Dispersed DNA variants underlie hearing loss in South Florida's minority population.

BACKGROUND: We analyzed the genetic causes of sensorineural hearing loss in racial and ethnic minorities of South Florida by reviewing demographic, phenotypic, and genetic data on 136 patients presenting to the Hereditary Hearing Loss Clinic at the University of Miami. In our retrospective chart review, of these patients, half self-identified as Hispanic, and the self-identified racial distribution was 115 (86%) White, 15 (11%) Black, and 6 (4%) Asian. Our analysis helps to reduce the gap in understanding the prevalence, impact, and genetic factors related to hearing loss among diverse populations. RESULTS: The causative gene variant or variants were identified in 54 (40%) patients, with no significant difference in the molecular diagnostic rate between Hispanics and Non-Hispanics. However, the total solve rate based on race was 40%, 47%, and 17% in Whites, Blacks, and Asians, respectively. In Non-Hispanic Whites, 16 different variants were identified in 13 genes, with GJB2 (32%), MYO7A (11%), and SLC26A4 (11%) being the most frequently implicated genes. In White Hispanics, 34 variants were identified in 20 genes, with GJB2 (22%), MYO7A (7%), and STRC-CATSPER2 (7%) being the most common. In the Non-Hispanic Black cohort, the gene distribution was evenly dispersed, with 11 variants occurring in 7 genes, and no variant was identified in 3 Hispanic Black probands. For the Asian cohort, only one gene variant was found out of 6 patients. CONCLUSION: This study demonstrates that the diagnostic rate of genetic studies in hearing loss varies according to race in South Florida, with more heterogeneity in racial and ethnic minorities. Further studies to delineate deafness gene variants in underrepresented populations, such as African Americans/Blacks from Hispanic groups, are much needed to reduce racial and ethnic disparities in genetic diagnoses.

Two novel heterozygous exonic deletions lead to Chanarin-Dorfman syndrome in a patient with congenital ichthyosis, sensorineural hearing loss, and liver dysfunction.

Chanarin-Dorfman syndrome is an autosomal recessively inherited disorder characterized by ichthyosis, sensorineural hearing loss, and hepatic dysfunction. We report on a 60-year-old female of Venezuelan descent who presented with congenital ichthyosis, progressive sensorineural hearing loss, and liver cirrhosis. We identify a heterozygous copy number deletion involving exon 1 and another heterozygous deletion involving exon 3 of the ABHD5 gene. Exon 2 is preserved. Both deletions were confirmed with RT-PCR. RNAseq from peripheral blood shows a reduction of ABHD5 expression overall and an absence of exon 3 expression, confirming the deleterious effects of the identified deletions. We present exonic deletions as a potentially common type of ABHD5 variation.

Transcranial Attenuation in Patients with Single-Sided Deafness.

Transcranial attenuation (TA) of bone-conducted sound has a high degree of variability by frequency and subject, which may play a role in the objective benefit of individuals with single-sided deafness (SSD) treated with a bone-anchored implant (BAI). This study sought to determine whether TA is predictive of benefit in individuals with SSD who receive a BAI. Adult, English-speaking patients with unilateral profound sensorineural hearing loss who underwent a BAI evaluation were included for study. Absolute TA values were consistent with previously published reports. Regression analysis indicated no correlation between TA values and aided speech-in-noise performance for any combined or individual frequencies. Measures of TA do not provide predictive value in determining behavioral outcomes in the SSD population. Specifically, low TA does not suggest improved outcomes with a BAI.

Comparison of packing material in an animal model of middle ear trauma.

PURPOSE: To compare the performance of absorbable gelatin sponge (AGS) with polyurethane foam (PUF) as middle ear packing material after mucosal trauma. MATERIALS AND METHODS: Using a randomized, controlled and blinded study design fifteen guinea pigs underwent middle ear surgery with mucosal trauma performed on both ears. One ear was packed with either PUF or AGS while the contralateral ear remained untreated and used as non-packed paired controls. Auditory brainstem response (ABR) thresholds were measured pre-operatively and repeated at 1, 2, and 6weeks postoperatively. Histological analysis of middle ear mucosa was done in each group to evaluate the inflammatory reaction and wound healing. Another eighteen animals underwent middle ear wounding and packing in one ear while the contralateral ear was left undisturbed as control. Twelve guinea pigs were euthanized at 2weeks postoperatively, and six were euthanized at 3days post-operatively. Mucosal samples were collected for analysis of TGF-ß1 levels by enzyme-linked immunosorbent assay. RESULTS: ABR recordings demonstrate that threshold level changes from baseline were minor in PUF packed and control ears. Threshold levels were higher in the AGS packed ears compared with both control and PUF packed ears for low frequency stimuli. Histological analysis showed persistence of packing material at 6weeks postoperatively, inflammation, granulation tissue formation, foreign body reaction and neo-osteogenesis in both AGS and PUF groups. TGF-ß1 protein levels did not differ between groups. CONCLUSION: PUF and AGS packing cause inflammation and neo-osteogenesis in the middle ear following wounding of the mucosa and packing.

Atypical radiographic features of skull base cholesterol granuloma.

Cholesterol granulomas (CGs) are the most common benign lesions of the petrous apex (PA) and have distinct computed tomography (CT) and magnetic resonance imaging (MRI) characteristics. On CT, CGs of the PA (PACG) present as expansile lesions with erosion of bony trabeculae. MRI shows a hyperintense lesion on T1-and T2-weighted images and do not enhance with gadolinium. The objective is to describe the radiographic features of CGs of the skull base that do not arise from the PA. This study is a retrospective review. Three patients were operated on for suspected recurrent endolymphatic sac tumor, intracranial cholesteatoma, and recurrent sphenoid wing meningioma based on CT and MRI findings. Pathology results were consistent with CG in all three cases. All patients had bone erosion on CT. These skull base CGs did not demonstrate similar MRI features. These lesions were hyperintense, iso-to-hyperintense, and hypointense on T1-weighted MRI, respectively. These CGs were hyperintense in two cases and iso-to-hyperintense in one case on T2-weighted MRI. These lesions either demonstrated central or rim enhancement after gadolinium administration. Skull base CGs that do not arise from the PA demonstrate a broad spectrum of radiographic characteristics on MRI that are not typical of PACG.

Middle fossa vestibular neurectomy for refractory vertigo: less is more.

OBJECTIVE: This study aimed to evaluate outcomes of the middle fossa (MF) superior vestibular neurectomy in unilateral Ménière's disease. PATIENTS AND METHODS: Case series with preoperative and postoperative analysis of the 1995 American Academy of Otolaryngology hearing stage and vertigo class, gait instability, and results of vestibular-evoked myogenic potentials (VEMP). RESULTS: Four out of the 5 patients had total vertigo control (class A) and 1 had near total control (class B) by the last visit (mean follow-up, 23.6 months). There were no changes in hearing thresholds and hearing stage. Four patients had resolution of their gait instability by 2 months after surgery. Postoperative VEMP responses were preserved in all 3 patients with positive VEMP preoperatively. CONCLUSION: This is the first report of the anatomical and functional preservation of the inferior vestibular nerve in vestibular neurectomy for the treatment of refractory vertigo in unilateral Ménière's disease, with VEMP testing before and after vestibular neurectomy. The modified technique limits the surgical dissection and may help avoid complications such as postoperative hearing loss and persistent gait instability. This approach is indicated when other more conservative measures have failed, and patient selection is paramount to avoid long-term complications.

Speech Discrimination Outcomes in Adult Cochlear Implant Recipients by Primary Language and Bilingual Hispanic Patients.

OBJECTIVE: Compare cochlear implant (CI) outcomes in English speakers, Spanish speakers, and bilingual Hispanics. STUDY DESIGN: Retrospective review. SETTING: Academic tertiary care center. METHODS: Eighty-five postlingually deafened adults unilaterally implanted between January 2014 and December 2018 were stratified by primary language. Primary outcomes were: (1) English consonant-nucleus-consonant and Spanish bisyllables word tests in quiet, and (2) English AzBio and Latin American Hearing In Noise Test (LA-HINT) sentence tests in quiet and in noise at multiple time-intervals postactivation. RESULTS: In the respective languages, primary Spanish speakers (n = 24), and English speakers (n = 61) experienced the greatest increases in average scores for word and sentence tests in quiet during the first 6 months postactivation, with gradual increases in average scores over time. English speakers performed significantly worse on AzBio tests in noise, compared to quiet, while the addition of noise did not significantly affect average LA-HINT scores in Spanish speakers across multiple time intervals. An early ceiling effect was also demonstrated for LA-HINT. Although not significant, bilingual Hispanics (n = 12) had lower average AzBio in quiet scores than English speakers and higher average LA-HINT in quiet scores than the Spanish speakers across multiple time intervals. CONCLUSION: English and Spanish CI users experienced the greatest increases in speech understanding in quiet the first few months after implant activation. An early ceiling effect is demonstrated with LA-HINT, indicating LA-HINT is not appropriate for evaluating longitudinal CI outcomes in Spanish speakers. Bilingual Hispanics represent a unique group, and further investigations are necessary to understand speech perception patterns in both languages and develop the best CI test strategies for these individuals.

Functional Hearing Preservation in Cochlear Implantation: The Miami Cocktail Effect.

OBJECTIVE: To investigate if pharmacological treatment with prednisone and L-N-acetylcysteine (STE + NAC) influence functional hearing preservation in cochlear implant (CI) surgery. STUDY DESIGNS: Preimplantation and postimplantation longitudinal case-control study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult recipients of CI with preimplantation functional hearing defined as an average of air-conducted thresholds at 125, 250, and 500 Hz (low-frequency pure-tone average [LFPTA]) <80 dB. INTERVENTIONS: Preimplantation and postimplantation audiometry. Weight-adjusted oral prednisone and L-N-acetylcysteine starting 2 days before surgery (Miami cocktail). Prednisone was continued for 3 days and L-N-acetylcysteine for 12 days after surgery, respectively. Cochlear implantation with conventional length electrodes. MAIN OUTCOME MEASURES: Proportion of patients with LFPTA <80 dB, and LFPTA change at 1-year postimplantation. RESULTS: All 61 patients received intratympanic and intravenous dexamethasone intraoperatively, with 41 patients receiving STE + NAC and 20 patients not receiving STE + NAC. At 1-year postimplantation, the proportion of functional hearing preservation was 83% in the STE + NAC group compared with 55% of subjects who did not receive STE + NAC ( p = 0.0302). The median LFPTA change for STE + NAC-treated and not treated subjects was 8.33 dB (mean, 13.82 ± 17.4 dB) and 18.34 dB (mean, 26.5 ± 23.4 dB), respectively ( p = 0.0401, Wilcoxon rank test). Perioperative STE + NAC treatment resulted in 10 dB of LFPTA better hearing than when not receiving this treatment. Better low-frequency preimplantation hearing thresholds were predictive of postimplantation functional hearing. No serious side effects were reported. CONCLUSION: Perioperative STE + NAC, "The Miami Cocktail," was safe and superior to intraoperative steroids alone in functional hearing preservation 1-year after cochlear implantation.

A schwannoma of the greater petrosal nerve located within the petrous apex and treated with stereotactic radiotherapy.

A 26 year-old female experienced progressive left sided pulsatile tinnitus and conductive hearing loss for two years, which following an extensive clinical workup, was diagnosed as a left greater petrosal nerve schwannoma located within the petrous apex of the temporal bone. Between neurosurgical management and radiation therapy, multiple therapeutic options were presented to the patient, who ultimately chose stereotactic radiotherapy as an alternative to surgical resection due to the potential morbidity associated with surgery. The patient received three fractions of 600 cGy without subsequent worsening of her symptoms, new onset neurologic symptoms or radiation induced side effects reported at a 3, 6 and 12 month clinic visits. A follow-up MRI at 6 and 12 months post radiation administration demonstrated no further tumor growth.

Genotype-phenotype associations in paragangliomas of the temporal bone in a multi-ethnic cohort.

BACKGROUND: Temporal bone paragangliomas are rare tumours with variable presentation that can be hereditary. Identification of clinical and genetic factors of aggressive tumour behaviour is important. OBJECTIVE: To determine the underlying genetic mutations and genotype/phenotype correlations in a multi-ethnic population of South Florida with sporadic temporal bone paragangliomas. METHODS: In a cohort of glomus tympanicum (GT) and glomus jugulare (GJ) cases, we assessed the frequency of pathogenic single nucleotide variants, insertions, deletions, and duplications in coding exons of genes that have been associated with paragangliomas (SDHB, SDHC, SDHD, SDHA, SDHAF2, RET, NF1, VHL, TMEM127, and MAX). RESULTS: None of the 12 GT cases had mutations. Among 13 GJ cases, we identified four mutation carriers (31%); two in SDHC, one in SDHB, and one in SDHD. All patients with pathogenic mutations were of Hispanic ethnicity, presented at a younger age (mean 27.5 versus 52.11 years), and with more advanced disease when compared to mutation-negative GJ cases.Conclusions and Significance: Mutations in the SDH genes are found in 31% of sporadic GJ. SDH-associated GJ had advanced disease and a 50% risk of metastasis. Our data supports emerging recommendations for genetic screening in all populations with GJ tumours as the genetic status informs management.

Cross-sectional Evaluation of Neurotology Fellowship Directors: A Present-day Snapshot of Leadership.

Objective: To identify demographic, training, and career trends of neurotology fellowship directors (FDs). Study Design: Cross-sectional study. Setting: United States. Subjects: All 26 neurotology FDs identified using the American Neurotology Society (ANS) ACGME Accredited Neurotology Fellowship Program Directory, accessed November 2021. Main Outcome Measures: Data were collected via CVs, institutional biographies, and emailed questionnaires. Data collected includes age, gender, race and ethnicity, residency and fellowship training institution, time since training completion until FD, length of time as FD, and Hirsch-index (h-index). Results: Twenty-six FDs were identified, and 17/26 (65.4%) FDs responded to the questionnaire. The majority (23/26; 88.5%) were male. The mean age of male and female FDs was 56 versus 47 years, respectively. Of the 17 that responded to the survey, 82.4% (14/17) self-identified as Caucasian. The mean h-index was 25.4. Older age correlated with a higher h-index (r = 0.46, P = 0.019). The duration (mean ± SD, years) from fellowship graduation to FD appointment was 10.7 ± 8.1 and 6.3 ± 4.8 from institutional hire. Six (23.1%) FDs had secondary graduate degrees, and 9 (34.6%) held a leadership position at a national otolaryngology organization. Conclusion: This observational study assesses demographic data on current neurotology FDs in the United States with an analysis of gender disparities. The objective measures identified can provide a baseline for growth in FD leadership.

Cross-sectional vestibular nerve analysis in vestibular neuritis.

OBJECTIVES: We examined the association between the size and cross-sectional area of the superior vestibular nerve as measured on constructive interference in steady-state (CISS) parasagittal magnetic resonance imaging (MRI) and the vestibular nerve function as measured by electronystagmography. METHODS: The retrospective observational cohort study took place at an academic tertiary referral center. Twenty-six patients who met established clinical and electronystagmographic criteria for vestibular neuritis and who underwent parasagittal CISS MRI were identified. Two blinded investigators measured vestibular nerve height and width bilaterally at the level of the fundus of the internal auditory canal and calculated the cross-sectional nerve areas. The inter-rater reliability and agreement were analyzed. Symptom duration, age, and gender were also examined. RESULTS: A statistically significant decrease was observed in both vestibular nerve cross-sectional area and height as compared to the contralateral vestibular nerve. A non-statistically significant trend was observed for a relative decreased cross-sectional nerve area with increased age, as well as a decrease in nerve area with an increase in symptom duration. CONCLUSIONS: Decreases in both vestibular nerve cross-sectional area and height are observed in patients with unilateral vestibular neuritis as measured on parasagittal CISS MRI.

Ethnic disparity in skin complications following bone-anchored hearing aid implantation.

OBJECTIVES: Sound processor loading after implantation of a bone-anchored hearing aid is often delayed by skin-site complications. This study examined the frequency of skin-site complications in various ethnic groups and determined factors that may lead to higher rates of skin-site complications resulting in delayed processor loading. METHODS: Adult, English-speaking patients who underwent implantation of a bone-anchored hearing aid between 2007 and 2010 were reviewed. Demographic data including ethnicity, tobacco use, diabetes mellitus, immunosuppression, and long-term steroid use were determined. Major and minor skin-site complications and the time to processor loading were recorded. RESULTS: The mean time to processor loading was 9.5 weeks, and the mean follow-up time was 23 months. There were no cases of osseointegration failure. African American patients had a significantly higher rate of major skin-site complications (p < 0.005) and a longer time to processor loading (mean, 17.6 weeks; p < 0.05) than the other ethnic groups. There was no significant difference in minor skin complication rates. There was no correlation between diabetes mellitus, long-term immunosuppression, or tobacco use and skin-site complications. CONCLUSIONS: Skin complications can delay processor loading following implantation of a bone-anchored hearing aid. There is a higher rate of major skin-site complications in African American patients, and these often delay processor loading. The risk of skin-site complications is not correlated with smoking, diabetes mellitus, or immunosuppression. An increased risk of skin-site complications is an important consideration for preoperative counseling.

Prophylactic valacyclovir in a patient with recurrent vestibular disturbances secondary to vestibular neuritis.

A 57-year-old woman with herpes labialis and previously diagnosed with vestibular neuritis experienced recurrences of vertigo and disequilibrium. Initially preceded by oral herpes outbreaks or upper respiratory infections, these recurrences became spontaneous and more frequent. Vestibular function demonstrated a 25% decrease in energy function in the right and the patient had left beating nystagmus on positional maneuver. Her reoccurrences of vestibular disturbances were followed up. Concurrently, she was prescribed daily valacyclovir (500 mg, 1 per day) given for the prevention of herpes labialis outbreaks by her primary care physician. Recurrences of disequilibrium stopped completely as well as oral herpes outbreaks.

Mesenchymal Stem Cells for Treatment of Delayed-Healing Tympanic Membrane Perforations Using Hyaluronate-based Laminas as a Delivery System: An Animal Model With Histopathologic Study.

HYPOTHESIS: Bone marrow derived-mesenchymal stem cells (BM-MSCs) improve the healing of chronic tympanic membrane perforations (cTMPs) in an animal model. BACKGROUND: cTMPs generate significant morbidity and reduced quality of life, usually requiring surgical assistance. With growing interest in alternative therapies, we sought to evaluate the effect of BM-MSC-therapy on the healing of cTMPs. METHODS: Sixty cTMPs were established in C57Bl/6 mice and randomized into four groups: hyaluronate scaffold as graft plus BM-MSCs (n = 19 ears), scaffold plus cell culture media (n = 16), scaffold plus phosphate-buffered saline (PBS, n = 12), and no intervention (n = 13). Hyaluronate scaffolds with or without BM-MSCs were applied on 8-week perforated eardrums. After a blinded assessment of perforation sizes at baseline and 2 weeks after treatment, mean perforation reduction rates (%) were compared. Histology characterization was then performed. RESULTS: Mean perforation size reduction rates were significantly higher for cTMPs that received scaffolds plus BM-MSCs (Student's t test, p = 0.0207, 12.3% [95% CI: 7.8-16.7]) and scaffolds plus cell culture media (p = 0.0477, 11.3% [95% CI: 4.4-18.2]) when compared with no intervention (4.2% [95% CI: 1.2-7.2]). This was not observed when treating eardrums with scaffolds plus PBS (7.3% [95% CI: 2.7-11.9]). On histology, BM-MSC-treated eardrums demonstrated restoration of the trilaminar configuration and reduced inflammatory changes, while other groups developed tissue architecture disorganization and hypercellular infiltrates surrounding the perforation site. CONCLUSIONS: BM-MSCs and cell culture media equivalently increased cTMP healing rates. Cell-based therapy conferred a restoration of the trilaminar configuration of the eardrum with relatively compact and organized fibrous layers.

Comparison of Speech Test Outcomes After Cochlear Implantation in Patients With and Without Asymmetric Hearing Loss.

OBJECTIVES: Determine whether asymmetric hearing loss (AHL) affects postoperative speech outcomes in cochlear implant (CI) patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary care hospital. PATIENTS: Adult English-speaking patients with unilateral CIs implanted between 2014 and 2018 were stratified into NonAHL and AHL groups based on preoperative AzBio scores in quiet from the nonimplanted ear (0-50% vs. 51-100%, respectively). INTERVENTIONS: CI surgery in the poorer performing ear. MAIN OUTCOME MEASURES: Postoperative consonant-nucleusconsonant (CNC) word and AzBio sentence test scores in quiet and/or noise at +5 dB signal-to-noise ratio (SNR). RESULTS: Of 512 patients, 33 non-AHL and 27 AHL patients were included. Average ages were 65.6 and 63.6 years, respectively. As expected, preoperative AzBio scores in quiet from the nonimplanted ear were higher in the AHL group (95% confidence interval [95%CI]: 66.4-76.4%) than the non-AHL group at baseline (95%CI: 12.3-23.6%). In both cohorts, AzBio scores in quiet from the implanted ear improved from baseline, with 24-month scores (95%CI: 73.8 - 84.9%) being higher than preoperative scores (95%CI: 13.2-23.1%). There were also significant differences in AzBio scores in quiet between cohorts overall (p  = 0.0120) on mixed model analysis, with the AHL group performing ∼6.4% better than the non-AHL group; however, differences were not significant when scores were stratified by time. In addition, there were no significant differences in CNC in quiet and AzBio scores in noise at +5 dB SNR between cohorts (p  = 0.1786 and p  = 0.6215, respectively). CONCLUSIONS: After CI, patients with AHL can achieve scores on word and sentence tests at least comparable to traditional CI candidates, supporting the expansion of CI candidacy to include patients with AHL.

Safety and Facial Nerve Outcomes of Intracisternal Papaverine Irrigation for Vestibular Schwannoma Resection.

BACKGROUND: Papaverine, a vasodilator approved for use by the U.S. Food and Drug Administration, has shown efficacy in treating vasospasm in cardiology, urology, and nephrology. The vasodilatory effect of papaverine is also hoped to protect the facial nerve from ischemic damage and nerve manipulation during vestibular schwannoma surgery. Our institution uses intracisternal papaverine irrigation during vestibular schwannoma resection to protect the facial nerve in patients with neuromonitoring changes. Our objective was to investigate the safety and facial nerve outcomes of intracisternal papaverine irrigation during vestibular schwannoma resection. METHODS: We retrospectively reviewed patients who underwent resection of vestibular schwannoma at our institution between 2008 and 2021. Patients received papaverine if the intraoperative facial nerve stimulation threshold increased above 0.05 mA. Postoperative outcomes were compared with control patients who did not receive papaverine. RESULTS: A total of 283 cases were included in our analysis. Patients who received papaverine (n = 60) had lower immediate postoperative House-Brackmann (HB) grades than did control individuals (mean, 1.54 vs. 1.95; P = 0.029) and a lower likelihood of immediate postoperative HB grade >1 (odds ratio, 0.514; P = 0.039). At long-term follow-up, there was no significant difference in HB grade. Papaverine use was not associated with increased rates of perioperative complications (P = 0.24). CONCLUSIONS: The off-label use of intracisternal papaverine irrigation during vestibular schwannoma resection can certainly be used safely for select cases. It is associated with improved immediate postoperative facial nerve outcomes, similar long-term facial nerve outcomes, and no significant increase in complications.

Microsurgical Management of Vestibular Schwannoma (Acoustic Neuroma): Facial Nerve Outcomes, Radiographic Analysis, Complications, and Long-Term Follow-Up in a Series of 420 Surgeries.

OBJECTIVE: The purpose of this study is to retrospectively evaluate the clinical and surgical outcomes of a large surgical series of vestibular schwannoma from North America over 20 years. METHODS: After institutional review board approval a retrospective review of the senior author's personal case logs to identify patients who had operations for vestibular schwannoma was performed. The clinical notes, operative record, preoperative and postoperative imagings, and long-term clinical follow-up notes were evaluated. RESULTS: A total of 415 patients who underwent 420 surgeries were identified from the years 1998-2021. The average length of follow-up was 3 years and 9 months. Overall, at last follow-up the rate of "good" facial nerve outcomes (House-Brackmann [HB] score I and II) was 86% and "poor" facial nerve outcomes (HB III-VI) was 14%. The amount of cerebellopontine angle extension (P = 0.023), tumor volume (P = 0.015), facial nerve consistency (P < 0.001), preoperative HB score (P < 0.001), and FN stimulation threshold at the end of the procedure (P < 0.001) were correlated to facial nerve function at the last follow-up. CONCLUSIONS: This study represents one of the largest recently reported surgical series of vestibular schwannoma in North American literature with available long term follow-up. Facial nerve outcomes correlated with cerebellopontine angle extension, tumor volume, facial nerve stimulation threshold, facial nerve consistency, preoperative facial nerve function, and history of a prior resection. Tumor recurrence remains significantly higher after subtotal resection. We believe the data supports a continuation of a strategy of general intent of gross total resection, greatly modifiable by intraoperative findings and judgment.

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

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

Head-shaking nystagmus predicts greater disability in unilateral peripheral vestibulopathy.

OBJECTIVES: The objective of this study was to determine the association of the bedside test of head-shaking nystagmus (HSN) with patients' self-perceived dizziness handicap as well as this test's sensitivity and specificity in unilateral peripheral vestibular hypofunction. STUDY DESIGN: A retrospective case-control study was performed. SETTING: The study was held at an academic, tertiary referral center. METHODS: Fifty-three adult patients with unilateral peripheral hypofunction defined by the caloric test of the videonystagmography with documented bedside HSN and who had completed questionnaires of self-perceived dizziness handicap were included. The sensitivity and specificity of the bedside HSN in patients and 10 healthy controls in diagnosing unilateral vestibular hypofunction defined by videonystagmographic caloric testing and by abnormal gain and symmetry of the vestibular-ocular reflex by rotary chair testing were determined. Scores of the screening test of the Dizziness Handicap Index and Functional Level Scale questionnaires were taken. RESULTS: When using the caloric irrigation test as the reference standard for unilateral vestibular hypofunction, the sensitivity, specificity, and positive predictive value of the bedside HSN were 31%, 96%, and 97%, respectively. When comparing with results of rotational chair testing (vestibular-ocular reflex gain and symmetry), the sensitivity of the HSN test increases to 71%. Patients with positive bedside HSN had higher scores (greater self-perceived dizziness handicap) of the Dizziness Handicap Index (P = .049) and higher (worse) scores of the Functional Level Scale (P = .0377) than those with negative bedside HSN (Wilcoxon rank test). CONCLUSIONS: Greater perceived handicap was correlated with a positive bedside HSN in patients with unilateral peripheral vestibulopathy. The HSN has sufficient sensitivity to be used as screening test of uncompensated vestibulopathy in this series. However, a negative HSN alone does not rule out the diagnosis of peripheral vestibular dysfunction.