Cochlear Implant Revisions Over Three Decades of Experience.

IMPORTANCE: The indications, technology, and surgical technique for cochlear implantation have evolved over the last three decades. Understanding the risk of cochlear implant revision (CIR) is important for patient counseling. OBJECTIVE: The objective of this study was to analyze the rates, indications, and audiologic outcomes for CIR over three decades of experience at a single academic medical center. DESIGN: A retrospective chart review was performed at a single academic medical center for individuals who underwent cochlear implantation between 1985 and 2022. SETTING: Single academic medical center. PARTICIPANTS: Three thousand twenty-five individuals who underwent 3,934 cochlear implant operations from 1985 to 2022. EXPOSURE: Cochlear implantation. MAIN OUTCOMES AND MEASURES: Rates, indications, risk factors, and audiologic outcomes for CIR. RESULTS: There were 276 cases of CIR after primary implantation and an overall revision rate of 7.6% (95% confidence interval, 6.8-8.5%) over 37 years of follow-up with many cases of CIR secondary to Advanced Bionics vendor B and field action failure groups. CIR rates increased sharply through the early and mid-2000s and have since remained stable. Hard or soft device failure was the most common indication for CIR, accounting for 73% of cases. Pediatric patient status and previous CIR were associated with an increased risk of CIR. Audiologic outcomes after CIR were similar to those before device failure. CONCLUSIONS AND RELEVANCE: CIR remains a common procedure most often performed for device failure. Pediatric patients and those who have undergone previous CIR are at the highest risk for future CIR. Audiologic outcomes remain stable after CIR, and these data will help providers counsel patients at the risk of future CIR and understand the risk factors associated with CIR.

Outcomes after revision of Advanced Bionics Clarion 1.2 cochlear implants.

INTRODUCTION: Cochlear implant reimplantation (CIR) for external processor upgrade or device failure is becoming increasingly common as the population of cochlear implant recipients ages. Patients with Advanced Bionics (AB) Clarion 1.2 cochlear implants may undergo CIR for device age/failure or desired technology upgrade so that they may use newer external processors that have improved connectivity features. The objective of this study was to evaluate audiologic outcomes for patients who were initially implanted with an AB Clarion 1.2 internal device and underwent CIR for technology upgrade or device failure. METHODS: Retrospective chart review was performed at a single academic medical center for patients (pediatric and adult) with an AB Clarion 1.2 internal device who underwent CIR to a later generation AB internal device and had available audiologic data. RESULTS: Forty-eight individuals with a Clarion 1.2 implant underwent CIR. Pre- and post-CIR speech understanding did not change for AzBio (p-value = 0.11, mean change = 12.1%, 95% CI = -2.9-27.2%), CNCw (p-value = 0.74, mean change = -1%, 95% CI = -10.4-12.4%), or HINT (p-value = 0.12, mean change = 19.9%, 95% CI = -2.6-42.4%) scores. Pure-tone averages improved following CIR (p-value < 0.01, mean change = 4.3 dB, 95% CI = 1.5-7.1 dB). CONCLUSIONS: Revision of AB Clarion 1.2 cochlear implants does not significantly worsen audiologic outcomes and may improve hearing in some individuals, but individual patient-level outcomes are variable.

Assessment of Sudden Sensorineural Hearing Loss After COVID-19 Vaccination.

IMPORTANCE: Emerging reports of sudden sensorineural hearing loss (SSNHL) after COVID-19 vaccination within the otolaryngological community and the public have raised concern about a possible association between COVID-19 vaccination and the development of SSNHL. OBJECTIVE: To examine the potential association between COVID-19 vaccination and SSNHL. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study and case series involved an up-to-date population-based analysis of 555 incident reports of probable SSNHL in the Centers for Disease Control and Prevention Vaccine Adverse Events Reporting System (VAERS) over the first 7 months of the US vaccination campaign (December 14, 2020, through July 16, 2021). In addition, data from a multi-institutional retrospective case series of 21 patients who developed SSNHL after COVID-19 vaccination were analyzed. The study included all adults experiencing SSNHL within 3 weeks of COVID-19 vaccination who submitted reports to VAERS and consecutive adult patients presenting to 2 tertiary care centers and 1 community practice in the US who were diagnosed with SSNHL within 3 weeks of COVID-19 vaccination. EXPOSURES: Receipt of a COVID-19 vaccine produced by any of the 3 vaccine manufacturers (Pfizer-BioNTech, Moderna, or Janssen/Johnson & Johnson) used in the US. MAIN OUTCOMES AND MEASURES: Incidence of reports of SSNHL after COVID-19 vaccination recorded in VAERS and clinical characteristics of adult patients presenting with SSNHL after COVID-19 vaccination. RESULTS: A total of 555 incident reports in VAERS (mean patient age, 54 years [range, 15-93 years]; 305 women [55.0%]; data on race and ethnicity not available in VAERS) met the definition of probable SSNHL (mean time to onset, 6 days [range, 0-21 days]) over the period investigated, representing an annualized incidence estimate of 0.6 to 28.0 cases of SSNHL per 100 000 people per year. The rate of incident reports of SSNHL was similar across all 3 vaccine manufacturers (0.16 cases per 100 000 doses for both Pfizer-BioNTech and Moderna vaccines, and 0.22 cases per 100 000 doses for Janssen/Johnson & Johnson vaccine). The case series included 21 patients (mean age, 61 years [range, 23-92 years]; 13 women [61.9%]) with SSNHL, with a mean time to onset of 6 days (range, 0-15 days). Patients were heterogeneous with respect to clinical and demographic characteristics. Preexisting autoimmune disease was present in 6 patients (28.6%). Of the 14 patients with posttreatment audiometric data, 8 (57.1%) experienced improvement after receiving treatment. One patient experienced SSNHL 14 days after receiving each dose of the Pfizer-BioNTech vaccine. CONCLUSIONS AND RELEVANCE: In this cross-sectional study, findings from an updated analysis of VAERS data and a case series of patients who experienced SSNHL after COVID-19 vaccination did not suggest an association between COVID-19 vaccination and an increased incidence of hearing loss compared with the expected incidence in the general population.

Catchment Profile of Large Cochlear Implant Centers in the United States.

OBJECTIVE: To characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States. STUDY DESIGN: Multi-institutional retrospective case series. SETTING: Tertiary referral CI centers. METHODS: Patients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients' residential zip codes were used to approximate travel distances and urban vs rural residential areas. RESULTS: Over the 6-year study period (2015-2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21-110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P < .001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P < .001). Overall, 91% of patients lived within a 200-mile radius of the institution, while 71% lived within a 100-mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P < .001, r2 = 0.2). CONCLUSIONS: While large CI centers serve geographically dispersed populations, most patients reside within a 200-mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

Perigeniculate arachnoid cysts and CSF fistulae of the fallopian canal: Histopathologic correlates of a rare clinical entity.

Cerebrospinal fluid (CSF) fistulae originating from the fallopian canal of the facial nerve is hypothesized to arise due to atypical patterns of subarachnoid space extension into the geniculate ganglion or more distal regions along the intratemporal course of the facial nerve, but its pathogenesis remains poorly understood. Although a rare etiology of CSF fistulae of the temporal bone, there are significant clinical ramifications due to the risk of recurrent meningitis, difficulty in identifying the anatomic location of the CSF leak, and technical challenges associated with surgical repair. We present three clinical cases of arachnoid cysts within the geniculate fossa with or without CSF fistulization and provide histopathologic correlates of this rare clinical phenomenon from a human temporal bone collection. The pediatric and adult patients presented suggest differential pathophysiologic mechanisms associated with CSF fistulae. Temporal bone histology reveals atypical patterns of subarachnoid space extension in the fallopian canal that may underlie arachnoid cyst formation and overt CSF leak from the geniculate region.

Immune-mediated ototoxicity associated with immune checkpoint inhibitors in patients with melanoma.

Immune checkpoint inhibitors (ICIs) are associated with a range of organ-specific toxicities known as immune-related adverse events (irAEs). Immune-mediated ototoxicity from ICIs is poorly described. Herein, we describe the clinical presentation, diagnostic evaluation and management of six ICI-treated patients who developed immune-mediated ototoxicity, identified by a multidisciplinary immune-related toxicity team. This is the largest case series to date and identifies bilateral high-frequency hearing loss and tinnitus as the most common reported symptoms and can be associated with abnormal speech reception thresholds and word recognition ability on audiogram in select patients. We propose multidisciplinary evaluation of patients with suspected otologic irAEs including referral to otolaryngology, audiometry evaluation±magnetic resonance imaging for evaluation of suspected immune-mediated ototoxicity.

Pilot Evaluation of Sheep as In Vivo Model for Cochlear Implantation.

OBJECTIVES: The rise in the use of cochlear implants (CIs) has continued to fuel research aimed at improving surgical approaches and the preservation of residual hearing. Current in vivo models involve small animals not suitable for evaluating full-sized CIs nor are prohibitively expensive nonhuman primates. The objective of this study was to develop and evaluate an in vivo model of cochlear implantation in sheep. METHODS: Eight adult, female sheep were implanted with full-sized CIs from three manufacturers using a retrofacial approach to the round window. Partial electrode insertions were performed to a depth of 10 to 12 mm before closure. Round window electrocochleography (ECoG) and auditory brainstem responses (ABR) were conducted during and after surgery. Following a 30-day implantation, cochleae were explanted and imaged using both x-ray microscopy and histology. RESULTS: The surgery was well tolerated although limited complications were observed in three of eight sheep. Electrode insertions were up to 12 mm before insertion resistance noted. ECoG and ABR responses were reduced postimplantation, reflecting changes in cochlear mechanics due to the presence of the implant, and/or insertion trauma. Histological and radiological image analysis showed the presence of intracochlear fibrosis as well as one instance of tip fold-over. CONCLUSIONS: The use of sheep presents a feasible live-animal model to study cochlear implantations. Full-sized implants as well as surgical techniques can be evaluated on functional outcomes such as ABR and ECoG as well as histological markers for residual hearing including intracochlear fibrosis. Use of this model and surgical approach has potential to evaluate CIs and surgical techniques in both the acute and chronic setting.

Hospital volume and failure to rescue after vestibular schwannoma resection.

BACKGROUND: Complication rates in many complex surgical procedures are associated with the volume of procedures performed. OBJECTIVES: To investigate the relationship between hospital volume and complications, mortality, and failure to rescue (FTR) rates in patients undergoing vestibular schwannoma (VS) surgery. DESIGN, SETTING, AND PARTICIPANTS: The Nationwide Inpatient Sample was used to identify 44,336 patients who underwent VS surgery in 1995-2011. Annual case volumes were stratified by quintiles and defined as very low (≤5 cases/year), low (6-12 cases/year) medium (13-22 cases/year), high (23-37 cases/year), and very high-volume (≥38 cases/year). MAIN OUTCOMES AND MEASURES: Relationships between hospital volume and in-hospital mortality, postoperative complications, as well as FTR rates, defined as death after a major complication, were examined using multivariate regression analysis. RESULTS: Postoperative medical and surgical complications occurred in 5.4% and 14.6% of cases, respectively, and did not differ significantly across volume quintiles. In-hospital mortality decreased with increasing hospital volume, with an incidence of 1.4% for hospitals in the lowest volume quintile compared to 0.1% for hospitals in the top volume quintile. After controlling for all other variables, the odds of in-hospital mortality were lower for medium (OR = 0.19 [0.04-0.93]) and very high-volume hospitals (OR = 0.07 [0.01-0.53]), but not high-volume hospitals (OR = 0.43 [0.05-3.77]). There was no association between hospital volume and the odds of postoperative surgical complications. FTR was associated with hospital volume, with decreasing odds for medium-volume (OR = 0.15 [0.02-0.93]), high-volume (OR = 0.17 [0.04-0.74]), and very high-volume (OR = 0.07 [0.04-0.74]) hospitals. CONCLUSIONS: Hospital volume does not appear to be associated with complication rates but is associated with decreased likelihood of FTR after VS surgery. LEVEL OF EVIDENCE: NA Laryngoscope, 130:1287-1293, 2020.

Temporal bone carcinoma: Treatment patterns and survival.

OBJECTIVES/HYPOTHESIS: Carcinomas of the temporal bone are rare, and appropriate treatment, staging, and survival data are limited. This study evaluates clinical characteristics and survival rates for patients with temporal bone carcinoma treated with resection at a single tertiary-care institution, with a focus on the outcomes of patients with locally advanced disease including skull base and/or dural invasion. STUDY DESIGN: Retrospective chart review. METHODS: Demographic, tumor-specific, and survival data were collected for patients with primary carcinomas of the external auditory canal with involvement of the temporal bone from 2003 to 2015. All patients were staged according to the modified Pittsburgh system. Kaplan-Meier and logistic regression analysis were used to calculate factor-specific survival outcomes. RESULTS: Sixty-seven patients met inclusion criteria; 85% were male. There were 43 squamous cell carcinomas (64%) and 24 basal cell carcinomas (BCCs) (36%). Tumor stage was 24 (36%) T2, 12 (18%) T3, and 31 (46%) T4 tumors; 53% had recurrent disease. Surgical management included 49 lateral temporal bone resections and 18 subtotal temporal bone resections. Kaplan-Meier analyses revealed more favorable 5-year survival rates associated with BCC histology (P = .01), lateral temporal bone resection compared to subtotal temporal bone resection (P < .01), lack of immunocompromise (P = .04), and absence of perineural/lymphovascular invasion (P = .01). Multivariate regression analysis did not yield statistically significant results. CONCLUSIONS: Factors predictive of more favorable survival include lack of immunocompromise, BCC histology, absence of perineural/lymphovascular invasion, and disease extent amenable to lateral temporal bone resection. Dural invasion is not an absolute contraindication to surgery, with a subset of patients surviving >5 years. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:E11-E20, 2020.

Relationship of a "Fundal Fluid Cap" and Vestibular Schwannoma Volume: Analysis of Preoperative Radiographic Findings and Outcomes.

OBJECTIVE: To evaluate the relationship between radiographic fundal fluid cap in the lateral internal auditory canal, preoperative clinical characteristics, and postoperative outcomes in patients with vestibular schwannoma who underwent microsurgical excision. STUDY DESIGN: Retrospective chart review. SETTING: Academic tertiary referral center. PATIENTS: Thirty-six consecutive patients (mean age 49.4 yr [range 29-74]) who underwent microsurgical vestibular schwannoma excision. INTERVENTIONS: Microsurgical excision. MAIN OUTCOME MEASURES: Linear fundal fluid size and tumor size calculated using volumetric analysis were measured on preoperative magnetic resonance imaging, and correlated to hearing status and postoperative facial nerve function. RESULTS: Mean fundal fluid size was 2.18 mm (range 0-7.32). Mean tumor volume was 5.58 cm (range, 0.210-40.3 cm). Short- and long-term postoperative House-Brackmann scores were 2.4 and 1.4, respectively. Fundal fluid size was associated with tumor volume (rs = 0.488, p = 0.003) but not preoperative hearing status (p = 0.333). The presence of fundal fluid and larger tumor volumes were statistically associated with poorer short-term and long-term postoperative facial nerve function (p < 0.05). CONCLUSIONS: Radiographic fundal fluid size is correlated to tumor volume.

Facial nerve decompression.

PURPOSE OF REVIEW: Facial nerve paralysis is a debilitating condition. Bell's palsy and temporal bone trauma are common causes of acute facial palsy, with recurrent idiopathic paralysis and Melkersson-Rosenthal syndrome accounting for a smaller subset of cases. Properly selected patients may benefit from facial nerve decompression. This article will review the relevant literature on facial nerve decompression. RECENT FINDINGS: The middle cranial fossa approach provides access to the primary site of lesion in Bell's palsy while preserving hearing. Patients with complete facial paralysis secondary to Bell's palsy or temporal bone trauma, more than 90% degeneration on electroneurography testing, and absent voluntary electromyography within 14 days of onset may benefit from facial nerve decompression. Facial nerve decompression may prevent future occurrences of recurrent forms of facial nerve paralysis. The return of facial nerve function following decompression will occur over weeks to months. SUMMARY: Appropriately selected patients with facial paralysis secondary to Bell's palsy or temporal bone trauma may benefit from facial nerve decompression. Patients should be counseled regarding the risks of decompression and that the return of maximal facial nerve function may be delayed up to 12 months.

Does a "Fundal Fluid Cap" Predict Successful Hearing Preservation in Vestibular Schwannoma Resections Via the Middle Cranial Fossa Approach?

OBJECTIVE: To determine the association between radiographic cerebrospinal fluid (CSF) cap in the lateral internal auditory canal (IAC) and likelihood of successful hearing preservation in middle cranial fossa (MCF) vestibular schwannoma (VS) surgery. STUDY DESIGN: Retrospective chart review. SETTING: Academic tertiary referral center. PATIENTS: One hundred thirty-eight consecutive patients (mean age/standard deviation, SD, 50/11 yr) who underwent MCF VS resection. INTERVENTIONS: MCF VS excision. MAIN OUTCOME MEASURES: Size of fundal fluid cap on preoperative magnetic resonance imaging (MRI), pre- and postoperative pure-tone average (PTA), and word recognition score (WRS). RESULTS: Mean tumor and fundal fluid sizes were 9.7 mm (SD, 3.9 mm) and 2.8 mm (SD, 1.7 mm), respectively. On bivariate analysis, fundal fluid size was associated with larger tumor size (p = 0.005) but not changes in postoperative PTA (p = 0.45) or WRS (p = 0.17). When fundal fluid size was stratified as none (<1 mm), small (≥1 mm and <4 mm), and large (≥4 mm), no significant differences were seen in rates of hearing preservation. Using multivariate linear regression models adjusting for patient age, sex, tumor nerve of origin, neurofibromatosis type II status, and preoperative PTA and WRS, superior vestibular nerve tumor origin but not increasing fundal fluid size was associated with preserved postoperative PTA or WRS. CONCLUSIONS: Presence or size of CSF fluid cap may not be a reliable prognostic indicator for hearing preservation in MCF VS resection, with important implications for patient counseling.

How Well Does Intraoperative Audiologic Monitoring Predict Hearing Outcome During Middle Fossa Vestibular Schwannoma Resection?

OBJECTIVE: To evaluate the association between signal changes during intraoperative audiologic monitoring and postoperative audiometric outcome in patients undergoing vestibular schwannoma (VS) resection via middle cranial fossa (MCF) approach. STUDY DESIGN: Retrospective chart review. SETTING: Academic tertiary referral center. PATIENTS: One hundred twenty-six consecutive patients (mean age 48.6 yrs, range 16-67; mean tumor size 9.9 mm, range 1.8-18.9 mm) who underwent MCF VS resection. INTERVENTIONS: Intraoperative audiologic monitoring using auditory brainstem response (ABR) and direct cochlear nerve action potential (CNAP). MAIN OUTCOME MEASURES: Intraoperative ABR wave V and CNAP N1 amplitudes and postoperative pure-tone average (PTA) and word recognition score (WRS). RESULTS: On ABR, absent wave V amplitude was associated with an 81.1% increase and 82.3% decrease in postoperative PTA and WRS, respectively. On CNAP, decreased or absent N1 amplitude was associated with 47.3 and 100% increase, respectively, in postoperative PTA, and 45.3% and 100% decrease, respectively, in postoperative WRS. Receiver-operating curve analysis showed that ABR combined with CNAP using a Cueva electrode achieved the highest diagnostic accuracy in predicting postoperative hearing decline when measured by WRS class (sensitivity 83.3%, specificity 100%), and performed better compared with each modality alone. CONCLUSION: Intraoperative ABR wave V and CNAP amplitude changes are associated with changes in postoperative hearing, and dual modality monitoring was more diagnostic of postoperative hearing decline compared with each modality alone during MCF VS resection. Overall, intraoperative ABR and CNAP were more specific than sensitive for postoperative hearing decline.

Surgical Management of Tumors Involving Meckel's Cave and Cavernous Sinus: Role of an Extended Middle Fossa and Lateral Sphenoidectomy Approach.

OBJECTIVE: To study the indications and outcomes of lateral sphenoidectomy as part of a combined skull base approach in the treatment of tumors involving Meckel's cave (MC) and cavernous sinus (CS). STUDY DESIGN: Retrospective patient series. SETTING: Tertiary referral center. PATIENTS: Twenty-two consecutive patients (mean age: 45 yr, range: 16-76) who underwent transzygomatic, extended middle fossa approaches for tumors involving MC and CS. INTERVENTIONS: Surgical access to MC and CS was achieved via extended middle fossa, trans-clinoid approach. Lateral sphenoidectomy was defined as drill-out of the greater sphenoid wing lateral to foramen rotundum and ovale, decompression of superior orbital fissure, and removal of anterior clinoid process. Reconstruction was achieved using combination of autologous and synthetic materials. Eleven patients (50%) received adjuvant radiation. MAIN OUTCOME MEASURES AND RESULTS: Tumor pathologies included meningioma (16 patients), epidermoid cyst (2), trigeminal schwannoma (2), invasive pituitary adenoma (1), and chondrosarcoma (1). Mean (range) preoperative tumor size was 4.0 cm (1.3-9). Mean (range) length of follow-up was 4 years (range 0.1-10). Overall tumor control and gross total resection were achieved in 95 and 23% of patients, respectively. Lateral sphenoidectomy was performed in 16 patients (73%) for enhanced surgical access and/or tumor extension to the infratemporal fossa (6 patients). Postoperatively, cranial nerve deficits occurred in 12 (55%) patients (V-9 patients; III, IV, or VI-4; VII-2; VIII-2). Cerebrospinal fluid leak and hydrocephalus occurred in two and four patients, respectively. CONCLUSION: In combination with middle fossa-based approaches to tumors involving MC and CS, lateral sphenoidectomy may play a viable role in tumor access and control.

Rhesus Cochlear and Vestibular Functions Are Preserved After Inner Ear Injection of Saline Volume Sufficient for Gene Therapy Delivery.

Sensorineural losses of hearing and vestibular sensation due to hair cell dysfunction are among the most common disabilities. Recent preclinical research demonstrates that treatment of the inner ear with a variety of compounds, including gene therapy agents, may elicit regeneration and/or repair of hair cells in animals exposed to ototoxic medications or other insults to the inner ear. Delivery of gene therapy may also offer a means for treatment of hereditary hearing loss. However, injection of a fluid volume sufficient to deliver an adequate dose of a pharmacologic agent could, in theory, cause inner ear trauma that compromises functional outcome. The primary goal of the present study was to assess that risk in rhesus monkeys, which closely approximates humans with regard to middle and inner ear anatomy. Secondary goals were to identify the best delivery route into the primate ear from among two common surgical approaches (i.e., via an oval window stapedotomy and via the round window) and to determine the relative volumes of rhesus, rodent, and human labyrinths for extrapolation of results to other species. We measured hearing and vestibular functions before and 2, 4, and 8 weeks after unilateral injection of phosphate-buffered saline vehicle (PBSV) into the perilymphatic space of normal rhesus monkeys at volumes sufficient to deliver an atoh1 gene therapy vector. To isolate effects of injection, PBSV without vector was used. Assays included behavioral observation, auditory brainstem responses, distortion product otoacoustic emissions, and scleral coil measurement of vestibulo-ocular reflexes during whole-body rotation in darkness. Three groups (N = 3 each) were studied. Group A received a 10 µL transmastoid/trans-stapes injection via a laser stapedotomy. Group B received a 10 µL transmastoid/trans-round window injection. Group C received a 30 µL transmastoid/trans-round window injection. We also measured inner ear fluid space volume via 3D reconstruction of computed tomography (CT) images of adult C57BL6 mouse, rat, rhesus macaque, and human temporal bones (N = 3 each). Injection was well tolerated by all animals, with eight of nine exhibiting no signs of disequilibrium and one animal exhibiting transient disequilibrium that resolved spontaneously by 24 h after surgery. Physiologic results at the final, 8-week post-injection measurement showed that injection was well tolerated. Compared to its pretreatment values, no treated ear's ABR threshold had worsened by more than 5 dB at any stimulus frequency; distortion product otoacoustic emissions remained detectable above the noise floor for every treated ear (mean, SD and maximum deviation from baseline: -1.3, 9.0, and -18 dB, respectively); and no animal exhibited a reduction of more than 3 % in vestibulo-ocular reflex gain during high-acceleration, whole-body, passive yaw rotations in darkness toward the treated side. All control ears and all operated ears with definite histologic evidence of injection through the intended site showed similar findings, with intact hair cells in all five inner ear sensory epithelia and intact auditory/vestibular neurons. The relative volumes of mouse, rat, rhesus, and human inner ears as measured by CT were (mean ± SD) 2.5 ± 0.1, 5.5 ± 0.4, 59.4 ± 4.7 and 191.1 ± 4.7 µL. These results indicate that injection of PBSV at volumes sufficient for gene therapy delivery can be accomplished without destruction of inner ear structures required for hearing and vestibular sensation.

Bilateral Vestibular Deficiency: Quality of Life and Economic Implications.

IMPORTANCE: Bilateral vestibular deficiency (BVD) causes chronic imbalance and unsteady vision and greatly increases the risk of falls; however, its effects on quality of life and economic impact are not well defined. OBJECTIVE: To quantify disease-specific and health-related quality of life, health care utilization, and economic impact on individuals with BVD in comparison with those with unilateral vestibular deficiency (UVD). DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional survey study of patients with BVD or UVD and healthy controls at an academic medical center. Vestibular dysfunction was diagnosed by means of caloric nystagmography. INTERVENTIONS: Survey questionnaire. MAIN OUTCOMES AND MEASURES: Health status was measured using the Dizziness Handicap Index (DHI) and Health Utility Index Mark 3 (HUI3). Economic burden was estimated using participant responses to questions on disease-specific health care utilization and lost productivity. RESULTS: Fifteen patients with BVD, 22 with UVD, and 23 healthy controls participated. In comparison with patients with UVD and controls, patients with BVD had significantly worse DHI (P < .001) and HUI3 scores. Statistically significant between-group differences were observed for overall HUI3 score (P < .001) and for specific attributes including vision, hearing, ambulation, emotion, and pain (P < .001 for all). Generalized linear model analysis of clinical variables associated with HUI3 scores after adjustment for other variables (including sex, race, education, age, and frequency of dizziness-related outpatient clinic visits) showed that the presence of UVD (P < .001) or BVD (P < .001), increased dizziness-related emergency room visits (P = .002), and increased dizziness-related missed work days (P < .001) were independently associated with worse HUI3 scores. Patients with BVD and UVD incurred estimated mean (range) annual economic burdens of $13,019 ($0-$48,830) and $3531 ($0-$48,442) per patient, respectively. CONCLUSIONS AND RELEVANCE: Bilateral vestibular deficiency significantly decreases quality of life and imposes substantial economic burdens on individuals and society. These results underscore the limits of adaptation and compensation in BVD. Furthermore, they quantify the potential benefits of prosthetic restoration of vestibular function both to these individuals and to society.

Treatment of chronic maxillary atelectasis using balloon dilation.

Balloon dilation may offer a more expedient and cost-effective treatment method compared with traditional endoscopic sinus surgery for chronic maxillary atelectasis. We sought to demonstrate the feasibility of balloon dilation of the maxillary os as a treatment modality for patients with chronic maxillary atelectasis by investigating the short-term outcomes in a retrospective case series of 4 patients representing 5 sinuses treated between 2011 and 2013. All sinuses were successfully balloon dilated without complications. Follow-up ranged from 1 week to 4 months. Aeration of the treated sinuses without restenosis was confirmed by postoperative endoscopy, sinus computed tomography, or both. All patients reported subjective symptomatic improvement. Balloon dilation of the maxillary os may be a feasible treatment option for maxillary sinus atelectasis. Longer follow-up and a larger study sample will be needed to validate the safety of this technique and determine the rate of restenosis.