Faculty Bibliography

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

BACKGROUND:Measurement of the angular depth of insertion (aDOI) of cochlear implant electrode arrays has numerous clinical and research applications. Plain-film radiographs are easily obtained intraoperatively and have been described as a means to calculate aDOI. CT imaging with 3D reformatting can also be used for this measurement, but is less conveniently obtained and requires higher radiation doses, a particular concern in pediatrics. The extent to which plain-film and 3D CT image-based measurements are representative of the true position of the electrode within the cochlea is unknown. METHODS:Cochlear implantation was performed on 10 cadaveric temporal bones. Five bones were implanted with perimodiolar electrodes (Contour Advance TM, Cochlear, Sydney, Australia) and five were implanted with lateral wall electrodes (Slim Straight, Cochlear). The insertion depths of the electrodes were varied. Each bone was imaged with a radiograph and CT. aDOI was measured for each bone in each imaging modality by a neurotologist and a neuroradiologist. To obtain a 'gold standard' estimate of aDOI, the implanted temporal bones were embedded in an epoxy resin and methodically sectioned at 100 μm intervals; histologic images were captured at each interval. A 3D stack of the images was compounded, and a MATLAB script used to calculate aDOI of the most apical electrode. Measurements in the three modalities (radiograph, CT, and histology) were then compared. RESULTS:The average aDOI across all bones was similar for all modalities: 423° for radiographs, 425° for CT scans, and 427° for histology, indicating that neither imaging modality resulted in large systematic errors. Using the histology-measured angles as a reference, the average error for CT-based measures (regardless of whether the error was in the positive or negative direction) was 12°, and that for radiograph-based measures was 15°. This small difference (12 vs 15° error) was not statistically significant. CONCLUSION/CONCLUSIONS:Based on this cadaveric temporal bone model, both radiographs and CTs can provide reasonably accurate aDOI measurements. In this small sample, and as expected, the CT-based estimates were more accurate than the radiograph-based measurements. However, the difference was small and not statistically significant. Thus, the use of plain radiographs to calculate aDOI seems judicious whenever it is desired to prevent unnecessary radiation exposure and expense.

Background/UNASSIGNED:Harmony is an important part of tonal music that conveys context, form and emotion. Two notes sounded simultaneously form a harmonic interval. In normal-hearing (NH) listeners, some harmonic intervals (e.g., minor 2nd, tritone, major 7th) typically sound more dissonant than others (e.g., octave, major 3rd, 4th). Because of the limited spectro-temporal resolution afforded by cochlear implants (CIs), music perception is generally poor. However, CI users may still be sensitive to relative dissonance across intervals. In this study, dissonance ratings for harmonic intervals were measured in 11 unilaterally deaf CI patients, in whom ratings from the CI could be compared to those from the normal ear. Methods/UNASSIGNED:Stimuli consisted of pairs of equal amplitude MIDI piano tones. Intervals spanned a range of two octaves relative to two root notes (F3 or C4). Dissonance was assessed in terms of subjective pleasantness ratings for intervals presented to the NH ear alone, the CI ear alone, and both ears together (NH + CI). Ratings were collected for both root notes for within- and across-octave intervals (1-12 and 13-24 semitones). Participants rated the pleasantness of each interval by clicking on a line anchored with "least pleasant" and "most pleasant." A follow-up experiment repeated the task with a smaller stimulus set. Results/UNASSIGNED:< 0.001). Ratings were similar between NH-only and NH + CI listening, with no significant binaural enhancement/interference. The follow-up tests showed that ratings were reliable for the least and most pleasant intervals. Discussion/UNASSIGNED:Although pleasantness ratings were less differentiated for the CI ear than the NH ear, there were similarities between the two listening modes. Given the lack of spectro-temporal detail needed for harmonicity-based distinctions, temporal envelope interactions (within and across channels) associated with a perception of roughness may contribute to dissonance perception for harmonic intervals with CI-only listening.

OBJECTIVE:Given rising scrutiny of healthcare expenditures, understanding intervention costs is increasingly important. This study aimed to compare and characterize costs for vestibular schwannoma (VS) management with microsurgery and radiosurgery to inform practice decisions and appraise cost reduction strategies. METHODS:In conjunction with medical records, internal hospital financial data were used to evaluate costs. Total cost was divided into index costs (costs from arrival through discharge for initial intervention) and follow-up costs (through 36 months) for 317 patients with unilateral VSs undergoing initial management between June 2011 and December 2015. A retrospective matched cohort based on tumor size with 176 patients (88 undergoing each intervention) was created to objectively compare costs between microsurgery and radiosurgery. The full sample of 203 patients treated with resection and 114 patients who underwent radiosurgery was used to evaluate a broad range of outcomes and identify cost contributors within each intervention group. RESULTS:Within the matched cohort, average index costs were significantly higher for microsurgery (100% by definition, because costs are presented as a percentage of the average index cost for the matched microsurgery group; 95% CI 93-107) compared to radiosurgery (38%, 95% CI 38-39). Microsurgery had higher average follow-up costs (1.6% per month, 95% CI 0.8%-2.4%) compared to radiosurgery (0.5% per month, 95% CI 0.4%-0.7%), largely due to costs incurred in the initial months after resection. A major contributor to total cost and cost variability for both resection and radiosurgery was the need for additional interventions in the follow-up period, which were necessary due to complications or persistent functional deficits. Although tumor size was not associated with increased total costs for radiosurgery, linear regression analysis demonstrated that, for patients who underwent microsurgery, each centimeter increase in tumor maximum diameter resulted in an estimated increase in total cost of 50.2% of the average index cost of microsurgery (95% CI 34.6%-65.7%) (p < 0.001, R2 = 0.17). There were no cost differences associated with the proportion of inpatient days in the ICU or with specific surgical approach for patients who underwent resection. CONCLUSIONS:This study is the largest assessment to date based on internal cost data comparing VS management with microsurgery and radiosurgery. Both index and follow-up costs are significantly higher when tumors were managed with resection compared to radiosurgery. Larger tumors were associated with increased resection costs, highlighting the incremental costs associated with observation as the initial management.

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 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 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.

OBJECTIVE A randomized trial that compares clinical outcomes following microsurgery (MS) or stereotactic radiosurgery (SRS) for patients with small- and medium-sized vestibular schwannomas (VSs) is impractical, but would have important implications for clinical decision making. A matched cohort analysis was conducted to evaluate clinical outcomes in patients treated with MS or SRS. METHODS The records of 399 VS patients who were cared for by 2 neurosurgeons and 1 neurotologist between 2001 and 2014 were evaluated. From this data set, 3 retrospective matched cohorts were created to compare hearing preservation (21 matched pairs), facial nerve preservation (83 matched pairs), intervention-free survival, and complication rates (85 matched pairs) between cases managed with SRS and patients managed with MS. Cases were matched for age at surgery (+/- 10 years) and lesion size (+/- 0.1 cm). To compare hearing outcomes, cases were additionally matched for preoperative Class A hearing according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines. To compare facial nerve (i.e., cranial nerve [CN] VII) outcomes, cases were additionally matched for preoperative House-Brackmann (HB) score. Investigators who were not involved with patient care reviewed the clinical and imaging records. The reported outcomes were as assessed at the time of the last follow-up, unless otherwise stated. RESULTS The preservation of preoperative Class A hearing status was achieved in 14.3% of MS cases compared with 42.9% of SRS cases (OR 4.5; p < 0.05) after an average follow-up interval of 43.7 months and 30.3 months, respectively. Serviceable hearing was preserved in 42.8% of MS cases compared with 85.7% of SRS cases (OR 8.0; p < 0.01). The rates of postoperative CN VII dysfunction were low for both groups, although significantly higher in the MS group (HB III-IV 11% vs 0% for SRS; OR 21.3; p < 0.01) at a median follow-up interval of 35.7 and 19.0 months for MS and SRS, respectively. There was no difference in the need for subsequent intervention (2 MS patients and 2 SRS patients). CONCLUSIONS At this high-volume center, VS resection or radiosurgery for tumors