Patient Characteristics Impacting Adherence to Serial Observation for Vestibular Schwannomas.

OBJECTIVE: To examine patient characteristics that impact serial observation adherence among vestibular schwannoma (VS) patients. STUDY DESIGN: Retrospective chart review. SETTING: 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: 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.

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.

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.

Prospective Assessment of Otologic Adverse Events due to Teprotumumab: Preliminary Results.

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.

Image Quality and Artifact Reduction of a Cochlear Implant With Rotatable Magnets.

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: Cadaveric study. SETTING: Tertiary referral center. PATIENTS: Five cadaveric heads. INTERVENTIONS: 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: 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.

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.

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.

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.

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.

Endoscopic Transcanal and Transmastoid Laser-Assisted Resection of Middle Ear Capillary Hemangioma.

OBJECTIVE: To present a rare case of a middle ear capillary hemangioma in an adult. PATIENT: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss. INTERVENTION: Endoscopic laser-assisted resection. MAIN OUTCOME MEASURES: Clinical, radiographic, and histopathological findings of a capillary hemangioma. RESULTS: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively. CONCLUSIONS: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization.

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.

Force and pressure measurements in temporal bones.

PURPOSE: Some cochlear implant (CI) patients lose their residual hearing during surgery. Two factors that might play a role in residual hearing loss are the change in intracochlear hydraulic pressure and force on the cochlear wall during electrode insertion. The aim of this study is to investigate whether a difference in peak hydraulic pressure and peak force on the cochlear wall exists during a CI electrode insertion with different insertion techniques. MATERIALS AND METHODS: Twenty fresh frozen temporal bones were used. Hydraulic pressure and force on the cochlear wall were recorded during straight electrode insertions with 1) slow versus fast insertion speed, 2) manual versus automatic insertion method and 3) round window approach (RWA) versus extended RWA (ERWA). RESULTS: When inserting with a slow compared to a fast insertion speed, the peak hydraulic pressure is 239% (95% CI: 130-399%) higher with a RWA and 58% (95% CI: 6-137%) higher with an ERWA. However, the peak force on the cochlear wall is a factor 29% less (95% CI: 13-43%) with a slow insertion speed. No effect was found of opening and insertion method. CONCLUSIONS: As contradictory findings were found for hydraulic pressure and force on the cochlear wall on insertion speed, it remains unclear which insertion speed (slow versus fast) is less traumatic to inner ear structure.

Cochlear Implant Outcomes in Neurofibromatosis Type 2: Implications for Management.

OBJECTIVE: To describe our institutional experience with cochlear implantation (CI) for rehabilitation of hearing loss in Neurofibromatosis type 2 (NF2) patients. STUDY DESIGN: Retrospective review between 1989 and 2019. SETTING: Tertiary-care center. PATIENTS: Twenty-four patients (67% female, mean age 45.6years) with NF2. Management of their ipsilateral vestibular schwannoma included microsurgery (n=12), stereotactic radiation (n=5), and observation (n=7). INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Ability to obtain open-set speech, daily device usage and long-term device benefit. RESULTS: All patients achieved some degree of sound awareness with CI. Nineteen patients (79%) achieved open-set speech understanding with a mean word-recognition score of 43% (range 0-88%). Patients with tumors 1.5 cm or less demonstrated the better speech understanding, without significant differences among treatment modalities. For tumors greater than 1.5 cm, patients who underwent microsurgery had a lower rate of open-set speech understanding compared to those treated with radiation or observation. Regular daily device use in 83% of patients was found. Long-term use (>10years) was observed in several patients, though some ultimately required reimplantation with an auditory brainstem implant due to progressive tumor growth. Mean follow-up duration was 4.1 years (range 0.4-15). CONCLUSIONS: Cochlear implantation can be an effective treatment for hearing loss in NF2 patients provided the cochlear nerve is intact, regardless of prior management for the ipsilateral tumor. The degree of benefit varies and is influenced by tumor size. Management strategies that preserve the cochlear nerve maximize the interval during which a CI could be of benefit to NF2 patients.

Cochlear Implantation in Sporadic Vestibular Schwannoma and Other Retrocochlear Pathology: A Case Series.

OBJECTIVE: To describe outcomes with cochlear implantation (CI) for rehabilitation of hearing loss in patients with sporadic vestibular schwannomas (VS) and other retrocochlear pathologies. STUDY DESIGN: Retrospective review. SETTING: Tertiary-care center. PATIENTS: Twenty three cases in 19 patients (53% men, mean age 55.8 yr) with non-neurofibromatosis type 2 related retrocochlear pathology. INTERVENTIONS: Unilateral or bilateral CI. MAIN OUTCOME MEASURES: Word recognition score, device usage. RESULTS: Etiology of deafness included sporadic VS (n = 9, 39%), radiation after head and neck or central nervous system (CNS) malignancy (n = 8, 35%), superficial siderosis (n = 3, 13%), neurosarcoidosis (n = 2, 9%), and pontine stroke (n = 1, 4%). Mean follow-up duration was 2.3 years (standard deviation [SD] 3.0; range, 0.2-9.4). Auditory perception was achieved in 20 out of 22 patients (91%) who have been activated. Mean WRS in patients with sporadic VS was 18% (SD 20; range, 0-44). Mean WRS in patients with non-VS retrocochlear pathology was 55% (SD 30; range, 0-94). Data logs showed 7.0 h/d of average use (SD 4.3; range, 0-13). CONCLUSIONS: Appropriately selected patients with retrocochlear pathology may benefit from CI so long as the patient has a cochlear fluid signal and an intact cochlear nerve. Patients with sporadic VS patients and normal contralateral hearing exhibited guarded outcomes with CI, whereas most patients with non-VS retrocochlear pathologies demonstrated open-set speech understanding scores comparable to or slightly worse than conventional CI candidates. Since variable performance benefit is observed with CI in patients with retrocochlear pathology, counseling is imperative to align patient expectations with realistic outcomes.

Stapes Surgery Outcomes in Patients With Concurrent Otosclerosis and Superior Semicircular Canal Dehiscence.

OBJECTIVE: To review outcomes of stapes surgery in patients with concurrent otosclerosis and superior semicircular canal dehiscence. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Patients with concurrent otosclerosis and superior canal dehiscence, confirmed by computed tomography (CT) imaging. INTERVENTION(S): Stapes surgery for conductive hearing loss. MAIN OUTCOME MEASURE(S): Postoperative air-bone gap (ABG), as well as the number of patients in whom surgery was deemed successful (postoperative ABG <10 dB HL). RESULTS: Five patients with superior canal dehiscence and concomitant otosclerosis who underwent surgical repair were identified. Mean preoperative ABG was 29.0 ±â€Š6.4 dB HL. Mean postoperative ABG was 13.0 ±â€Š13 dB HL. Three patients (60%) had a successful outcome, defined as postoperative ABG less than 10. One patient experienced unmasking of superior canal dehiscence vestibular symptoms. CONCLUSIONS: Patients with concurrent otosclerosis and superior canal dehiscence appear to have a lower likelihood of successful hearing restoration following stapes surgery. Patients should be counseled accordingly. Routine preoperative CT imaging before stapes surgery may be helpful to identify patients at risk for poor outcomes.