increased susceptibility to informational masking among individuals with single-sided deafness (SSD). To demonstrate a reduction in informational masking when SSD is treated with an integrated bone conduction hearing aid (IBC). To identify the acoustic cues that contribute to IBC-aided masking release. To determine the effects of device experience on the IBC advantage. DESIGN: Informational masking was evaluated with the coordinate-response measure. Participants performed the task by reporting color and number coordinates that changed randomly within target sentences. The target sentences were presented in free field accompanied by zero to three distracting sentences. Target and distracting sentences were spoken by different talkers and originated from different source locations, creating two sources of information for auditory streaming. Susceptibility to informational masking was inferred from the error rates of unaided SSD patients relative to normal controls. These baseline measures were derived by testing inexperienced IBC users without the device on the day of their initial fitting. The benefits of IBC-aided listening were assessed by measuring the aided performance of users who had at least 3 months' device experience. The acoustic basis of the listening advantage was isolated by correlating response errors with the voice pitch and location of distracting sentences. The effects of learning on cue effectiveness were evaluated by comparing the error rates of experienced and inexperienced users. RESULTS: Unaided SSD participants (inexperienced users) performed as well as normal controls when tested without distracting sentences but produced significantly higher error rates when tested with distracting sentences. Most errors involved responding with coordinates that were contained in distracting sentences. This increased susceptibility to informational masking was significantly reduced when experienced IBC users were tested with the device. The listening advantage was most strongly correlated with the availability of voice pitch cues, although performance was also influenced by the location of distracting sentences. Directional asymmetries appear to be dictated by location-dependent cues that are derived from the distinctive transmission characteristics of IBC stimulation. Experienced users made better use of these cues than inexperienced users. CONCLUSIONS: These results suggest that informational masking is a significant source of communication impairment among individuals with SSD. Despite the lateralization of auditory function, unaided SSD subjects experience informational masking when distractors occur in either the deaf or normal spatial hemifield. Restoration of aural sensitivity in the deaf hemifield with an IBC enhances speech intelligibility under complex listening conditions, presumably by providing additional sound-segregation cues that are derived from voice pitch and spatial location. The optimal use of these cues is not immediate, but a significant listening advantage is observed after 3 months of unstructured use.

Cochlear Implant Electrode Array Design and Speech Understanding.

OBJECTIVE: Cochlear implant electrode arrays are categorized based on their design as lateral wall (LW) and perimodiolar (PM) electrode arrays. The objective of this study was to investigate the effect of LW versus PM designs on postoperative speech perception across multiple manufacturers and over long follow-up durations. DESIGN: Retrospective cohort study. SETTING: Single academic medical center. PARTICIPANTS: A total of 478 adult cochlear implant recipients, implanted between the years 1992 and 2017. INTERVENTIONSS: PM versus LW cochlear implants. MAIN OUTCOMES AND MEASURES: Postoperative Consonant-Nucleus-Consonant Word (CNC-w) and Hearing in Noise Test (HINT) scores between 6 months and 5 years. RESULTS: Across 478 patients, approximately one-third received LW (n = 176, 36.8%), whereas 302 patients received a PM array (63.2%). The PM group had higher CNC-w scores from 6 months to 2 years (52 [interquartile range, 38-68] versus 48 [31-62], p = 0.036) and from 2 to 5 years (58 [43-72] versus 48 [33-66], p < 0.001). Multivariable analysis of patient-averaged scores indicated that the PM group had greater improvement from preoperative scores at all time points after the initial 6 months for both CNC-w ( ß = 4.4 [95% confidence interval, 0.6-8.3], p = 0.023) and HINT testing ( ß = 4.5 [95% confidence interval, 0.3-8.7], p = 0.038). CONCLUSIONS: This study indicates that PM electrode arrays are associated with small increases in postoperative speech perception scores, relative to LW arrays, when assessed across manufacturers, over long time durations, and using multiple outcome instruments. These findings may help guide surgeon selection and patient counseling of cochlear implant arrays.

Cochlear Implant Revisions Over Three Decades of Experience.

IMPORTANCE: The indications, technology, and surgical technique for cochlear implantation have evolved over the last three decades. Understanding the risk of cochlear implant revision (CIR) is important for patient counseling. OBJECTIVE: The objective of this study was to analyze the rates, indications, and audiologic outcomes for CIR over three decades of experience at a single academic medical center. DESIGN: A retrospective chart review was performed at a single academic medical center for individuals who underwent cochlear implantation between 1985 and 2022. SETTING: Single academic medical center. PARTICIPANTS: Three thousand twenty-five individuals who underwent 3,934 cochlear implant operations from 1985 to 2022. EXPOSURE: Cochlear implantation. MAIN OUTCOMES AND MEASURES: Rates, indications, risk factors, and audiologic outcomes for CIR. RESULTS: There were 276 cases of CIR after primary implantation and an overall revision rate of 7.6% (95% confidence interval, 6.8-8.5%) over 37 years of follow-up with many cases of CIR secondary to Advanced Bionics vendor B and field action failure groups. CIR rates increased sharply through the early and mid-2000s and have since remained stable. Hard or soft device failure was the most common indication for CIR, accounting for 73% of cases. Pediatric patient status and previous CIR were associated with an increased risk of CIR. Audiologic outcomes after CIR were similar to those before device failure. CONCLUSIONS AND RELEVANCE: CIR remains a common procedure most often performed for device failure. Pediatric patients and those who have undergone previous CIR are at the highest risk for future CIR. Audiologic outcomes remain stable after CIR, and these data will help providers counsel patients at the risk of future CIR and understand the risk factors associated with CIR.

Trends in Timing and Provision of Pediatric Cochlear Implant Care During COVID-19.

Objectives: To identify trends in timing of pediatric cochlear implant (CI) care during COVID-19. Study Design: Retrospective cohort. Setting: Tertiary care center. Methods: Patients under 18 years of age who underwent CI between 1/1/2016 and 2/29/2020 were included in the pre-COVID-19 group, and patients implanted between 3/1/2020 and 12/31/2021 comprised the COVID-19 group. Revision and sequential surgeries were excluded. Time intervals between care milestones including severe-to-profound hearing loss diagnosis, initial CI candidacy evaluation, and surgery were compared among groups, as were the number and type of postoperative visits. Results: A total of 98 patients met criteria; 70 were implanted pre-COVID-19 and 28 during COVID-19. A significant increase in the interval between CI candidacy evaluation and surgery was seen among patients with prelingual deafness during COVID-19 compared with pre-COVID-19 (µ = 47.3 weeks, 95% confidence interval [CI]: 34.8-59.9 vs µ = 20.5 weeks, 95% CI: 13.1-27.9; p < .001). Patients in the COVID-19 group attended fewer in-person rehabilitation visits in the 12 months after surgery (µ = 14.9 visits, 95% CI: 9.7-20.1 vs µ = 20.9, 95% CI: 18.1-23.7; p = .04). Average age at implantation in the COVID-19 group was 5.7 years (95% CI: 4.0-7.5) versus 3.7 years in the pre-COVID-19 group (95% CI: 2.9-4.6; p = .05). The time interval between hearing loss confirmation and CI surgery was on average 99.7 weeks for patients implanted during COVID-19 (95% CI: 48.8-150) versus 54.2 weeks for patients implanted pre-COVID (95% CI: 39.6-68.8), which was not a statistically significant difference (p = .1). Conclusion: During the COVID-19 pandemic patients with prelingual deafness experienced delays in care relative to patients implanted before the pandemic.

Outcomes after revision of Advanced Bionics Clarion 1.2 cochlear implants.

INTRODUCTION: Cochlear implant reimplantation (CIR) for external processor upgrade or device failure is becoming increasingly common as the population of cochlear implant recipients ages. Patients with Advanced Bionics (AB) Clarion 1.2 cochlear implants may undergo CIR for device age/failure or desired technology upgrade so that they may use newer external processors that have improved connectivity features. The objective of this study was to evaluate audiologic outcomes for patients who were initially implanted with an AB Clarion 1.2 internal device and underwent CIR for technology upgrade or device failure. METHODS: Retrospective chart review was performed at a single academic medical center for patients (pediatric and adult) with an AB Clarion 1.2 internal device who underwent CIR to a later generation AB internal device and had available audiologic data. RESULTS: Forty-eight individuals with a Clarion 1.2 implant underwent CIR. Pre- and post-CIR speech understanding did not change for AzBio (p-value = 0.11, mean change = 12.1%, 95% CI = -2.9-27.2%), CNCw (p-value = 0.74, mean change = -1%, 95% CI = -10.4-12.4%), or HINT (p-value = 0.12, mean change = 19.9%, 95% CI = -2.6-42.4%) scores. Pure-tone averages improved following CIR (p-value < 0.01, mean change = 4.3 dB, 95% CI = 1.5-7.1 dB). CONCLUSIONS: Revision of AB Clarion 1.2 cochlear implants does not significantly worsen audiologic outcomes and may improve hearing in some individuals, but individual patient-level outcomes are variable.

Candidacy for Cochlear implantation: Validating a novel Cochlear implant candidacy calculator against gold-standard, in-clinic audiometric assessments.

Objectives: Cochlear implants (CI) are reliable implantable devices that are highly cost-effective in reducing the burden of hearing loss at an individual and societal scale. However, only 10% of CI candidates are aware of their candidacy and receive a CI. A web-based screening tool to assess CI candidacy may make many more individuals aware of their candidacy for cochlear implantation. The objective of this study was to validate and optimize the online Cochlear Implant Candidacy Calculator against in-clinic audiometric testing. Methods: Audiogram data and word discrimination scores for 132 patients who underwent initial CI consultation at the Johns Hopkins Cochlear Implant Center in 2020 were inputted into the calculator. Candidacy results from the calculator were compared against formal clinical diagnoses provided by the audiologist at the time of visit. Receiver Operating Characteristic (ROC) and Area Under the Curve (AUC) analyses were performed to identify optimal diagnostic thresholds. Results: Of the resulting 132 patients, 54 presented with single-sided deafness (SSD), and 114 were clinically determined to be CI candidates. ROC AUC analyses identified optimal thresholds of high-frequency PTA ≥65 dB and word discrimination score ≤ 50%. To maximize sensitivity at the expense of specificity, diagnostic thresholds of high-frequency PTA ≥ 65 dB and word discrimination score ≤ 70% were chosen, which yielded accuracy, sensitivity, specificity, and ROC AUC of 0.90, 0.94, 0.82, and 0.88, respectively. Conclusion: The novel online CI Candidacy Calculator exhibits high sensitivity and accuracy, and moderate specificity. The calculator may thereby be useful in increasing awareness of potential CI candidacy, increasing prevalence of CIs, and decreasing the burden of hearing loss.

Are Speech Perception Scores in Cochlear Implant Recipients Consistent Across Different Tests?

OBJECTIVE: Cochlear implant (CI) candidacy and postoperative outcomes are assessed using sets of speech perception tests that vary from center to center, limiting comparisons across institutions and time periods. The objective of this study was to determine if scores on one speech perception test could be reliably predicted from scores on another test. STUDY DESIGN: Arizona Biomedical (AzBio) Sentence Test, Consonant-Nucleus-Consonant word (CNCw), and Hearing in Noise Test (HINT) scores in quiet for the implanted ear were collected for individuals who received a CI between 1985 and 2019. Scores collected during the same testing session were analyzed using Bland-Altman plots to assess agreement between testing methods. Simple linear regression with logit transformation was used to generate predictive functions and 95% confidence intervals for expected mean and individual scores. SETTING: Single academic medical center. PATIENTS: A total of 1,437 individuals with a median age of 59.9 years (range, 18-95 yr) and 46% (654 of 1,437) male. INTERVENTIONS: N.A. MAIN OUTCOME MEASURES: Agreement as a function of test score, mean, variance, and correlation coefficients. RESULTS: A total of 2,052 AzBio/CNCw, 525 AzBio/HINT, and 7,187 CNCw/HINT same-session score pairings were identified. Pairwise test comparisons demonstrated limited agreement between different tests performed in the same session, and a score correlation between different speech tests revealed large variances. CONCLUSION: Transformation functions between test batteries were predictive of mean scores but performed poorly for prediction of individual scores. Point-wise comparisons of scores across CI test batteries should be used with caution in clinical and research settings.

Catchment Profile of Large Cochlear Implant Centers in the United States.

OBJECTIVE: To characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States. STUDY DESIGN: Multi-institutional retrospective case series. SETTING: Tertiary referral CI centers. METHODS: Patients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients' residential zip codes were used to approximate travel distances and urban vs rural residential areas. RESULTS: Over the 6-year study period (2015-2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21-110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P < .001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P < .001). Overall, 91% of patients lived within a 200-mile radius of the institution, while 71% lived within a 100-mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P < .001, r2 = 0.2). CONCLUSIONS: While large CI centers serve geographically dispersed populations, most patients reside within a 200-mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

Continuous vestibular implant stimulation partially restores eye-stabilizing reflexes.

BACKGROUNDBilateral loss of vestibular (inner ear inertial) sensation causes chronically blurred vision during head movement, postural instability, and increased fall risk. Individuals who fail to compensate despite rehabilitation therapy have no adequate treatment options. Analogous to hearing restoration via cochlear implants, prosthetic electrical stimulation of vestibular nerve branches to encode head motion has garnered interest as a potential treatment, but prior studies in humans have not included continuous long-term stimulation or 3D binocular vestibulo-ocular reflex (VOR) oculography, without which one cannot determine whether an implant selectively stimulates the implanted ear's 3 semicircular canals.METHODSWe report binocular 3D VOR responses of 4 human subjects with ototoxic bilateral vestibular loss unilaterally implanted with a Labyrinth Devices Multichannel Vestibular Implant System vestibular implant, which provides continuous, long-term, motion-modulated prosthetic stimulation via electrodes in 3 semicircular canals.RESULTSInitiation of prosthetic stimulation evoked nystagmus that decayed within 30 minutes. Stimulation targeting 1 canal produced 3D VOR responses approximately aligned with that canal's anatomic axis. Targeting multiple canals yielded responses aligned with a vector sum of individual responses. Over 350-812 days of continuous 24 h/d use, modulated electrical stimulation produced stable VOR responses that grew with stimulus intensity and aligned approximately with any specified 3D head rotation axis.CONCLUSIONThese results demonstrate that a vestibular implant can selectively, continuously, and chronically provide artificial sensory input to all 3 implanted semicircular canals in individuals disabled by bilateral vestibular loss, driving reflexive VOR eye movements that approximately align in 3D with the head motion axis encoded by the implant.TRIAL REGISTRATIONClinicalTrials.gov: NCT02725463.FUNDINGNIH/National Institute on Deafness and Other Communication Disorders: R01DC013536 and 2T32DC000023; Labyrinth Devices, LLC; and Med-El GmbH.

A Cohort Study of Hearing Outcomes Between Middle Fossa Craniotomy and Transmastoid Approach for Surgical Repair of Superior Semicircular Canal Dehiscence Syndrome.

OBJECTIVE: To compare postoperative hearing outcomes between transmastoid and middle fossa craniotomy (MFC) approaches for surgical repair of superior semicircular canal dehiscence syndrome (SCDS) in a tertiary referral center. STUDY DESIGN: Historical cohort study. SETTING: Tertiary referral center. PATIENTS: Twelve consecutive SCDS cases who underwent transmastoid plugging of the superior canal; "controls" were 18 audiogram-matched patients who underwent MFC plugging and resurfacing. MAIN OUTCOME MEASURES: Differences between preoperative, 7-day postoperative, and long-term (>6 wk) postoperative air and bone conduction, speech discrimination scores (SDS), and pure-tone averages (PTA) in TM cases versus MFC controls. METHODS: MFC controls were selected by matching preoperative bone conduction (BC) pure-tone thresholds from the TM cases within 10-dBs NHL in ≥80% of recorded frequencies. Wilcoxon signed-rank tests were performed to compare primary outcomes between matches, with a Bonferroni corrected p value of 0.004 (n = 13 variables measured at each time period). RESULTS: No statistically significant differences were found in long-term postoperative air conduction and BC thresholds at any frequency both during the immediate postoperative period as well as at long-term follow-up (p > 0.004). Similarly, there were no differences in long-term SDS or PTA (p > 0.004). CONCLUSIONS: In this pilot study, there were no long-term significant differences in hearing outcomes between the two repair techniques for SCDS. We recommend continuing with the established practice for recommending surgical repair based on individual patient characteristics and preferences in managing both vestibular and auditory function.

Safe Intraoperative Neurophysiologic Monitoring During Posterior Spinal Fusion in a Patient With Cochlear Implants.

OBJECTIVE: Cochlear implants are generally considered a contraindication for any procedure requiring electrical stimulation near the implant. We present a case of a patient undergoing intraoperative transcranial electrical motor-evoked potential monitoring with a cochlear implant without adverse outcomes. PATIENT: A 12-year-old girl with a history of VACTERL presented with worsening congenital kyphosis and bilateral severe-to-profound hearing loss. Since age 7 the patient used a cochlear implant in the right ear and hearing aid in the left ear. Physical examination and magnetic resonance imaging in 2016 revealed a left-sided 66-degree thoracolumbar kyphosis at T11 making the patient a candidate for surgical correction. INTERVENTIONS: She underwent a posterior spinal fusion surgery, performed with intraoperative transcranial electrical motor-evoked potential monitoring. Steps were taken to mitigate electrical stimulation of the patient's cochlear implant. MAIN OUTCOME MEASURES: Postoperative impedance of individual channels, audiometry, and neural response testing were compared with preoperative measurements. RESULTS: Significant (>10%) impedance changes were observed postoperatively in channels 1, 2, 4, and 6; however, the net variation across all the channels was low (3%). The patient reported no hearing changes, and no significant changes in hearing threshold were seen in postoperative audiometric testing or neural response testing. CONCLUSION: We present a case of successful posterior spinal fusion with intraoperative neurophysiological monitoring via transcranial electrical stimulation, in a patient with a cochlear implant. With proper precautions, motor-evoked potential monitoring can be safely performed in a patient with a cochlear implant.

Safety of radiofrequency ablation for adenotonsillectomy after cochlear implantation.

OBJECTIVE: While a cadaveric animal study has suggested that radiofrequency ablation can be safely used in patients with cochlear implants, no in vivo studies have been published to confirm that radiofrequency ablation does not alter the integrity of the cochlear implant device. METHODS: Cochlear implant impedance and functional performance were studied through a prospective case series in five children with seven functioning multichannel implants before and after radiofrequency ablation adenotonsillectomy. RESULTS: There were 4 females and 1 male patient, aged 6-10 years (mean 8.5 ±â€¯1.95 years) with 7 functioning implants. Pre- and post-surgical impedance testing revealed all electrodes were within normal operating limits. There was no statistically significant difference between the mean pre and post-operative impedances in 5 of the 7 tested implants (P = 0.2-0.8). The other two implants showed statistically significant improvement in impedance values which were not clinically significant (P = 0.02 and P < 0.001). Speech perception was unchanged as was functional performance for all 7 tested implants. CONCLUSIONS: We found that radiofrequency ablation used in the oropharynx during adenotonsillectomy did not alter the integrity of the cochlear implant devices when assessed using electrode impedance testing, audiometry and speech perception evaluation. These results confirm those reported in previous in vitro studies and confirm the safety of radiofrequency ablation adenotonsillectomy for children who have undergone previous cochlear implant placement.

Amplification in the rehabilitation of unilateral deafness: speech in noise and directional hearing effects with bone-anchored hearing and contralateral routing of signal amplification.

OBJECTIVE: Vibromechanical stimulation with a semi-implantable bone conductor (Entific BAHA device) overcomes some of the head-shadow effects in unilateral deafness. What specific rehabilitative benefits are observed when the functional ear exhibits normal hearing versus moderate sensorineural hearing loss (SNHL)? DESIGN: The authors conducted a prospective trial of subjects with unilateral deafness in a tertiary care center. PATIENTS: This study comprised adults with unilateral deafness (pure-tone average [PTA] > 90 dB; Sp.D. < 20%) and either normal monaural hearing (n = 18) or moderate SNHL (PTA = 25-50 dB: Sp.D. > 75%) in the contralateral functional ear (n = 5). INTERVENTIONS: Subjects were fit with contralateral routing of signal (CROS) devices for 1 month and tested before (mastoid) implantation, fitting, and testing with a bone-anchored hearing aid (BAHA). OUTCOME MEASURES: Outcome measures were: 1) subjective benefit; 2) source localization tests (Source Azimuth Identification in Noise Test [SAINT]); 3) speech discrimination in quiet and in noise assessed with Hearing In Noise Test (HINT) protocols. RESULTS: There was consistent satisfaction with BAHA amplification and poor acceptance of CROS amplification. General directional hearing decreased with CROS use and was unchanged by BAHA and directional microphone aids. Relative to baseline and CROS, BAHA produced significantly better speech recognition in noise. Twenty-two of 23 subjects followed up in this study continue to use their BAHA device over an average follow-up period of 30.24 months (range, 51-12 months). CONCLUSION: BAHA amplification on the side of a deaf ear yields greater benefit in subjects with monaural hearing than does CROS amplification. Advantages likely related to averting the interference of speech signals delivered to the better ear, as occurs with conventional CROS amplification, while alleviating the negative head-shadow effects of unilateral deafness. The advantages of head-shadow reduction in enhancing speech recognition with noise in the hearing ear outweigh disadvantages inherent in head-shadow reduction that can occur by introducing noise from the deaf side. The level of hearing impairment correlates with incremental benefit provided by the BAHA. Patients with a moderate SNHL in the functioning ear perceived greater increments in benefit, especially in background noise, and demonstrated greater improvements in speech understanding with BAHA amplification.

Predictive models for cochlear implantation in elderly candidates.

OBJECTIVE: An aging American population carries a high prevalence of profound sensorineural hearing loss. We examined the performance of multichannel cochlear implant recipients in a large database of adult subjects. DESIGN: Nonconcurrent prospective study of a national cohort with multivariate regression analysis of preoperative and postoperative performance variables in multichannel cochlear implant recipients. We applied models of prediction established in previous studies to the observed results. SETTING: Referral centers with active cochlear implant programs. PATIENTS: Adolescents and adults with profound hearing loss (N = 749; age range, 14-91 years). MAIN OUTCOME MEASURE: Postoperative monosyllabic word recognition. RESULTS: The population 65 years and older demonstrated a clinically insignificant 4.6%-smaller postoperative word score compared with the population younger than 65 years. When duration of deafness exceeded 25 years, elderly recipients demonstrated higher word scores than their younger counterparts. A more significant factor affecting outcomes is the ratio of duration of deafness to age at implantation. CONCLUSIONS: Age at implantation carried relatively little predictive value for postoperative performance in subjects 65 years and older. Although a small decrement in mean speech recognition scores was evident, the clinical significance of this difference is questionable when all of the results observed in elderly patients are considered. A shorter percentage of life spent in severe-to-profound sensorineural hearing loss suggests a foundation of acoustic/auditory processing in the elderly cohort that may mitigate potential physiological effects associated with advanced age. This study confirms and extends previous observations that duration of profound deafness and residual speech recognition carry higher predictive value than the age at which an individual receives an implant.

Serial audiometry in a clinical trial of AIED treatment.

OBJECTIVE: We analyzed pure-tone and speech audiometric results from a prospective trial of anti-inflammatory treatment of subjects with active autoimmune inner ear disease (AIED). We sought to characterize the pattern and size of the treatment effect as reflected in clinical audiometry and to identify audiometric predictors of response to steroid treatment of AIED. SUBJECTS: Adult participants demonstrated clinically established criteria for AIED (n = 116). Eligibility required audiometric evidence of active AIED as indicated by idiopathic sensorineural hearing loss with threshold elevations within 3 months of enrollment. METHODS: We evaluated audiometric changes after 4 weeks of treatment with pharmacologic doses (60 mg/day) of prednisone. We examined the relationship between audiometric pure-tone thresholds at baseline and changes in word intelligibility score (WIS) using parametric and nonparametric analyses. Magnitudes of change were assessed using independent or paired t-tests. Separate analyses were performed on subgroups that did or did not show improved WIS score with steroid treatment. RESULTS: Overall mean pure-tone averages improved from baseline to closeout of prednisone treatment in better hearing ears from 52.4 to 48.3 dB (p < .0001). Mean WIS improved in the better ear from 71.4% to 78.1% (p < .0001). Of pure-tone measures, only the six-tone average showed significant correlation with both the absolute improvements in WIS and with the percentage change in WIS after treatment. Individual frequencies at baseline showed no significant relationship with changes in WIS score after treatment. In 69 (59.5%) of 116 subjects, WIS improved (range, 2-80%) in the better ear. In these subjects, the baseline pure-tone thresholds and pure-tone averages correlated significantly and positively with improvement in WIS. CONCLUSIONS: Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

The Safety and Efficacy of PF-04958242 in Age-Related Sensorineural Hearing Loss: A Randomized Clinical Trial.

IMPORTANCE: To our knowledge, this is the first study to assess the potential to pharmacologically improve auditory function in adults with age-related sensorineural hearing loss. OBJECTIVE: To explore the potential for the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid potentiator mechanism to affect auditory function in individuals with mild to moderate age-related sensorineural hearing loss. DESIGN, SETTING, AND PARTICIPANTS: A randomized, double-blind, placebo-controlled, single-dose, 3-way crossover study was conducted in 3 academic ear, nose, and throat clinics and 2 private clinical research centers between December 22, 2011, and February 26, 2013. Participants were 50- to 75-year-old men and women of nonchildbearing potential with mild to moderate sensorineural hearing loss. INTERVENTIONS: Three single doses of PF-04958242, an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid glutamate-positive allosteric modulator, and placebo. MAIN OUTCOMES AND MEASURES: Pure-tone average, speech discrimination score, and speech in noise testing change from baseline at 1 and 5 hours after a single dose of PF-04958242. RESULTS: The treatment was safe and well tolerated. The estimates for the primary end point change from baseline in pure-tone average compared with placebo at 1 hour were -0.77 (95% CI, -2.14 to 0.59) and 0.37 (95% CI, -0.97 to 1.72) for 0.27 and 0.35 mg, respectively. At 5 hours the estimates were -0.57 (95% CI, -2.43 to 1.29) and -0.56 (95% CI, -2.45 to 1.33) for 0.27 and 0.35 mg, respectively. No significant change from baseline was demonstrated compared with placebo in the primary or secondary study end points at 1 or 5 hours after receiving treatment. CONCLUSIONS AND RELEVANCE: To our knowledge, this clinical trial is the first study of a pharmacologic treatment for age-related sensorineural hearing loss and provides information with regard to study design, end points, variability, data characteristics, and operational feasibility to guide the design of future hearing loss trials. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01518920.

Cochlear implant outcome is not influenced by the choice of ear.

OBJECTIVES: This study tested the hypothesis that patients with residual hearing in the nonimplanted ear had the same cochlear implant benefit whether the implanted ear had profound or severe hearing loss. DESIGN: A retrospective chart review of adult cochlear implant recipients with postlingual hearing loss. Patients were categorized according to the pure-tone average of the implanted and contralateral ears as (a) bilateral profound, (b) severe-profound, and (c) bilateral severe. The results of a test battery of spoken language measures were compared among patients belonging to these hearing categories at 6, 12, and 24 months after surgery, using a t-test and multivariate regression analyses. RESULTS: The presence of residual hearing in one or both ears was associated with significantly higher postoperative speech perception scores compared with participants with bilateral profound hearing loss. Among participants with similar amounts of residual hearing in the nonimplanted ear, however, there was no difference in speech recognition scores between those with profound and those with severe hearing loss in the implanted ear. CONCLUSIONS: Among participants with asymmetric hearing loss, there is no additional benefit to implanting the better-hearing ear that can be preserved for use with a hearing aid for better speech understanding in noise and sound localization. These results suggest that the additional benefit received by patients with residual hearing is mediated by trophic effects on crossed pathways in the central nervous system and is independent of the preoperative functional status of the implanted ear.