Faculty Bibliography

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 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: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/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.

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/METHODS:Retrospective case series. SETTING/METHODS:Tertiary referral hospital. PATIENTS/METHODS:One hundred three patients who underwent cochlear implantation between June 2020 and August 2021. INTERVENTIONS/METHODS:Intraoperative electrophysiologic monitoring (electrode impedances, neural response telemetry, and TIM measurement) and modified Stenver's view plain film radiographs. MAIN OUTCOME MEASURES/METHODS: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/CONCLUSIONS:TIM measurements were able to accurately identify tip foldovers. More research is needed to define the adjunctive role of TIM as an intraoperative measure.

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/UNASSIGNED: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/CONCLUSIONS:Cochlear implantation in patients with concomitant retrocochlear pathology generally results in improved speech discrimination scores sustained over time.

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

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.

OBJECTIVE:To present a rare case of a middle ear capillary hemangioma in an adult. PATIENT/METHODS:A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss. INTERVENTION/METHODS:Endoscopic laser-assisted resection. MAIN OUTCOME MEASURES/METHODS: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.