Making a racket in America's fastest growing sport: Evaluation of noise exposure in pickleball.

OBJECTIVE: To measure noise exposure present on pickleball courts and assess the risk of noise-induced hearing loss (NIHL) per guidelines put forward by the National Institute of Occupational Safety and Health (NIOSH). METHODS: Observational study measuring noise levels at multiple recreational pickleball courts in the Richmond, VA area, documenting LAeq, LASmax, and LCpeak at courtside and waiting areas of pickleball courts. Measurements were completed using the NIOSH SLM application on an iPhone 13 with iMM-6 Calibrated Measurement Microphone (equivalent to IEC 61672-1 Class II) that was calibrated using ND-9 Sound Level Calibrator (IEC942 Class I). RESULTS: Average sound levels recorded at waiting areas adjacent to the courts, measured in LAeq, LASmax, and LCpeak, were 69.1 dBA, 92.0 dBA, and 112.1 dBC, respectively, while courtside measurements were 69.7 dBA, 92.2 dBA, and 115.6 dBC, respectively. These measurements were within NIOSH and OSHA recommendations. CONCLUSION: The data demonstrates that randomly sampled pickleball courts have noise levels that do not increase risk for NIHL for participants or bystanders alike based on NIOSH guidelines. However, prolonged noise exposure and ambient noise pollution may have other health implications and warrant further investigations. LEVEL OF EVIDENCE: Level 2.

Intraoperative Correction of Cochlear Implant Electrode Translocation.

INTRODUCTION: Translocation of precurved cochlear implant (CI) electrodes reduces hearing outcomes, but it is not known whether it is possible to correct scalar translocation such that all electrodes reside fully in the scala tympani (ST). METHODS: Six cadaveric temporal bones were scanned with CT and segmented to delineate intracochlear anatomy. Mastoidectomy with facial recess was performed. Precurved CI electrodes (CI532; Cochlear Limited) were implanted until scalar translocation was confirmed with postoperative CT. Then, electrodes were removed and replaced. CT scan was repeated to assess for translocation correction. Scalar position of electrode contacts, angular insertion depth (AID) of the electrode array, and M- (average distance between each electrode contact and the modiolus) were measured. An in vivo case is reported in which intraoperative translocation detection led to removal and replacement of the electrode. RESULTS: Five of 6 cadaveric translocations (83%) were corrected with 1 attempt, resulting in full ST insertions. AID averaged 285 ± 77° for translocated electrodes compared to 344 ± 28° for nontranslocated electrodes (p = 0.109). M- averaged 0.75 ± 0.18 mm for translocated electrodes and 0.45 ± 0.11 mm for nontranslocated electrodes (p = 0.016). Reduction in M- with translocation correction averaged 38%. In the in vivo case, translocation was successfully corrected in a single attempt. CONCLUSION: Scalar translocation of precurved CI electrodes can be corrected by removal and reinsertion. This significantly improves the perimodiolar positioning of these electrodes. There was a high rate of success (83%) in this cadaveric model as well as a successful in vivo attempt.

Predictors of sleep paralysis and relationship of sleep paralysis with sleep quality in university students of Islamabad.

OBJECTIVE: To determine the frequency of sleep paralysis among university students, and to determine the association between sleep paralysis and sleep quality along with different predictive factors leading to sleep paralysis. METHODS: The descriptive cross-sectional study was conducted at the National University of Modern Languages, Islamabad, Pakistan, from June to October, 2019, and comprised adult students of either gender who had experienced at least one episode of sleep paralysis in the past. Demographic variables were obtained using Google Form, while experiences of sleep paralysis were assessed using the Waterloo Unusual Sleep Experience Questionnaire, and sleep quality was assessed with the Sleep Condition Indicator. Data was analysed using SPSS 20. RESULTS: Initially, 440 students were assessed/ Of them, 233(53%) were males, 207(47%) were females, 208(47.3%) were aged 20-23 years, and 129(29.3%) were studying in the third year of their university programme. Of the total, 130(29.5%) subjects had experienced sleep paralysis at least once in their lifetime. The most frequent sensation experienced was feeling of floating 22(16.9%). Mean age at the time of the first experience was 9.7±3.1 years, and 66(50.8%) had experienced the episodes while lying on their back, while 32(24.6%) had positive family history of sleep paralysis. Sleep paralysis had significant relationship with sleep quality (p<0.001). CONCLUSIONS: About one-third of the sample had experienced sleep paralysis once in their lifetime. The relationship between sleep paralysis and insomnia was significant.

Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Morphology and cochlear implantation in enlarged vestibular aqueduct.

PURPOSE: The purpose of this work is to explore audiometry following cochlear implantation (CI) in patients with enlarged vestibular aqueduct (EVA) and to investigate the effects of inner ear morphological variation on post CI audiometry. METHODS: This was a retrospective review of both natural and cochlear-implant-aided audiometry results, using all available measurements in a mixed-effects model accounting for longitudinal change and the grouping structure of ears. Patients who visited our tertiary academic medical center between 2000 and 2016 were identified as having EVA according to Cincinnati criteria on radiological examination; patients eligible for CI were then selected for analysis. RESULTS: Multivariable modeling showed a statistically significant hearing improvement in ears with EVA undergoing CI with regards to pure tone average (-64.0 dB, p < 0.0001), speech reception threshold (-57.90 dB, p < 0.0001), and word score (34.8%, p > 0.0001). Vestibular aqueduct midpoint size and the presence of incomplete partition type II (IP II) did not have significant independent associations with audiometric findings. However, multivariable modeling revealed a statistically significant interaction between IP II and CI such that IP II ears demonstrated a decrease in WS improvement of 30.2% (p = 0.0059) compared to non-IP II ears receiving CI. CONCLUSION: There is a statistically significant audiometric benefit to ears with EVA receiving CI. Morphology, specifically the presence of IP II, may hinder CI benefit in terms of word score however this finding needs clinical validation. This data improves personalization of surgical counseling and planning for patients with EVA considering CI.

Spontaneous resolution of hypercalcemia.

BACKGROUND: Primary hyperparathyroidism (PHPT) is a frequently encountered endocrine disorder due to benign neoplastic lesions or gland hyperplasia. It is often discovered incidentally when routine lab work reveals hypercalcemia. METHODS: This case presents a 55-year-old male with a neck mass and electrolyte irregularities consistent with PHPT. However, his laboratory values suddenly normalized prior to surgery. RESULTS: Post-operative pathologic analysis of the specimen demonstrated massive infarction of the affected gland, and explained the spontaneous resolution of the patient's electrolyte derangements. CONCLUSIONS: The objective of this case study is to demonstrate the importance of further investigation in patients with fluctuating lab values and emphasize the potential dangers of gland infarction.

Long-term outcomes of endolymphatic sac shunting with local steroids for Meniere's disease.

OBJECTIVES: To evaluate the long-term efficacy of endolymphatic sac shunt techniques with and without local steroid administration. STUDY DESIGN: Retrospective case series and patient survey. SETTING: Tertiary university hospital. PATIENTS: Meniere's disease (MD) patients that failed medical therapy and subsequently underwent an endolymphatic sac shunt procedure. All patients had definitive or probable MD and at least 18-months of follow-up. INTERVENTIONS: Three variations on endolymphatic sac decompression with shunt placement were performed: Group A received no local steroids, Group B received intratympanic dexamethasone prior to incision, and Group C received dexamethasone via both intratympanic injection and direct endolymphatic sac instillation. MAIN OUTCOME MEASURE(S): Vertigo control, hearing results, and survey responses. RESULTS: Between 2002 and 2013, 124 patients with MD underwent endolymphatic sac decompression with shunt placement. 53 patients met inclusion criteria. Groups A, B, and C had 6 patients, 20 patients, and 27 patients, respectively. Mean follow-up was 56months. Vertigo control improved in 66%, 83%, and 93% of Groups A, B, and C. Functional level improved for Group B (-2.0) and Group C (-2.2) but was unchanged in Group A. Pure-tone average and speech discrimination scores changed by +22dB and -30%, +6dB and -13%, and +6dB and -5% in Groups A, B, and C. The long-term hearing results were significantly better with steroids (Groups B and C) according to the AAO-HNS 1995 criteria but did not meet significance on non-parametric testing. CONCLUSIONS: Endolymphatic sac shunt procedures may benefit from steroid instillation at the time of shunt placement.

Hearing loss in enlarged vestibular aqueduct and incomplete partition type II.

PURPOSE: The purpose of this work is to identify the role of incomplete partition type II on hearing loss among patients with enlarged vestibular aqueduct (EVA). BACKGROUND: EVA is a common congenital inner ear malformation among children with hearing loss, where vestibular aqueduct morphology in this population has been shown to correlate to hearing loss. However, the impact of incomplete partition between cochlear turns on hearing loss has not been, despite meaningful implications for EVA pathophysiology. METHODS: A retrospective review of radiology reports for patients who had computed tomography (CT) scans with diagnoses of hearing loss at a tertiary medical center between January 2000 and June 2016 were screened for EVA. CT scans of the internal auditory canal (IAC) for those patients with EVA were examined for evidence of incomplete partition type II (IP-II), measurements of midpoint width and operculum width a second time, and patients meeting Cincinnati criteria for EVA selected for analysis. Statistical analysis including chi-square, Wilcoxon rank-sum, and t-tests were used to identify differences in outcomes and clinical predictors, as appropriate for the distribution of the data. Linear mixed models of hearing test results for all available tests were constructed, both univariable and adjusting for vestibular aqueduct morphometric features, with ear-specific intercepts and slopes over time. RESULTS: There were no statistically significant differences in any hearing test results or vestibular aqueduct midpoint and operculum widths. Linear mixed models, both univariable and those adjusting for midpoint and operculum widths, did not indicate a statistically significant effect of incomplete partition type II on hearing test results. CONCLUSIONS: Hearing loss due to enlarged vestibular aqueduct does not appear to be affected by the presence of incomplete partition type II. Our results suggest that the pathophysiological processes underlying hearing loss in enlarged vestibular aqueduct may not be a result of cochlear malformation, and instead are more likely to involve vestibular aqueduct or cellular and molecular-level mechanisms of hearing loss.

Successful endovascular management of venous sinus thrombosis complicating trans-labyrinthine removal of vestibular schwanomma.

Cerebral venous sinus thrombosis (CVST) is a rare complication of surgical treatment of vestibular schwanomma. We present a rare case of extensive venous sinus thrombosis after trans-labyrinthine approach that was refractory to systemic anti-coagulation. Mechanical aspiration thrombectomy was utilized to re-canalize the venous sinuses and resulted in successful resolution of neurological symptoms. Indications of utilizing endovascular approaches are discussed that will enable skull base surgeons to address this uncommon yet potentially fatal complication.

Functional Outcome of Complex Elbow Fracture Managed With the Boyd Approach.

INTRODUCTION: The Boyd approach allows excellent access to the elbow and is used to treat complex elbow injuries using a single incision approach. MATERIALS AND METHODS: In this study, we retrospectively evaluated 16 patients with complex elbow injuries treated with open reduction and internal fixation using the Boyd approach between 2016 and 2018. RESULTS: All fractures were well united in anatomical position. Postoperatively, the range of motion was not significantly different between the affected and unaffected elbows. The mean Mayo Elbow Performance Index score was 95 ± 5 (range 90 to 100). All study participants had satisfactory results and recovered to full activity. There was no incidence of posttraumatic arthritis of the elbow joint or synostosis of the radius and ulna. CONCLUSION: Thus, according to our study, the Boyd elbow approach is a safe and effective method of treating elbow injuries.

Endoscopic-Assisted Presigmoid Approach to the Internal Auditory Canal. A Feasibility Study.

HYPOTHESIS: The retrolabyrinthine (presigmoid) approach has been utilized in various skull base surgeries but has not been fully utilized in the management of internal auditory canal (IAC) lesions, such as vestibular schwannoma (VS). Microsurgical retrolabyrinthine approach provides limited visualization of the IAC, while endoscopic-assisted techniques allow for further lateral exposure with labyrinthine preservation. BACKGROUND: Traditional approaches to the IAC have the disadvantage of hearing sacrifice or retraction of brain tissue. With the introduction of endoscopic techniques and enhanced visualization, access to this region of complex anatomy is possible. METHODS: Radiomorphometric and anatomical dissection was performed on two cadaveric temporal bones. High-resolution computed tomography was used to segment and delineate the volume of the IAC. Projected accessible IAC was compared to actual postdissection data with preservation of the posterior semicircular canal (PSCC) via the retrolabyrinthine corridor. RESULTS: While preserving the PSCC, the 0° and 30° endoscopes visualized 57.1% and 78.6% of the IAC for cadaver 1, and 64.0% and 76.0% of the IAC for cadaver 2, respectively. Sacrificing the PSCC, the 0° and 30° endoscopes provided visualization of 78.6% 85.7% of the IAC for cadaver 1, and 88.0% and 95.1% of the IAC for cadaver 2, respectively. CONCLUSIONS: Retrolabyrinthine approach to resection of VS is a potentially viable hearing-preserving alternative to traditional approaches. This approach provides access to the majority of the IAC, while angled endoscopes or sacrifice of the PSCC can provide additional access toward the fundus. Further studies are needed to determine the clinical feasibility of this approach.

Outcomes of Near-Total and Subtotal Resection of Sporadic Vestibular Schwannoma: A Systematic Review and Meta-Analysis.

OBJECTIVE: To evaluate tumor control and facial nerve outcomes after gross-total (GTR), near-total (NTR), and subtotal resection (STR) of sporadic vestibular schwannomas (VS). DATA SOURCES: PubMed, Cochrane Central Register of Controlled Trials, Web of Science, and Scopus databases were searched in August 2021 through inception following PRISMA guidelines. REVIEW METHODS: English language articles reporting tumor control and facial nerve outcomes of adults (≥18 years) with NTR and STR of VS were evaluated. Study characteristics, demographics data, tumor characteristics, type of surgical intervention, and outcome measures on tumor control and facial nerve function were collected. Pooled relative risk (RR) estimates for tumor recurrence and facial nerve outcomes were calculated and stratified by extent of resection. RESULTS: From an initial search of 2504 articles, 48 studies were included in the analysis. When comparing 1108 patients who underwent NTR to 3349 patients with GTR, the pooled RR of recurrence in the NTR cohort was 2.94 (95% confidence interval [CI] 1.65-5.24, P = .0002). When comparing 1016 patients who underwent STR to 6171 patients with GTR, the pooled RR of recurrence in the STR cohort was 11.50 (95% CI 6.64-19.92, P < .0001). Estimates for risk of tumor regrowth for less-than-complete resection are presented. There was no elevated risk of adverse facial nerve outcome (defined as House-Brackmann grade III and above) in each category of extent of resection compared to GTR. CONCLUSION: Extent of resection predicts risk of tumor recurrence/regrowth following microsurgical resection. Favorable facial nerve outcome should be weighed against the increased risk of regrowth and the potential need for further treatment.

Increased Incidence of Vestibular Disorders in Patients With SARS-CoV-2.

Objective: Determine the incidence of vestibular disorders in patients with SARS-CoV-2 compared to the control population. Study Design: Retrospective. Setting: Clinical data in the National COVID Cohort Collaborative database (N3C). Methods: Deidentified patient data from the National COVID Cohort Collaborative database (N3C) were queried based on variant peak prevalence (untyped, alpha, delta, omicron 21K, and omicron 23A) from covariants.org to retrospectively analyze the incidence of vestibular disorders in patients with SARS-CoV-2 compared to control population, consisting of patients without documented evidence of COVID infection during the same period. Results: Patients testing positive for COVID-19 were significantly more likely to have a vestibular disorder compared to the control population. Compared to control patients, the odds ratio of vestibular disorders was significantly elevated in patients with untyped (odds ratio [OR], 2.39; confidence intervals [CI], 2.29-2.50; P < 0.001), alpha (OR, 3.63; CI, 3.48-3.78; P < 0.001), delta (OR, 3.03; CI, 2.94-3.12; P < 0.001), omicron 21K variant (OR, 2.97; CI, 2.90-3.04; P < 0.001), and omicron 23A variant (OR, 8.80; CI, 8.35-9.27; P < 0.001). Conclusions: The incidence of vestibular disorders differed between COVID-19 variants and was significantly elevated in COVID-19-positive patients compared to the control population. These findings have implications for patient counseling and further research is needed to discern the long-term effects of these findings.

Doxycycline-Induced Intracranial Hypertension Presenting as Unilateral Pulsatile Tinnitus.

Background: Pulsatile tinnitus (PT) is increasingly recognized as a cardinal symptom of idiopathic intracranial hypertension (IIH). However, clinicians should remain aware of other causes of nonidiopathic or secondary intracranial hypertension manifesting as PT. We present 2 patients with isolated PT (without accompanying headache, blurred vision, and papilledema) thought to be secondary to tetracycline-induced intracranial hypertension. To our knowledge, these are the first cases of PT as the presenting symptom of this condition. Cases: A 41-year-old female (body mass index [BMI] 29 kg/m2) with ocular rosacea was initially treated with minocycline. Shortly after transitioning to oral doxycycline and erythromycin eye ointment, she noted left-sided PT. Her PT resolved after discontinuing doxycycline. In a second case, a 39-year-old female (BMI 19 kg/m2) with acne presented with a three-year history of left-sided PT while on long-term oral doxycycline for many years. She denied visual or auditory changes and atypical headaches. MRI findings were concerning for intracranial hypertension. Three months later, the patient was seen by neuro-ophthalmology, with findings suggesting prior papilledema. The patient reported PT improvement after discontinuing doxycycline. Conclusions: This case series highlights 2 cases of isolated PT as the sole symptom of intracranial hypertension that resolved with tetracycline cessation. The presentation and unexpected improvement following tetracycline discontinuation are atypical compared with previous reports of tetracycline-induced intracranial hypertension. Clinicians should maintain a high index of suspicion for all types of intracranial hypertension (idiopathic and secondary), even in patients with a lower BMI. Current and prior medications should be reviewed when considering the etiology of intracranial hypertension.

Temporal Bone Resection for Squamous Cell Carcinoma of the Lateral Skull Base: Systematic Review and Meta-analysis.

OBJECTIVE: Temporal bone squamous cell carcinoma (TBSCC) is rare and often confers a poor prognosis. The aim of this study was to synthesize survival and recurrence outcomes data reported in the literature for patients who underwent temporal bone resection (TBR) for curative management of TBSCC. We considered TBSCC listed as originating from multiple subsites, including the external ear, parotid, and external auditory canal (EAC), or nonspecifically from the temporal bone. DATA SOURCES: PubMed, Cochrane Library, Embase, and manual search of bibliographies. REVIEW METHOD: A systematic literature review conducted in December 2020 according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: Survival data were collected from 51 retrospective studies, resulting in a pooled cohort of 501 patients with TBSCC. Compared to patients undergoing lateral TBR (LTBR), patients undergoing subtotal (SBTR) or total (TTBR) TBR exhibited significantly higher rates of stage IV disease (P < .001), positive surgical margins (P < .001), facial nerve involvement (P < .001), and recurrent disease (P < .001). A meta-analysis of 15 studies revealed a statistically significant 97% increase in mortality in patients who underwent STBR or TTBR. On multivariate analysis, recurrent disease was independently associated with worse overall survival (P < .001). On univariate analysis, facial nerve involvement was also associated with decreased overall survival (P < .001). CONCLUSION: Recurrent disease was associated with risk of death in patients undergoing TBR. Larger prospective multi-institutional studies are needed to ascertain prognostic factors for a wider array of postoperative outcomes, including histology-specific survival and recurrence outcomes.

Reducing ICU Length of Stay: The Impact of a Multidisciplinary Perioperative Pathway in Vestibular Schwannoma.

Objective This study was aimed to evaluate the impact of a multidisciplinary perioperative pathway on length of stay (LOS) and postoperative outcomes after vestibular schwannoma surgery. Setting This study was conducted in a tertiary skull base center. Main Outcome Measures The impact of the pathway on intensive care unit (ICU) LOS was evaluated as the primary outcome measure of the study. Overall resource LOS, postoperative complications, and readmission rates were also evaluated as secondary outcome measures. Methods Present study is a retrospective review. Results A universally adopted perioperative pathway was developed to include standardization of preoperative education and expectations, intraoperative anesthetic delivery, postoperative nursing education, postoperative rehabilitation, and utilization of stepdown and surgical floor units after ICU stay. Outcomes were measured for 95 consecutive adult patients who underwent surgical resection for vestibular schwannoma (40 cases before implementation of the perioperative pathway and 55 cases after implementation). There were no significant differences in the two groups with regard to tumor size, operative time, or medical comorbidities. The mean ICU LOS decreased from 2.1 in the preimplementation group to 1.6 days in the postimplementation group ( p = 0.02). There were no significant differences in overall resource LOS postoperative complications or readmission rates between groups. Conclusion Multidisciplinary, perioperative neurotologic pathways can be effective in lowering ICU LOS in patients undergoing vestibular schwannoma surgery without compromising quality of care. Further research is needed to continue to sustain and continuously improve these and other measures, while continuing to provide high-quality care to this patient population.

Comparative Analysis of Recidivism After Endoscopic and Microscopic-Based Cholesteatoma Resection.

OBJECTIVES: 1) To analyze outcomes of cholesteatoma resection utilizing postauricular microscopic and endoscopic ear surgery (EES) approaches.2) To analyze predictors of residual and recurrent cholesteatoma. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Three hundred seventy-five adult and pediatric patients with cholesteatoma (2012-2017). INTERVENTIONS: Patients underwent surgical resection of cholesteatoma with EES (n = 122) and microscopic (n = 253) approach. MAIN OUTCOME MEASURES: Residual cholesteatoma, recurrent cholesteatoma, second-look procedures. RESULTS: The endoscopic cohort included significantly more pediatric cases (p = 0.0008). There was no difference in laterality, gender distribution, congenital or acquired cholesteatoma, and revision cases between the cohorts. Out of 122 EES cases, 16 (13%) developed residual disease and 9 (7%) developed recurrent disease. Of 253 microscopic cases 16 (6%) developed residual disease while 11 (4%) developed recurrent disease. Second look procedures were more commonly used in EES cohort (50 vs 18%). Single predictor analysis revealed 12 predictors for residual disease and 5 for recurrent disease. Multivariable model identified pediatric case distribution and higher disease stage to be significant predictors for both residual (p = 0.04, 0.007) and recurrent disease (p = 0.02, 0.01). EES approach was associated with a weak significance for residual disease (p = 0.049) but not recurrent disease (p = 0.34). CONCLUSIONS: EES approach for cholesteatoma resection seems to perform similarly to microscopic approach with no difference in rates of recurrent disease. However, it is associated with a higher rate of residual disease; this may be a reflection of a greater rate of second look procedures done in this group.

Hearing loss patterns in enlarged vestibular aqueduct syndrome: Do fluctuations have clinical significance?

OBJECTIVES: To reveal hearing loss patterns in patients with enlarged vestibular aqueduct (EVA) syndrome according to demographic and clinical characteristics. METHODS: A retrospective, longitudinal study design was utilized to identify patients with EVA. Ears of patients were categorized into one of four cohorts: progressive fluctuating, progressive non-fluctuating, stable fluctuating, and stable non-fluctuating patterns. Pairwise and group comparisons were performed with non-parametric tests to assess vestibular aqueduct (VA) morphology, clinical, and demographic variables between hearing loss pattern cohorts. Rates of hearing loss in the subgroups were determined utilizing a mixed linear effects model. RESULTS: 44 patients (25 female, 19 male, median diagnosis age: 8.06 years) met inclusion criteria. 16 individuals demonstrated unilateral EVA and 29 had bilateral EVA, resulting in 74 total ears with EVA. Amongst the four cohorts, differences in operculum widths amongst groups were statistically significant (p = 0.049) while VA midpoint widths were not (p = 0.522). Progressive hearing loss ears without fluctuations demonstrated a 3.20 dB per year (p < 0.001) progression while progressive hearing loss ears with fluctuations reported a rate of 3.52 dB loss per year (p < 0.001). CONCLUSION: Hearing fluctuations occur similarly in EVA patients with stable and progressive hearing loss. With the exception of increased rates of hearing loss progression for fluctuating progressive hearing loss patients, vestibular aqueduct morphology, demographic, and clinical characteristics commonly reported are likely not strong predictors for whether patients will or will not experience fluctuating patterns of hearing loss.

Predictive Modeling and Risk Stratification of Patients With Enlarged Vestibular Aqueduct.

OBJECTIVES/HYPOTHESIS: To investigate patient-specific characteristics that independently predict for progressive hearing loss in patients with enlarged vestibular aqueduct (EVA). Utilize multivariable predictive models to identify subgroups of patients with significantly different progression risks. STUDY DESIGN: Retrospective analysis of patients evaluated at an academic tertiary care center. Cohort included 74 ears of patients with a diagnosis of EVA as defined by the Cincinnati criteria. METHODS: Hearing trajectories were characterized, and a Kaplan-Meier estimator was utilized to determine progressive phenotype probabilities across the first 10 years after diagnosis. Cox proportional hazard regression was used to identify patient characteristics that independently altered this probability. Stratified risk groups were delineated from generated nomogram scores. RESULTS: Male gender was associated with a 4.53 hazard ratio for progressive hearing loss (95% confidence interval [CI], 2.53 to 12.59). Each millimeter increase in operculum size was independently associated with an 80.40% increase in expected hazard (95% CI, 40.18 to 120.62). Each dB increase in air pure tone average at time of diagnosis decreased expected hazard by 1.59% (95% CI, -3.02 to -0.17). The presence of incomplete partition type II was associated with a 2.44 hazard ratio (95% CI, 1.04 to 5.72). Risk groups stratified by median nomogram score evidenced the discriminative ability of our model with the progression probability in the high-risk group being six times higher at 1 year, nearly five times greater at 3 years, and three times greater at 9 years. CONCLUSIONS: EVA patient characteristics can be used to predict hearing loss probability with a high degree of accuracy (C-index of 0.79). This can help clinicians make more proactive management decisions by identifying patients at high risk for hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1439-1445, 2022.