Preliminary Evidence for the Effects of Gentamicin on Vertical Semicircular Canals.

INTRODUCTION: Gentamicin is a vestibulotoxic antibiotic often used in patients with Ménière's disease for its vestibular ablative effects. Gentamicin's effect on the horizontal semicircular canal does not always correlate with the degree of vertigo control achieved by patients; its effect on the vertical semicircular canals remains unknown. We sought to examine the effect of intratympanic gentamicin on vertical semicircular canal function in patients with Ménière's disease using video head impulse testing. METHODS: A retrospective case series was carried out at a tertiary academic center. Patients with Ménière's disease who received ≥1 intratympanic gentamicin injection from 2019-2022 and had video head impulse testing performed were included. Outcomes of interest were vertical semicircular canal function following intratympanic gentamicin, correlations between vertical semicircular canal function and horizontal semicircular canal function, and residual symptoms following injection. RESULTS: Ten patients met inclusion criteria. Twenty percent had abnormal V-SCC function prior to any injection and 40% following the first injection. There was an association between abnormal vertical and horizontal semicircular canal function following the first intratympanic gentamicin injection, though the relationship did not reach statistical significance (p = 0.058). While patients with abnormal vertical semicircular canal function following the first injection were less likely to report ongoing vertigo attacks, the relationship was not statistically significant (p = 0.260). CONCLUSIONS: Intratympanic gentamicin leads to changes in vertical semicircular canal function in at least a proportion of patients with Ménière's disease. Further study is required to better assess correlations between vertical semicircular canal function and symptom control following intratympanic gentamicin.

Pure tone audiometry as assessed by a commercially-available mobile phone application compared to formal audiometry.

PURPOSE: Comparison of audiometric measurements of commercially available smartphone audiogram application thresholds as compared to gold standard audiometric evaluation. MATERIALS AND METHODS: A single-institution, original contribution. Ninety consecutive adult patients presenting to a tertiary care auditory clinic with auditory complaints were evaluated using standard audiometric testing and an application-based hearing test. Correlation between app results and standard audiogram for air conduction pure tone thresholds was evaluated. RESULTS: Mimi™ (Berlin, Germany) results for audiometric thresholds were moderately correlated with standard audiogram (r = 0.51-0.68) depending on severity. The percentage of patients whose hearing loss severity on formal audiometry results were accurately reflected in the Mimi™ (app-based hearing test: ABHT)1 results ranged from 18.2 to 80 %. Among patients whose results were at the extremes of hearing performance, app and standard audiogram results were similar. ABHT yielded an overall sensitivity of 35.5 % and specificity of 97.1 % for normal hearing, and an overall sensitivity of 80 % and specificity of 96 % for severe hearing loss. CONCLUSIONS: Results from an audiometric smart phone application showed accurate categorization of hearing loss at the high and extremes as compared to standard audiometry. However, correlation of pure tone values was more variable and dependent on hearing level.

Socioeconomic determinants impact quality of life at vestibular schwannoma diagnosis.

BACKGROUND: Socioeconomic variables including race, education, and income have been shown to affect vestibular schwannoma incidence, treatment, and outcomes. We sought to determine the impact of socioeconomic factors on quality of life at the time of vestibular schwannoma diagnosis. METHODS: Retrospective cohort study conducted at a tertiary academic center. All patients evaluated for vestibular schwannoma from March 1, 2010 to December 31, 2021 who completed at least one Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire at presentation or prior to any intervention were included. PANQOL scores were compared across income quintiles, racial groups, and health insurance categories. RESULTS: Two-hundred and ninety-six patients who had non-missing information on variables of interest were included. Compared to White/Caucasian patients (84.5 %), Black/African American patients (4.7 %) had significantly lower PANQOL total scores (b = -12.8[-21.7, -4.0], p = 0.005). Compared to patients with Commercial insurance (53 %), patients who were Uninsured/ Self-pay (1.7 %) had significantly lower PANQOL total scores (b = -16.7[-31.4, -1.9], p = 0.027). Patients in higher income quintiles had significantly higher PANQOL total scores (b = 11.7[3.9, 19.5], p = 0.004 comparing highest income quintile to lowest). After controlling for potential confounders, income quintile (b = 9.6[1.3, 17.9], p = 0.023 comparing highest quintile to lowest) and insurance (b = -17.0[-31.9, -21], p = 0.025 comparing Uninsured/Self-pay to Commercial insurance) remained predictors of total PANQOL score. CONCLUSIONS: Socioeconomic factors including race, health insurance, and income appear to contribute to quality of life at the time of vestibular schwannoma diagnosis. These variables are interrelated and the effects of race may be mediated in part by differences in income and health insurance coverage.

Reassessing the Utility of Surgical Intervention for Skull Base Osteomyelitis: A 16-Year Experience.

OBJECTIVE: The role of surgery in lateral skull base osteomyelitis (SBO) is controversial. Surgical intervention is often requested by consulting services in the interest of additional culture data to inform medical management. However, whether surgery alters subsequent antibiotic treatment or modifies disease outcome remains unknown. The aim of this study was to investigate the role of surgical intervention in the treatment of SBO by (1) comparing nonsurgical and surgical culture data and (2) assessing clinical outcomes and treatment course following surgical intervention. STUDY DESIGN: Retrospective. SETTING: Tertiary care center. METHODS: The electronic record was queried for all patients with SBO who presented to a single institution over a 16-year period (2007-2023). Information recorded included history and exam, bedside and intraoperative culture data, antibiotic course, and disease outcomes. Primary outcome measures included change in medical management based on intraoperative cultures, recurrence rates, and mortality rates. RESULTS: Forty patients (41 ears, average age 73 ± 13 years) met inclusion criteria. Out of 13 (32%) patients who underwent surgical intervention, one intraoperative culture changed the antibiotic course due to identification of resistance to the original antibiotic used. Surgery did not demonstrate a benefit in overall mortality (23% vs 18%, P = 0.36) or facial nerve function (33% vs 50%, P = 0.56) compared to medical management, and was associated with increased recurrence rates (54% vs 11%, P = 0.05). CONCLUSION: Surgical cultures rarely changed antibiotic selection. Surgical debridement in treatment-refractory SBO was also not associated with improvement in recurrence or mortality rates, though this may reflect underlying differences in disease severity.

An Update on the Epidemiology and Clinicodemographic Features of Meniere's Disease.

OBJECTIVE: To characterize the estimated prevalence and clinicodemographic features of Ménière's disease (MD) using current diagnostic criteria. METHODS: A cross-sectional study was undertaken at our tertiary academic referral center. All patients seen in Otolaryngology clinic with ICD-10 diagnoses of MD, from January 1, 2013 to July 31, 2022 were identified. Chart review was undertaken to determine the estimated prevalence of MD meeting AAO-HNS diagnostic criteria. Clinicodemographic features were evaluated against a comparator group without MD seen in our health system. RESULTS: Of 806 ICD-10 diagnoses of MD, we identified 480 MD cases meeting diagnostic criteria (168 definite). Mean age at presentation for MD cases was 49 years. Forty-seven percent of cases were male. A significantly higher proportion of MD cases than comparators were white (76% vs. 66%, p < 0.001). Mean time since MD symptom onset was 6.7 years, with a mean attack duration of 4.6 h; 7.5% of MD cases reported a positive family history, and 7% had bilateral disease. The odds of reporting migraine were significantly greater among MD patients than comparators (OR 1.74 [1.26-2.42]); the odds of having autoimmune conditions were lower (OR 0.45 [0.28-0.74]); and the odds of reporting allergies were no different (OR 0.96 [0.74-1.25]) versus comparator patients after controlling for demographic characteristics. CONCLUSIONS: Among MD diagnoses, there is a low estimated prevalence of MD meeting diagnostic criteria, and an even lower prevalence of definite MD. Compared to a comparator group of patients seen for any disorder, patients with MD are more likely to be white, male, and have a history of migraine. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3310-3315, 2024.

A multi-institutional machine learning algorithm for prognosticating facial nerve injury following microsurgical resection of vestibular schwannoma.

Vestibular schwannomas (VS) are the most common tumor of the skull base with available treatment options that carry a risk of iatrogenic injury to the facial nerve, which can significantly impact patients' quality of life. As facial nerve outcomes remain challenging to prognosticate, we endeavored to utilize machine learning to decipher predictive factors relevant to facial nerve outcomes following microsurgical resection of VS. A database of patient-, tumor- and surgery-specific features was constructed via retrospective chart review of 242 consecutive patients who underwent microsurgical resection of VS over a 7-year study period. This database was then used to train non-linear supervised machine learning classifiers to predict facial nerve preservation, defined as House-Brackmann (HB) I vs. facial nerve injury, defined as HB II-VI, as determined at 6-month outpatient follow-up. A random forest algorithm demonstrated 90.5% accuracy, 90% sensitivity and 90% specificity in facial nerve injury prognostication. A random variable (rv) was generated by randomly sampling a Gaussian distribution and used as a benchmark to compare the predictiveness of other features. This analysis revealed age, body mass index (BMI), case length and the tumor dimension representing tumor growth towards the brainstem as prognosticators of facial nerve injury. When validated via prospective assessment of facial nerve injury risk, this model demonstrated 84% accuracy. Here, we describe the development of a machine learning algorithm to predict the likelihood of facial nerve injury following microsurgical resection of VS. In addition to serving as a clinically applicable tool, this highlights the potential of machine learning to reveal non-linear relationships between variables which may have clinical value in prognostication of outcomes for high-risk surgical procedures.

A Rare Case of Cochlear Implant Electrode Array Misplacement Into the Posterior Semicircular Canal.

Cochlear implant electrode array misplacement is a rare but serious complication that may result in failure of hearing rehabilitation, non-auditory percepts, vestibular disturbance, or damage to adjacent neurovascular structures. We present a case of an elderly patient who suffered electrode array misplacement into the posterior semicircular canal, resulting in vestibular symptoms and severe downstream sequelae. The risk of misplacement may be higher in patients with a history of chronic otitis media or prior otologic surgery, and with the use of pre-curved electrode arrays. Electrophysiological testing and intraoperative imaging may allow for early detection and intervention in these cases. Laryngoscope, 133:175-177, 2023.

Predictive Ability of First-Side Cochlear Implant Performance in Adult Sequential Bilateral Cochlear Implantation.

OBJECTIVE: Predictors of second-side cochlear implant performance have not been well studied. We sought to assess whether speech recognition scores from first-side cochlear implant (CI1) could predict second-side cochlear implant (CI2) scores in sequential bilaterally implanted adults. STUDY DESIGN: Retrospective review using a prospectively collected database. SETTING: Academic tertiary care hospital. PATIENTS: Fifty-seven adults with postimplantation speech recognition testing performed at least 12 months after CI2. INTERVENTION: Sequential bilateral CI. MAIN OUTCOME MEASURES: CI2 performance at ≥12 months as measured using consonant-nucleus-consonant (CNC) words and AzBio sentences in quiet and +10 dB signal-to-noise ratio (S/N). RESULTS: CI1 performance scores at ≥12 months were independently associated with CI2 performance scores at ≥12 months for CNC words (ß = 0.371 [0.136-0.606], p = 0.003), AzBio sentences in quiet (ß = 0.614 [0.429-0.80], p < 0.0001), and AzBio +10 dB S/N (ß = 0.712 [0.459-0.964], p < 0.0001). CI1 scores on AzBio in quiet at 0 to 6 months were also independently associated with CI2 AzBio in quiet scores at ≥12 months (ß = 0.389 [0.004-0.774], p = 0.048). Hearing loss etiology and duration, age at implantation, interval between CI1 and CI2, duration of hearing aid use, and preimplantation speech recognition testing scores were not consistently associated with CI2 scores at ≥12 months. CONCLUSIONS: CI1 performance is an independent predictor of second-side performance as measured ≥12 months postimplantation. This may be a clinically useful metric when considering adult sequential bilateral implantation.

Caloric function as a predictor of cochlear implant performance.

OBJECTIVES: Changes in vestibular function have been demonstrated following cochlear implantation (CI). The functional impact of these changes on CI performance has not been well-studied. We sought to assess whether caloric changes postimplantation could predict CI performance. METHODS: Retrospective review of a prospectively collected database at a tertiary care hospital. Patients who underwent CI over a 22-year period (1999-2021) and had videonystagmography (VNG) testing pre- and postimplantation were included. Caloric responses were compared pre- versus post-implantation, and assessed for their ability to predict CI performance as evaluated using AzBio +10 decibels signal-to-noise ratio (dB S/N) scores. RESULTS: 43 CI recipients were included. There was a significant difference in the average maximal slow-phase velocity in response to caloric irrigation in the implanted ear pre- versus post-operatively (21.2 vs. 18.5 deg/s; p = 0.02). Controlling for age and pre-implantation speech recognition performance, pre- and post-implantation caloric responses in the implanted ear significantly predicted CI performance at 0-6 months and >6 months post-implantation. Caloric changes following implantation were not significantly correlated with CI performance. CONCLUSION: CI impacts vestibular function as evidenced by changes in caloric responses. Vestibular function pre- and possibly post-CI may be clinically useful metrics for predicting some proportion of CI performance variability.

Long-Term Hearing Outcomes After Hybrid Cochlear Implantation.

OBJECTIVE: To examine long-term (>5 yr) low-frequency hearing preservation after hybrid cochlear implantation. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Tertiary care center outpatient clinic. PATIENTS: All patients older than 21 years implanted with a Cochlear Hybrid L24 device from 2014 to 2021. MAIN OUTCOME MEASURE: Changes in low-frequency pure-tone average (LFPTA) were calculated at each of several time points relative to the date of implantation. The proportion of patients with preserved LFPTA at last follow-up and Kaplan-Meier estimates for loss of residual hearing were calculated in addition to hazard ratios for hearing loss according to patient- and surgery-specific factors. RESULTS: Thirty ears in 29 patients underwent hybrid cochlear implantation and were eligible for inclusion (mean age, 59 yr; 65% female). Mean preoperative LFPTA was 31.7 dB. Mean LFPTA across all implanted ears at first follow-up was 45.1 dB; no patient had experienced loss of residual hearing at first follow-up. Six patients had loss of residual hearing during the follow-up period, with Kaplan-Meier probability estimates of preserved hearing of 100% at 1 month, 90% at 12 months, 87% at 24 months, and 80% at 48 months. There was no association between loss of residual hearing and patient age, preoperative LFPTA, surgeon, or use of topical steroids intraoperatively (hazard ratios, 1.05 [0.96-1.15], 0.97 [0.88-1.05], 1.39 [0.20-9.46], 0.93 [0.09-9.74], respectively). CONCLUSIONS: Long-term (>5 yr) outcomes after hybrid cochlear implantation demonstrate good preservation of low-frequency hearing, with only modest decline in the long-term postimplantation, and a low proportion of loss of residual low-frequency hearing.

Socioeconomic Status Among Cochlear Implant Candidates and Association With Surgical Pursuance.

Importance: Despite the sizeable global burden of hearing loss, cochlear implants have poor penetrance among eligible hearing-impaired individuals. Identifying populations who may benefit from a cochlear implant but among whom penetrance is poor is an important aim in reducing the health-related and economic effects of hearing loss on both individuals and society. Objectives: To explore the association of socioeconomic status (SES) with cochlear implant candidacy and the decision to undergo cochlear implantation. Design, Setting, and Participants: This retrospective cohort study was performed in a tertiary academic center. All adult patients evaluated for cochlear implant candidacy from January 1, 1999, through December 31, 2022, were included in the analysis. Exposures: Household income quintile and rural or urban residence were used as proxies for SES based on zip code linkage to US Census and US Department of Agriculture data. Main Outcomes and Measures: Odds of cochlear implant candidacy and surgery. Results: A total of 754 individuals underwent candidacy evaluations and were included in the analysis (386 [51.2%] women; mean [SD] age, 64.0 [15.7] years). Of these, 693 (91.9%) were cochlear implant candidates, and 623 candidates (89.9%) underwent cochlear implantation. Multivariable analyses demonstrated that individuals in the highest income quintile had lower odds of cochlear implant candidacy compared with those in the lowest income quintile (odds ratio [OR], 0.26 [95% CI, 0.08-0.91]), and candidates in the highest income quintile had greater odds of undergoing cochlear implant surgery compared with those in the lowest quintile (OR, 2.59 [95% CI, 1.14-5.86]). Living in a small town or a micropolitan or rural area was associated with lower odds of undergoing cochlear implant surgery compared with living in a metropolitan core (OR, 0.18 [95% CI, 0.04-0.83]) after controlling for distance to the primary implant center. Conclusions and Relevance: The findings of this cohort study suggest that individuals with higher SES are less likely to qualify for a cochlear implant; however, those who qualify are more likely to undergo surgery compared with those with lower SES. These findings highlight a hearing health care disparity that should be addressed through further studies to guide population-based initiatives.

Diagnostic Yield and Utility of Radiographic Imaging in the Evaluation of Pulsatile Tinnitus: A Systematic Review.

Objective: The objective of this study is to assess diagnostic yield of imaging modalities used to evaluate patients presenting with pulsatile tinnitus (PT). Databases Reviewed: PubMed, Embase, and Scopus were queried using the search terms "pulsatile tinnitus," "pulse-synchronous tinnitus," and "pulse synchronous tinnitus" with no date limitations. Methods: Studies that reported diagnostic imaging for patients presenting with PT were included. Data were reviewed for sample size, gender, age, imaging study, indications, and diagnoses. The primary outcome measure from aggregated data was the yield of positive diagnoses made with each imaging modality. The quality of evidence was assessed for risk of bias. Results: From an initial search of 1145 articles, 17 manuscripts met inclusion criteria, of which 12 studies evaluated individual imaging modalities. The number of unique patients included was 1232. The diagnostic yield varied between modalities: carotid ultrasound (21%, 95% confidence interval [CI]: 12%-35%), CT temporal bone (65%, CI: 20%-93%), computed tomographic angiography (86%, CI: 80%-90%), and MRI/magnetic resonance angiography (58%, CI: 43%-72%). Conclusion: Studies on the diagnostic approach to PT are limited by heterogeneity in both inclusion criteria and reporting standards. A wide range of imaging modalities are used in practice during the initial evaluation of PT, and the diagnostic yield for imaging can be improved by utilizing more specific clinical indications.

Lateral Skull Base Chordoma Mimicking a Paraganglioma.

We present an unusual case of chordoma arising entirely from the lateral skull base with imaging features suggestive of a paraganglioma. Clinical history, management, histopathology, and imaging characteristics are described, including a review of gallium-dotate PET scanning somatostatinreceptor-positive tumors. We further provide a review of management options, including a summary of our approach with surgical biopsy via retrosigmoid and resection via transtemporal approaches. Based on radiologic characteristics and location, lateral skull base chordoma may arise with isolated lateral skull base involvement and has the potential to be misidentified as a glomus jugulare on initial workup.

Audiometric and Surgical Outcomes of a Novel Bone-Conduction Hearing Aid.

OBJECTIVE: To report the audiometric and surgical outcomes of a series of patients having undergone implantation of a novel transcutaneous bone conduction implant (t-BCI). STUDY DESIGN: Retrospective case series. SETTING: Single academic tertiary referral center. PATIENTS: Adults (≥18 yr) implanted between December 1, 2019, and August 1, 2021, with audiometric data available before and after device implantation and a minimum of 4 weeks follow-up. INTERVENTIONS: Surgical t-BCI. MAIN OUTCOME MEASURES: Change in aided pure tone average (PTA) after implantation. Secondary outcomes include average operative time, and adverse events. RESULTS: Twenty-three patients underwent implantation of the t-BCI via either a conventional or minimally invasive surgical approach. The most common indication for implantation was unilateral conductive hearing loss with a history of chronic otitis media. The mean operative time was 59 minutes. The mean preimplantation unaided air conduction PTA was 65 dB, and mean postimplantation was 27.2 dB. The mean change in PTA was 37.8 dB, which was significant ( p < 0.0001). There were 30.4% of the patients that suffered from adverse events, the most common of which were pain (8.7%) and device-related complications (13%). One major adverse event occurred, involving magnet displacement that impaired device activation and required reoperation for replacement. CONCLUSION: Forming the largest series of patients implanted with this t-BCI in the published literature, our data demonstrate that implantation of the device is feasible via either a traditional or minimally invasive surgical approach, with good audiometric benefit and a favorable safety profile.

Comparison of Outcomes of Surgical Repair of Spontaneous Temporal Bone CSF Leaks and Encephaloceles Using Bone Cement and Autologous Material.

OBJECTIVE: To compare outcomes transmastoid repair of spontaneous middle fossa cerebrospinal fluid (CSF) leak using only bone cement (BC) versus only autologous material (AM) or combined materials (CM) with both bone cement and autologous material. STUDY DESIGN: Retrospective Chart Review. SETTING: Tertiary Care Hospital. PATIENTS: Forty-three adult patients undergoing transmastoid repair of spontaneous middle fossa CSF leak between 2014 and 2020 (BC:12, AM:15, CR:16). INTERVENTIONS: Cortical mastoidectomy, identification of defect, and repair with BC (Cranios® hydroxyapatite), AM (local bone, fascia, fat, and/or cartilage), or CM (Cranios® combined with autologous materials). MAIN OUTCOME MEASURES: Successful repair without recurrent CSF leak or encephalocele throughout follow up. RESULTS: Fifty-one percent of subjects were female. Mean age at repair was 58.6 years (SD 10.9). Mean BMI was 35.4 (SD 7.6; BC:36.3, AM:36.5, CM:33.6). Forty (93%) patients had successful repair without known recurrent CSF leak or encephalocele since surgery (BC:11, 91.6%; AM:14, 93.3%; CM:15, 93.8%; p = 0.49) over a mean length of follow up of 49.6 months (BC: 37.9, AM: 59.2, CR: 49.5). The difference in mean operative time amongst the groups was faster for patients using bone cement (BC: 100.2 min, AM: 182.8, CM: 133.2; p < 0.00001). CONCLUSIONS: BC, AM, and CM techniques each demonstrate effective and sustained means of repair for middle fossa CSF leak and encephalocele, even in the presence of multiple defects. Use of isolated BC offers a significant decrease in operative time with a noninferior outcome. Active CSF leak at the time of surgery is associated with increased risk of recurrence.

Rates of Sensorineural Hearing Loss and Revision Surgery After Stapedotomy: A Single-institution Experience Using the Nitinol Prosthesis.

Background: Historically, stapedectomy complication rates are quoted as 1% profound postoperative sensorineural hearing loss (SNHL), 5%-10% nonprofound SNHL, and 5%-10% revision surgery. Objective: We sought to reassess rates of post-stapedotomy complications based on our experience using contemporary surgical technique. Methods: A retrospective case series was carried out at an academic tertiary referral center. Adult patients undergoing stapedotomy from 2013 to 2020 were included. Primary outcomes were rates of hearing loss and revision surgery. Rates of dizziness, tinnitus, dysgeusia, and proportions of patients who achieved air-bone gap (ABG) closure at 8-12 weeks postoperatively were also assessed. Results: Four hundred sixty-eight stapedotomies in 399 patients with a median follow-up duration of 99 days (range, 11-5134) were reviewed. One patient (0.21%) suffered profound SHNL and 15 (3.20%) patients suffered nonprofound SNHL. The revision rate for stapedotomies from our institution was 4.49% (21 total revision surgeries). In 277 operations (59.19%), the patient had closure of the ABG within 10 dB. A further 132 (28.21%) had closure of the ABG between 10 and 20 dB. Air pure-tone audiometry scores improved by an average of 25.03 dB. Eighty-three (17.74%) patients complained of postoperative dizziness, which resolved by the time of the first follow-up appointment in all but 26 (5.56%). Seventeen patients (3.63%) complained of tinnitus, and 22 (4.70%) complained of dysgeusia. Conclusions: SNHL, complications, and revision rates for stapedotomy in the modern era may be substantially lower than those currently presented to patients based on classic techniques and historical data.

Profound Sudden Sensorineural Hearing Loss in Hematologic Malignancy: A Case for Urgent Cochlear Implantation With Discussion and Systematic Review of the Literature.

OBJECTIVE: To perform a systematic review of sensorineural hearing loss (SNHL) in hematologic malignancy; to describe an illustrative case of urgent cochlear implantation for bilateral profound SNHL and vestibular hypofunction in hyperviscosity syndrome; to suggest an approach to management of hyperviscosity syndrome-associated deafness with cochlear implantation. DATA SOURCES: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, systematic search of PubMed and Embase databases was undertaken for articles detailing clinical information about SNHL caused directly by hematologic malignancies. RESULTS: A total of 37 studies from 1989 to 2020 were qualitatively reviewed, the majority of which were case studies or case series. Causes of hearing loss in hematologic malignancy were found to include hyperviscosity syndrome, labyrinthine hemorrhage, infiltration, and infection. Patients with profound SNHL in hematologic malignancies from hyperviscosity syndrome may be candidates for cochlear implantation, and are also at increased risk for cochlear ossification. We review previous cases for their diagnostic approach, treatment paradigm, and outcomes data, and propose an approach to management. CONCLUSION: Bilateral sudden profound SNHL and vestibular hypofunction is a presenting symptom of hyperviscosity syndrome in hematologic malignancy. Although this entity is rare and previous reports have suggested improvement in hearing with oncologic treatment, cases with profound hearing loss are unlikely to recover serviceable hearing. We advocate for early magnetic resonance imaging with attention to fluid signal in the inner ear and serial audiometric follow-up to guide clinical decisions. We advise early consideration for cochlear implantation.

Failure and Revision Surgery After Cochlear Implantation in the Adult Population: A 10-year Single-institution Retrospective and Systematic Review of the Literature.

OBJECTIVE: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure. STUDY DESIGN: Retrospective chart review and systematic review of the literature using PubMed and Embase. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Four hundred ninety-eight devices in 439 distinct adult patients. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Implant failure rate and etiology. RESULTS: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9-77.1 vs mean 47.6 yr, CI: 39.9-55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful.In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%). CONCLUSIONS: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation.

Predictors of Postoperative Electrode Deactivation Among Adult Cochlear Implantees.

OBJECTIVE: To characterize postoperative electrode functionality after adult cochlear implantation; to identify rationale and risk factors for electrode deactivation. STUDY DESIGN: Retrospective Chart Review. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Five hundred nineteen cochlear implants in 433 adult patients over 5 years. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Rate of electrode deactivation after adult cochlear implantation. RESULTS: One hundred twenty (27.7%) patients experienced electrode deactivation postoperatively, involving a total of 447 electrodes. The most common reasons for deactivation were bothersome nonauditory symptoms (n = 170, 38.0%), perceived benefit by patients (n = 64, 14.3%), and bothersome auditory symptoms (n = 60, 13.4%). Four hundred nineteen (93.7%) of involved electrodes remained deactivated at most recent follow-up, whereas 28 (6.3%) were able to be reactivated. Deactivation was most likely to occur within the first 4 weeks after activation (n = 90 patients,75.0%; p < 0.01). Among affected patients, the average number of electrodes deactivated was 3.44 (range 1-13; SD 2.50). Age was not associated with electrode deactivation. CONCLUSIONS: While 98% of cochlear implants had full insertions, more than a quarter of implantees may experience electrode deactivation postoperatively for a multitude of reasons, with bothersome nonauditory symptoms most prevalent. Deactivation of five or more electrodes and simultaneous deactivation of two or three electrodes seems to increase the odds of subsequent device failure. However, deactivation encompasses a wide range of issues that likely include patient factors, surgical technique, and device-specific issues. Prognosis varies greatly at the individual level and further evaluation is required to better identify the issues underlying deactivation and identify true predictors of failure.

Impact of Reconstruction With Hydroxyapatite Bone Cement on CSF Leak Rate in Retrosigmoid Approach to Vestibular Schwannoma Resection: A Review of 196 Cases.

OBJECTIVE: To assess the impact of reconstructive technique on the incidence of cerebrospinal fluid (CSF) leak following retrosigmoid approach to acoustic neuroma resection. STUDY DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: A total of 1,200 patients with acoustic neuromas presented to our institution from 2005 to 2018. Of these, 196 patients underwent surgical resection via a retrosigmoid approach. INTERVENTION: At our institution, internal auditory canal (IAC) reconstruction following a retrosigmoid approach was performed with bone wax and muscle plug or Norian hydroxyapatite bone cement from 2005 to 2013. Starting in 2014, a newer model of bone cement, Cranios hydroxyapatite, was used exclusively for reconstruction. MAIN OUTCOME MEASURES: Rates of CSF leak were evaluated across different methods of IAC reconstruction and types of bone cement. Patients whose leaks were attributable to the craniectomy site were excluded from analysis. RESULTS: The postoperative CSF leak rate among patients who did not receive bone cement for IAC reconstruction was 15.6% (n.5). The leak rate amongst patients who received Norian bone cement was 6.3% (n.4). After introduction of Cranios bone cement, the total leak rate decreased to 1% (n.1). Compared with all other types of closure, Cranios had a significantly reduced rate of postoperative CSF leak (p < 0.005). The leak rate following Cranios versus Norian was also significantly reduced (p < 0.05). Leak rate was not affected by tumor size (p.0.30) or age (p.0.43). CONCLUSION: CSF leak rate following acoustic neuroma resection was significantly reduced by introduction of Cranios hydroxyapatite bone cement.