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.

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.

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.

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.

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.

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.

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.

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.

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.

Genetics and the Individualized Therapy of Vestibular Disorders.

Background: Vestibular disorders (VDs) are a clinically divergent group of conditions that stem from pathology at the level of the inner ear, vestibulocochlear nerve, or central vestibular pathway. No etiology can be identified in the majority of patients with VDs. Relatively few families have been reported with VD, and so far, no causative genes have been identified despite the fact that more than 100 genes have been identified for inherited hearing loss. Inherited VDs, similar to deafness, are genetically heterogeneous and follow Mendelian inheritance patterns with all modes of transmission, as well as multifactorial inheritance. With advances in genetic sequencing, evidence of familial clustering in VD has begun to highlight the genetic causes of these disorders, potentially opening up new avenues of treatment, particularly in Meniere's disease and disorders with comorbid hearing loss, such as Usher syndrome. In this review, we aim to present recent findings on the genetics of VDs, review the role of genetic sequencing tools, and explore the potential for individualized medicine in the treatment of these disorders. Methods: A search of the PubMed database was performed for English language studies relevant to the genetic basis of and therapies for vestibular disorders, using search terms including but not limited to: "genetics," "genomics," "vestibular disorders," "hearing loss with vestibular dysfunction," "individualized medicine," "genome-wide association studies," "precision medicine," and "Meniere's syndrome." Results: Increasing numbers of studies on vestibular disorder genetics have been published in recent years. Next-generation sequencing and new genetic tools are being utilized to unearth the significance of the genomic findings in terms of understanding disease etiology and clinical utility, with growing research interest being shown for individualized gene therapy for some disorders. Conclusions: The genetic knowledge base for vestibular disorders is still in its infancy. Identifying the genetic causes of balance problems is imperative in our understanding of the biology of normal function of the vestibule and the disease etiology and process. There is an increasing effort to use new and efficient genetic sequencing tools to discover the genetic causes for these diseases, leading to the hope for precise and personalized treatment for these patients.

Resection of a meningioma, vestibular schwannoma, and placement of auditory brainstem implant using translabyrinthine approach.

The authors present the case of a 34-year-old patient with neurofibromatosis type 2 (NF-2) who underwent a left translabyrinthine approach for resection a meningioma, vestibular schwannoma, and placement of an auditory brainstem implant (ABI). They review the preoperative workup, technical nuances of the surgery, and cadaveric dissections with anatomical diagrams, and provide a review on ABIs. The patient remained neurologically intact and had improvement in lip reading when using the ABI device in the postoperative period. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID2163.

The Epidemiological and Financial Effects of Pneumococcal Vaccination on Otitis Media Related Admissions in the United States.

OBJECTIVE: To quantify the prevalence of hospital admissions, the financial impact, and the trends in surgical procedure rates for AOM and CAOM for all ages before and after 13-valent pneumococcal conjugate vaccine (PCV13) introduction. METHODS: Retrospective analysis of the National Inpatient Sample (NIS) from 1998 to 2013 to determine the prevalence of AOM/CAOM related admissions and weighted frequencies of AOM/CAOM related International Classification of Diseases, ninth revision (ICD-9) hospital diagnoses. Prevalence of surgical procedures to treat CAOM, cost of admission, length of stay, and cost per day of admission were tabulated. Trend analysis of this data was performed. RESULTS: A total of 46 580 patients were hospitalized with AOM in the designated time period, of which 37 366 had CAOM. The prevalence of hospital admission due to AOM had the most pronounced decrease from pre-vaccine era (1998) to post-PCV13 implementation (2013) in age group 0 to 4 (32%) followed by age group 5 to 19 (7%). Age groups 20-64 and 65+ showed slight increases in prevalence. The trend in prevalence of admissions due to CAOM mirrors that of overall admissions with an 18% and 5.8% decrease in age groups 0-4 and 5-19, respectively, and a 1% increase in ages 20+. The inflation adjusted mean cost of admission did not significantly increase between 2001 and 2013. The total cost per admission was $4428 and $7546 for those with AOM and CAOM, respectively. Mastoidectomy rates increased by 17% in hospitalized children during the post-vaccine era but decreased in the elderly population. CONCLUSION: The prevalence of AOM/CAOM hospital admissions decreased from the pre-vaccine era (1998) to post-PCV13 implementation (2013) in pediatric patients. Surgical procedure utilization and cost of hospital admission for AOM/CAOM did not increase throughout the study period.

Predicting Recidivism for Acquired Cholesteatoma: Evaluation of a Current Staging System.

OBJECTIVE: Assess the utility and prognostic capabilities of the European Academy of Otology and Neurotology (EAONO) and Japanese Otological Society (JOS) cholesteatoma classification system, specifically for retraction pocket cholesteatoma. STUDY DESIGN: Retrospective. SETTING: Tertiary referral hospital. PATIENTS: Adults and children with retraction pocket cholesteatoma. INTERVENTIONS: Primary and planned second-look tympanoplasty with mastoidectomy. MAIN OUTCOME MEASURES: Incidence of recurrent or residual cholesteatoma at planned second-look surgery. Independent variables of age, gender, size of canal defect, and mucosa status were assessed. Additionally, the cholesteatoma classification, stage, and extent according to the EAONO/JOS system were recorded during the primary surgery. RESULTS: A total of 125 cases were included. Twelve (9.6%) cases had recidivism over an average time of 7.5 months: the recurrence rate was 4% (n = 5), residual rate was 5% (n = 6), and one patient had both recurrent and residual disease (0.8%). Residual cholesteatoma occurred more frequently in children (p = 0.04, RR = 7.9 [1.0, 63.6]). Supratubal recess (S1) disease was associated with both recurrent cholesteatoma (p = 0.04, RR = 5.9 [1.3, 27.2]) and recidivism (p = 0.01, RR = 4.2 [1.5, 11.9]). Larger canal defects also showed an association with residual disease (p = 0.017). CONCLUSION: Younger patients and those with large ear canal defects tend to have residual disease at second-look surgery. Supratubal recess disease is also associated with recurrence. Despite the utility of the EAONO/JOS classification and staging system for cholesteatoma description and type, the prognostic value remains uncertain.

Effect of age, electrode array, and time on cochlear implant impedances.

Objectives: To determine the impact of age, electrode array, and time on impedance patterns in cochlear implant (CI) patients. Methods: A retrospective case review was performed on 98 patients implanted with the CI24RE perimodiolar (PM) and CI422 lateral wall (LW) arrays between 2010 and 2014 to assess impedances at the 1 week and 3-6 month visit after initial stimulation (IS). Results: With respect to age, impedances were higher in young patients compared to older patients in the middle and apical turns. With time, there were significant reductions in impedances across most electrodes. Electrode array type also had a significant impact on impedance measurements with PM and LW arrays having higher impedances in the basal turn and apical turns, respectively. Furthermore, PM arrays demonstrated significantly lower impedances in the middle and apical turn with time, when compared to LW arrays. Conclusions: Age, electrode array, and time can independently affect CI impedances. Moreover, we show that PM arrays may be advantageous to LW arrays, due to demonstrated lower impedances in the middle and apical turns long term. Understanding the impact of impedance on speech discrimination and determining the intracochlear processes that contribute to differences in impedance are future research directions.

Animal Model of Chronic Tympanic Membrane Perforation.

Chronic tympanic membrane perforations (TMP) can be a source of significant morbidity from hearing loss, recurrent middle ear infections, changes in lifestyle, and risk of cholesteatoma formation. Laboratory experiments of TMP have been fraught by the rapid and high rate of spontaneous healing observed in animal models. There is controversy on the minimal time that perforations in animal models must have in order to be considered chronic TMP and thus have clinical relevance, with authors suggesting time periods of perforation patency of 8-12 weeks. In this article, we sought to create a clinically significant experimental model that could yield a high rate of perforation patency for at least 8 weeks. Animals undergoing acute TMP were exposed to three different experimental situations to delay the healing of the perforation: fractionated radiation, topical lipopolysaccharide application, and a combined dexamethasone and mitomycin C (DXM/MC) solution. In our study, the use of DXM/MC reliably produced TMP lasting at least 8 weeks in 86.48% of the cases without the need to reopen the perforation, infolding the edges of the membrane, or using physical barriers to prevent TMP closure. Histologically, the resulting perforated tympanum showed hyaline changes of the remnant tympanum and hyperkeratosis of the squamous epithelia of the external auditory canal. We believe that this model is reproducible and has potential use in experiments of delayed healing of TMP. Anat Rec, 303:619-625, 2020. © 2019 American Association for Anatomy.

Cervical VEMP tuning changes by Meniere's disease stages.

OBJECTIVE: To determine if changes in cervical vestibular-evoked myogenic potential (cVEMP) testing reflect the different stages of cochlea-saccular hydrops in Meniere's disease (MD). METHODS: This is a case-control retrospective series. Forty-seven patients with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery diagnostic and staging criteria, and 30 with non-MD vertigo as control. Meniere patients were further classified based on symptoms at the time of testing as active or stable. Subsequently, patients underwent cVEMP testing by tone-burst stimuli at 500 and 1,000 Hz. The main outcome measure was to compare the cVEMP 1,000 and 500 Hz amplitude ratio in ears with MD and non-MD vertigo, and in active versus stable MD. RESULTS: The cVEMP 1,000/500 Hz amplitude ratio was higher in Meniere's ears (mean = 1.14 µV, SD = 0.25) than in non-Meniere's ears (mean = 0.96 µV, SD = 0.2) (Student's t test, P = .001), and higher in active (mean = 1.22 µV, SD = 0.25) than in stable MD (mean = 1.00 µV, SD = 0.18) (P = .0035). The diagnostic value of cVEMP 1,000/500 Hz amplitude ratio to differentiate MD versus non-MD vertigo was evaluated with a receiver-operating characteristics (ROC) curve and the area under the curve (AUC) was 0.716 (95% confidence interval [CI] [0.591, 0.829]). The ideal cutoff point was 0.9435 with sensitivity and specificity values of 83% and 53%, respectively. The sensitivity and specificity values for this test to differentiate active versus stable MD were 68% and 81%, respectively, with AUC 0.746 (95% CI [0.607, 0.885]) and cutoff value of 1.048. In all ears, the 1,000/500 Hz amplitude ratio increased by a decrease of the 500 Hz amplitude with increasing age. CONCLUSION: The cVEMP 1,000/500 Hz amplitude ratio is elevated in ears with MD but not in those with non-MD vertigo. After corrected by age, this ratio is higher in active but not in stable MD, probably reflecting dynamic changes in saccular membrane motion mechanics in hydrops, and may be a useful marker of disease progression and the effect of therapy. LEVEL OF EVIDENCE: IV.

The pattern of hearing outcome following surgery of the semicircular canals.

OBJECTIVE: To analyze demographic, clinical, surgical, and audiometric factors that may affect hearing outcome following surgery for the semicircular canals (SCC). METHOD: This is a retrospective case review of adults who underwent surgeries for superior SCC (SSCC), lateral SCC (LSCC), or posterior SCC (PSCC) and whose data were extracted and analyzed for factors affecting the hearing outcome in these procedures. RESULTS: Thirteen patients underwent surgery for SSCC, seven cases for the LSCC, one for the PSCC, and one case of combined PSCC/SSCC surgery. The mean age was 49.8 ± 12 years (21-66). There was no difference between the preoperative and postoperative pure tone average (PTA) thresholds at 0.5-3 kHz. Higher thresholds were noted at 4, 6, and 8 kHz postoperatively. Deterioration (>10 dB) in the bone-conduction (BC) PTA was demonstrated in 3 of 22 (13.6%) cases with no significant difference in the demographic, clinical, surgical, and preoperative audiometric parameters relative to the cases without PTA BC change. A significantly larger difference in PTA BC (pre- vs. postoperative) was seen for males. Small effect size was noted for Air conduction (AC) PTA in males, and moderate effect size for Word Recognition Score (WRS) in surgery for the LSCC compare to SSCC. CONCLUSIONS: SCC surgeries carry a relatively low risk of deterioration in PTA BC. High frequency thresholds should also be included in postoperative hearing outcome assessment. Cases of LSCC for intractable Meniere's disease and surgery in males carry higher risk of poor postoperative hearing outcomes. Level of Evidence: 4.