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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.

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).

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.

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.

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.

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.

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.

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.

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.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.Both patients underwent stent-assisted coil embolization of the venous diverticula.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.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.

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.

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).Ovid-MEDLINE, EMBASE, Scopus, Cochrane CENTRAL, and manual bibliographic searches.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.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%).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.

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.

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. Electronic supplementary material The online version of this article (10.1186/s13063-018-3099-0) contains supplementary material, which is available to authorized users.

Objectives/Hypothesis To describe the clinical experience and characterize the outcomes of cochlear implantation (CI) in children with isolated enlarged vestibular aqueduct (IEVA) as compared to children with enlarged vestibular aqueduct (EVA) associated with other bony labyrinth abnormalities. Study Design Single, tertiary care, institutional retrospective review over 2 decades. Methods The clinical course and outcomes of 55 children with EVA undergoing CI between 1991 and 2013 were reviewed. Test measures included open and closed set speech perception tests, and various speech and language measures. Results In 18 children (32.7%), IEVA was the only defect present. In 33 children (60%), EVA occurred concomitantly with incomplete partition type 2 (IP 2) bilaterally, and three children with incomplete partition type 1 bilaterally. Ninety-two percent (51 of 55) occurred bilaterally and had matching bony defects. Mean age of CI was 73.4 months. A statistically significant defect-related and linguistic-status pattern was noted, impacting the timing of implantation: IEVA = 112.8 months, IP 2 = 58.4 months (P

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.

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.

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.

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.

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).Retrospective chart review.Tertiary referral center.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.Cochlear implantation.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).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.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.

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.

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.

To report the pretreatment and posttreatment population characteristics and the overall stability of the audiologic outcomes found during the Sudden Hearing Loss Clinical Trial (ClinicalTrials.gov: Identifier NCT00097448).Multicenter, prospective randomized noninferiority trial of oral versus intratympanic (IT) steroid treatment of sudden sensorineural hearing loss (SSNHL).Fifteen academically based otology practices.Two hundred fifty patients with unilateral SSNHL presenting within 14 days of onset with 50 dBHL or greater pure tone average hearing threshold in the affected ear.Either 60 mg/d oral prednisone for 14 days with a 5-day taper (121 patients) or 4 IT doses for 14 days of 40 mg/ml methylprednisolone (129 patients).Primary end point was change in hearing [dB PTA] at 2 months after treatment. Noninferiority was defined as less than 10 dB difference in hearing outcome between treatments. In this article, pretreatment and posttreatment hearing findings will be reported in detail.A general (and stable) effect of treatment and a specific effect of greater improvement at low frequencies were found in both treatment groups.Hearing improvements are stable, and a significantly greater improvement occurs with lower frequency after either oral or IT steroid treatment of SSNHL.

Objective To evaluate serum antibody to heat shock protein (HSP) 70 as a marker for autoimmune sensorineural hearing loss (AISNHL). Design Sera from 20 patients with rapidly progressive sensorineural HL and 20 control volunteers without HL were tested for antibody reactivity against multiple HSP 70 substrates. Substrates included recombinant human HSP (rHuHSP) 72, purified bovine brain heat shock cognate (HSC) 73 and HSP 72, as well as heat-shocked and non-heat-shocked protein extracts from bovine kidney (MDBK) cells. All serum donors were previously tested for antibody to guinea pig inner ear supporting cells; 17 of 20 patients but none (0 of 20) of the controls were positive. Methods Sera were tested using Western blots. Results Reactivity with rHuHSP 70 was observed in 16 patients and 17 controls. Similarly, 15 of 20 patients and 17 of 20 controls stained for both HSP 72 and HSC 73 from the bovine brain. When tested against the heat-shock-induced and control MDBK extracts, six patients and nine controls had greater reactivity with the induced HSP 72. Conclusion The frequency of antibodies to HSP substrates did not differ in patients and controls. Prior studies reported that HSP 72 is the 68 kD antigen commonly detected by AISNHL sera. However, we show that HSP 72 antibodies are no more prevalent in patients than in normal controls. Thus, it is unlikely that the 68 kD protein is HSP 72. Therefore, HSPs are not appropriate substrates for serodiagnosis of AISNHL.

(2010). The Predictive Value of Transtympanic Promontory EABR in Congenital Temporal Bone Malformations. Cochlear Implants International: Vol. 11, Proceedings of the 9th European Symposium on Paediatric Cochlear Implantation, Warsaw, 2009, pp. 181-186.

To report the adverse effects associated with prolonged high-dose prednisone for the treatment of autoimmune inner ear disease (AIED).Prospective data collected as part of a multicenter, randomized, controlled trial for the treatment of corticosteroid-responsive AIED with methotrexate.Tertiary referral centers.One hundred sixteen patients with rapidly progressive, bilateral sensorineural hearing loss.All patients completed a 1-month course of prednisone (60 mg/d). In Phase 2, 67 patients with improvement in hearing underwent a monitored 18-week prednisone taper, resulting in 22 weeks of prednisone therapy at an average dose of 30 mg per day. Thirty-three patients were randomized to receive methotrexate in Phase 2. Thirty-four patients received prednisone and placebo.Adverse events (AE) in patients treated with prednisone only.Of 116 patients, 7 had to stop prednisone therapy during the 1-month challenge phase due to AE. Of 34 patients, 5 were unable to complete the full 22-week course of prednisone due to AE. The most common AE was hyperglycemia, which occurred in 17.6% of patients participating in Phase 2. Weight gain was also common, with a mean increase in body mass index of 1.6 kg/m2 (95% confidence interval, 0.77-2.3) during the 22-week steroid course. Patients entering Phase 2 were followed for a mean of 66 weeks. No fractures or osteonecrosis were reported.Although high-dose corticosteroids are associated with known serious side effects, prospective data in the literature are limited. The present study suggests that with appropriate patient selection, monitoring, and patient education, high-dose corticosteroids are a safe and effective treatment of AIED.

Objectives/Hypothesis: Choline transporter-like protein 2 (CTL2), a 68–72 kDa inner-ear membrane glycoprotein, is a candidate target antigen in autoimmune hearing loss (AIHL). The objective of this study was to test recombinant human CTL2 as a potential target for the detection of human autoantibodies in patients with AIHL. Study Design: In vitro assay development. Methods: Human inner ear CTL2 mRNA was cloned into baculovirus and used to infect insect cells. Immunofluorescence and western blotting were used to determine optimal expression of recombinant human CTL2 (rHuCTL2) in insect cells. AIHL patient sera of known reactivity with guinea pig inner ear were tested for antibodies to purified rHuCTL2 on western blots. Sera from normal hearing donors were used as controls. Results: The rHuCTL2 protein migrated as three bands: a core protein of 62kDa and two N-glycosylated bands at 66 and 70 kDa. Sera from 6/12 (50%) of AIHL patients with antibody to the 68–72 kDa inner-ear protein or to supporting cells also have antibody to rHuCTL2. Four of the four patients with antibody to rHuCTL2 responded to corticosteroids, whereas 4/8 that lacked antibody to rHuCTL2 did not. Among normal human sera, 80% were negative; binding was barely detectable in 3/15 (20%). Conclusions: The rHuCTL2 protein can be produced efficiently and used as a substrate for testing human sera. Antibodies to rHuCTL2 were detected in 50% of inner-ear–reactive AIHL sera. Additionally, circulating antibody to rHuCTL2 is with associated response to corticosteroids in some AIHL patients. Laryngoscope, 2009

OBJECTIVE To evaluate the usefulness of promontory electric auditory brainstem response (EABR) testing in children with inner ear malformations before cochlear implantation indicated by postoperative speech performance. STUDY DESIGN Retrospective analysis. SETTING Tertiary academic cochlear implant center. PATIENTS Forty-three children with congenital inner ear malformations, of which 39 received a cochlear implant. Age at implant ranged from 12 months to 13 years. Patients were categorized into 3 groups based on the type of malformations. Groups 1 and 2 involved malformations of the cochlea and/or vestibular organ. Group 3 consisted of children with narrow internal auditory canal. INTERVENTIONS Preoperative EABR testing with threshold, Wave V amplitude, and latency determination. MAIN OUTCOME MEASURES Postoperative speech perception performance was measured using Glendonald Auditory Speech Perception Tests for words and sentences, Northwestern University-Children's Perception of Speech test, and minimal pairs test. Patients were further categorized into a speech perception category based on these test results. RESULTS Mean values for EABR threshold, Wave V amplitude, and latency for Group 1 (11 patients) were 485 microA, 0.21 microV, and 4.51 milliseconds, respectively. Mean values for Group 2 (20 patients) were 556 microA, 0.26 microV, and 4.45 milliseconds, respectively. Mean values for Group 3 (8 patients) were 500 microA, 0.11 microV, and 4.65 milliseconds, respectively. Open-set sentence recognition was possible in 73% in Group 1, 30% in Group 2, and 38% in Group 3 by 36 months after implant activation. Patients with lower preoperative EABR threshold (

Choline transporter-like protein 2 (CTL2) is a multi-transmembrane protein expressed on inner ear supporting cells that was discovered as a target of antibody-induced hearing loss. Its function is unknown. A 64 kDa band that consistently co-precipitates with CTL2 from inner ear extracts was identified by mass spectroscopy as cochlin. Cochlin is an abundant inner ear protein expressed as multiple isoforms. Its function is also unknown, but it is suspected to be an extracellular matrix component. Cochlin is mutated in individuals with DFNA9 hearing loss. To investigate the CTL2–cochlin interaction, antibodies were raised to a cochlin-specific peptide. The antibodies identify several cochlin polypeptides on western blots and are specific for cochlin. We show that the heterogeneity of the cochlin isoforms is caused, in part, by in vivo post-translational modification by N-glycosylation and, in part, caused by alternative splicing. We verified that antibody to CTL2 co-immunoprecipitates cochlin from the inner ear and antibody to cochlin co-immunoprecipitates CTL2. Using cochlear cross-sections, we show that CTL2 is more widely distributed than previously described, and its prominent expression on cells facing the scala media suggests a possible role in homeostasis. A prominent but previously unreported ribbon-like pattern of cochlin in the basilar membrane was demonstrated, suggesting an important role for cochlin in the structure of the basilar membrane. CTL2 and cochlin are expressed in close proximity in the inner sulcus, the spiral prominence, vessels, limbus, and spiral ligament. The possible functional significance of CTL2–cochlin interactions remains unknown.

Objective To evaluate hearing outcomes and effects of stimulation rate on performance with the Nucleus Freedom cochlear implant (Cochlear Americas, Denver, CO). Study Design and Setting Randomized, controlled, prospective, single-blind clinical study using single-subject repeated measures (A-B-A-B) design at 14 academic centers in the United States and Canada and comparison with outcomes of a prior device by the same manufacturer. Patients Seventy-one severely/profoundly hearing impaired adults. Results Seventy-one adult recipients were randomly programmed in two different sets of rate: ACE or higher rate ACE RE. Mean scores for Consonant Nucleus Consonant words is 57%, Hearing in Noise Test (HINT) sentences in quiet 78%, and HINT sentences in noise 64%. Sixty-seven percent of subjects preferred slower rates of stimulation, and performance did not improve with higher rates of stimulation using this device. Conclusions Subjects performed well, and there was no advantage to higher stimulation rates with this device. Significance Higher stimulation rates do not necessarily result in improved performance.

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.

Background Sudden sensorineural hearing loss is infrequent, with an estimated incidence of 5 to 20 cases yearly per 100 000 people. Although multiple etiologies have been identified, infection and idiopathic SNHL are most common. Sudden sensorineural hearing loss after nonotologic noncardiac surgery is highly unusual, particularly after spinal surgery. Case Description We report a case of unilateral sudden SNHL after lumbar spinal fusion with review of the literature. Potential etiologies, treatment, and prognosis are summarized. Conclusion Multiple etiologies have been proposed for SNHL after nonotologic noncardiac surgery. Excessive positive upper airway pressure during induction of anesthesia or Valsalva maneuvers can result in SNHL from LMR. Aberrant NO accumulation in the middle ear cavity during general anesthesia with subsequent LMR is also a potential etiology, as is excessive leakage of CSF causing a form of endolymphatic hydrops. There is no definitive treatment of postoperative SNHL, although middle ear exploration may be beneficial for suspected overpressure injury to the middle ear, causing a perilymphatic fistula. Corticosteroids have been beneficial in certain cases of idiopathic SNHL; however, its effectiveness for postoperative SNHL is undocumented.

Objective: To highlight a case of cochlear implantation in the setting of ipsilateral petrous apex chondrosarcoma. Background: A patient with bilateral progressive hearing loss was incidentally found to have a destructive right petrous apex lesion on computed tomography before cochlear implantation. The patient had no associated symptoms and a magnetic resonance imaging scan was obtained, narrowing the differential diagnosis. A middle cranial fossa approach was performed for synchronous biopsy of the lesion and cochlear implantation. Results: Frozen sections revealed a low-grade chondroid lesion, and a Med-El Combi 40+ cochlear implant with a split electrode array was inserted via the middle fossa. Final pathologic examination revealed a Grade I chondrosarcoma. The patient suffered no complications postoperatively and was followed-up over 5years with serial computed tomographic scans and clinical examinations. No additional treatment was administered. Eighteen months postoperatively, the patient experienced episodic vertigo. There were no new findings on computed tomography, and the vertigo improved with a low-salt diet. Otherwise, the patient had excellent hearing results, and the lesion has not progressed under observation. Conclusion: The implications of observing low-grade chondrosarcomas in well-selected patients and the unique aspect of cochlear implantation on the affected side are discussed.

Objective To evaluate surgical results using the middle cranial fossa approach for hearing preservation vestibular schwannoma surgery. Study design Retrospective case review. Setting Tertiary referral academic center. Patients Seventy-three consecutive patients with vestibular schwannoma operated on using the middle cranial fossa approach between February 1999 and February 2005. Interventions The tumors were removed via the middle cranial fossa approach with modifications to improve exposure. Standard auditory brainstem and facial nerve monitoring were used. Main outcome measures Pre- and postoperative hearing measures and facial function, tumor size, and postoperative complications. Hearing status was categorized into Classes A, B, C, and D as described by the American Academy of Otolaryngology-Head and Heck Surgery "Guidelines for the Evaluation of Hearing Preservation in Acoustic Neuroma, 1995." Results Thirty-four patients presented with Class A hearing preoperatively. Among patients presenting with Class A hearing, a total of 27 (80%) maintained Class A or B hearing postoperatively. Of these, 21 (62%) remained in Class A, 6 (18%) deteriorated slightly to Class B, and 7 (20%) deteriorated to Class D postoperatively. Twenty-eight patients presented with Class B hearing preoperatively. Of these, 18 (64%) remained in Class B, 3 (11%) deteriorated to Class C, and 7 (25%) deteriorated to Class D. Three patients had Class C hearing preoperatively. Of these, 2 (66%) remained in Class C and 1 (33%) deteriorated to Class D. Eight patients presented in Class D and one of these improved to Class C postoperatively. Overall, 62 patients presented with useful (Class A or B) hearing and 45 (73%) remained in Class A or B. Nineteen patients had tumors larger than 10 mm in greatest dimension and had Class A or B hearing preoperatively. Of these, 11 (58%) retained Class A or B hearing postoperatively. At 4 months or greater follow-up, facial nerve outcome were excellent in 96%: House-Brackmann Grade I in 61 (85%), Grade II in 8 (11%), and Grade III in 3 (4%). There were no Grade IV, V, or VI results on final follow-up. Six (8%) patients developed cerebrospinal fluid leaks. Conclusion By achieving excellent exposure and using meticulous microsurgical technique, it is possible to resect small vestibular schwannomas via the middle fossa approach, with preservation of hearing at excellent or preoperative levels in the majority of patients, with excellent or satisfactory facial nerve outcomes in 96% of patients.

We analyzed pure-tone and speech audiometric results from a prospective trial of anti-inflammatory treatment of subjects with active autoimmune inner ear disease (AIED). We sought to characterize the pattern and size of the treatment effect as reflected in clinical audiometry and to identify audiometric predictors of response to steroid treatment of AIED.Adult participants demonstrated clinically established criteria for AIED (n = 116). Eligibility required audiometric evidence of active AIED as indicated by idiopathic sensorineural hearing loss with threshold elevations within 3 months of enrollment.We evaluated audiometric changes after 4 weeks of treatment with pharmacologic doses (60 mg/day) of prednisone. We examined the relationship between audiometric pure-tone thresholds at baseline and changes in word intelligibility score (WIS) using parametric and nonparametric analyses. Magnitudes of change were assessed using independent or paired t-tests. Separate analyses were performed on subgroups that did or did not show improved WIS score with steroid treatment.Overall mean pure-tone averages improved from baseline to closeout of prednisone treatment in better hearing ears from 52.4 to 48.3 dB (p.0001). Mean WIS improved in the better ear from 71.4% to 78.1% (p.0001). Of pure-tone measures, only the six-tone average showed significant correlation with both the absolute improvements in WIS and with the percentage change in WIS after treatment. Individual frequencies at baseline showed no significant relationship with changes in WIS score after treatment. In 69 (59.5%) of 116 subjects, WIS improved (range, 2-80%) in the better ear. In these subjects, the baseline pure-tone thresholds and pure-tone averages correlated significantly and positively with improvement in WIS.Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

To describe the presentation, evaluation, and management of patients with geniculate ganglion hemangioma.Retrospective case review.Tertiary-care academic medical center.Six patients with hemangiomas of the geniculate ganglion, evaluated and managed at a single institution.All patients underwent imaging and follow-up, with five of the patients undergoing operative intervention.Tumor size and extent, facial nerve and hearing function, histopathologic findings, and complications are discussed as a function of observation versus microsurgical excision.Six patients with ossifying hemangiomas of the geniculate ganglion were evaluated at a single institution over a 10-year period. These patients underwent operative intervention when their facial nerve function began to decline. Hearing was preserved in five of the six cases. Resection and grafting of the facial nerve was required in five of six cases. Two of the six cases were found to have histologic evidence of facial nerve infiltration on pathologic examination.The majority of these tumors infiltrated the facial nerve and could not be completely removed without excision of the nerve itself. Based on this experience, it would seem reasonable to defer surgical excision until facial nerve function has declined to grade 3 or worse. Whether earlier subtotal excision would result in better outcomes or not remains unanswered. This study presents several complicated scenarios that illustrate the difficulty of clinical decision-making in this disease. Associated clinical dilemmas and controversies are discussed.

Objective: To evaluate surgical techniques and complications associated with external auditory canal (EAC) closure in cochlear implant surgery. Study Design: Retrospective case review. Setting: Tertiary referral center with a large cochlear implant program. Patients: Twenty-eight patients (8 adults and 20 children) underwent multichannel cochlear implantation with EAC closure. The follow-up periods ranged between 1 and 10 years. Closure of the EAC was performed in conjunction with implantation of ears with chronic suppurative otitis media or cochlear drill-out procedures for ossification, or for access to the cochlea in patients with abnormal temporal bone anatomy. A modified Rambo technique was used for EAC closure in all but one case. Interventions: Cochlear implantation with EAC closure and subsequent clinical and radiologic follow-up. Main Outcome Measures: Postoperative complications or the development of cholesteatoma in the implanted ear. Results: Cholesteatoma developed in the implanted ear in two patients. Breakdown of EAC closure occurred in one of these patients. The details of these patients are reviewed. Conclusion: Closure of EAC can be done with relatively low risk. Close and careful follow-up is required for early detection of a develooine cholesteatoma.

Involvement of the superior semicircular canal (SSC) in benign paroxysmal positional vertigo (BPPV) is rare. SSC BPPV is distinguished from the more common posterior semicircular canal (PSC) variant by the pattern of nystagmus triggered by the Dix-Hallpike position: down-beating torsional nystagmus in SSC BPPV versus up-beating torsional nystagmus in PSC BPPV. SSC BPPV may be readily treated at the bedside, which is a key component in excluding central causes of down-beating nystagmus. We present an unusual video case report believed to represent refractory SSC BPPV based on the pattern of nystagmus and the absence of any other central signs.

Objective: Document the use of transmastoid labyrinthectomy to treat disabling vertigo secondary to a lesion in the internal auditory canal. Patient: A 69-year-old man with nonserviceable left hearing experienced disabling attacks of vertigo refractory to medical measures. Magnetic resonance imaging revealed a small left intracanalicular lesion with an irregular configuration and modest enhancement, suggesting either an unusual acoustic neuroma or a cavernoma. Tumor size remained stable on serial imaging, and the patient declined microsurgical resection, stereotactic radiation, or intratympanic gentamicin therapy. Intervention: Transmastoid labyrinthectomy followed by a customized vestibular rehabilitation program. Main Outcome Measure: Comparison of patient symptoms preoperatively and at 5 and 8 months after surgery. Results: Complete relief of vertigo was achieved, but the patient has moderate imbalance postoperatively. Conclusion: Transmastoid labyrinthectomy alone may be a viable treatment option in patients with an internal auditory canal neoplasm causing disabling attacks of vertigo.

Objective To determine whether long-term vestibular compensation (VC) and clinical outcomes differ after transmastoid labyrinthectomy (TML) versus retrolabyrinthine vestibular neurectomy (RVNS). Study Design Prospective, observational study. Setting Tertiary care, university hospital. Patients Twenty-one subjects were studied several years after they were relieved of spontaneous episodic vertigo caused by peripheral vestibular disease by TML or RVNS. Interventions All patients had undergone TML or RVNS more than 2.5 years before the study and returned for physiologic and functional studies of vestibular compensation. Main Outcome Measures Completeness of physiologic VC, as assessed by electronystagmography and rotational chair testing; performance on computerized dynamic posturography; pure-tone and speech audiometry; self-assessment of balance and hearing function with validated survey instruments. Results There were no differences in the incidence of physiologic VC or functional recovery between the TML and RVNS subjects. Although a majority of subjects in each group had evidence of incomplete vestibular compensation, there was no difference in self-assessment of balance or hearing handicap at long-term follow-up. Conclusions Long-term clinical balance and hearing outcomes are equivalent when TML and RVNS successfully cure spontaneous, episodic vertigo. There is a high incidence of incomplete VC after both procedures, though this does not usually produce a significant balance handicap.

Disorders affecting the peripheral vestibular system commonly involve the peripheral auditory system causing hearing loss. There are a number of disorders, however, that selectively involve the peripheral vestibular system causing dizziness without hearing loss. These disorders include benign paroxysmal positional vertigo, vestibular neuritis, recurrent vestibulopathy, familial vestibulopathy, and bilateral idiopathic vestibulopathy. This article reviews these disorders and their diagnosis and management.

Objective To determine the risk of recurrent tumor in patients after incomplete resection of acoustic neuroma. Study design Retrospective case review. Setting A tertiary referral center. Patients Thirty-nine patients were identified who underwent incomplete resection of acoustic neuroma between January 1988 and December 1993, and had a minimum follow-up of 3.5 years, at a mean of 6.2 years (range 3.5-10.2 years). Intervention Regular follow-up. using either computed axial tomography or magnetic resonance imaging yearly. Main outcome measures Growth of residual tumor as determined by the increase in its greatest dimension on follow-up imaging studies and the necessity for additional treatment. Results Tumor regrowth occurred in 17 patients (44%). Ten patients (26%) required additional treatment during the follow-up period. Conclusion This study suggests that incomplete resection of acoustic neuroma is associated with a significant risk of recurrent tumor requiring subsequent intervention.

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.1 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. Key Words: Mondini, sensorineural, hearing loss, familial, inner ear.

Using two-dimensional (2-D) gel electrophoresis, human perilymph and cerebrospinal fluid have been shown to be highly enriched for an acidic protein with Mr 30 000, we designated it as AP30. The protein exhibits charge heterogeneity, with at least eight isoforms visible between pI 4.5 to 5.5 on 2-D gels. Purification of the protein was carried out by ammonium sulfate precipitation, polybuffer exchanger column chromatofocusing, and acetone fractional precipitation. The resulting preparation also contains eight spots in the acidic area of 2-D gels, and one broad band located at Mr 30 000 by SDS-PAGE. Digestion of AP30 with neuraminidase causes the isoforms to shift to a more basic position and to consolidate into two primary spots, indicating that AP30 is a variably sialylated glycoprotein. Amino acid analysis of AP30 revealed an amino acid content very similar to that of human apolipoprotein D. Attempts to determine the amino acid sequence demonstrated that the N-terminus is blocked. Edman sequencing of two peptide fragments, generated by cyanogen bromide cleavage of AP30, both revealed sequences having 100% identity to human apolipoprotein D. Western blot analysis of AP30 with the antibody against authentic human apolipoprotein D demonstrated a high degree of cross-reactivity. These studies indicate that AP30 from human perilymph and cerebrospinal fluid is a member of the apolipoprotein D family.