Single-cell multi-omic analysis of the vestibular schwannoma ecosystem uncovers a nerve injury-like state.

Vestibular schwannomas (VS) are benign tumors that lead to significant neurologic and otologic morbidity. How VS heterogeneity and the tumor microenvironment (TME) contribute to VS pathogenesis remains poorly understood. In this study, we perform scRNA-seq on 15 VS, with paired scATAC-seq (n = 6) and exome sequencing (n = 12). We identify diverse Schwann cell (SC), stromal, and immune populations in the VS TME and find that repair-like and MHC-II antigen-presenting SCs are associated with myeloid cell infiltrate, implicating a nerve injury-like process. Deconvolution analysis of RNA-expression data from 175 tumors reveals Injury-like tumors are associated with larger tumor size, and scATAC-seq identifies transcription factors associated with nerve repair SCs from Injury-like tumors. Ligand-receptor analysis and in vitro experiments suggest that Injury-like VS-SCs recruit myeloid cells via CSF1 signaling. Our study indicates that Injury-like SCs may cause tumor growth via myeloid cell recruitment and identifies molecular pathways that may be therapeutically targeted.

Relationship between clinical measures of hearing and clinical measures of vestibular function.

PURPOSE: Patients often have basic audiometry (BA) but not objective diagnostic tests of the vestibular system (VNG) when complaining of symptoms of a vestibular disorder. The relationship of BA results to VNG results is unknown. This study sought to determine if BA scores are related to impaired VNG scores. MATERIALS AND METHODS: We reviewed electronic medical records at a tertiary care center, for patients seen between 2015 and 2021 who had had both a BA and a VNG (n = 651). BA subtests were pure tone averages, word recognition, and tympanogram. VNG subtests were cervical vestibular evoked myogenic potentials, Dix-Hallpike maneuvers, and bi-thermal caloric tests. All tests were summarized as normal/abnormal. RESULTS: More subjects had abnormal BA than abnormal VNG scores. Age but not sex was significantly related to abnormal scores. High BP was a significant comorbidity in 15 % of the sample, more in patients with abnormal than normal VNG scores. Although the abnormal BA and abnormal VNG were significantly related, pure tone averages and tympanogram scores were not related to VNG subtests. Abnormal word recognition with both ears combined was significantly related to normal and abnormal bi-thermal caloric tests. CONCLUSIONS: If the clinician needs to know of any VNG impairment, in general, then performing a BA without a VNG might suffice. If the clinician needs information about the details of possible vestibular impairment, then a VNG should be performed.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Approach to Cochlear Implantation in Patients With Ventriculoperitoneal Shunts.

OBJECTIVE: To evaluate the safety and outcomes of cochlear implantation (CI) in patients with ventriculoperitoneal (VP) shunts to inform clinical practice. STUDY DESIGN: Historical cohort study. SETTING: Tertiary referral centers. METHODS: A multi-institutional historical cohort of patients with VP shunts and CI was identified and analyzed. RESULTS: A total of 46 patients (median age 8 years [interquratile range, IQR: 2-46]) with VP shunts and CI were identified. Of these, 41 (89%) patients had a VP shunt prior to CI. Based on institutional preference and individual patient factors, CI was performed contralateral to a pre-existing VP shunt in 24 of these 41 cases (59%) and ipsilateral in 17 (41%). Furthermore, pre-CI relocation of the VP shunt was performed in 3 cases (7%), and 2 patients (5%) underwent planned revision of their VP shunt concurrent with CI. In total, 2 of 27 pediatric patients (7%) required unanticipated revision shunt surgery, both contralateral to CI device placement, given VP shunt malfunction. One of 19 adult patients (5%) required shunt revision during CI due to shunt damage noted intraoperatively. Among 43 patients with available follow-up, 38 (88%) are regular CI users, with a median consonant-nucleus vowel-consonant word: score of 58% (IQR: 28-72). CONCLUSION: CI can be performed at low risk, either contralateral or ipsilateral, to a VP shunt, and does not mandate shunt revision in most cases. Additional considerations regarding CI receiver-stimulator placement are necessary with programmable shunts to mitigate device interaction. Preoperative planning, including coordination of care with neurosurgery, is important to achieving optimal outcomes.

Quality of Life After Modified Rambo Ear Canal Closure in Pediatric and Adult Patients.

OBJECTIVE: To present the surgical and quality of life (QOL) outcomes of patients who underwent blind sac closure of the external auditory canal (EAC) via a modified Rambo approach. STUDY DESIGN: Retrospective case review. SETTING: Tertiary academic referral center. METHODS: All patients who underwent EAC closure with a modified Rambo approach between 2015 and 2021 were evaluated. Complication rates, QOL estimations from a validated survey, and subjective cosmetic reports were analyzed. RESULTS: Thirty-five ears were closed in 31 patients. The most common indication for surgery was related to cochlear implantation and cochlear malformation or cholesteatoma (31.4%). No case involved an immediate complication requiring revision surgery, and 4 ears (11.4%) were suspected of having cholesteatoma within the surgical cavity at a mean 28.6-month follow-up. Adults (≥18 years) had significantly higher QOL scores than children in the medical resource subscale of the Chronic Ear Survey (P < .01), and patients undergoing concurrent EAC closure and skull base tumor removal scored higher than others (P = .04). Females reported higher cosmetic scores than males (P = .04). QOL and cosmetic scores compared favorably to previously published data involving the management of otologic disease. CONCLUSIONS: Ear canal closure can be a useful technique for select adult and pediatric patients. Patient and surgeon concerns regarding QOL and cosmesis in ear canal closure should be explored prior to employing this surgical technique, though the present data suggest that the modified Rambo approach to closure is generally associated with reasonable outcomes in both areas.

Monopolar Cautery Use in Pediatric Cochlear Implant Users.

OBJECTIVES: To determine the incidence and impact of monopolar cautery use in a cohort of pediatric cochlear implant (CI) users. STUDY DESIGN: Case series from a retrospective chart review and a systematic review of the literature. SETTING: Tertiary academic referral center. METHODS: CI patient charts from 2012 to 2021 were reviewed from a single pediatric hospital system to determine if monopolar cautery was used during a subsequent surgical procedure. In addition, a systematic review of the literature was performed to identify additional, relevant patients. Postoperative CI function was the primary outcome measure. RESULTS: In total, 190 patients underwent a surgical procedure following cochlear implantation in a single pediatric hospital system. Fifteen patients (7.9%) and 17 distinct surgical procedures were identified in which monopolar cautery was used. Seven of these 17 cases (41.2%) involved the head and neck, and 10 were performed below the clavicles. No patients experienced a device failure or a decline in CI performance following surgery. A systematic review identified an additional 4 patients who underwent a surgery that used monopolar cautery following cochlear implantation, and no change in CI function was identified. CONCLUSIONS: The present study adds additional support to the notion that monopolar cautery does not necessarily injure CI functionality. While the most risk adverse strategy when planning a surgical procedure for a CI patient is to avoid monopolar cautery use altogether, the use of cautery should not immediately be associated with implant dysfunction.

Predicting surgical decision-making in vestibular schwannoma using tree-based machine learning.

OBJECTIVE: Vestibular schwannomas (VSs) are the most common neoplasm of the cerebellopontine angle in adults. Though these lesions are generally slow growing, their growth patterns and associated symptoms can be unpredictable, which may complicate the decision to pursue conservative management versus active intervention. Additionally, surgical decision-making can be controversial because of limited high-quality evidence and multiple quality-of-life considerations. Machine learning (ML) is a powerful tool that utilizes data sets to essentialize multidimensional clinical processes. In this study, the authors trained multiple tree-based ML algorithms to predict the decision for active treatment versus MRI surveillance of VS in a single institutional cohort. In doing so, they sought to assess which preoperative variables carried the most weight in driving the decision for intervention and could be used to guide future surgical decision-making through an evidence-based approach. METHODS: The authors reviewed the records of patients who had undergone evaluation by neurosurgery and otolaryngology with subsequent active treatment (resection or radiation) for unilateral VS in the period from 2009 to 2021, as well as those of patients who had been evaluated for VS and were managed conservatively throughout 2021. Clinical presentation, radiographic data, and management plans were abstracted from each patient record from the time of first evaluation until the last follow-up or surgery. Each encounter with the patient was treated as an instance involving a management decision that depended on demographics, symptoms, and tumor profile. Decision tree and random forest classifiers were trained and tested to predict the decision for treatment versus imaging surveillance on the basis of unseen data using an 80/20 pseudorandom split. Predictor variables were tuned to maximize performance based on lowest Gini impurity indices. Model performance was optimized using fivefold cross-validation. RESULTS: One hundred twenty-four patients with 198 rendered decisions concerning management were included in the study. In the decision tree analysis, only a maximum tumor dimension threshold of 1.6 cm and progressive symptoms were required to predict the decision for treatment with 85% accuracy. Optimizing maximum dimension thresholds and including age at presentation boosted accuracy to 88%. Random forest analysis (n = 500 trees) predicted the decision for treatment with 80% accuracy. Factors with the highest variable importance based on multiple measures of importance, including mean minimal conditional depth and largest Gini impurity reduction, were maximum tumor dimension, age at presentation, Koos grade, and progressive symptoms at presentation. CONCLUSIONS: Tree-based ML was used to predict which factors drive the decision for active treatment of VS with 80%-88% accuracy. The most important factors were maximum tumor dimension, age at presentation, Koos grade, and progressive symptoms. These results can assist in surgical decision-making and patient counseling. They also demonstrate the power of ML algorithms in extracting useful insights from limited data sets.

Racial and Socioeconomic Disparities in Patients With Meningioma: A Retrospective Cohort Study.

BACKGROUND: Meningiomas are the most common intracranial neoplasms. Although genomic analysis has helped elucidate differences in survival, there is evidence that racial disparities may influence outcomes. African Americans have a higher incidence of meningiomas and poorer survival outcomes. The etiology of these disparities remains unclear, but may include a combination of pathophysiology and other factors. OBJECTIVE: To determine factors that contribute to different clinical outcomes in racial populations. METHODS: We retrospectively reviewed 305 patients who underwent resection for meningiomas at a single tertiary care facility. We used descriptive statistics and univariate, multivariable, and Kaplan-Meier analyses to study clinical, radiographical, and histopathological differences. RESULTS: Minority patients were more likely to present through the emergency department than an outpatient clinic (P < .0001). They were more likely to present with more advanced clinical symptoms with lower Karnofsky Performance scores, more frequently had peritumoral edema (P = .0031), and experienced longer postoperative stays in the hospital (P = .0053), and African-American patients had higher hospitalization costs (P = .046) and were more likely to be publicly insured. Extent of resection was an independent predictor of recurrence freedom (P = .039). Presentation in clinic setting trended toward an association with recurrence-free survival (P = .055). We observed no significant difference in gross total resection rates, postoperative recurrence, or recurrence-free survival. CONCLUSION: Minority patients are more likely to present with severe symptoms, require longer perioperative hospitalization, and generate higher hospitalization costs. This may be due to socioeconomic factors that affect access to health care. Targeting barriers to access, especially to subspecialty care, may facilitate more appropriate and timely diagnosis, thereby improving patient care and outcomes.

Effects of Age on Delayed Facial Palsy After Otologic Surgery: A Systematic Review and Meta-Analysis.

OBJECTIVES: To report 4 cases of delayed facial palsy (DFP) after pediatric middle ear (ME) surgery and systematically review and analyze the associated literature to evaluate the effects of age on DFP etiology, management, and prognosis. METHODS: Systematic review of PubMed, Cochrane Library, and Embase for articles related to DFP after cochlear implantation (CI) was performed. These articles were assessed for level of evidence, methodological limitations, and number of cases. Meta-analysis was performed to assess the effects of age on DFP incidence. Furthermore, a comprehensive list of all pediatric DFP cases after otologic surgery was assembled through a multi-institutional retrospective review and systematic review of the literature. RESULTS: Twenty-nine articles fit the criteria for inclusion in the meta-analysis. The incidence of DFP after CI was 0.23% and 1.01% for pediatric and adult cases, respectively. This difference was statistically significant (P < .001, odds ratio 4.36). Twenty-three cases, adding to the 4 presented herein, were suitable for a comprehensive list. The mean age was 6.9 years. Average postoperative day of paresis onset was 5.4, with an average maximum House-Brackmann grade of 3.5. All patients obtained full facial recovery after an average of 23.5 days. CONCLUSIONS: The systematic review demonstrates that DFP after pediatric CI is rare and occurs at a significantly lower rate than in adults, further supporting the viral reactivation hypothesis of DFP. The prognosis for pediatric DFP after otologic surgery is excellent, with a high rate of full recovery in a short time frame. However, steroid administration can be considered. LEVEL OF EVIDENCE: IIa.

The posterior ligament of the incus ("white dot"): A reliable surgical landmark for the facial recess.

OBJECTIVE: There is a void in the literature describing reliable surgical landmarks that aid in the dissection of the facial recess in the absence of skeletonizing the mastoid segment of the facial nerve. The posterior ligament of the incus is a readily distinguishable "white dot" along the incus buttress that has been used to guide dissection in a safe and efficient manner. The goal of our study is to describe a surgical approach that utilizes this surgical landmark to drill the facial recess and to take anatomical measurements demonstrating the safety and reliability of this approach. MATERIALS AND METHODS: After cortical mastoidectomies were performed in 10 cadaveric temporal bones, the white dot was identified at the junction of short process of the incus and the incus buttress. Using the white dot for anatomical reference, a 2 mm diamond drill bit was used to open the facial recess without first identifying the facial nerve or chorda tympani nerve. After photographs were taken, the facial and chorda tympani nerves were definitively identified and skeletonized to delineate the confines of the facial recess. Photographs were once again acquired in a consistent manner for comparison. Finally, calibrated anatomic measurements were acquired from the 10 distinct image sets. RESULTS: The facial recess was successfully drilled in 10 temporal bones using the posterior ligament as a surgical landmark without injury to the chorda tympani or facial nerve. The median angle taken from the axis of the short process of the incus to the facial nerve - chorda tympani junction was 139.2° (IQR 136.8-141). At the widest point in the facial recess, median distances anterior and posterior to an imaginary line connecting the white dot to the facial nerve - chorda tympani junction were 1.6 mm (IQR 1.5-1.7) and 1.6 mm (IQR 1.6-1.7; p = 0.57), indicating at this point, the white dot reference reliably bisects the facial recess width. Similarly, at the level of the round window niche, median anterior and posterior distances from an imaginary line connecting the white dot to the facial nerve - chorda tympani junction were 1.1 mm (IQR 1.1-1.3) and 1.3 mm (IQR 1.1-1.7; p = 0.07), respectively, once again demonstrating the white dot reliably bisecting the facial recess. CONCLUSIONS: The white dot, representing the posterior ligament of the incus, is a reliable surgical landmark that aids in safe and efficient drilling of the facial recess without first skeletonizing the facial nerve.

Absent pyramidal eminence and stapedial tendon associated with congenital stapes footplate fixation: Intraoperative and radiographic findings.

OBJECTIVE: Report an association between congenital stapes footplate fixation (CSFF) and radiological absence of the pyramidal eminence and stapedial tendon. PATIENTS: Children and adults with intraoperatively confirmed CSFF and an absent stapedial tendon. INTERVENTIONS: Computed tomography (CT); exploratory tympanotomy with stapedotomy. MAIN OUTCOME MEASURES: Absence of a pyramidal eminence and stapedial tendon aperture identified on preoperative CT that was confirmed intraoperatively. RESULTS: Eight patients with intraoperative confirmation of CSFF and absent stapedial tendon were retrospectively identified. The average preoperative bone conduction and air conduction pure tone averages were 19.6 dB (SD 15.6 dB) and 55.9 dB (SD 23.6 dB), respectively. The average air-bone gap was 36.3 dB (SD 17.9 dB) preoperatively. In the seven patients who underwent preoperative CT, all were consistently identified to have an absent or hypoplastic pyramidal eminence and absent stapedial tendon aperture at the pyramidal eminence. In six cases, the stapedial footplate appeared normal, while in one case the footplate appeared abnormal which correlated with severe facial nerve prolapse observed intraoperatively. All eight cases underwent exploratory tympanotomy and demonstrated intraoperative stapes footplate fixation, absent stapedial tendon and either absent or hypoplastic pyramidal eminence, which correlated with preoperative CT findings. CONCLUSIONS: This study identifies a clinically pragmatic association between an absent pyramidal eminence identified on high-resolution CT and the diagnosis of CSFF. In a condition that otherwise generally lacks distinctive radiological features, the absence of a pyramidal eminence on CT in a patient with nonprogressive, congenital conductive hearing loss may strengthen clinical suspicion for CSFF.

Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas.

OBJECTIVES: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. METHODS: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures. RESULTS: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. CONCLUSIONS: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

Usefulness of Exam Questions and Vital Signs for Predicting the Outcome of Objective Vestibular Tests.

OBJECTIVES/HYPOTHESIS: To determine the value of standard clinic screening questions and vital signs in predicting abnormal vestibular function, indicated by standard objective diagnostic tests. STUDY DESIGN: Retrospective records review. METHODS: We reviewed electronic medical records of 150 patients seen by the neurotologists or the physician assistant they supervised, in an out-patient tertiary care clinic, between June 2018 and March 2020, and subsequently referred for the complete objective vestibular test battery (VB). RESULTS: Of standard questions asked during the initial exam about vertigo, disequilibrium, lightheadedness and oscillopsia, only vertigo predicted an abnormal response on the VB. More males than females had abnormal VB responses, P < .05. Pulse was not related to VB score. Significantly more subjects with blood pressure in the range for stage 2 hypertension (blood pressure [BP] stage 2) had abnormal than normal results on the VB, P < .00001. Subjects with BP stage 2 had high rates of diabetes (34.2%) and hypertension (68.4%) as diagnosed by their primary care physicians or cardiologists. CONCLUSION: Complaints of subjective vertigo and BP in the range of hypertension stage 2 are most likely to predict abnormal findings on the VB. Therefore, during an examination of a patient who comes in complaining of dizziness, two measures may be the most useful for screening: BP in the range of hypertension type 2, when BP is taken by a nurse, and a question to determine whether or not the patient has true vertigo. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:1382-1385, 2021.

Prevalence of and Associations With Distress and Professional Burnout Among Otolaryngologists: Part II, Attending Physicians.

OBJECTIVE: To ascertain the prevalence of and associations with distress and professional burnout among academic otolaryngology attending physicians. STUDY DESIGN: Cross-sectional survey. SETTING: Twelve US academic otolaryngology programs. METHODS: A questionnaire was administered that encompassed sociodemographic and professional features, the Expanded Physician Well-being Index for distress, the 2-item Maslach Burnout Inventory for professional burnout, the Patient Health Questionnaire-2 screen for major depressive disorder, and the Generalized Anxiety Disorder-2 screen for generalized anxiety disorder. RESULTS: The survey response rate was 56% and included 186 attending physicians. The average respondent age was 47 years; 72% were men; 93% were married or partnered; and 86% had children. Distress was present in 40%, professional burnout in 26%, positive depression screening in 8%, and positive anxiety screening in 11%. In a univariable setting, age, hours worked in a typical week, nights on call in a typical week, and years of practice were significantly associated with distress, although in a multivariable setting, only hours worked in a typical week remained significantly associated with a positive Expanded Physician Well-being Index screen (odds ratio for each 10-hour increase, 2.61; 95% CI, 1.73-3.93; P < .001). In a univariable setting, hours worked in a typical week was significantly associated with a positive Maslach Burnout Inventory screen. CONCLUSION: Distress or professional burnout occurs in more than a quarter of academic otolaryngology attending physicians, whereas the prevalence of depression or anxiety is approximately 10%. The number of hours worked per week had the strongest association with distress and burnout. These findings may be used to develop and implement programs to promote physician well-being and mitigate professional burnout.

Prevalence of and Associations With Distress and Professional Burnout Among Otolaryngologists: Part I, Trainees.

OBJECTIVE: To assess the prevalence of distress and burnout in otolaryngology trainees, including associations with relevant sociodemographic and professional factors, and to compare these results with those of attending otolaryngologists. STUDY DESIGN: A cross-sectional survey of trainees and attending physicians. SETTING: Twelve academic otolaryngology programs. METHODS: Distress and burnout were measured with the Expanded Physician Well-being Index and the 2-item Maslach Burnout Inventory. The Patient Health Questionnaire-2 and Generalized Anxiety Disorder-2 were used to screen for depressive disorders and anxiety disorders, respectively. Associations with sociodemographic and professional characteristics were assessed. RESULTS: Of the 613 surveys administered to trainees and attending physicians, 340 were completed (56%). Among 154 trainees, distress was present in 49%, professional burnout in 35%, positive depressive disorder screening in 5%, and positive anxiety disorder screening in 16%. In univariable analysis, female gender, hours worked in a typical week (HW), and nights on call in a typical week (NOC) were significantly associated with distress. In multivariable analysis, female gender (odds ratio, 3.91; P = .001) and HW (odds ratio for each 10 HW, 1.89; P = .003) remained significantly associated with distress. Female gender, HW, and NOC were significantly associated with burnout univariably, although only HW (odds ratio for each 10 HW, 1.92; P = .003) remained significantly associated with burnout in a multivariable setting. Attending physicians had less distress than trainees (P = .02) and felt less callous and less emotionally hardened than trainees (P < .001). CONCLUSION: Otolaryngology trainees experience significant work-place distress (49%) and burnout (35%). Gender, HW, and NOC had the strongest associations with distress and burnout.

Otosclerosis in an Urban Population.

OBJECTIVE: To investigate the evolving prevalence of otosclerosis in a large urban population. METHODS: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population. RESULTS: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio [OR] = 3.69, [95% confidence interval [CI], 2.02-6.76], p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics. CONCLUSION: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The "imported" otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.

The Potential for Preserved Cochleovestibular Function in the Setting of Prolonged Pneumolabyrinth.

OBJECTIVE: To discuss the natural history and management of pneumolabyrinth, with particular attention to illustrative cases where hearing improved after a fluid-filled vestibule was restored. PATIENTS: Adults at two academic medical centers with documented pneumolabyrinth on computed tomography (CT) imaging. INTERVENTION: Middle ear surgery or observation. MAIN OUTCOME MEASURES: Cochlear and vestibular symptoms are reported. Inner ear function was documented with audiometry and vestibular testing. Resolution of pneumolabyrinth was documented on imaging for a subset of patients. RESULTS: All patients presented with severe hearing loss following temporal bone fracture (three cases), penetrating trauma (one case), or stapedectomy (three cases). The radiographic finding was typically seen when imaging was obtained in the first few days after the injury. In two cases, vestibular symptoms resolved and partial recovery of hearing was seen despite a relatively long time interval between the inciting event and repair. CONCLUSION: Management of pneumolabyrinth is individualized based on the mechanism of injury, severity of symptoms, and timing of the imaging study. In most cases, the barrier between the middle ear and inner ear is expected to heal with rapid resolution of pneumolabyrinth. Late repair is demonstrated to result in favorable recovery in selected cases. The presence of air in the vestibule cannot be assumed to be indicative of irreversible inner ear damage.

Cochlear implantation after traumatic brain injury without otic capsule fracture: A case report and literature review.

OBJECTIVE: The aim of this study was to report a case of cochlear implantation (CI) for a patient with an otic capsule-sparing traumatic brain injury (TBI) and to review the relevant literature. METHODS: A patient with history of TBI received a CI for bilateral profound hearing loss. A systematic review of the literature was performed to identify and compare similar cases. RESULTS: A 36-year-old male with a history of hearing loss from right acute labyrinthitis was referred for bilateral profound sensorineural hearing loss (SNHL) after a fall with associated injury to the central auditory nervous system (CANS) including the brainstem. On the right, behavioral acoustic threshold measurements were in the profound range with absent OAEs. On the left, testing revealed no measurable behavioral acoustic thresholds and variable physiologic measures. A right unilateral cochlear implant was performed with most recent follow-up demonstrating speech awareness thresholds of 25 dB HL with excellent detection of all 6 Ling sounds. However, the patient also continues to suffer from other neurologic sequelae related to his TBI, which challenge his ability to demonstrate objective and subjective benefit. A systematic review of the literature demonstrates variable outcomes for patients with TBI and SNHL. CONCLUSIONS: Patients with profound SNHL and TBI present a distinct rehabilitative challenge for clinicians. CI may provide meaningful benefit in this population, though care should be taken in patient selection and counseling.