Cochlear Implantation in Children Under 9 Months of Age: Safety and Efficacy.

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: Single-center retrospective review. SETTING: Tertiary referral center. PATIENTS: Children 5 to 8 months of age who received a CI between 2011 and 2021. INTERVENTIONS: Therapeutic-CI. MAIN OUTCOME MEASURES: 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.

Facial Reanimation After Intratemporal Facial Nerve Schwannoma Resection: A Systematic Review.

Objective: To systematically analyze the outcomes of reanimation techniques that have been described for patients undergoing non-fascicle sparing resection of intratemporal facial schwannomas. Methods: A systematic review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines of the PubMed, MEDLINE, and Cochrane Central Register of Controlled Trials databases. Results: Eight hundred forty studies were screened with 22 meeting inclusion criteria comprising 266 patients. Most facial nerve reanimations (81.2%) were performed using an interposition nerve graft. The remaining patients underwent hypoglossal-facial nerve transposition (13.9%), primary anastomosis (3.4%), and free muscle transfer (0.1%). Of the reported interposition grafts, the two most utilized were the great auricular (113/199) and sural (86/199) nerves. Interposition nerve grafts resulted in significantly better outcomes in facial nerve function postoperatively than hypoglossal-facial transposition (3.48 vs. 3.92; p < 0.01). There was no difference between interposition grafts. Conclusion: This study systematically reports that interposition nerve grafts, after resection of intratemporal facial schwannoma, result in superior outcomes than hypoglossal-facial nerve transposition in these patients.

Acceptance and Benefit of Electroacoustic Stimulation in Children.

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.

Bilateral cochlear implantation after immunotherapy-related profound hearing loss: A case report.

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.

Slim Modiolar Electrode Placement in Candidates for Electroacoustic Stimulation.

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.

HiRes ultra series cochlear implant field recall: failure rates and early outcomes.

OBJECTIVE: 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: 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: Of 113 at-risk devices, 20 devices (17.7%) in 18 patients (two bilaterally implanted) were identified as failures. All devices were with mid-scala electrodes. Eleven patients (61.1%) were children and 7 (38.9%) adults. Twelve patients were found to have failing devices after reporting subjective performance decline; the remainder were prompted by manufacturer notification. All were revised, with the majority (83.3%) choosing the same manufacturer. All had uncomplicated original and revision insertions. Among adults, average word scores on the revised side were stable pre- to post-revision (P = 0.95). DISCUSSION: 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: 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.

Interferon-Driven Immune Dysregulation in Common Variable Immunodeficiency-Associated Villous Atrophy and Norovirus Infection.

PURPOSE: About 15% of patients with common variable immunodeficiency (CVID) develop a small intestinal enteropathy, which resembles celiac disease with regard to histopathology but evolves from a distinct, poorly defined pathogenesis that has been linked in some cases to chronic norovirus (NV) infection. Interferon-driven inflammation is a prominent feature of CVID enteropathy, but it remains unknown how NV infection may contribute. METHODS: Duodenal biopsies of CVID patients, stratified according to the presence of villous atrophy (VA), IgA plasma cells (PCs), and chronic NV infection, were investigated by flow cytometry, multi-epitope-ligand cartography, bulk RNA-sequencing, and RT-qPCR of genes of interest. RESULTS: VA development was connected to the lack of intestinal (IgA+) PC, a T helper 1/T helper 17 cell imbalance, and increased recruitment of granzyme+CD8+ T cells and pro-inflammatory macrophages to the affected site. A mixed interferon type I/III and II signature occurred already in the absence of histopathological changes and increased with the severity of the disease and in the absence of (IgA+) PCs. Chronic NV infection exacerbated this signature when compared to stage-matched NV-negative samples. CONCLUSIONS: Our study suggests that increased IFN signaling and T-cell cytotoxicity are present already in mild and are aggravated in severe stages (VA) of CVID enteropathy. NV infection preempts local high IFN-driven inflammation, usually only seen in VA, at milder disease stages. Thus, revealing the impact of different drivers of the pathological mixed IFN type I/III and II signature may allow for more targeted treatment strategies in CVID enteropathy and supports the goal of viral elimination.

The Frailty Phenotype in Older Adults Undergoing Cochlear Implantation.

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.

Deciphering imprints of impaired memory B-cell maturation in germinal centers of three patients with common variable immunodeficiency.

Common variable immunodeficiency (CVID), characterized by recurrent infections, low serum class-switched immunoglobulin isotypes, and poor antigen-specific antibody responses, comprises a heterogeneous patient population in terms of clinical presentation and underlying etiology. The diagnosis is regularly associated with a severe decrease of germinal center (GC)-derived B-cell populations in peripheral blood. However, data from B-cell differentiation within GC is limited. We present a multiplex approach combining histology, flow cytometry, and B-cell receptor repertoire analysis of sorted GC B-cell populations allowing the modeling of distinct disturbances in GCs of three CVID patients. Our results reflect pathophysiological heterogeneity underlying the reduced circulating pool of post-GC memory B cells and plasmablasts in the three patients. In patient 1, quantitative and qualitative B-cell development in GCs is relatively normal. In patient 2, irregularly shaped GCs are associated with reduced somatic hypermutation (SHM), antigen selection, and class-switching, while in patient 3, high SHM, impaired antigen selection, and class-switching with large single clones imply increased re-cycling of cells within the irregularly shaped GCs. In the lymph nodes of patients 2 and 3, only limited numbers of memory B cells and plasma cells are formed. While reduced numbers of circulating post GC B cells are a general phenomenon in CVID, the integrated approach exemplified distinct defects during GC maturation ranging from near normal morphology and function to severe disturbances with different facets of impaired maturation of memory B cells and/or plasma cells. Integrated dissection of disturbed GC B-cell maturation by histology, flow cytometry, and BCR repertoire analysis contributes to unraveling defects in the essential steps during memory formation.

Cochlear Implantation Outcomes in Patients With Retrocochlear Pathology: A Systematic Review and Pooled Analysis.

OBJECTIVE: To review the current literature regarding cochlear implantation in patients with retrocochlear pathologies and extract speech perception scores between 6 months and 1 year after surgery. DATABASES REVIEWED: PubMed/MEDLINE, Embase and Cochrane CENTRAL via Ovid, CINAHL Complete via Ebsco, and Web of Science. METHODS: The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search strategies included keywords and subject headings to maximize retrieval and reflect cochlear implants and retrocochlear pathologies. Patients with previously resected vestibular schwannoma (VS) were excluded. RESULTS: There were 2,524 abstracts screened against inclusion criteria, and 53 studies were included, with individual data available for 171 adult patients. Pathologies included were either observed or irradiated VS (previously operated tumors were excluded) (n = 99, 57.9%), superficial siderosis (n = 39, 22.8%), neurosarcoidosis (n = 11, 6.4%), and previous central nervous system or skull base radiation (n = 22, 12.9%). Mean (standard deviation) postoperative consonant-nucleus-consonant (CNC) word scores were 45.4% (24.2) for observed VS, 44.4% (20.8) for irradiated VS, 43.6% (21.0) for superficial siderosis, 89.5% (3.0) for neurosarcoidosis, and 30.0% (30.2) in patients with previous central nervous system or skull base irradiation. Irradiated compared with observed VS had similar postoperative CNC word scores (effect size, 0.06; p = 0.71). Age, sex, maximal tumor dimension, and neurofibromatosis type 2 status did not significantly impact cochlear implant performance in patients with VS. Eighty-two percent of patients with reported device usage were daily users, and overall, 82% of cases benefitted from cochlear implantation. CONCLUSION: Cochlear implantation in patients with concomitant retrocochlear pathology generally results in improved speech discrimination scores sustained over time.

The Natural History of Primary Inner Ear Schwannomas: Outcomes of Long-Term Follow-Up.

OBJECTIVE: To describe the natural history of primary inner ear schwannomas over a long follow-up period. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Patients with primary inner ear schwannomas with serial audiometric and radiologic follow-up. MAIN OUTCOME MEASURES: 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 (American Academy of Otolaryngology-Head and Neck Surgery 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: 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.

Predictive Value of Transimpedance Matrix Measurements to Detect Electrode Tip Foldover.

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.

Modern Hearing Preservation Outcomes After Vestibular Schwannoma Stereotactic Radiosurgery.

BACKGROUND: For patients with vestibular schwannoma (VS), stereotactic radiosurgery (SRS) has proven effective in controlling tumor growth while hearing preservation remains a key goal. OBJECTIVE: To evaluate hearing outcomes in the modern era of cochlear dose restriction. METHODS: During the years 2013 to 2018, 353 patients underwent Gamma knife surgery for VS at our institution. We followed 175 patients with pre-SRS serviceable hearing (Gardner-Robertson Score, GR 1 and 2). Volumetric and dosimetry data were collected, including biological effective dose, integral doses of total and intracanalicular tumor components, and hearing outcomes. RESULTS: The mean age was 56 years, 74 patients (42%) had a baseline GR of 2, and the mean cochlear dose was 3.5 Gy. The time to serviceable hearing loss (GR 3-4) was 38 months (95% CI 26-46), with 77% and 62% hearing preservation in the first and second years, respectively. Patients optimal for best hearing outcomes were younger than 58 years with a baseline GR of 1, free canal space ≥0.041 cc (diameter of 4.5 mm), and mean cochlear dose <3.1 Gy. For such patients, hearing preservation rates were 92% by 12 months and 81% by 2 years, staying stable for >5 years post-SRS, significantly higher than the rest of the population. CONCLUSION: Hearing preservation after SRS for patients with VS with serviceable hearing is correlated to the specific baseline GR score (1 or 2), age, cochlear dose, and biological effective dose. Increased tumor-free canal space correlates with better outcomes. The most durable hearing preservation correlates with factors commonly associated with smaller tumors away from the cochlea.

Cochlear Implants in Veterans: 10-Year Experience at a Single Referral Center.

PURPOSE: Severe hearing loss is particularly prevalent among Veterans. In response, the Veterans Affairs (VA) provides hearing aids and cochlear implants as part of their comprehensive hearing loss treatment. The VA's national health care system and integrated electronic health records offer a unique opportunity to examine patterns and outcomes in the management of severe hearing loss. METHOD: We retrospectively reviewed an urban VA center's database for all Veterans who underwent cochlear implantation between 2010 and 2019. We collected preoperative demographic and audiological data including cochlear implant outcomes. RESULTS: During the study period, 61 Veterans received a cochlear implant, totaling 70 ears implanted. Median age at implantation was 70.2 years. Over 90% of Veterans reported preoperative hearing aid use, and median preoperative four-frequency pure-tone average and aided word recognition score in the ear to be implanted were 100 dB HL and 7%, respectively. The median onset of severe hearing loss bilaterally was over 4 years prior to cochlear implant evaluation based on available audiometric data. Postoperative word recognition score was negatively correlated with self-reported duration of hearing loss. CONCLUSIONS: This study provides unique insights into how Veterans with severe hearing loss are managed at one VA center. Compared to data published on cochlear implant recipients in the civilian population, Veterans in this cohort were older and had higher rates of preoperative hearing aid use. Available VA data suggest most Veterans were appropriate candidates for cochlear implant referral years prior to undergoing an evaluation. Reasons for treatment delays in this population remain to be studied.

Distinct CD8 T Cell Populations with Differential Exhaustion Profiles Associate with Secondary Complications in Common Variable Immunodeficiency.

PURPOSE: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency, with heterogeneous clinical presentation. Our goal was to analyze CD8 T cell homeostasis in patients with infection only CVID, compared to those additionally affected by dysregulatory and autoimmune phenomena. METHODS: We used flow and mass cytometry evaluation of peripheral blood of 40 patients with CVID and 17 healthy donors. RESULTS: CD8 T cells are skewed in patients with CVID, with loss of naïve and increase of effector memory stages, expansion of cell clusters with high functional exhaustion scores, and a highly activated population of cells with immunoregulatory features, producing IL-10. These findings correlate to clinically widely used B cell-based EURO classification. Features of exhaustion, including loss of CD127 and CD28, and expression of TIGIT and PD-1 in CD8 T cells are strongly associated with interstitial lung disease and autoimmune cytopenias, whereas CD8 T cell activation with elevated HLA-DR and CD38 expression predict non-infectious diarrhea. CONCLUSION: We demonstrate features of advanced differentiation, exhaustion, activation, and immunoregulatory capabilities within CD8 T cells of CVID patients. Assessment of CD8 T cell phenotype may allow risk assessment of CVID patients and provide new insights into CVID pathogenesis, including a better understanding of mechanisms underlying T cell exhaustion and regulation.

Modifications to Implanting the OSIA® 2 Bone Conduction Hearing Implant: How I Do it.

This "How I Do It" report describes modifications made to the OSIA bone conduction hearing implant surgery in order to reduce wound complications. Laryngoscope, 132:1850-1854, 2022.

Cochlear Implant Outcomes in CHARGE Syndrome: Updated Perspectives.

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.

Stimulating the Cochlear Apex Without Longer Electrodes: Preliminary Results With a New Approach.

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.

Delayed cochlear implantation in congenitally deaf children-identifying barriers for targeted interventions.

OBJECTIVES: Age of cochlear implantation (CI) is an important predictor of language development in those with congenital sensorineural hearing loss. Despite universal newborn hearing screening initiatives and the known benefits of early CI, a subset of congenitally deaf children continue to be evaluated for cochlear implants later in childhood. This study aims to identify the barriers to early cochlear implantation in these children. METHODS: A retrospective review was conducted for all pediatric cochlear implants aged 3 years or older performed at a single academic institution between 2013 and 2017. Children implanted before the age three, those with a prior unilateral cochlear implant, and those with progressive or sudden hearing loss were excluded. Variables included newborn hearing screen results, age at hearing loss diagnosis, time of initiation and documented benefit of hearing aids, age of implantation, pre/post-implantation evaluation scores, and reason for delayed referral for cochlear implantation. RESULTS: Thirty-one patients were identified meeting these inclusion criteria. Twenty-one children were subject to UNBS in the U.S. Fourteen of those children failed their newborn hearing screening. Average age at implantation was 6.2 years. Four reasons were identified for increased age at cochlear implantation. Two categories represent delays related to (1) Amplification continually prescribed even though the range of hearing loss and speech development assessment suggests CI may have been more appropriate well before referral (N = 13) (2) Patients were not subject to newborn hearing screening and/or timely diagnosis of their hearing loss (N = 8). In other cases, patients were appropriately fit with hearing aids until evidence that they derived limited benefit and then referred for CI (N = 8). Lastly, in a few cases, records were indeterminate with regards to the timing and appropriate diagnosis of their hearing loss (N = 2). CONCLUSION: Understanding the reasons for delayed cochlear implantation in congenitally deaf children might allow the development of targeted interventions to improve outcomes. Specifically, those children who were not referred before age 3 despite use of amplification with limited benefit offer one potential target population for earlier CI.

Outcomes of Salvage Resection and Radiosurgery Following Failed Primary Treatment of Vestibular Schwannomas.

OBJECTIVE: To evaluate outcomes following salvage microsurgery (MS) and salvage stereotactic radiosurgery (SRS) after failure of primary treatment for vestibular schwannomas (VS). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. METHODS: Patients with more than 1 intervention for their VS were divided into 4 groups: MS followed by SRS (n = 61), MS followed by MS (n = 9), SRS followed by MS (n = 7), and SRS followed by SRS (n = 7), and outcomes were evaluated. RESULTS: A total of 77 patients were included (84 procedures). In group 1 (MS then SRS), 3% developed a decline in facial function, 3% developed trigeminal sensory loss, and 13% patients had gradual improvement of facial nerve function following SRS. Group 2 (MS then MS) had the highest rates of facial nerve deterioration, although all but 1 patient achieved a House-Brackmann score of II or III. Gross-total resection (GTR) was achieved in 56% of patients. When a different approach was used for salvage resection, GTR occurred more commonly, and facial nerve outcomes were similar. In group 3 (SRS then MS), GTR occurred in 43% of cases, and 2 of 7 patients developed worsened facial function. In group 4 (SRS then SRS), no patient developed facial weakness after reirradiation, and 1 developed a trigeminal nerve deficit. CONCLUSIONS: For MS recurrences/residuals, SRS is the mainstay of treatment and does not preclude facial function recovery. If salvage microsurgery is required, an alternate approach should be considered. For SRS failures, when MS is required, less-than GTR may be preferable, and reirradiation is a potential safe alternative.