Faculty Bibliography

Objective: To assess the complication and migration rates associated with fixation of cochlear implant receiver stimulators using a subperiosteal tight pocket without either suture fixation or bone recession. Study Design: Dual institution retrospective case review Methods: A retrospective case review was conducted at two tertiary referral centers. All patients who underwent cochlear implantation with device fixation using a subperiosteal tight pocket without suturing over the device or recessing of the receiver stimulator in bone were identified. There was a minimum follow-up period of six months. Outcome measures included intraoperative and postoperative complications, including evidence of device migration associated with interference with external device use or need for revision surgery. Other outcome measures included soft tissue flap complications. Results: Sixty-two patients were identified with a mean age of 39 years, (range 1.5-95 years). The average follow-up period was 32.6 months (range 6-120 months). Device manufacturers included Cochlear Corporation (n=44), MED-EL (n=12) and Advanced Bionics (n=6). There were no associated intraoperative complications related to subperiosteal pocket fixation of the receiver stimulator, and no cases of migration were identified. Conclusion: Fixation of the cochlear implant receiver stimulator using a subperiosteal tight pocket without either suture fixation or bone recession has been demonstrated to be feasible across a range of patient demographics and cochlear implant devices. This method of fixation appears to allow for an efficient and minimally invasive approach without compromising patient safety or device performance.

OBJECTIVE:To examine patient characteristics that impact serial observation adherence among vestibular schwannoma (VS) patients. STUDY DESIGN/METHODS:Retrospective chart review. SETTING/METHODS:Single tertiary care center. METHODS:We selected for VS patients from 201 to 2020 who elected for serial observation as initial management. Patients under 18, with previous management, bilateral or intralabyrinthine VS, and neurofibromatosis type 2 were excluded. Demographics, tumor characteristics, and follow-up status were extracted. Single and multiple logistic regression was used to identify patient characteristics impacting follow-up. RESULTS:We identified 507 VS patients who chose serial observation as initial management. Most were female (56.0%), white (73.0%), and married (72.8%). The mean age was 59.3 and most had private insurance (56.4%). Median Charlson Comorbidity Index was 2.00. Mean pure tone audiometry (PTA) average was 41.7 Hz. Average tumor size was 9.04 mm. Of 507 patients, 358 (70.6%) returned for at least one follow-up. On multiple logistic regression analysis, patients with private insurance (odds ratio [OR]: 0.39, confidence interval [CI]: 0.22-0.68; P = .001), racial minority background (OR: 0.54, CI: 0.35-0.83; P = .005), worse PTA averages (OR: 0.99, CI: 0.98-1.00; P = .044), and older age at diagnosis (OR: 0.97, CI: 0.95-1.00; P = .038) were less likely to follow-up. CONCLUSION/CONCLUSIONS:Private health insurance, racial minority background, worse PTA average, and older age were associated with decreased follow-up among adult VS patients electing serial observation. Patients with these characteristics may require additional support to ensure serial observation adherence.

OBJECTIVE:To assess the safety of cochlear implants (CIs) in children under 9 months of age to better understand expected postoperative complication rates, and to provide a preliminary look at efficacy. STUDY DESIGN/METHODS:Single-center retrospective review. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Children 5 to 8 months of age who received a CI between 2011 and 2021. INTERVENTIONS/METHODS:Therapeutic-CI. MAIN OUTCOME MEASURES/METHODS:The primary outcome was 6-month postoperative complication rate. Secondary outcomes included length of stay, device explantation/reimplantation, rehabilitation supports, and hearing and language outcomes. Complications of children implanted under than 9 months of age was compared with a cohort of children implanted 9 to 18 months of age between the years 2011 and 2016 using a chi-squared test (p < 0.05). RESULTS:One hundred six children under 9 months of age were implanted (204 CIs) at a mean age of 6.6 months (range: 5-8). Postoperative complications occurred in 28 patients (26%) and were often minor. There were no mortalities. There was no statistically significant difference in complications, including reimplantation rates, between patients implanted under 9 months of age and those implanted 9 to 18 months of age. Speech discrimination outcomes were excellent, and mean spoken language outcomes were near normative for typically developing children. CONCLUSIONS:Cochlear implantation can be a safe and effective treatment option for children 5 to 8 months of age. Early hearing screening and referral for infants to receive appropriate intervention will continue to play a critical role in optimizing speech and language outcomes.

OBJECTIVES:Immune-checkpoint inhibitors (ICI) are being utilized with increasing frequency and may be linked to neurologic and audiovestibular toxicities. This report aimed to describe a case of ICI-induced sensorineural hearing loss ultimately requiring bilateral cochlear implantation. METHODS:A 42-year-old female with stage IV metastatic melanoma of the perianal skin was treated with ipilimumab (blocker of cytotoxic T-lymphocyte associated protein 4 [CTLA-4]) and nivolumab (anti-programmed cell death protein 1 [PD1]). After 21 weeks of therapy, she developed sudden vertigo and bilateral hearing loss. A full workup including MRI and lumbar puncture ruled out intracranial parenchymal metastases, leptomeningeal metastases, stroke and intracranial infection. ICI-associated aseptic meningoencephalitis was therefore diagnosed. The patient received systemic steroids as well as intratympanic steroids, which temporarily improved hearing, but eventually developed permanent, bilateral profound sensorineural hearing loss. RESULTS:The patient received bilateral cochlear implants and has demonstrated good performance one year after implantation. DISCUSSION:ICI are being increasingly used to treat a variety of advanced malignancies. This is the first report of bilateral cochlear implants in the context of profound hearing loss after an immunotherapy induced meningoencephalitis. CONCLUSION:ICI carries the risk of potential ototoxicity, including profound SNHL as depicted in our case. Cochlear implantation proved to be beneficial and may be considered in patients with ICI-related hearing loss.

OBJECTIVES:To determine rates of hearing preservation and performance in patients who met candidacy for electroacoustic stimulation (EAS) and were implanted with a slim modiolar electrode (CI532 or CI632). DESIGN:Adult patients meeting Food and Drug Administration criteria for electroacoustic stimulation (preoperative low-frequency pure-tone average [LFPTA] less than 60 dB at 125, 250, and 500 Hz and monosyllabic word scores between 10% and 60% in the ear to be implanted), who received a slim modiolar electrode were included. Main outcome measures included rates of hearing preservation, defined as a LFPTA ≤80 dB at 125, 250, and 500 Hz, as well as postoperative low-frequency pure-tone threshold shifts, consonant-Nucleus-Consonant (CNC) word scores and AzBio sentences in noise scores. RESULTS:Forty-six patients met inclusion criteria during a 4-year period. Mean (standard deviation) preoperative LFPTA was 34.5 (13.0) dB, and 71.7% had preserved hearing at initial activation. The mean LFPTA shift in patients who preserved hearing at initial activation was 19.7 (14.6) dB, compared with 62.6 (17.7) dB in patients who did not preserve hearing as per our definition. Perioperative steroid use was not different in patients with and without preserved hearing (X 2 (1, N = 46) = 0.19, p = .67, V = 0.06). One year after surgery, 57% of patients had a decline in LFPTA >80 dB and were no longer considered candidates for EAS, with 34.7% still retaining low-frequency thresholds ≤80 dB. CNC word scores at 1 year were 69.9% and 61.4% among individuals with and without preserved low-frequency hearing respectively, measured in their CI ear alone, in their regular listening condition of EAS or electric only ( t (32) = 1.13, p = 0.27, d = 0.39, 95% CI = -6.51, 22.86). Device use time did not differ between groups. Among adults with preserved residual hearing at 1 year (n = 16), 44% used EAS, although there was no significant difference in performance between EAS users and nonusers with preserved hearing. Loss of residual hearing over time did not result in a decline in speech perception performance. CONCLUSION:The present study demonstrated favorable early rates of hearing preservation with a slim modiolar array. Performance was not significantly different in individuals with and without preserved low-frequency acoustic hearing, independent of EAS use. Compared with reports of short electrode use, the loss of residual hearing in patients implanted with this array did not impact speech perception performance.

OBJECTIVE:To assess a series of patients receiving teprotumumab therapy and objectively quantify the rates of otologic adverse events. STUDY DESIGN:A prospective cohort study of adult patients receiving teprotumumab between May 2020 and January 2022. SETTING:Tertiary referral center. METHODS:Prior to treatment initiation, an ototoxicity-specific audiometric battery was completed, which included conventional audiometry (frequencies 250-8000 Hz), ultrahigh-frequency audiometry (9000-20,000 Hz), tympanometry, speech discrimination scores, and distortion product otoacoustic emissions (DPOAEs). Testing was then repeated after treatment completion. RESULTS:In total, 35 patients were recruited, with a median (range) age of 48.5 years (21-74), and 8 (22.8%) were male. The most common subjective symptom reported was a hearing decline (25.7%), followed by aural fullness (17.1%) and tinnitus (14.3%). Fourteen patients had both pre- and posttreatment audiometric data. Among them, 3 patients (21.4%) were found to have changes in standard frequency audiometry, and 10 (71.4%) had changes in high-frequency audiometry, with 2 patients having changes in both. Less than half (n = 5) of the 11 patients with changes in standard or high-frequency pure tone hearing noted subjective hearing decline. Changes in DPOAE were noted in 4 patients out of 13 (30.7%). Two patients discontinued treatment due to hearing decline. Finally, 3 patients (8.6%) were diagnosed with patulous eustachian tube (PET) by an otolaryngologist, and another 3 patients are suspected to have PET based on symptom description during ophthalmologic follow-up. CONCLUSION:In our cohort, a high incidence of otologic symptoms was found to be associated with teprotumumab usage. Subjective hearing decline, changes in ultrahigh-frequency hearing as well as eustachian tube dysfunction may be encountered and suggest the potential ototoxicity of teprotumumab.

OBJECTIVE:To determine if metal reduction magnetic resonance imaging sequences and changes in implant placement minimize artifact from cochlear implants and improve visualization of intracranial structures. STUDY DESIGN/METHODS:Cadaveric study. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Five cadaveric heads. INTERVENTIONS/METHODS:Specimens were implanted with Advanced Bionics HiRes Ultra3D devices at nasion-external auditory canal angles of 90, 120, and 160 degrees, and distances from the external auditory canal of 9 or 12 cm. Standard brain/internal auditory canal (IAC) sequences with metal artifact reducing technique were acquired in a 1.5T scanner. MAIN OUTCOME MEASURES/METHODS:The primary outcome was visibility of 14 intracranial structures graded on a 4-point scale (1, structures <50% visible; 2, >50% visible with some areas nonvisible from artifact; 3, artifact present but adequate for diagnosis; and 4, high quality). Scores were determined by experienced head and neck radiologists and compared with one-way analysis of variance. RESULTS:Imaging sequences included axial 5-mm whole-brain turbo spin echo (TSE) T2 with right to left and anterior to posterior encoding, fluid-attenuation inversion recovery high bandwidth, axial 5-mm whole-brain slice-encoding metal artifact correction (SEMAC), axial IAC constructive interference in steady state, and axial 3-mm T1 IAC with and without fat saturation. T1 IACs in axial and coronal planes were best for ipsilateral structures overall (mean [standard deviation {SD}], 3.8 [0.6] and 3.8 [0.5]). SEMAC (mean [SD], 3.5 [0.8]) was superior to TSE with anterior to posterior encoding (mean [SD], 3.5 [0.9) for ipsilateral cortex, cerebellopontine angle, and brainstem/cerebellum, and equivalent for the inner ear. Constructive interference in steady state and T1 with fat saturation were poor for all ipsilateral structures (mean, 2.8 [ p < 0.01]; mean, 3.1 [ p < 0.01]). The 120 degrees/12 cm position was overall best, although the 120 degrees/9 cm position still afforded visualization of ipsilateral structures; other angles and distances conferred slight advantages for specific structures of interest. CONCLUSIONS:SEMAC and T2 TSE with anterior to posterior encoding sequences provide artifact suppression while retaining excellent image quality. Different placement angles did not confer improvement in visualization, although placement distances provided slight advantages for some structures.

OBJECTIVE:To describe the natural history of primary inner ear schwannomas over a long follow-up period. STUDY DESIGN/METHODS:Retrospective case series. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Patients with primary inner ear schwannomas with serial audiometric and radiologic follow-up. MAIN OUTCOME MEASURES/METHODS:Patterns of hearing loss, rate of hearing decline, presence of vestibular symptoms, and rate of tumor growth. RESULTS:A total of 12 patients with 13 tumors were identified. The mean duration of follow-up was 7 years. Forty-six percent of tumors were intracochlear, 15% were intravestibular, 23% were transmodiolar, and 15% were intravestibular-cochlear. Hearing loss was the most common presenting symptom, occurring in all patients. Among patients with serviceable hearing (AAO Class A or B) at the time of presentation, the average time to decline to a nonserviceable hearing level was 57.3 months (range, 21-117 mo). Hearing loss was sudden in 31% of patients, progressive in 61% and fluctuating in 8%. No patients had intractable vertigo; however, two required vestibular physiotherapy. On initial magnetic resonance imaging, the mean largest tumor dimension was 3.1 mm (standard deviation, 1.2 mm), and the mean largest dimension on most recent magnetic resonance imaging was 4.4 mm (standard deviation, 1.1 mm). Two tumors exhibited no growth over a follow-up of 11.3 and 2.8 years, respectively. Overall, the mean growth was 0.25 mm per year followed. Two patients underwent cochlear implantation with simultaneous tumor resection and had favorable outcomes. CONCLUSION/CONCLUSIONS:Long-term follow-up suggests a conservative approach, with possible hearing rehabilitation at the time of deterioration, is a safe management strategy for primary inner ear schwannomas.

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/METHODS:Prospective cohort study. SETTING/METHODS:Tertiary care hospital. PATIENTS/METHODS:Adults older than 65 years undergoing cochlear implantation. INTERVENTIONS/METHODS: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/METHODS: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.

OBJECTIVE/UNASSIGNED:Evaluate rates of Advanced Bionics Ultra 3D/Ultra cochlear implant failure in the setting of a worldwide device recall and report surgical and auditory outcomes after revision. METHODS/UNASSIGNED:Retrospective chart review was performed for adult and pediatric patients implanted with at risk devices at our center from 2016 to 2020. Device failure rates, surgical, and auditory outcomes were recorded and analyzed. RESULTS/UNASSIGNED: = 0.95). DISCUSSION/UNASSIGNED:Patients with device failure due to this field action performed well after revision implantation. Patients with bilateral at-risk devices but evidence of unilateral failure may elect to undergo simultaneous empiric revision of the contralateral device. Three patients who elected to change device manufacturers on revision have variable results that require further investigation. CONCLUSIONS/UNASSIGNED:Patients requiring revision for a device field action overall perform well. At-risk devices continue to require monitoring as a growing number are likely to fail over time.