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Objectives/hypothesis: To determine the mode of inheritance of familial nonsyndromic Mondini dysplasia.Study Design: Correlative clinical genetic analysis of a single kindred.Methods: Clinical history, physical examination, audiologic analysis, computed tomography of the temporal bones, and cytogenetic analysis.Results: The male proband, three affected sisters, and an affected brother are offspring of unaffected parents. The mother and an unaffected brother have audiologic findings suggestive of heterozygous carrier status for a recessive hearing loss gene.Conclusions: Pedigree analysis indicates autosomal recessive inheritance in this family. The observed inheritance and clinical, audiologic, and radiologic findings are different from those previously described for another family with nonsyndromic Mondini dysplasia. The phenotype in this study family therefore represents a distinct subtype, indicating clinical and genetic heterogeneity of this disorder. This information should facilitate future molecular linkage analyses and genetic counselling of patients with inner ear malformations. [ABSTRACT FROM AUTHOR]

Objective: Evaluate the clinical utility of several simple measures of static and dynamic equilibrium in human subjects. In particular, one proposed clinical measure, the Clinical Test of Sensory Integration and Balance (CTSIB) was compared with dynamic posturography for the measurement of postural control capabilities.Study Design: Cross-sectional study of normal subjects and prospective observational study of the same performance measures in vestibular disorder patients.Setting: Academic tertiary care referral center.Participants: Data were collected for all test measures from a group of normal subjects (ages, 20 to 79 years), as well as for a group of patients undergoing treatment for vestibular dysfunction.Results: Data suggest that several semiquantitative clinical tests of static and dynamic equilibrium can be helpful in evaluating and monitoring patients with chronic vestibular dysfunction. The CTSIB results seem to correlate well with dynamic posturography, suggesting that this measure may be useful in identifying patients with abnormal postural control. Formal dynamic posturography testing appears to be more sensitive in detecting abnormal postural control and more exact in defining the specific pattern of dysfunction.Conclusion: Simple clinical measures of static and dynamic equilibrium can reliably distinguish vestibular disorder patients from normal subjects. Dynamic posturography continues to play an important role in the functional evaluation and management of vestibular disorder patients. [ABSTRACT FROM AUTHOR]

Objective: To determine the degree to which electrocochleography (ECoG) correlates with auditory and vestibular outcomes after repair of superior semicircular canal dehiscence (SSCD) via transmastoid (TM) and middle cranial fossa (MCF) approaches., Study Design: Retrospective review., Setting: Academic tertiary referral center., Patients: Adults with SSCD who underwent repair between 2005 and 2019., Intervention: Pre-, intra-, and postoperative ECoG., Main Outcome Measures: Patient-reported vestibular and auditory symptoms; pre-, intra-, and postoperative ECoG measures, dizziness handicap inventory (DHI) scores., Results: Forty-six patients underwent SSCD repair (40 unilateral, six bilateral) between 2005 and 2019, including 24 MCF and 28 TM approaches. There were no differences in preoperative, intraoperative, or postrepair ECoG SP/AP values between the MCF and TM groups (p 0.12, 0.77, 0.58). Patients had subjective improvement in vestibular symptoms (or stable vestibular function in patients operated for predominantly auditory manifestations) with both approaches (MCF: 87.5%; TM: 92.3%; p 0.64). A successful outcome correlated with intraoperative SP/AP ratio normalization (p 0.0005). Similarly, DHI scores were similar in both groups preoperatively (p 0.66) and returned to their preoperative baseline postoperatively with both (p 0.52). Reported vestibular symptoms persisted or worsened more often in patients with migraine (66.6% vs. 28.9%, p 0.03), and with persistently abnormal ECoG measures, though the latter was not statistically significant in this population (38% vs. 15%, p 0.10). Patients had subjective improvement or stability in auditory symptoms using either approach (MCF: 96%; TM: 100%; p 0.62), also correlating with SP/AP ratio normalization (p 0.008)., Conclusions: Correction of abnormal preoperative ECoG reliably correlates to patient symptom improvement after SSCD repair. No significant differences in postoperative outcomes were noted between patients undergoing TM versus MCF repair. Circumspection regarding the likelihood of an ideal outcome after SSCD repair should be exercised when counseling patients with concomitant migraine., Define Professional Practice Gap and Educational Need: It is not certain whether outcomes differ between the two dominant approaches for SSCD repair. Surgeons and patients would benefit from an intraoperative metric that reflects satisfactory plugging of SSCD., Learning Objective: To highlight the reliability and unique utility of intraoperative ECoG and demonstrate the correlation between ECoG correction and symptom improvement for SSCD repair., Desired Result: To report subjective and objective outcomes following SSCD repair and encourage adoption of intraoperative ECoG monitoring., Level of Evidence: Level V., Indicate Irb or Iacuc: IRB review considers this study exempt (HUM00169949)., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Importance: Benign paroxysmal positional vertigo of the posterior canal (PC-BPPV) is a common disorder that is diagnosed using the Dix-Hallpike test (DHT) and treated with the canalith repositioning maneuver (CRM). Patients often seek out information about BPPV self-management, but studies to develop and evaluate patient-centered instructional resources are limited., Objective: To develop and preliminarily evaluate a patient-oriented PC-BPPV self-management instructional video., Methods: We assembled a multidisciplinary team and used an iterative process to develop a theory-based instructional video for self-performing the DHT and CRM. We recruited individuals searching online for information about dizziness to complete a survey and review the video. Patients rated the video by scoring seven questions that measure behavioral intent to perform the DHT or CRM (attitudes/acceptability, perceived self-efficacy, and social norms) using a 10-point scale (higher scores = more favorable ratings). A multilevel linear regression model was used to determine the association of age, sex, race, and education with video ratings., Results: Of the 771 participants who completed the survey, 124 (16%) also reviewed and evaluated the PC-BPPV instructional video. The video review participants were typically more than or equal to 55 years old (70%; 93/124), women (70%; 87/124), and White (70%; 88/124). These participants also generally reported acute-subacute and moderate-to-severe dizziness, and 60% (75/124) reported typical BPPV triggers. The median scores for the seven questions about attitudes/acceptability, self-efficacy, and social norms on the PC-BPPV instructional video were all more than or equal to 9 out of 10 with interquartile ratios that ranged from 7 to 9 at the 25th percentile to 10 at the 75th percentile. Female sex was the only demographic variable associated with higher video ratings (coefficient, 1.21, 95% CI 0.60-1.83)., Conclusion: This study found that participants rated the PC-BPPV self-management video favorably on measures that contribute to behavioral intent to perform the DHT or CRM. The findings provide support that the video is appropriate to use in future studies that evaluate patient self-performance accuracy and outcomes., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: To describe postoperative hearing outcomes following transmastoid (TM) and middle cranial fossa (MCF) approaches for semicircular canal dehiscence (SSCD) repair., Study Design: Retrospective review., Setting: Academic, tertiary referral center., Patients: Adults with SSCD who underwent repair between 2005 and 2019., Interventions: Pure tone audiometry pre- and postoperatively after SSCD repair., Main Outcome Measures: Change in air-bone gap (ABG) at 250 and 500 Hz, pure tone average (PTA), bone conduction (BC), and air conduction (AC) thresholds at 500, 1000, 2000, and 4000 Hz for patients undergoing TM and MCF approaches for SSCD repair., Results: The average change in BC PTA for patients undergoing TM (n = 26) and MCF (n = 24) SSCD repair was not significantly different between the two groups. The first and final postoperative PTAs were recorded an average of 1.7 (range 0.30-3.0) and 29.1 (range 3.5-154) months postoperatively. For patients who underwent MCF repair, the average BC PTAs increased (+) by 2.2 dB HL (p 0.43) and 0.57 dB HL (p 0.88) at the first and final audiograms respectively compared to +1.27 dB HL (p 0.53) and a decrease (-) of 0.57 dB HL (p 0.63) for the TM group. The average changes in low frequency ABG for patients undergoing MCF repair were -4.7 dB (p 0.08) and -6.9 dB (p 0.15) at first and final audiograms respectively compared to -4.9 dB (p 0.06) and -4.1 dB (p 0.36) for patients who underwent TM repair. There was a high frequency hearing loss noted at 8000 Hz for the MCF (30.0 dB ± 18.7 preop; 41.7 dB ± 21.7 postop; p 0.01) and TM (32.1 dB ± 23.2 preop; 44.3 dB ± 29.6 postop; p 0.001) groups which persisted on long term follow up., Conclusions: Both TM and MCF approaches to SSCD repair can be performed with long-term preservation of hearing. ABGs were reduced in each treatment group but did not reach significance. A high frequency hearing loss (8000 Hz) may be expected with either approach., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Patients with single sided deafness (SSD) struggle with sound localization and speech in noise. Existing treatment options include contralateral routing of signal (CROS) systems, percutaneous bone conduction hearing devices (BCHDs), passive transcutaneous BCHDs, active BCHDs, and cochlear implants. Implanted devices provide benefits in speech in noise compared to CROS devices. Percutaneous BCHDs transmit sound efficiently but have aesthetic drawbacks and skin complications. Scalp attenuation impacts passive transcutaneous BCHD performance. Active BCHDs overcome these issues and provide benefits for speech in noise. Cochlear implantation is the only existing option that restores binaural input but introduces electrical rather than acoustic stimuli to the deaf ear. Active BCHDs have been designed to maintain efficient sound transmission and avoid chronic skin irritation and cosmetic concerns that may occur with percutaneous BCHDs. Cochlear implantation may be a superior option for recently deafened SSD patients, though this requires further study. The duration of deafness, patient age and comorbidities, and a shared decision-making model among patients, surgeons, and audiologists should be considered in device selection. The aim of this manuscript is to review available devices, discuss surgical considerations for implantable devices, review available published results for speech in noise and sound quality with each device, and provide an overview to guide shared decision making for patients and providers. This review consolidates available literature and reviews experience with a newer active transcutaneous active BCHD available for use in the SSD population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Vestibular physical therapy (VPT) is a specialized form of evidence-based therapy designed to alleviate primary (vertigo, dizziness, imbalance, gait instability, falls) and secondary (deconditioning, cervical muscle tension, anxiety, poor quality of life, fear of falling/fear avoidance behavior) symptoms related to vestibular disorders. This article provides an overview of VPT, highlighting various exercise modalities used to treat a variety of vestibular disorders. Patient safety and fall prevention are paramount; therefore, fall risk assessment and treatment are also addressed., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: To identify intraoperative neurophysiologic measures predictive of delayed progressive sensorineural hearing loss in the operative ear after a middle fossa approach (MCF) for resection of vestibular schwannoma (VS)., Study Design: Retrospective review., Setting: Academic, tertiary referral center., Patients: Subjects with vestibular schwannoma who underwent a MCF microsurgical resection of VS were analyzed for individuals whose hearing was initially preserved but subsequently developed progressive sensorineural hearing loss in the operative ear. Thirty-seven patients were identified for whom audiologic and neurophysiologic data was available., Intervention: Intraoperative neurophysiologic changes will correlate with delayed sensorineural hearing loss in the operative ear., Main Outcome Measures: Audiometric evaluations, intraoperative electrocochleography (ECoG), and auditory brainstem response (ABR) measures., Results: Twenty-five subjects experienced stable hearing or hearing loss in the operative ear comparable to the contralateral ear. Twelve subjects suffered a significant increase in the hearing asymmetry between ears. Deterioration in the amplitude of wave V of the ABR persisting at the close of tumor resection correlated with delayed sensorineural hearing loss in the operative ear (p 0.02, 5% mean improvement in the stable hearing group, versus a 14% decline with progressive asymmetry), but changes in ECoG or other auditory brainstem response parameters (p > 0.05) were not predictive., Conclusions: Persisting amplitude reduction of wave V of the intraoperative ABR best correlates with delayed progressive sensorineural hearing loss in the operative ear. Neither persistent changes in ECoG, other ABR parameters, nor transient changes, correlated with delayed progressive sensorineural hearing loss in the operative ear., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: To define a complication of the translabyrinthine surgical approach to the posterior fossa related to a rare variant of the anterior inferior cerebellar artery (AICA) that penetrated into the petrous temporal bone., Patient: A healthy 59-year-old male with a unilateral sporadic vestibular schwannoma., Intervention: The patient elected to undergo a translabyrinthine approach for resection of a vestibular schwannoma. An aberrant loop of AICA was encountered during the temporal bone dissection within the petrous portion of the temporal bone., Outcomes: The patient suffered a presumed ischemic insult resulting in a fluctuating ipsilateral facial paresis and atypical postoperative nystagmus., Results: MRI demonstrated an ischemic lesion in the vascular distribution of the right anterior-inferior cerebellar artery, including the lateral portion of the right cerebellar hemisphere, middle cerebellar peduncle, and bordering the right cranial nerve VII nucleus. His functional recovery was excellent, essentially identical to the anticipated course in an otherwise uncomplicated surgery., Conclusions: This case highlights the irregular anatomy of the AICA as well as the importance of thorough neurological exams in the postsurgical lateral skull base patient.

Objective: A new active transcutaneous bone conduction hearing implant system that uses piezoelectric technology has been developed: an active osseointegrated steady-state implant system (OSI). This was the first clinical investigation undertaken to demonstrate clinical performance, safety, and benefit of the new implant system., Study Design and Setting: A multicenter prospective within-subject clinical investigation was conducted., Patients: Fifty-one adult subjects with mixed and conductive hearing loss (MHL/CHL, n = 37) and single-sided sensorineural deafness (SSD, n = 14) were included., Main Outcome Measure: Audiological evaluations included audiometric thresholds, speech recognition in noise, and quiet. Hearing and health-related patient-reported outcomes (PROs; health utilities index [HUI], abbreviated profile of hearing aid benefit [APHAB], and speech, spatial of qualities of hearing scale [SSQ]), daily use, surgical and safety parameters were collected., Results: Intra- and postoperative complications were few. One implant was removed before activation due to post-surgical infection. Compared with the preoperative softband tests, a significant improvement in speech recognition-in-noise was observed in the MHL/CHL group (-7.3 dB, p ≤ 0.0001) and the SSD group (-8.1 dB, p = 0.0008). In quiet, word recognition improved in the MHL/CHL group, most markedly at lower intensity input of 50 dB SPL (26.7%, p ≤ 0.0001). The results of all PROs showed a significant improvement with the new device compared with preoperative softband in the MHL/CHL group. In the SSD group significant improvements were observed in the APHAB and SSQ questionnaires., Discussion: The results confirmed the clinical safety, performance, and benefit of this new treatment modality for subjects with CHL, MHL, and SSD.

Study Objective: We evaluated a strategy to increase use of the test (Dix-Hallpike's test [DHT]) and treatment (canalith repositioning maneuver [CRM]) for benign paroxysmal positional vertigo in emergency department (ED) dizziness visits., Methods: We conducted a stepped-wedge randomized trial in 6 EDs. The population was visits with dizziness as a principal reason for the visit. The intervention included educational sessions and decision aid materials. Outcomes were DHT or CRM documentation (primary), head computed tomography (CT) use, length of stay, admission, and 90-day stroke events. The analysis was multilevel logistic regression with intervention, month, and hospital as fixed effects and provider as a random effect. We assessed fidelity with monitoring intervention use and semistructured interviews., Results: We identified 7,635 dizziness visits during 18 months. The DHT or CRM was documented in 1.5% of control visits (45/3,077; 95% confidence interval 1% to 1.9%) and 3.5% of intervention visits (159/4,558; 95% confidence interval 3% to 4%; difference 2%, 95% confidence interval 1.3% to 2.7%). Head CT use was lower in intervention visits compared with control visits (44.0% [1,352/3,077] versus 36.9% [1,682/4,558]). No differences were observed in admission or 90-day subsequent stroke risk. In fidelity evaluations, providers who used the materials typically reported positive clinical experiences but provider engagement was low at facilities without an emergency medicine residency program., Conclusion: These findings provide evidence that an implementation strategy of a benign paroxysmal positional vertigo-focused approach to ED dizziness visits can be successful and safe in promoting evidence-based care. Absolute rates of DHT and CRM use, however, were still low, which relates in part to our broad inclusion criteria for dizziness visits., (Copyright © 2019 American College of Emergency Physicians. Published by Elsevier Inc. All rights reserved.)

Importance: Benign paroxysmal positional vertigo (BPPV) is a common cause of acute dizziness. Strong evidence exists for diagnosing BPPV using the Dix-Hallpike Test (DHT) and treating it with the canalith repositioning maneuver (CRM). Despite this, both are infrequently used in the emergency department (ED)., Objective: As an early method to evaluate a BPPV-focused educational intervention, we evaluated whether an educational intervention improved ED provider performance on hypothetical stroke and BPPV cases delivered by vignette., Design: A randomized, controlled, educational intervention study in ED physicians. The intervention aimed to promote the appropriate use of the DHT and CRM. A BPPV vignette, a stroke-dizziness (safety) vignette, and vignette scoring schemes (higher scores indicating more optimal care) used previously established vignette methodology., Setting: We recruited participants at the exhibitor hall of an emergency medicine annual meeting., Participants: We recruited 48 emergency physicians. All were board certified or residency trained and board eligible. All were engaged in the active practice of emergency medicine. None were trainees., Interventions: Intervention group: a narrated, educational presentation by computer followed by the clinical vignettes., Control Group: Received no educational intervention and completed the clinical vignettes-intended to mirror current clinician practice., Main Outcome Measure: Primary endpoint: total score (out of 200 points) on a vignette-based scoring instrument assessing the performance of history, physical, and diagnostic testing on hypothetical stroke and BPPV cases., Results: The efficacy threshold was crossed at the interim analysis. The intervention group had higher performance scores compared with controls (113.2 versus 68.6, p < 0.00001). BPPV and safety subscores were both significantly higher in the intervention group. Sixty-two percent of the intervention group planned to use the DHT versus 29% of controls. After the vignette described characteristic BPPV nystagmus, 100% of the intervention group planned to use the CRM versus 17% of controls., Conclusions and Relevance: The educational intervention increased provider performance in dizziness vignettes, including more frequent appropriate use of the DHT/CRM. These findings indicate the intervention positively influenced planned behavior. Future work is needed to implement and evaluate this intervention in clinical practice.

Background: Benign paroxysmal positional vertigo (BPPV) is the most common peripheral vestibular disorder, and accounts for 8% of individuals with moderate or severe dizziness. BPPV patients experience substantial inconveniences and disabilities during symptomatic periods. BPPV therapeutic processes - the Dix-Hallpike Test (DHT) and the Canalith Repositioning Maneuver (CRM) - have an evidence base that is at the clinical practice guideline level. The most commonly used CRM is the modified Epley maneuver. The DHT is the gold standard test for BPPV and the CRM is supported by numerous randomized controlled trials and systematic reviews. Despite this, BPPV care processes are underutilized., Methods/design: This is a stepped-wedge, randomized clinical trial of a multi-faceted educational and care-process-based intervention designed to improve the guideline-concordant care of patients with BPPV presenting to the emergency department (ED) with dizziness. The unit of randomization and target of intervention is the hospital. After an initial observation period, the six hospitals will undergo the intervention in five waves (two closely integrated hospitals will be paired). The order will be randomized. The primary endpoint is measured at the individual patient level, and is the presence of documentation of either the Dix-Hallpike Test or CRM. The secondary endpoints are referral to a health care provider qualified to treat dizziness for CRM and 90-day stroke rates following an ED dizziness visit. Formative evaluations are also performed to monitor and identify potential and actual influences on the progress and effectiveness of the implementation efforts., Discussion: If this study safely increases documentation of the DHT/CRM, this will be an important step in implementing the use of these evidenced-based processes of care. Positive results will support conducting larger-scale follow-up studies that assess patient outcomes. The data collection also enables evaluation of potential and actual influences on the progress and effectiveness of the implementation efforts., Trial Registration: ClinicalTrials.gov, ID: NCT02809599 . The record was first available to the public on 22 June 2016 prior to the enrollment of the first patients in October 2016.

: Chronic suppurative otitis media can have long-term effects on hearing if not managed effectively. When combined with cholesteatoma the condition may require creation of an open mastoid cavity. Recurrence of cholesteatoma is a concern when cochlear implantation is performed with overclosure of the external auditory meatus. A 61-year-old female with recurrent cholesteatoma in this setting was treated using a preauricular approach to provide adequate visualization while preventing the need to remove the implant or risking injury to the internal components. This technique would be useful in similar patients to prevent morbidity from removal and reinsertion of a cochlear implant.

Objective: To analyze the immediate postoperative and long-term hearing outcome data in patients who have undergone hearing preservation attempts with the middle cranial fossa (MCF) approach for the resection of sporadic vestibular schwannoma., Study Design: Retrospective review of a surgical patient cohort., Setting: Tertiary academic referral center., Patients: Adult patients with unilateral sporadic vestibular schwannoma., Intervention: Surgical treatment with a MCF approach., Main Outcome Measure: Comparison of pre- and postoperative audiometric data in accordance with the 1995 and 2012 American Academy of Otolaryngology/Head and Neck Surgery guidelines on reporting hearing outcomes and with a word recognition score only scale. Long-term hearing outcome data were analyzed in the following postoperative groups: 3 to 5, 6 to 8, 9 to 11, and 12+ years after surgery., Results: From 1999 to 2016, 174 patients underwent the MCF approach and 155 of them met inclusion criteria for analysis of hearing outcome data. Class A or B hearing was preserved in 70% of the entire cohort after recovery. Seventy-one patients with measurable hearing met criteria for long-term data analysis. The rate of American Academy of Otolaryngology/Head and Neck Surgery class A or B hearing preservation was 82% at 3 to 5 years and declined thereafter. The rate of word recognition score class I or II hearing preservation was 98% at 3 to 5 years and declined less rapidly thereafter. Patients with preoperative Class A hearing had significantly higher rates of successful hearing preservation at all postoperative intervals., Conclusion: Delayed hearing loss occurs in a progressively increasing fashion but speech understanding remains durable for a majority of patients whose hearing is initially preserved with the MCF approach.

Background: The traditional approach to dizziness encourages providers to emphasize the type of dizziness. However, symptom types might substantially overlap in individual patients, thus limiting the clinical value of this approach. We aimed to describe the overlap of types of dizziness using a US nationally representative sample., Methods: The 2008 US National Health Interview Survey was examined for prevalence and overlap of types of dizziness. The data were also separately examined among people who otherwise had typical features of traditionally vertigo-based disorders (ie, benign paroxysmal positional vertigo and Meniere's disease). Data analysis also included exploratory factor analysis., Results: Twelve-month prevalence of problems with dizziness or balance was 14.8%, representing 33.4 million individuals. The mean number of dizziness symptoms was 2.4 (95% confidence interval [CI], 2.3-2.4), with 61.1% reporting more than one type. Of subjects who otherwise had typical features of traditionally vertigo-based disorders, the mean number of dizziness types was 3.1 (95% CI, 3.0-3.3), and only 24.6% (95% CI, 21.0%-28.7%) reported vertigo as the primary type. Exploratory factor analysis found that symptom types loaded onto a single factor without other clinical or demographic variables., Conclusions: Substantial overlap of dizziness types exists among US adults with dizziness. People otherwise having features of traditionally vertigo-based disorders also typically report multiple dizziness types and do not typically report vertigo as the primary type. Symptom types correlate more strongly with each other than with other clinical or demographic variables. These findings suggest that the traditional emphasis on dizziness types is likely of limited clinical utility., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To report two cases of pulsatile tinnitus caused by complex venous diverticula with successful treatment via coil embolization and stent placement followed by complete resolution of symptoms. We also review the literature pertaining to venous diverticula causing pulsatile tinnitus treated using endovascular techniques., Patients: Two women patients, aged 27 and 29 years, presented to our institution with 3-month histories of pulsatile tinnitus. In each case, non-invasive imaging and conventional digital subtraction angiography (DSA) confirmed the presence of a complex right transverse-sigmoid sinus junction diverticulum., Intervention: Both patients underwent stent-assisted coil embolization of the venous diverticula., Main Outcome and Results: Clinical and DSA follow-up at 6 and 12 months confirmed resolution of symptoms with obliteration of the venous diverticulum. We also performed a PubMed database search for the period January 1995 through June 2016 using the terms pulsatile tinnitus, venous aneurysm/diverticulum, stent-assisted coil embolization, and endovascular treatment and identified reports of 14 additional patients treated using endovascular techniques., Conclusions: Venous sinus diverticula causing pulsatile tinnitus can be successfully treated with stent-assisted coil embolization with complete resolution of clinical symptoms. This is in concordance with 13 case reports in the literature involving 14 patients with venous sinus diverticula treated using endovascular techniques.

Background: The test and treatment for benign paroxysmal positional vertigo (BPPV) are evidence-based practices supported by clinical guideline statements. Yet these practices are underutilized in the emergency department (ED) and interventions to promote their use are needed. To inform the development of an intervention, we interviewed ED physicians to explore barriers and facilitators to the current use of the Dix-Hallpike test (DHT) and the canalith repositioning maneuver (CRM)., Methods: We conducted semi-structured in-person interviews with ED physicians who were recruited at annual ED society meetings in the United States. We analyzed data thematically using qualitative content analysis methods., Results: Based on 50 interviews with ED physicians, barriers that contributed to infrequent use of DHT/CRM that emerged were (1) prior negative experiences or forgetting how to perform them and (2) reliance on the history of present illness to identify BPPV, or using the DHT but misattributing patterns of nystagmus. Based on participants' responses, the principal facilitator of DHT/CRM use was prior positive experiences using these, even if infrequent. When asked which clinical supports would facilitate more frequent use of DHT/CRM, participants agreed supports needed to be brief, readily accessible, and easy to use, and to include well-annotated video examples., Conclusions: Interventions to promote the use of the DHT/CRM in the ED need to overcome prior negative experiences with the DHT/CRM, overreliance on the history of present illness, and the underuse and misattribution of patterns of nystagmus. Future resources need to be sensitive to provider preferences for succinct information and video examples.

SLC44A2 was discovered as the target of an antibody that causes hearing loss. Knockout mice develop age related hearing loss, loss of sensory cells and spiral ganglion neurons. SLC44A2 has polymorphic sites implicated in human disease. Transfusion related acute lung injury (TRALI) is linked to rs2288904 and genome wide association studies link rs2288904 and rs9797861 to venous thromboembolism (VTE), coronary artery disease and stroke. Here we report linkage disequilibrium of rs2288904 with rs3087969 and the association of these SLC44A2 SNPs with Meniere's disease severity. Tissue-specific isoform expression differences suggest that the N-terminal domain is linked to different functions in different cell types. Heterozygosity at rs2288904 CGA/CAA and rs3087969 GAT/GAC showed a trend for association with intractable Meniere's disease compared to less severe disease and to controls. The association of SLC44A2 SNPs with VTE suggests that thrombi affecting cochlear vessels could be a factor in Meniere's disease., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To minimize discomfort, time, and costs, vestibular laboratories may perform monothermal caloric irrigations and discontinue testing if responses are symmetric. This systematic review aimed to determine the diagnostic accuracy of the monothermal caloric screening test (MCST) for unilateral vestibular dysfunction compared with bithermal caloric testing (BCT)., Data Sources: Ovid-MEDLINE, EMBASE, Scopus, Cochrane CENTRAL, and manual bibliographic searches., Review Methods: Inclusion criteria specified concurrent MCST and BCT performance and reporting of test measures (monothermal caloric asymmetry, unilateral weakness). The primary outcomes were between-measure correlation, sensitivity, and specificity. Meta-analysis was performed with hierarchical bivariate and univariate random-effects models. Heterogeneity was assessed with the I(2) statistic., Results: Fifteen studies (n = 5572 participants) met inclusion criteria. Thresholds varied between studies. Asymmetries calculated by MCST and BCT were strongly correlated, but a subgroup analysis showed no correlation for those with mild unilateral weakness. The sensitivity and specificity of the MCST ranged from 0.54 to 1.00 and 0.25 to 0.96, respectively. Predictably, higher sensitivity resulted from lower cutoff points for the MCST, higher thresholds for the BCT, and additional test positivity criteria. Warm irrigations yielded higher sensitivity than cool. Studies excluding participants with severe unilateral weakness yielded lower sensitivity estimates. After pooling by threshold, temperature, and risk of bias, most performance estimates remained substantially heterogeneous (I(2) > 60%)., Conclusion: Accuracy of the MCST is lacking precisely where it is needed most-at the border of normal and abnormal vestibular function. To guide clinical practice, research should include analysis of subgroups with varying levels of function and employ standardized testing parameters., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.)

Objectives: To evaluate whether monothermal caloric screening can reduce the number of caloric irrigations required in the vestibular testing battery while maintaining diagnostic accuracy., Design: Prospective controlled cohort study. Three hundred and ninety patients referred for vestibular testing at this tertiary referral health system over a 1-year period were evaluated; 24 patients met exclusion or failure criteria and 366 patients were included in the study. Population was 35.6% male; average age was 50.4 years old. Each patient underwent caloric testing using either warm or cool water irrigation initially and this data was used for monothermal screening data. All patients then completed bithermal binaural caloric testing to obtain the "gold standard" bithermal data for comparison. The sensitivity and specificity of monothermal cool or monothermal warm caloric tests were calculated using a receiver operating characteristic curve analysis., Results: Using a monothermal interear difference threshold of 25%, warm monothermal screening had sensitivity of 98.0%, specificity of 91.3%, false negative rate of 2%, and false positive rate of 8.7%. Cool monothermal screening also had excellent sensitivity (92.3%) and specificity (95.3)%, with a false negative rate of 7.7%, and a false positive rate of 4.7%. The diagnosis associated with the single false negative warm monothermal caloric test was compensated vestibular paresis. In the study population, 71.9% had a negative monothermal screen; if the monothermal data were accepted, 2 fewer irrigations would have been performed resulting in an average saving of $264 (typical Medicare reimbursement for 2 irrigations) billed per patient screened as well as shortening the average testing battery by about 15 min., Conclusions: Warm monothermal caloric screening can reduce time and cost of vestibular testing while nearly matching the diagnostic accuracy of bithermal testing.

Context: As of 2015, more than 23 million scuba diver certifications have been issued across the globe. Given the popularity of scuba diving, it is incumbent on every physician to know and understand the specific medical hazards and conditions associated with scuba diving., Evidence Acquisition: Sources were obtained from PubMed, MEDLINE, and EBSCO databases from 1956 onward and ranged from diverse fields including otologic reviews and wilderness medicine book chapters., Study Design: Clinical review., Level of Evidence: Level 5., Results: Otologic hazards can be categorized into barotrauma-related injuries or decompression sickness., Conclusion: When combined with a high index of suspicion, the physician can recognize these disorders and promptly initiate proper treatment of the potentially hazardous and irreversible conditions related to scuba diving.

Objectives: To describe the rationale, intraoperative details, and histopathologic findings discovered when treating an unusual case of apogeotropic horizontal canal positional vertigo with a transmastoid labyrinthectomy., Patient: A single case report., Intervention: Therapeutic., Main Outcome Measures: Resolution of apogeotropic nystagmus and improvement of positional vertigo., Results: The apogeotropic variant of horizontal canal positional vertigo can be a difficult entity to treat. This report describes a patient who developed profound sensorineural hearing loss and vertigo after an acute left labyrinthitis. Ten months later, she developed vertigo with apogeotropic positional nystagmus involving the left horizontal semicircular canal. Particle repositioning maneuvers and vestibular physical therapy were unsuccessful. In addition, she developed intermittent positional vertigo affecting the ipsilateral vertical semicircular canals. Given the persistence of her vertigo, multiple canal involvement, and patient preference for definitive treatment, a transmastoid labyrinthectomy was performed. Intraoperatively, the ampulla of the horizontal canal as well as that of the other canals was grossly abnormal as later confirmed on histology. After surgery, her apogeotropic nystagmus and vertigo resolved, and her balance ability gradually improved to a highly functional level., Conclusion: This case illustrates a unique form of positional vertigo that developed and persisted after acute labyrinthitis. Conservative measures were unsuccessful and a transmastoid labyrinthectomy documented dense inflammatory tissue involving all three ampullae. We postulate that the post-labyrinthitic inflammatory changes resulted in mass loading of the membranous ampullae, causing abnormal nystagmus patterns and positional vertigo, which resolved after the labyrinthectomy.

Semicircular canal dehiscence (SCD) is a condition of the inner ear wherein the temporal bone adjacent to the vestibular apparatus thins or erodes completely. It has no clear epidemiological predisposing factors that have been identified to date. Its diagnosis is made difficult by a clinical presentation that often overlaps with other pathologies of the ear. Symptoms of SCD are believed to result from a "third-window" phenomenon, which disrupts the transmission of acoustic energy in the inner ear. Symptoms, when present, may change or worsen over time, confounding the diagnosis. We present a case of SCD of the posterior canal that mimicked Meniere's disease for several decades. We also discuss the clinical findings that may steer clinical suspicion towards this diagnosis., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: The objective of this study is to describe the presentation and management of a rare site of cholesteatoma recurrence at the bony-cartilaginous junction after intact canal wall (ICW) mastoidectomy., Study Design: Retrospective case series, Setting: Tertiary referral center, Patients: Patients with cholesteatoma formation arising from the bony-cartilaginous (BC) junction of the external auditory canal (EAC) requiring surgical intervention were retrospectively identified across a 5-year period., Intervention(s): All patients were treated surgically to eradicate the disease and reconstruct the bony defect when possible., Main Outcome Measure(s): This observational study details the presentation, risk factors, and management of a rare site of cholesteatoma recurrence after ICW mastoidectomy., Results: After ICW mastoidectomy, eight patients were identified with fistulae in the lateral EAC near the BC junction. Seven patients had associated iatrogenic cholesteatomas arising at this site, and one patient had a dry fistula with bony stenosis of the EAC. All patients had a history of chronic otitis media and previous surgery. Patients averaged 9 years between surgery and recidivism. Reconstruction of the bony defect was completed using hydroxyapatite reconstruction plates in four patients with 75% success, soft wall reconstruction in two patients using temporalis muscle, and canal wall down mastoidectomy in two patients who had extensive disease and exposed dura. No recurrent disease was evident during an average follow-up of 16 months. EAC reconstruction was successful in 83% of cases., Conclusion: This case series reports a novel pattern of iatrogenic cholesteatoma formation near the BC junction of the EAC that can occur years after ICW mastoidectomy. In properly selected cases, this condition can be managed with revision ICW mastoidectomy and reconstruction.

Importance: To compare the efficacy of treatments commonly offered to patients with Ménière's disease who fail conservative medical therapy including diuretics and a sodium-restricted diet., Objectives: This study compared three second-echelon treatments: the Meniett device, endolymphatic sac decompression, and intratympanic gentamicin injections to determine their comparative effectiveness and capacity to mitigate against the necessity of a surgical labyrinthectomy., Design, Setting, and Participants: Retrospective observational study at an academic tertiary care center. Patients with Ménière's disease who failed primary medical management were evaluated after treatment with a Meniett device (n=20), endolymphatic sac decompression (n=23) or intratympanic gentamicin injections (n=17). Cases were included if auditory and vertigo control data were available before and a minimum of two years after treatment, in patients without previous otologic surgery or intratympanic injections. Average age ranged from 54 to 75 years., Interventions: Use of the Meniett device, endolymphatic sac shunt decompression surgery or intratympanic gentamicin injections using variable doses and injection schedules., Main Outcomes and Measures: Proportion of patients with vertigo control and hearing preservation by a modified version of the AAO-HNS criteria after second-echelon treatment, thus not requiring definitive labyrinthectomy., Results: Despite endolymphatic sac surgery demonstrating a longer duration (61 months) prior to labyrinthectomy, no differences were found between the 3 treatment options in terms of patients going on to definitive labyrinthectomy or in the number of months of symptom relief following treatment. There was also no difference in residual auditory perception across the 3 groups., Conclusions and Relevance: No significant therapeutic differences were found between the studied second-echelon treatments for symptom relief of Ménière's disease.

Objective: Children with auditory neuropathy (AN) have variable hearing on pure tone testing, and the presence of speech and language delays often play a major role in the decision to offer cochlear implantation (CI) in this population. Despite this fact, the speech and language outcomes in this group after CI are not well described. This study compares speech and language outcomes after CI in a subset of the pediatric AN population that does not have a confounding cognitive disorder with those of their peers with cochlear hearing loss (CoHL)., Study Design: Retrospective chart review., Setting: Tertiary referral center., Patients: Seventeen pediatric patients with AN who received a CI and a group of children with CoHL who received a CI were the subjects of this study. The 2 groups demonstrated similar ages at implant. Children with cognitive delays were excluded from each group., Intervention: Cochlear implantation., Main Outcome Measures: All subjects were evaluated preoperatively and postoperatively with standardized age appropriate speech and language measures, including the Expressive Vocabulary Test (EVT), Peabody Picture Vocabulary Test (PPVT), and Preschool Language Scale (PLS)., Results: There was no significant difference between the groups on age of activation of the CI. Children with a diagnosis of AN had a significantly lower unaided pure tone average preoperatively as compared with children with cochlear hearing loss; however, there was no significant difference between the groups on either their preimplantation or postimplantation speech and language scores., Conclusion: Children with a diagnosis of AN without associated cognitive or developmental disorders have speech and language outcomes comparable to other children who received a CI.

Object: The middle cranial fossa (MCF) approach is a microsurgical technique described as a primary option in the treatment of small, intracanalicular schwannomas involving the eighth cranial nerve. Excellent rates of complete tumor resection, hearing preservation, preservation of facial nerve function, and low complication rates have been reproduced using this technique. However, the durability of hearing preservation attained using the various treatment options has not been adequately assessed. The purpose of this study was to evaluate the durability of long-term hearing preservation in patients with vestibular schwannoma (VS) treated via the MCF approach. The authors hypothesize that hearing preservation in these patients will prove to be durable years after treatment in a high percentage of cases., Methods: Retrospective medical chart review was performed in 103 consecutive patients undergoing resection of VS via a modified MCF approach between 1999 and 2008. Patients in whom surgical goals were gross-total resection and hearing preservation were included. Preoperative and postoperative hearing assessment was performed using standard audiometric testing, and classified according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines as a primary outcome measure. Outcomes and neurological complications initially, and at 1, 3, and 5 years following operation were analyzed., Results: Initial hearing preservation rates were in keeping with the best previously published results. At initial postoperative audiometric follow-up, of the patients presenting with Class A hearing, 67% remained Class A, 17% were Class B, 1% were Class C, and 15% were Class D. Of patients presenting with Class B hearing, 24% were Class A, 53% remained Class B, 6% were Class C, and 18% were Class D. Of patients presenting with Class C hearing, 100% remained Class C. To assess the durability of hearing preservation in our patients, the authors evaluated hearing function at regular intervals after the initial postoperative audiometric follow-up. Audiometric data were available for 56 patients at 5-year follow-up. Of the 20 patients with Class A hearing at initial postoperative follow-up with 5-year follow-up, 13 (65%) remained Class A, 6 (30%) were Class B, and 1 (5%) was Class C. Of the 12 patients with Class B hearing at initial postoperative follow-up with 5-year follow-up, 4 (33%) were Class A, 4 (33%) remained Class B, and 4 (33%) were Class C. Of the 3 patients with Class C hearing at initial postoperative follow-up with 5-year follow-up, all 3 (100%) remained Class C., Conclusions: A majority of patients with preserved hearing following the MCF approach for treatment of VS experience durability of their preserved hearing at 5-year follow-up. The initial AAO-HNS classification was preserved in 13 (65%) of the 20 patients who had Class A hearing at 5 years, and in 8 (67%) of the 12 who had Class B hearing at 5 years. Overall, a decline in AAO-HNS classification was noted in 15% of patients with preserved Class A hearing, and in 33% of those with preserved Class B hearing. Facial nerve function was preserved in 91% of cases. Superior hearing preservation as well as good outcomes in facial nerve function and few serious complications can be accomplished using the MCF approach for resection of small VSs.

Objective: Auditory neuropathy (AN) is a heterogeneous clinical entity for which the optimal method of auditory rehabilitation has been a matter of some debate. Such patients often do not receive sufficient benefit from hearing aids. Previous studies have shown that select AN patients may benefit from cochlear implantation (CI), but reported outcomes are variable and likely are a reflection of the heterogeneous patient population included under the umbrella diagnosis of AN. This study compares CI outcomes in a subset of the pediatric AN population who do not have a confounding cognitive disorder with their cochlear hearing loss peers. Additionally, it examines the impact of a confounding cognitive or developmental disorder on CI outcomes within the AN population., Study Design: Retrospective chart review., Setting: Tertiary referral center., Patients: Twenty-six pediatric patients with AN who received a CI were the subjects of this study. Seventeen of these children had a diagnosis of AN alone, and their CI outcomes were compared with those of a similar group of children with cochlear hearing loss. The remaining 9 children had a diagnosis of AN in association with a confounding cognitive or developmental disorder, and their CI outcomes were compared with those of children with isolated AN., Intervention: Cochlear implantation., Main Outcome Measures: All subjects were evaluated preoperatively and postoperatively with age-appropriate speech perception testing., Results: Children with a diagnosis of AN alone performed comparably to their peers with cochlear hearing loss. The presence of a confounding cognitive or developmental disorder within the AN population was correlated with significantly poorer CI outcomes as compared with those of children with isolated AN., Conclusion: Children with a diagnosis of AN without associated cognitive or developmental disorders perform at a level comparable to other children requiring a CI. Children with a diagnosis of AN associated with other developmental anomalies derive some benefit from CI but are significantly more likely to continue to rely on nonoral/aural methods of communication after implantation.

Objective: To describe and communicate data collected in the CHEER (Creating Healthcare Excellence through Education and Research) infrastructure proof-of-concept study to facilitate understanding of the potential capabilities of practice-based research networks and to present pilot data for development of future research initiatives., Study Design: Prospective observational study of CHEER infrastructure operational capacity using a convenience sample of all patients presenting to the practices with tinnitus, dizziness, or a combination of these symptoms., Setting: The CHEER network of community and academic practice sites., Subjects and Methods: The data collection exercise collected demographic, clinical, treatment, and health-related quality-of-life surveys on tinnitus, dizziness, and migraine disorders. Descriptive analysis of the data is presented., Results: Of the sites in the CHEER network, 73% (16/22) successfully enrolled subjects; a total of 1532 patients were enrolled in 8 months. Tinnitus alone, dizziness alone, and both occurred in 28%, 34%, and 29%, respectively. Patients complaining of tinnitus and dizziness had lower quality of life than those sufferers with 1 disorder. Migraine was associated with 27% of patients. The most frequent diagnoses for patients with tinnitus and dizziness were Ménière disease (34%), vertiginous migraine (18%), and benign paroxysmal positional vertigo (16%)., Conclusion: Descriptive data on patients with common disorders can be rapidly collected within the framework of a practice-based research network. The data in this study provide valuable pilot information on the targeted disorders, providing a baseline for development of future epidemiological data and clinical trials.

Context: Idiopathic sudden sensorineural hearing loss has been treated with oral corticosteroids for more than 30 years. Recently, many patients' symptoms have been managed with intratympanic steroid therapy. No satisfactory comparative effectiveness study to support this practice exists., Objective: To compare the effectiveness of oral vs intratympanic steroid to treat sudden sensorineural hearing loss., Design, Setting, and Patients: Prospective, randomized, noninferiority trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of onset of 50 dB or higher of pure tone average (PTA) hearing threshold. The study was conducted from December 2004 through October 2009 at 16 academic community-based otology practices. Participants were followed up for 6 months., Intervention: One hundred twenty-one patients received either 60 mg/d of oral prednisone for 14 days with a 5-day taper and 129 patients received 4 doses over 14 days of 40 mg/mL of methylprednisolone injected into the middle ear., Main Outcome Measures: Primary end point was change in hearing at 2 months after treatment. Noninferiority was defined as less than a 10-dB difference in hearing outcome between treatments., Results: In the oral prednisone group, PTA improved by 30.7 dB compared with a 28.7-dB improvement in the intratympanic treatment group. Mean pure tone average at 2 months was 56.0 for the oral steroid treatment group and 57.6 dB for the intratympanic treatment group. Recovery of hearing on oral treatment at 2 months by intention-to-treat analysis was 2.0 dB greater than intratympanic treatment (95.21% upper confidence interval, 6.6 dB). Per-protocol analysis confirmed the intention-to-treat result. Thus, the hypothesis of inferiority of intratympanic methylprednisolone to oral prednisone for primary treatment of sudden sensorineural hearing loss was rejected., Conclusion: Among patients with idiopathic sudden sensorineural hearing loss, hearing level 2 months after treatment showed that intratympanic treatment was not inferior to oral prednisone treatment., Trial Registration: clinicaltrials.gov Identifier: NCT00097448.

Vertigo provoked by head rotation is a classic symptom of rotational vertebrobasilar ischemia (RVBI). Inner ear disease can cause positional vertigo and mimic RVBI. We review the case of a patient with vertigo consistently triggered by leftward head rotation when supine. Computed tomography angiogram and dynamic arteriogram failed to show compression of the vertebral arteries with head rotation. Further evaluation revealed benign paroxysmal positional vertigo (BPPV) as the underlying etiology. Treatment of her BPPV led to complete resolution of her symptoms. A succinct overview of this common otologic disorder is provided, and strategies to help distinguish it from RVBI are discussed., (Copyright 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved.)