Faculty Bibliography

OBJECTIVE/UNASSIGNED:To better characterize the cochlear apex in relation to surgically relevant landmarks to guide surgeons and improve procedural success of apical electrode placement. STUDY DESIGN/UNASSIGNED:Retrospective image analysis. SETTING/UNASSIGNED:Tertiary referral center. PATIENTS/UNASSIGNED:Cochlear implant recipients with available preoperative computed tomography (CT) imaging. INTERVENTION/UNASSIGNED:None. MAIN OUTCOME MEASURE/UNASSIGNED:Cochlear dimensions and cochlear apex distance measures to surgically relevant middle ear landmarks and critical structures. RESULTS/UNASSIGNED:Eighty-two temporal bone CT scans were analyzed utilizing multiplanar reformats. The average lateral width of promontory bone over the cochlear apex was 1.2 mm (standard deviation [SD], 0.3). The anteroposterior distance from the round window (avg, 4.2 mm; SD, 0.5), oval window (avg, 3.3 mm; SD, 0.3), cochleariform process (avg, 2.3; SD, 0.5), and superior-inferior distance from the cochleariform process (avg, -0.9; SD, 0.8) to the cochlear apex were measured. The relationship of the cochlear apex to critical structures was highly variable.A newly developed stapes vector was created and found to mark the posterior/superior boundary of the apex in 94% of patients. When a vector parallel to the stapes vector was drawn through the round window, it marked the anterior/inferior boundary of the cochlear apex in 89% of patients. CONCLUSIONS/UNASSIGNED:This study assists in characterizing cochlear apex anatomy and its relation to surrounding structures as a means of improving procedural accuracy and reducing trauma during apical cochleostomy. Understanding both distance relationships and expected boundaries of the apex could help to inform future surgical approaches.

OBJECTIVES/UNASSIGNED:This study aims to synthesize current knowledge and outcomes related to pediatric auditory brainstem implantation (ABI) in children with severe inner ear malformations (IEMs). It highlights the clinical management practices, challenges, and potential future directions for consensus development in this field. METHODS/UNASSIGNED:A systematic review of findings presented at the Third International Pediatric ABI Symposium organized by the Hacettepe Cochlear Implant team between 3 and 5 September 2020 was conducted, incorporating data from 41 departments across 19 countries. Relevant clinical outcomes, imaging techniques, surgical approaches, and rehabilitation strategies were analyzed to identify key trends and variability in practices. RESULTS/UNASSIGNED:The review indicates that children receiving ABIs exhibit diverse auditory outcomes influenced by individual anatomical variations and developmental factors. Early implantation, particularly before the age of three, positively correlates with better auditory and language development. Multicenter experiences underscore the necessity of tailored decision-making, which considers both surgical candidacy and comprehensive rehabilitation resources. DISCUSSION:/UNASSIGNED:The variability in outcomes emphasizes the need for improved consensus and guidelines regarding eligibility, surgical techniques, and multidisciplinary rehabilitation approaches. Notable complications and the necessity for thorough imaging assessments were also identified as critical components affecting clinical decisions. CONCLUSION/UNASSIGNED:A formal consensus statement is warranted to standardize best practices in ABI management. This will not only enhance patient outcomes but also guide future research efforts to address the remaining challenges in the treatment of children with severe IEMs. Enhanced collaboration among team members will be pivotal in achieving these objectives.

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 To characterize treatment and hearing outcomes for cerebellopontine angle (CPA) meningiomas with inherent risks of hearing loss and identify predictors of hearing loss for surgically treated lesions. Design Retrospective chart review. Setting Tertiary care medical center. Participants Adult patients with CPA meningiomas impinging upon cranial nerve VIII and/or pretreatment hearing loss managed with microsurgery or stereotactic radiosurgery (SRS) with Gamma Knife at our center between 2012 and 2023. Main Outcome Measures Hearing preservation rate was determined from analysis of patients with pretreatment serviceable hearing for whom hearing-preserving treatment was attempted. Surgical patients were further analyzed using multivariable Cox proportional hazards regression models to identify factors predictive of postoperative hearing loss. Results We identified 80 patients with CPA meningiomas meeting inclusion criteria who were managed with either microsurgery (43, 54%) or radiosurgery (37, 46%). Following SRS, hearing was preserved in 88% of cases. Following microsurgery, hearing was preserved in 71% of patients-all patients who lost hearing had tumors involving the internal auditory canal (IAC). Among surgical patients only, multivariable analysis accounting for preoperative hearing, recurrence status, lesion size, and patient age, the preoperative imaging finding that the CPA meningioma surrounded the vestibulocochlear nerve was significantly associated with hearing loss (hazard ratio: 10.3, 95% confidence interval: 1.3-81.4, p = 0.02). Conclusion Most patients with meningiomas of the CPA can experience preservation of hearing, even when there is risk of hearing loss based on pretreatment evaluation. IAC invasion and surrounding of eighth nerve by tumor may portend poorer hearing outcomes in surgically managed patients.

Hearing loss is common among Veterans, and extensive hearing care resources are prioritized within the Veterans Administration (VA). Severe hearing loss poses unique communication challenges with speech understanding that may not be overcome with amplification. We analyzed data from the VA Audiometric Repository between 2005 and 2017 and the relationship between hearing loss severity with speech recognition scores. We hypothesized that a significant subset of Veterans with severe or worse hearing loss would have poor unaided speech perception outcomes even with adequate audibility. Sociodemographic characteristics and comorbidities were compiled using electronic medical records as was self-report measures of hearing disability. We identified a cohort of 137,500 unique Veterans with 232,789 audiograms demonstrating bilateral severe or worse hearing loss (four-frequency PTA > 70 dB HL). The median (IQR; range) age of Veterans at their first audiogram with severe or worse hearing loss was 81 years (74 to 87; 21-90+), and a majority were male (136,087 [99%]) and non-Hispanic white (107,798 [78.4%]). Among those with bilateral severe or worse hearing loss, 41,901 (30.5%) also had poor speech recognition scores (<50% words), with greater hearing loss severity correlating with worse speech perception. We observed variability in speech perception abilities in those with moderate-severe and greater levels of hearing loss who may derive limited benefit from amplification. Veterans with communication challenges may warrant alternative approaches and treatment strategies such as cochlear implants to support communication needs.

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.

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.

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 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.