Otology Neurotology

Inner Ear Proteins as Potential Biomarkers

01-02-2020 – Mulry, Erin; Parham, Kourosh

Journal Article

Objective: The purpose of this manuscript is to identify proteins exclusive to the inner ear based on published research to identify potential candidate biomarkers and guide future inner ear research.
Data Sources: Literature on inner ear proteins published on Pubmed, Google Scholar, and Scopus was reviewed using key words such as “inner ear molecule,” “inner ear exclusive protein,” and several specific protein searches such as “prestin” based on findings from the initial searches.
Study Selections: Studies were selected for abstract review based on title relevance, and full text was chosen for review based on abstract relevance. Several related studies cited in initially reviewed literature were also chosen to compile more detailed information on specific molecules with the goal of at least two to three published articles for each protein.
Data Extraction & Synthesis: Proteins that were cited to have only been found within the inner ear were included in this review, including some proteins that were later identified outside the inner ear. Information regarding their size, location, function, and clinical significance was recorded.
Conclusions: Based on this literature search, eight proteins exclusive to the inner ear were identified including otolin-1, otoconin 90/95, prestin, otoancorin, otogelin, α-tectorin, β-tectorin, and cochlin. Proteins initially found to be exclusive to the inner ear though later identified outside of the inner ear included oncomodulin, otospiralin, and otoraplin. This literature review may serve as a focused guide for future research on proteins exclusive to the inner ear as potential biomarkers for diseases of the inner ear.

Systematic Review and Network Meta-analysis of Cognitive and/or Behavioral Therapies (CBT) for Tinnitus

01-02-2020 – Landry, Evie C.; Sandoval, Xochitl Citlalli Romo; Simeone, Calla N.; Tidball, Glynnis; Lea, Jane; Westerberg, Brian D.

Journal Article

Objective: To evaluate the efficacy of cognitive and/or behavioral therapies in improving health-related quality of life (HRQOL), depression, and anxiety associated with tinnitus.
Data Sources: EMBASE, MEDLINE, PubMed, Psyc
INFO, and the Cochrane Registry were used to identify English studies from database inception until February 2018.
Study Selection: Randomized controlled trials (RCTs) comparing cognitive and/or behavioral therapies to one another or to waitlist controls for the treatment of tinnitus were included.
Data Extraction: Quality and risk were assessed using GRADE and Cochranes Risk of Bias tool respectively.
Data Synthesis: Pairwise meta-analysis (12 RCTs: 1,144 patients) compared psychological interventions to waitlist controls. Outcomes were measured using standardized mean differences (SMDs) and 95% confidence intervals (CI). I2 and subgroup analyses were used to assess heterogeneity. Network meta-analysis (NMA) (19 RCTS: 1,543 patients) compared psychological therapies head-to-head. Treatment effects were presented by network diagrams, interval plots, and ranking diagrams indicating SMDs with 95% CI. Direct and indirect results were further assessed by inconsistency plots.
Conclusions: Results are consistent with previously published guidelines indicating that CBT is an effective therapy for tinnitus. While guided self-administered forms of CBT had larger effect sizes (SMD: 3.44; 95% CI: −0.022, 7.09; I2: 99%) on tinnitus HRQOL, only face-to-face CBT was shown to make statistically significant improvements (SMD: 0.75; 95% CI: 0.53, 0.97; I2: 0%). Guided self-administered CBT had the highest likelihood of being ranked first in improving tinnitus HRQOL (75%), depression (83%), and anxiety (87%), though statistically insignificant. This NMA is the first of its kind in this therapeutic area and provides new insights on the effects of different forms of cognitive and/or behavioral therapies for tinnitus.

Genetic Epidemiology of Hearing Loss in the 22 Arab Countries: A Systematic Review

01-02-2020 – Sidenna, Mariem; Fadl, Tasnim; Zayed, Hatem

Journal Article

Background: Hearing loss (HL) is a heterogeneous condition that causes partial or complete hearing impairment. Hundreds of variants in more than 60 genes have been reported to be associated with Hereditary HL (HHL). The HHL prevalence is thought to be high in the Arab population; however, the genetic epidemiology of HHL among Arab populations is understudied. This study aimed to systematically analyze the genetic epidemiology of HHL in Arab countries.
Methods: We searched four literature databases (PubMed, Scopus, Science Direct, and Web of Science) from the time of inception until January 2019 using broad search terms to capture all the reported epidemiological and genetic data related to Arab patients with HHL.
Findings: A total of 2,600 citations were obtained; 96 studies met our inclusion criteria. Our search strategy yielded 121,276 individuals who were tested for HL over 52 years (1966–2018), of whom 8,099 were clinically diagnosed with HL and belonged to 16 Arab countries. A total of 5,394 patients and 61 families with HHL were genotyped, of whom 336 patients and 6 families carried 104 variants in 44 genes and were from 17/22 Arab countries. Of these variants, 72 (in 41 genes) were distinctive to Arab patients. Arab patients manifested distinctive clinical phenotypes. The incidence of HHL in the captured studies ranged from 1.20 to 18 per 1,000 births per year, and the prevalence was the highest in Iraq (76.3%) and the lowest in Jordan (1.5%).
Interpretation: This is the first systematic review to capture the prevalence and spectrum of variants associated with HHL in an Arab population. There appears to be a distinctive clinical picture for Arab patients with HHL, and the range and distribution of variants among Arab patients differ from those noted in other affected ethnic groups.

Spatial Hearing as a Function of Presentation Level in Moderate-to-Severe Unilateral Conductive Hearing Loss

01-02-2020 – Thompson, Nicholas J.; Kane, Stacey L. G.; Corbin, Nicole E.; Canfarotta, Michael W.; Buss, Emily

Journal Article

Hypothesis: Patients with moderate-to-severe unilateral conductive hearing loss (UCHL) can make use of binaural difference cues when stimuli are presented at a high enough intensity to provide audibility in the affected ear.
Background: Spatial hearing is essential for listening in complex environments and sound source localization. Patients with UCHL have decreased access to binaural difference cues, resulting in poorer spatial hearing abilities compared with listeners with normal hearing.
Methods: Twelve patients with moderate-to-severe UCHL, most due to atresia (83.3%), and 12 age-matched controls with normal hearing bilaterally participated in this study. Outcome measures included: 1) spatial release from masking, and 2) sound source localization. Speech reception thresholds were measured with target speech (Pediatric Az
Bio sentences) presented at 0 degree and a two-talker masker that was either colocated with the target (0 degree) or spatially separated from the target (symmetrical, ±90 degrees). Spatial release from masking was quantified as the difference between speech reception thresholds in these two conditions. Localization ability in the horizontal plane was assessed in a 180 degree arc of 11 evenly-spaced loudspeakers. These two tasks were completed at 50 and 75 d
Results: Both children and adults with UCHL performed more poorly than controls when recognizing speech in a spatially separated masker or localizing sound; however, this group difference was larger at 50 than 75 d
Conclusion: Patients with UCHL experience improved spatial hearing with the higher presentation level, suggesting that the auditory deprivation associated with a moderate-to-severe UCHL does not preclude exposure to—or use of—binaural difference cues.

Hemodynamic Changes in the Sigmoid Sinus of Patients With Pulsatile Tinnitus Induced by Sigmoid Sinus Wall Anomalies

01-02-2020 – Liu, Zhaohui; He, Xueying; Du, Runshuang; Wang, Guopeng; Gong, Shusheng; Wang, Zhenchang

Journal Article

Objective: This study is to investigate the hemodynamic changes of pulsatile tinnitus (PT) patients induced by sigmoid sinus wall anomalies (SSWA).
Study Design: Prospective study.
Setting: Tertiary referral university hospital.
Patients: Fifteen unilateral PT patients with SSWA identified on computed tomography images and surgery and 15 age-, sex-, and body mass index-matched healthy volunteers underwent velocity-encoded, cine magnetic resonance imaging.
Intervention: Hemodynamic data in sigmoid sinus were obtained from velocity-encoded, cine magnetic resonance imaging, and compared between PT patients and controls.
Main Outcome Measures: Heart rate was recorded. Cross-sectional area (CSA), peak positive velocity (PPV), average positive flow volume per beat (APFV/beat), average flow volume per beat (AFV/beat), peak negative velocity (PNV), and average negative flow volume per beat (ANFV/beat) were measured. Average flow volume per minute (AFV/min), average positive flow volume per minute (APFV/min), average negative flow volume per minute (ANFV/min), average positive velocity (APV), average negative velocity (ANV), and regurgitation fraction (RF) were calculated.
Results: APV at PT side of patients was 13.4 ± 3.3 cm/s, which was significantly slower than that at corresponding side of controls (15.8 ± 2.6 cm/s). PNV and RF at PT side of patients were 21.0 ± 15.4 cm/s and 2.4% respectively, which were significantly higher than those values at corresponding side of controls (both of them were 0). HR, CSA, PPV, APFV/beat, APFV/min, AFV/beat, AFV/min, ANV, ANFV/beat, and ANFV/min were 69.8 ± 9.4 beat/min, 48.4 ± 17 mm2, 31.4 ± 5.9 cm/s, 5.4 ± 1.8 ml/beat, 373.9 ± 117.7 ml/min, 5.1 ± 2.0 ml/beat, 352.0 ± 134.6 ml/min, 2 (0–4.9) cm/s, 1 (0–2.7) ml/beat, and 4.1 (0–141.3) ml/min at PT side of patients, and 67.4 ± 7.8 beat/min, 38.2 ± 18 mm2, 29.9 ± 3.9 cm/s, 5.3 ± 2.0 ml/beat, 350.3 ± 125.3 ml/min, 5.1 ± 1.9 ml/beat, 340.5 ± 117.9 ml/min, 0 (0–2.1) cm/s, 0 (0–0.8) ml/beat, and 0 (0–55.4) ml/min at corresponding side of controls. These hemodynamics were not significantly different between groups.
Conclusion: APV, PNV, and RF changes take place in SSWA patients, which may be associated with the occurrence of PT and have the potential value to improve accurate etiological diagnosis and predict treatment success.

Intraoperative Recording of Auditory Brainstem Responses for Monitoring of Floating Mass Transducer Coupling Efficacy During Revision Surgery—Proof of Concept

01-02-2020 – Fröhlich, Laura; Rahne, Torsten; Plontke, Stefan K.; Oberhoffner, Tobias; Dziemba, Oliver; Gadyuchko, Maria; Hoth, Sebastian; Mir-Salim, Parwis; Müller, Alexander

Journal Article

Objective: The objective of the study was to measure auditory brainstem responses elicited by stimulation via a semi-implantable active middle ear implant with an electromagnetically driven floating mass transducer to quantify the coupling efficacy (=vibroplasty in situ thresholds – bone conduction thresholds) in a patient during a revision surgery.
Patients: One patient, reimplanted with an active middle ear implant in a revision surgery.
Intervention(s): Diagnostic.
Main Outcome Measure(s): Intraoperative auditory brainstem responses evoked by stimulation via an active middle ear implant in a calibrated set-up directly indicating the coupling efficacy magnitude (auditory brainstem response threshold = coupling efficacy), as well as pre- and postoperative bone conduction and vibroplasty in situ thresholds.
Results: The intraoperative auditory brainstem response threshold was detected at 0 d
B n
HL, i.e., the magnitude of coupling efficacy was determined intraoperatively to be 0 d
B. The actual postoperative coupling efficacy (=postoperative vibroplasty in situ – postoperative bone conduction thresholds) was –2.5 d
Conclusions: The coupling efficacy determined intraoperatively was consistent with the postoperative coupling efficacy. The described method seems to be a promising tool to objectively quantify the magnitude of coupling efficacy in active middle ear implant surgeries.

A Case Series of Patients With Concurrent Otosclerosis and Superior Semicircular Canal Dehiscence

01-02-2020 – Dewyer, Nicholas A.; Quesnel, Alicia M.; Santos, Felipe

Journal Article

Objective: To describe the presentations and treatment results for patients with concurrent otosclerosis and superior semicircular canal dehiscence (SCD).
Study Design: Retrospective case series and literature review.
Setting: Tertiary academic medical center.
Patients: Patients with concurrent diagnoses of otosclerosis (radiographically and/or surgically confirmed) and SCD (radiographically confirmed) in the same ear.
Intervention(s): Review of medical records.
Main Outcome Measure(s): Clinical presentations, outcomes following stapedotomy.
Results: Eight patients with 10 affected ears were identified. All patients presented with slowly progressive conductive hearing loss, normal otoscopy, absent acoustic reflexes, and without other symptoms of SCD syndrome. Seven patients were treated with stapedotomy and 1 with hearing aids. Of those treated with stapedotomy, a persistent conductive hearing loss was the most common hearing result. One patient had near-complete closure of their air bone gap. None had a profound sensorineural hearing loss. Four patients had unmasking of SCD symptoms.
Conclusions: The clinical and audiometric presentations of patients with concurrent otosclerosis and SCD are often indistinguishable from those of patients with only otosclerosis. Computed tomography of the temporal bone is the only way to identify concurrent SCD. Stapedotomy in these patients typically results in a persistent conductive hearing loss, though 14 to 33% of patients experience near-complete closure of their air bone gap. SCD symptoms are unmasked in 57 to 63% of patients who undergo stapedotomy. Further work is needed to delineate the utility of routine preoperative computed tomography scan in otosclerosis patients, and to identify prognostic factors for patients with concurrent otosclerosis and SCD who wish to undergo stapedotomy.

Systematic Review of Pathogenic GJB2 Variants in the Latino Population

01-02-2020 – Bouzaher, Malek H.; Worden, Cameron P.; Jeyakumar, Anita

Journal Article

Objectives: Define the extent to which GJB2-related hearing loss is responsible for non-syndromic hearing loss (NSHL) in the Latino population.
Methods: Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed. PubMed and MEDLINE were accessed from 1966 to 2019 using permutations of the Me
SH terms: “Hearing Loss,” “Hearing Impairment,” “Deafness,” “Latin American,” “Latino,” “GJB2,” and “Genetic.” Additionally, countries designated as Latino by the US Office of Management and Bureau were cross-referenced as key terms against the aforementioned search criteria. Exclusion criteria included non-English publications, a non-Latino study population, and literature not investigating GJB2. An allele frequency analysis of pathogenic GJB2 variants in the Latino population was performed and stratified by country of origin and reported ethnicity.
Results: One hundred twenty two unique studies were identified of which 64 met our inclusion criteria. Forty three studies were included in the GJB2 systematic review. A total of 38 pathogenic GJB2 variants were identified across 20 countries in the Latino population. The prevalence of pathogenic GJB2 variants varied by country; however, were generally uncommon with the exception of c.35del
G (p.
Gly12Valfs*) which displayed an allele frequency of 3.1% in the combined Latino population; ranging from 21% in Colombia to 0% in Guatemala.
Conclusion: Variation in the prevalence of pathogenic GJB2 variants by country likely reflect the heterogeneous nature of ethnic ancestral contributions to the Latino population. Additional research utilizing next generation sequencing might aid in the development of assays for high throughput diagnosis of inherited hearing loss in the multitude of ethnic sub-groups that comprise this and other traditionally marginalized populations.

Factors Influencing Time to Cochlear Implantation

01-02-2020 – Dornhoffer, James R.; Holcomb, Meredith A.; Meyer, Ted A.; Dubno, Judy R.; McRackan, Theodore R.

Journal Article

Objective: To determine demographic and audiologic factors associated with time to treatment with cochlear implantation.
Methods: Retrospective review of a prospectively maintained adult cochlear implant database. A total of 492 patients were implanted from 2012 to 2017. Time to implantation, preimplantation audiologic outcomes, and demographic data were collected. Multivariate analysis was undertaken to establish demographic/audiologic factors that predict time to cochlear implantation.
Results: Using multivariate analysis, nonwhite race (hazard ratio 0.157, p = 0.038) and increased age (hazard ratio 0.970, p = 0.038) were associated with increased time to cochlear implantation. Nonwhite patients had significantly higher pure-tone averages and lower speech recognition scores (consonant–nucleus–consonant words and Az
Bio sentences in quiet) and were less likely to use hearing aids as compared with white patients (all p < 0.001). Sex (p = 0.188), health insurance type (p = 0.255), preoperative hearing aid use (p = 0.174), and audiologic outcomes were not significant predictors of time to implantation.
Conclusion: Nonwhite patients have poorer preoperative hearing and speech recognition and lower hearing aid use and are at risk for delay in referral and treatment for severe to profound sensorineural hearing loss. Other demographic factors, notably health insurance status, did not significantly predict time to cochlear implantation. Given the observed hearing healthcare disparities, special outreach programs may be needed to ensure timely cochlear implantation and effective hearing screening and rehabilitation.

Relations Between Scalar Shift and Insertion Depth in Human Cochlear Implantation

01-02-2020 – Zelener, Freddi; Majdani, Omid; Roemer, Ariane; Lexow, G. Jakob; Giesemann, Anja; Lenarz, Thomas; Warnecke, Athanasia

Journal Article

Objective: The intracochlear position of an electrode array may influence the outcome after cochlear implantation. The design of the electrode array can increase the risk of trauma causing penetration of the basilar membrane or shift of the electrode array into the scala vestibuli. The aim of the present study was to identify a scalar shift after implantation of two different electrode arrays developed by one manufacturer.
Study Design: Retrospective analysis.
Setting: Tertiary referral center.
Patients and Intervention: Cochlear implant recipients implanted between 2010 and 2014 and receiving either a mid-scala (n = 30) or a perimodiolar (n = 30) electrode array.
Main Outcome Measure: Occurrence of scalar shift in association with the electrode type.
Results: Scalar shift occurred in 26.7% (8 of 30) of the patients implanted with a perimodiolar electrode array and in 6.7% (2 of 30) of the patients implanted with the mid-scala electrode array. The mean insertion depth in the patients experiencing scalar shift after implantation of the mid-scala electrode was much deeper (21.59 ± 0.34 mm) when compared with the mean insertion depth of the patients with scalar shift after implantation with a perimodiolar electrode array (17.85 ± 2.19 mm). There tends to be a correlation between the cochlear length and the occurrence of a scalar shift. However, the number of patients with scalar shift in the mid-scala group is rather small.
Conclusion: Based on the presented data, more patients implanted with a perimodiolar electrode array have a scalar shift when compared with the midscalar electrode array.

Electrode Array Type and Its Impact on Impedance Fluctuations and Loss of Residual Hearing in Cochlear Implantation

01-02-2020 – Thompson, Nicholas J.; Dillon, Margaret T.; Buss, Emily; Park, Lisa R.; Pillsbury, Harold C. III; O’Connell, Brendan P.; Brown, Kevin D.

Journal Article

Hypothesis/Objective: Determine variables associated with electrode impedance fluctuations and loss of residual hearing in cochlear implant (CI) recipients.
Background: CI recipients with postoperative hearing preservation demonstrate superior speech perception with an electric-acoustic stimulation (EAS) device as compared with a CI-alone device. Maintaining superior speech perception over time relies on long-term hearing preservation; therefore, understanding variables that may contribute to loss of residual hearing is needed. Recent reports suggest a relationship between changes in electrode impedance and loss of residual hearing. The variables influencing this relationship have yet to be determined.
Methods: Review of pediatric and adult CI cases from 2013 to 2016 who presented with preoperative residual hearing. Regression analysis was performed to evaluate effects of array type (lateral wall vs. perimodiolar), manufacturer, age at implantation, and preoperative hearing on impedance. The correlation between peak impedance change and change in low-frequency hearing was determined.
Results: One hundred forty-six CI recipients presented with preoperative residual hearing. A multivariate regression analysis demonstrated a statistically significant association between preoperative hearing thresholds (p = 0.017), device manufacturer (p = 0.011), and array type (p = 0.038) on postoperative impedance changes. Hearing preservation rates and change in impedance differed by electrode array type. The association between peak impedance changes and loss of residual hearing differed between manufacturers (R2 = 0.208, p = 0.029 vs. R2 = 0.016, p = 0.609).
Conclusion: Impedance fluctuation appears to be a marker for loss of residual hearing for specific electrode array types and manufacturers. Specific arrays may affect the cochlear microenvironment differently, with different effects on postoperative hearing preservation.

Radial Forearm Free Flap for Cochlear Implant Coverage in a Post-Irradiated Field

01-02-2020 – Dewyer, Nicholas A.; Sethi, Rosh K.; Yamasaki, Alisa; Lee, Daniel J.

Journal Article

Objective: To describe a novel use of a radial forearm free flap (RFFF) for soft tissue coverage over a cochlear implant (CI) in a post-irradiated field.
Patients: Single patient case report of a woman with a history of radiation therapy for brainstem astrocytoma who suffered from repeated CI extrusion despite tympanomastoid obliteration and locoregional pedicled flap soft tissue coverage.
Intervention(s): Sequential bilateral, single-staged revision cochlear implantation with RFFF soft tissue coverage.
Main Outcome Measure(s): Postoperative wound healing complications including infection or device extrusion.
Results: There have been no further issues with wound healing, infection, or device extrusion with a follow up period of 3 years on one side and 8 months on the second side. Both CIs are functioning well with the flaps being sufficiently thin to allow for the use of typical external processor magnets.
Conclusions: A RFFF can be used to provide robust soft tissue coverage over a CI in a post-irradiated field. The RFFF and CI may safely be performed in a single operation.

Predicting Reduced Tinnitus Burden After Cochlear Implantation in Adults

01-02-2020 – Dixon, Peter R.; Crowson, Matthew; Shipp, David; Smilsky, Kari; Lin, Vincent Y.; Le, Trung; Chen, Joseph M.

Journal Article

Objective: Explore patient characteristics associated with tinnitus improvement after cochlear implantation.
Study Design: Retrospective cohort.
Setting: Tertiary referral.
Patients: Adults with bilateral severe-to-profound hearing loss and tinnitus.
Interventions: Unilateral cochlear implantation.
Results: From 1996 to 2018, 358 patients endorsed pre-implant tinnitus and had ascertainable tinnitus status at 1-year. Clinically significant improvement in Tinnitus Handicap Inventory (THI) (reduction by at least 7-points) was observed in 262 (73.2%) patients, of whom 155 (59.2%) reported complete resolution. Of the 24 characteristics explored, four were identified as independent predictors of improved tinnitus in logistic regression models. In a multivariable model including identified independent predictors, each 10-percentage point increase in baseline hearing in noise test was associated with an 14% reduction in odds of tinnitus resolution or clinically significant improvement (odds ratio OR 0.86, 95% confidence limits CL 0.77, 0.96) and preoperative use of a hearing aid in the contralateral ear was associated with a 72% reduction (OR 0.28; 95% CL 0.11, 0.73). Each 10-point increase in baseline Hearing Handicap Inventory for Adults (HHI) score was associated with a 28% increase in odds of tinnitus improvement (OR 1.28; 95% CL 1.07, 1.54). Higher baseline burden of tinnitus was associated with higher odds of tinnitus improvement (OR 1.21 per 10-point THI increase, 95% CL 1.04, 1.40).
Conclusions: Worse residual hearing and higher baseline hearing and tinnitus handicap are associated with higher odds of tinnitus improvement. Expectations of patients seeking reduced tinnitus burden following cochlear implantation should be managed by counselling regarding odds of tinnitus improvement compared to those with similar residual hearing and tinnitus burden.

Cochlear Enhancement May Precede Cochlear Obliteration After Vestibular Schwannoma Excision

01-02-2020 – Erbele, Isaac D.; Miller, Laura S.; Mankekar, Gauri; Morel, Christian E.; Anderson, Dwayne T.; Son, Leslie S.; Arriaga, Moises A.

Journal Article

Objective: Cochlear obliteration after vestibular schwannoma excision has been noted, with implications on cochlear implantation. Early postoperative cochlear enhancement with gadolinium on magnetic resonance imaging (MRI) has also been observed. Timing of enhancement and association with obliteration is described here.
Study Design: Retrospective case review.
Setting: Tertiary referral center, ambulatory.
Patients: Patients receiving vestibular schwannoma excision surgery by the senior author performed at one institution between January 2015 and July 2017 with postoperative MRIs
Intervention: Diagnostic.
Main Outcome Measure(s): The imaging characteristics on postoperative MRIs examined were loss of fluid signal on postoperative T2 images and cochlear enhancement on gadolinium enhanced T1 images. In the patients receiving labyrinthine sparing procedures, presence of postoperative hearing was evaluated.
Results: Of the 42 patients evaluated, 24 received the translabyrinthine approach and 18 received a labyrinth sparing surgery. Twenty-nine had evidence of cochlear enhancement on T1 with gadolinium contrast, and 27 had evidence of cochlear obliteration on T2 images. The odds ratio of patients with cochlear enhancement having obliteration was 30.0:1 (p < 0.0001). Intense cochlear enhancement (n = 21) appeared a median of 163 days after surgery, and complete or near complete obliteration (n = 18) appeared a median of 480 days after surgery, a statistically significant difference (p < 0.001). Within the labyrinth sparing group, there was no statistically significant association between hearing loss and cochlear obliteration or enhancement.
Conclusions: Cochlear enhancement is correlated with cochlear obliteration and may precede it.

Postoperative Healthcare Utilization of Elderly Adults After Cochlear Implantation

01-02-2020 – Raymond, Mallory J.; Dong, Andy; Naissir, Samir Ballestas; Vivas, Esther X.

Journal Article

Objective: To determine the association between geriatric age and postoperative healthcare utilization after cochlear implantation.
Study Design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: Older adults (>59 yr) who underwent unilateral cochlear implantation from 2009 until 2016.
Intervention(s): Standard electrode length cochlear implantation.
Main Outcome Measure(s): Postoperative surgical and audiological visit rate after cochlear implantation for those aged 60 to 69, 70 to 79, and 80+ years.
Results: Fifty-nine older adult patients were included in the study with a mean age of 71.5 ± 6.9 years (range, 60–88 yr), mean duration of hearing loss of 25.4 ± 19.6 years (range, 0.25–67 yr), and mean length of follow up of 37 ± 24.6 months (range, 6–107 mo). There was no significant difference in the mean number of surgical and audiological visits over both the first and second postoperative years between those aged 60 to 69, 70 to 79, and 80+ years. Additionally, on one-way multivariate analysis of covariance (MANCOVA), there was no significant difference in cumulative postoperative healthcare utilization measures between each age group, when controlling for postoperative Az
Bio scores, estimated household income, and driving distance to the hospital.
Conclusions: Older geriatric adults do not have higher rates of postoperative healthcare utilization after cochlear implantation than their younger, geriatric hearing impaired counterparts, despite presumed higher rates of frailty and comorbidity.

Feasibility of Pediatric Robotic Cochlear Implantation in Phantoms

01-02-2020 – Bom Braga, Gabriela O’Toole; Schneider, Daniel; Muller, Fabian; Hermann, Jan; Weber, Stefan; Caversaccio, Marco

Journal Article

Objective: To demonstrate the feasibility of robotic cochlear implant surgery in subject specific pediatric phantoms.
Study Design: Pilot study.
Materials and Methods: Computed tomographic preoperative encrypted data of 10 pediatric subjects (total of 20 sides) between 8 months and 48 months old, who underwent cochlear implant surgery were studied. Four datasets (n = 8 sides) were selected for investigation of the complete robotic procedure including middle and inner ear access and electrode insertion.
Results: The planning of the safe trajectory for the robotic approach was possible in 17 of the cases. In three sides, planning the trajectory was not possible due to the small size of the facial recess. Bone thickness study demonstrated average sufficient bone thickness at the site of screw implantation in general. The complete robotic procure including the drilling and insertion was successfully carried out on all the created phantoms.
Conclusion: With this work we have demonstrated the feasibility of planning and performing a robotic middle and inner ear access and cochlear implantation (CI) in phantom models of pediatric subjects. To develop and validate the proposed procedure for use in children, next stage optimization of the current surgical workflow and adaptation of the surgical material to pediatric population is necessary.

Monitoring Cochlear Nerve Integrity During Vestibular Schwannoma Microsurgery in Real-Time Using Cochlear Implant Evoked Auditory Brainstem Response and Streaming Neural Response Imaging

01-02-2020 – Patel, Neil S.; Saoji, Aniket A.; Olund, Amy P.; Carlson, Matthew L.

Journal Article

Objective: Intraoperative far-field auditory brainstem response (ABR) and direct cochlear nerve action potential monitoring using neural response imaging (NRI) are techniques for monitoring the cochlear nerve during vestibular schwannoma (VS) surgery. A new paradigm has developed where the cochlear nerve is anatomically preserved during tumor removal to facilitate cochlear implantation in select circumstances. This report describes the use of an unmodified commercial cochlear implant (CI) to elicit electrically-evoked direct cochlear nerve and far-field ABR monitoring to evaluate the status of the cochlear nerve during tumor resection in real time.
Patients: Adult female with unilateral, sporadic VS.
Intervention(s): Cochlear implantation followed by translabyrinthine resection of VS in single operation.
Main Outcome Measures: During tumor resection intra-cochlear electrodes were used to deliver electrical stimulation and measure NRI or the cochlear nerve action potential. Electrically-evoked ABR (e
ABR) was measured using surface electrodes and wave V was monitored (far-field ABR) during surgery.
Results: A 61-year-old female was evaluated for a unilateral, enlarging intracanalicular VS with asymmetric SNHL. The patient opted for microsurgery and due to her bilateral hearing loss was a candidate for CI. Cochlear implantation with an unmodified, commercially available lateral wall electrode was performed. The remainder of the approach and tumor resection was performed under continuous e
ABR and NRI monitoring. Gross total resection was achieved with intact e
ABR and NRI at the conclusion of the case. Changes in ABR and NRI consistently recovered after modifying dissection strategy. The patient reported auditory percepts with her cochlear implant postoperatively.
Conclusions: This report demonstrates the feasibility of using real-time NRI and e
ABR with a CI to facilitate preservation of the cochlear nerve during VS microsurgery. Using this method to mitigate cochlear nerve trauma during microsurgery may preserve the option of CI for hearing rehabilitation.

Speech Perception, Dichotic Listening, and Ear Advantage in Simultaneous Bilateral Cochlear Implanted Children

01-02-2020 – Koopmann, Moritz; Lesinski-Schiedat, Anke; Illg, Angelika

Journal Article

Objective: To study the development of dichotic listening, ear advantage, and speech perception in cochlear implant (CI) recipients using five audiometric tests.
Study Design: Prospective cohort analysis.
Setting: University Hospital.
Patients: One hundred twenty one participants who received simultaneous bilateral CIs and had at least 12 months of regular bilateral CI use. Age at time of testing ranged from 4 to 18 years with a mean age of 9 years (standard deviation SD: 3.0). Mean duration of CI use was 7.073 years (SD: 2.86).
Intervention: Bilateral simultaneous cochlear implantation.
Main Outcome Measure: Goettinger and Freiburger monosyllabic word test, Hochmair, Schulz and Desoyer sentence (HSM) test in noise and in quiet conditions and the dichotic listening test by Uttenweiler were applied according to the childrens’ age. The results were evaluated using a two-sided t test for dependent samples.
Results: In all tests applied, we could not show significant differences in performance between left and right CI and therefore could not demonstrate an ear advantage. Dichotic listening was challenging, only one subject produced results required for normal hearing subjects to pass the test. The older the CI recipients get and duration of CI use increases, the more results in all speech perception tests improve.
Conclusions: Our results cannot lend further support to the existence of ear advantage in CI recipients. We do not recommend the Uttenweiler dichotic listening test to determine lateralization. Further research to gather more information on dichotic listening and ear advantage in CI recipients is necessary and should use consonant-vowel stimuli as more comparable stimuli.

The Image Fusion Technique for Cochlear Implant Imaging: A Study of its Application for Different Electrode Arrays

01-02-2020 – Sipari, Sini; Iso-Mustajärvi, Matti; Könönen, Mervi; Löppönen, Heikki; Dietz, Aarno

Journal Article

Objectives: To investigate the benefits of the image fusion technique for precise postoperative assessment of intracochlear placement with six different electrode arrays.
Study Design: Consecutive retrospective case study.
Settings: Tertiary referral center.
Patients: Analyses of imaging data of 30 patients implanted with six different electrode arrays.
Interventions: Electrode reconstructions obtained from postoperative cone-beam computed tomography (CBCT) were overlaid onto preoperative magnetic resonance imaging (MRI) and/or high-resolution computed tomography (HRCT) registrations to create artefact-free images.
Main Outcome Measures: Each electrodes intracochlear position was analyzed with the image fusion reconstructions and compared with the results obtained by CBCT alone. The electrode location was classified according to its position in relation to the basal membrane at four different insertion angles.
Results: In 40 out of 151 measurements (26.5%), the location grading obtained by CBCT alone changed after the assessment with the image fusion reconstructions. A significant association was found between deep insertions (over 360 degrees) and the effectiveness of image fusion (p = 0.019). The difference between the impact of the fusion technique for the basal turn versus the apical part was highly significant (p = 0.001). There was no significant difference between the effectiveness of the image fusion and the different electrodes.
Conclusions: By utilizing an image fusion technique, a more accurate assessment of electrode placement could be achieved for all types of electrodes. Image fusion was especially beneficial for insertions beyond 360 degrees.

Same-Day Patient Consultation and Cochlear Implantation: Innovations in Patient-Centered Health Care Delivery

01-02-2020 – Nassiri, Ashley M.; Yawn, Robert J.; Gifford, René H.; Holder, Jourdan T.; Stimson, C. J.; Eavey, Roland D.; Haynes, David S.

Journal Article

Objective: To develop and implement a streamlined, patient-centered service delivery model for patients referred for cochlear implantation (CI) at a high-volume academic center.
Patients: CI candidate adults.
Interventions: CI, implementation of new CI delivery model.
Main Outcome Measures: Referral-to-surgery time, patient travel burden.
Results: Data from 206 adults that underwent CI were used to develop a process map of the initial operational state from referral date to day of surgery (referral-to-surgery time). The initial referral-to-surgery time was 136 days on average, yet the average total work time by all involved providers was 17.6 hours. Prolonged wait times were associated with the following preoperative tasks: appointment scheduling, insurance approval, device ordering and shipment, and surgical scheduling. Patients traveled to the institution on at least two occasions for appointments. A new bundled, patient-centered CI delivery model was developed to address prolonged wait times, travel burden, and process inefficiencies. The new model implemented an interactive electronic medical record, coordinated appointments with same-day surgery, and stocked device inventory to reduce the referral-to-surgery time to 24 days—an improvement of 112 days. In the new model, new patient consultation and surgery were completed in one day, reducing the patient travel burden to the institution.
Conclusions: The new CI program demonstrates that delivery innovations can have a substantial impact on measures of patient convenience and experience, and that these results are achievable without new technologies or changes in medical management. With a focus on patient-centered design, health care delivery models can be augmented to increase value for patients.

Duration of Processor Use Per Day Is Significantly Correlated With Speech Recognition Abilities in Adults With Cochlear Implants

01-02-2020 – Holder, Jourdan T.; Dwyer, Nichole C.; Gifford, René H.

Journal Article

Objective: Quantify the relationship between average hours of processor use per day and measures of speech recognition in post-lingually deafened adults with cochlear implants.
Setting: Cochlear implant (CI) program at a tertiary medical center.
Patients: Three hundred adult (mean age = 64, 130 women) CI users were included.
Main Outcome Measures: Correlation analyses were completed for CI-aided speech recognition (Consonant-Nucleus-Consonant CNC monosyllables and Az
Bio sentences) at approximately 12 months post-implantation and average hours of processor use per day, which was extracted from the CI programming software.
Results: Average processor use was 10.2 hours per day (range, 0.1–22.7), and average speech recognition scores were 49.9 and 61.7% for CNC and Az
Bio sentence recognition, respectively. We found a strong, significant correlation between hours of processor use per day and consonant-nucleus-consonant (CNC) word recognition (rs = 0.61, p < 0.0001) and Az
Bio sentence recognition (rs = 0.56, p < 0.0001).
Conclusions: Results suggest that highest speech recognition outcomes are correlated with greater than 10 hours of CI use per day. Further research is needed to assess the causal link between daily CI use and speech recognition abilities.

The Management of Tympanic Membrane Perforation With Endoscopic Type I Tympanoplasty

01-02-2020 – Marchioni, Daniele; Gazzini, Luca; De Rossi, Stefano; Di Maro, Flavia; Sacchetto, Luca; Carner, Marco; Bianconi, Luca

Journal Article

Objective: The objective of this study is to describe what we consider to be the state-of-the-art procedure for the treatment of tympanic membrane perforations, and to present the results attained in our institution.
Study Design: A retrospective cohort study, using data of Verona University Hospital, Italy. This medical record includes the data of 98 patients who underwent 100 transcanal endoscopic type I tympanoplasties from November 2014 to October 2017.
Setting: Tertiary referral center University Hospital of Verona, Italy.
Patients: Were enrolled 109 patients, that underwent endoscopic type I tympanoplasty in the period considered. Out of the selected patients, 11 (10.1%) were lost to long-term follow-up, and were therefore excluded from our study. Other exclusion criteria were surgical approaches that included other procedures. Patients whose follow-up was shorter than 6 months were excluded from this study.
Intervention: The technique is based on an endoscopic placement of underlay graft of temporal fascia or tragal cartilage. We consider the data of four surgeons from Verona University ENT department.
Main Outcome Measure: In the study we considered the reduction of the Air Bone Gap as functional outcome and the integrity of the reconstruction as anatomical outcome of success.
Results: No major intraoperative complications were observed. The closure rate was 86%. The mean surgery time was 48.6 minutes. The air bone gap was improved within 20 DB HL in 89% of patient. Only 8% of patients needed revision surgery, and none needed a third surgical evaluation.
Conclusion: Endoscopic ear surgery is by now a reality that has replaced in many cases exclusive microscopic ear surgery. Transcanal endoscopic type I tympanoplasty can be considered nowadays as an alternative technique for tympanic membrane perforations.

Reducing Postoperative Call Volume Through Verbal Preoperative Education

01-02-2020 – Chern, Alexander; Haynes, David S.; Bennett, Marc L.

Journal Article

Objective: To improve patient satisfaction and understanding of what to expect after chronic ear surgery and reduce call volume to an otology clinic at an academic tertiary referral center.
Study Design: Quality improvement initiative.
Setting: A single-academic tertiary referral center.
Patients: One hundred and ten patients who underwent chronic ear surgery in March to May 2018.
Intervention: Preoperative counseling over the phone 1 week before surgery.
Main Outcome Measures: Patient call volume to the clinic within a 2-week postoperative window, patient understanding, and satisfaction of perioperative course.
Results: There was a significant increase in patient satisfaction (10.1% increase, 9.8 intervention vs. 8.9 no intervention, p = 0.0032) and in patient understanding of what to expect after surgery (6.7% increase, 9.5 intervention vs. 8.9 no intervention, p = 0.0275). There was a significant decrease in mean number of calls per patient to the clinic (57.6% decrease, 0.31 intervention vs. 0.72 no intervention, p = 0.0105) and in percentage of patients who made any number of calls to the clinic (20% intervention vs. 46%, no intervention, p = 0.00438).
Conclusions: Verbal preoperative counseling over the phone was effective in significantly reducing unnecessary call volume to the clinic and in improving patient satisfaction and overall understanding of what to expect after surgery.

Comparison of Opioid Prescription Patterns and Consumption Following Otologic Surgery

01-02-2020 – Ngombu, Stephany; Hooks, Andrea; Rock, Alexander N.; Nogan, Stephen; Adunka, Oliver F.; Dodson, Edward E.; Moberly, Aaron C.; Mattingly, Jameson K.

Journal Article

Objective: To examine opioid prescribing patterns and consumption among patients undergoing common otologic surgeries.
Study Design: Retrospective cohort study with chart review and telephone survey.
Setting: Tertiary academic medical center.
Methods: Retrospective chart review and telephone survey of those undergoing tympanoplasty, tympanomastoidectomy, stapedectomy, and cochlear implantation in 2018. The survey consisted of questions regarding the details of the number of pills taken, duration of opioid use, subjective pain control, the use of over-the-counter pain medications, opioid disposal, and their history of substance abuse.
Results: Sixty-one patients were able to be contacted and agreed to participate in the study. Fifty-nine (96.7%) stated that their pain was controlled, and 10 (16.4%) did not take any opioids postoperatively despite their prescription. The mean morphine milligram equivalent (MME) prescribed was 99.9 (44.3) and MME taken was 45.2 (SD 46.3) (p  0.05). Analysis of the MME and pills prescribed and taken among the different surgeries (tympanoplasty, stapes surgery, tympanomastoidectomy, and cochlear implantation) revealed no statistically significant interactions (p > 0.05). Pain control was achieved for 50% of patients with 5 pills (MME = 25 mg), for 75% with 12 pills (MME = 60 mg), and for 90% with 24 pills (MME = 135 mg).
Conclusion: The opioid epidemic continues to be an ongoing issue in the United States, and prescription opioid abuse is a large contributor. There is increasing literature to suggest a practice of overprescribing in multiple surgical specialties. This same finding appears to be present in common otologic surgeries, where on average patients took less than half of the prescribed MME/pills, and 75% of patients had their pain controlled with 12 pills or fewer. Otolaryngologists performing otologic surgery should strongly consider adjusting their postoperative regimens to reflect these findings.
Level of Evidence: 2b

Comparison of Spontaneous Temporal Bone Cerebrospinal Fluid Leaks From the Middle and Posterior Fossa

01-02-2020 – Cooper, Timothy; Choy, Matthew H.; Gardner, Paul A.; Hirsch, Barry E.; McCall, Andrew A.

Journal Article

Objectives: To compare patients surgically managed for spontaneous cerebrospinal fluid (CSF) leaks of the temporal bone arising from the middle cranial fossa (MCF) and posterior cranial fossa (PCF) and to describe the surgical management of posterior fossa CSF leaks.
Study Design: Retrospective case review.
Setting: Academic tertiary center.
Patients: Adult patients presenting with spontaneous temporal bone CSF leaks undergoing operative repair between January 2010 and August 2018. Patients with a history of trauma, previous mastoid surgery, and iatrogenic CSF leaks were excluded.
Intervention: Transmastoid or MCF CSF leak repair.
Main Outcome Measures: Patient demographics, body mass index (BMI), comorbidities, presenting features, and lumbar puncture opening pressures were compared between groups and the management of the PCF CSF leaks described.
Results: Forty-six patients (26 women, 20 men) were included. The mean age at the time of repair was 58.0 ± 12.9 years (±SD). The origin of the CSF leak was from the PCF in three patients and MCF in 43 patients. All three patients with PCF leaks presented with an acute history of meningitis compared with only seven (16%) in the MCF group. This difference was statistically significant (p = 0.01, Fishers exact test). There were no statistically significant differences in age, sex, BMI, or lumbar puncture opening pressures. The PCF leaks were repaired using a transmastoid approach with multilayer closure of the bony defect and fat graft obliteration of the mastoid.
Conclusions: Spontaneous CSF leaks arising from the PCF are rare and may present more commonly with meningitis. Identification requires careful review of imaging.

Use of IL-5 Inhibitor Benralizumab as a Novel Therapy for Eosinophilic Otitis Media: Clinical Capsule and Review of Literature

01-02-2020 – Chow, Kevin; Cosetti, Maura K.

Journal Article

Objective: We report a case of recurrent eosinophilic otitis media (EOM) successfully treated with the novel monoclonal IL-5 inhibitor benralizumab. We also review literature relevant to EOM diagnosis and potential for biologic treatment.
Patient: A 24-year-old woman with bilateral otitis media with effusion unresponsive to standard treatment.
Intervention(s): Multidisciplinary treatment using subcutaneous benralizumab after diagnosis of EOM.
Main Outcome Measure(s): Behavioral audiometry, peripheral serology, otomicroscopy, and eosinophilic otitis media symptom severity.
Results: Recalcitrant otitis media with effusion (OME) in the setting of nasal polyposis and asthma prompted clinical suspicion for EOM, which was confirmed by pathologic examination of middle ear effusion and soft tissue biopsy. Treatment with benralizumab was initiated by pulmonology and well-tolerated. Within 2 months of treatment onset there was a cessation of EOM symptoms, a reduction of peripheral eosinophil levels, and an improvement in conductive hearing loss.
Conclusions: Monoclonal antibodies such as benralizumab may be an effective treatment option for EOM. A high level of clinical suspicion in patients with bronchial asthma, nasal polyposis, and recalcitrant OME may allow early diagnosis of EOM. Awareness of emerging biologic treatment options is important in the management of this challenging entity and may prevent long-term sequelae.

“The Importance of the Temporal Bone 3T MR Imaging in the Diagnosis of Menières Disease”

01-02-2020 – Paškoniene, Aiste; Baltagalviene, Renata; Lengvenis, Givi; Beleškiene, Vilma; Ivaška, Justinas; Markeviciute, Vetra; Mickeviciene, Vaiva; Lesinskas, Eugenijus

Journal Article

Background: The aim of this study was to evaluate endolymphatic hydrops using the 3T temporal bone magnetic resonance imaging (MRI), performed according to the chosen protocol, and determine whether it could be applied as an objective diagnostic tool for Menières disease.
Methods: 105 participants diagnosed with probable (n = 50) and definite (n = 55) Menières disease were included in this prospective study at Vilnius University Hospital, Santaros Clinics. Audiometry, vestibular function tests, videonystagmography, and computer posturography were performed before MRI. The 3T MRI with gadolinium contrast was performed to evaluate the endolymphatic hydrops. Imaging protocol consisted of 3D-FLAIR and 3D T2DRIVE sequences. Vestibular endolymphatic sac was interpreted as enlarged if occupied more than 50% of the vestibular area.
Results: 78.1% of subjects had abnormal MRI findings other than hydrops, and it was more than 90% (50/55) of patients in the definite MD group (p < 0.001). Changes in caloric test were observed in 63.8% of subjects in general, and in 76.4% of patients with a definite Menières disease. The side of the endolymphatic hydrops observed on MR imaging corresponded to the clinical diagnosis of the Menières disease based on the results of audiometry (p < 0.001) and unilateral weakness (p < 0.001). Endolymphatic hydrops on MRI and directional preponderance in caloric test were two independent predictors of the definite Menières disease.
Conclusions: Temporal bone 3T MRI with gadolinium contrast is clinically superior to confirm the diagnosis of Menières disease. Grade II endolymphatic hydrops on MRI, directional preponderance, and unilateral weakness on caloric test were independent predictors for the definite Menières disease.

“On the Relationship Between Menières Disease and Endolymphatic Hydrops”

01-02-2020 – Gluth, Michael B.

Journal Article

The relationship between Menières disease and endolymphatic hydrops is ambiguous. On the one hand, the existence of cases of endolymphatic hydrops lacking the classic symptoms of Menières disease has prompted the assertion that endolymphatic hydrops alone is insufficient to cause symptoms and drives the hypothesis that endolymphatic hydrops is a mere epiphenomenon. Yet, on the other hand, there is considerable evidence suggesting a relationship between the mechanical pressure effects of endolymphatic hydrops and resultant disordered auditory physiology and symptomatology. A critical appraisal of this topic is undertaken, including a review of key histopathologic data chiefly responsible for the epiphenomenon hypothesis. Overall, a case is made that A) the preponderance of available evidence suggests endolymphatic hydrops is likely responsible for some of the auditory symptoms of Menières disease, particularly those that can be modulated by mechanical manipulation of the basilar membrane and cochlear microphonic; B) Menières disease can be reasonably considered part of a larger spectrum of hydropic inner ear disease that also includes some cases that lack vertigo. C) The relationship with endolymphatic hydrops sufficiently robust to consider its presence a hallmark defining feature of Menières disease and a sensible target for diagnostic detection.

Associations of Vestibular Tests With Penn Acoustic Neuroma Quality of Life Scores After Resection of Vestibular Schwannoma

01-02-2020 – Brown, Clifford Scott; Cooper, Matthew W.; Peskoe, Sarah B.; Risoli, Thomas Jr.; Kaylie, David M.

Journal Article

Objective: Determine associations between preoperative caloric testing and video head impulse testing (v
HIT) with baseline and postoperative Penn Acoustic Neuroma Quality of Life (PANQOL) scores following resection of vestibular schwannoma (VS).
Study Design: Retrospective case series.
Setting: Two tertiary referral hospitals.
Patients: Adult patients with unilateral VS, preoperative calorics, v
HIT, and dizziness handicap inventory (DHI) score.
Interventions: Surgical resection of VS and postoperative surveys.
Main Outcome Measures: PANQOL scores.
Results: Forty-three patients were included (58.1% women) with a median age of 54 years (range, 28–82). Mean tumor size was 14.8 mm (σ=8.6), and 28 (65.1%) were right-sided. Average preoperative v
HIT gain was 0.7 (σ = 0.3). Covert and overt saccades were present in 8 (25%) and 14 (42.4%) patients, respectively. Average preoperative unilateral weakness was 47% (σ = 33.2). Translabyrinthine approach was performed in 26 (60.5%) patients. No significant difference of PANQOL scores was noted at baseline or over time between patients with normal (>0.8) or abnormal (50%) had significantly higher baseline PANQOL scores compared with those with < 25% or 25 to 50% (p = 0.02), but had significant improvement in scores over time (p = 0.01). Higher preoperative DHI preoperatively was significantly associated with worse PANQOL scores at all timepoints (β=0.57, p = 0.0064). No differences in PANQOL scores amongst surgical approaches were observed.
Conclusion: Preoperative vestibular testing with v
HIT, calorics, DHI, and baseline PANQOL surveys may allow for patient counseling regarding postoperative quality of life over time.

Lesion Patterns and Possible Implications for Recovery in Acute Unilateral Vestibulopathy

01-02-2020 – Navari, Elena; Casani, Augusto Pietro

Journal Article

Objective: To assess patterns of end-organ involvement in acute unilateral vestibulopathy (AUV) and to define the recovery.
Study Design: Retrospective chart review.
Setting: Tertiary academic referral hospital.
Patients and Interventions: Fifty-nine patients fulfilling clinical criteria for AUV and evaluated using the caloric vestibular test, video head impulse test, and both cervical and ocular vestibular evoked myogenic potentials, up to 10 days from symptoms onset were included.
Main Outcome Measures: Vestibular tests and requirements for vestibular rehabilitation were analyzed.
Results: The most affected end-organ was the horizontal canal (97%), followed by the anterior canal (83%), utricle (73%), posterior canal (46%), and saccule (44%). Nineteen (32%) patients exhibited complete receptors lesion, while 13 (22%) exhibited damage to receptors connected with the superior division of the vestibular nerve (VN). The proportion of patients who underwent vestibular rehabilitation was higher in the latter two groups (58% for both). Partial involvement of the receptors innervated by both the division of the VN, rather than by its superior division, was detected in 22 and 24% of patients, respectively. Total or partial damage to receptors innervated by the inferior VN was not found.
Conclusion: Deeper understanding of AUV may be achieved through identification of its patterns. Slightly more than one-half of AUV cases seemed to be associated with a nerve lesion, with a worse clinical outcome. The remaining patients exhibited selective involvement of vestibular receptors, more probably consistent with an intralabyrinthine lesion pattern and experienced better spontaneous recovery.

The Conditional Probability of Vestibular Schwannoma Growth at Different Time Points After Initial Stability on an Observational Protocol

01-02-2020 – Sethi, Mantegh; Borsetto, Daniele; Cho, Yeajoon; Gair, Juliette; Gamazo, Nicola; Jefferies, Sarah; Joannides, Alexis; Mannion, Richard; Helmy, Adel; Axon, Patrick; Donnelly, Neil; Tysome, James R.; Bance, Manohar

Journal Article

Objective: The natural history of vestibular schwannomas (VS) is well documented in the literature, with tumour growth being paramount to decision making for both surveillance and treatment of these patients. Most previous studies refer to the risk of VS growth over a given period of time; however, this is not useful for counselling patients at different stages of their follow-up, as the risk of tumour growth is likely to be less following each subsequent year that a tumour does not grow. Accordingly, we investigated the conditional probability of VS growth at particular time-points, given a patient has not grown thus far. This Bayesian method of risk stratification allows for more tailored and accurate approximations of the risk of growth versus nongrowth of VS.
Methods: Retrospective analysis of a prospectively collected database in a tertiary referral skull base unit, containing all patients diagnosed between 2005 and 2014 with sporadic unilateral VS and a minimum of 5-year surveillance.
Results: A total of 341 patients met the inclusion criteria. The mean age at diagnosis was 67 years, the sizes of the VS at diagnosis were intracanalicular in 49%, small in 39%, medium in 11%, and large in 1%. Over the entire 5-year surveillance period, a total of 139 tumours were seen to grow (41%) and 202 did not grow (59%). At 1 year, the probability of growth given that the tumour had not grown to date was seen to be 21%, at 2 years 12%, at 3 years 9%, at 4 years 3%, and at 5 years 2%. The conditional probability of growth of extracanalicular VS was significantly higher in the first year when compared with intracanalicular VS (29% versus 13%, p = 0.01), but there was no such difference in years 2, 3, 4 or 5 (p = 0.60, 0.69, 0.36, 0.39, respectively).
Conclusion: This is the first study in the literature concerned specifically with the conditional probability of VS growth. The data presented here can be used to better inform VS patients of their risk of growth at particular time points in their disease—the longer VS have been observed to be stable, the lower the risk of subsequent growth in a given year. Further, an extracanalicular vestibular schwannoma is more likely to grow in the first year compared with an intracanalicular vestibular schwannoma. Our data also adds support to surveillance protocols with increasingly infrequent MRI scans, as after 4 years of not growing, the risk of growth in year 5 falls to <2%.

Quantifying Tertiary Referral Center Bias in Vestibular Schwannoma Research

01-02-2020 – Saba, Elias S.; Marinelli, John P.; Lohse, Christine M.; Link, Michael J.; Carlson, Matthew L.

Journal Article

Objective: The majority of research influencing our understanding of vestibular schwannoma (VS) comes from large tertiary referral centers, and as a consequence, is inherently prone to referral bias. The objective of the current study was to characterize tertiary referral center bias in VS research.
Study design: Single-institution retrospective clinical, audiometric, and radiologic review.
Setting: One tertiary referral center.
Patients: One hundred twenty-three patients with sporadic VS treated at our institution, consisting of a local cohort of all 41 VS patients residing in the same US county as our medical center and a referral cohort of 82 patients from outside counties matched 2:1 based on age, sex, and year of diagnosis.
Intervention: Surgical resection, stereotactic radiosurgery (SRS), observation.
Main outcome measures: Clinical manifestations, audiometric and radiologic findings, elected treatment modality.
Results: Local patients had significantly longer follow-up after initial presentation compared to referral patients (median 4.0 vs 2.2 yr; p = 0.007). Referral patients were significantly less likely to have an incidental VS diagnosis (1% vs. 29%; p < 0.001) and presented with worse symptomatology such as hearing loss (80% vs. 54%; p = 0.002), dizziness (72% vs. 44%; p = 0.002), headache (29% vs. 15%; p = 0.075), and other cranial nerve dysfunction (11% vs. 0%; p = 0.029). Audiometrically, referral patients had significantly poorer word recognition scores at presentation (median 70% vs. 90%; p = 0.043). Local patients were more likely to have tumors confined to the IAC compared with referral patients, and had significantly smaller tumors when restricted to this region (median 4.0 vs 6.5 mm; p = 0.005). Referral patients were significantly more likely to undergo definitive management with either radiosurgery or microsurgery following primary evaluation compared to local patients (48% vs. 24%; p = 0.013).
Conclusion: These data suggest that the majority of existing literature surrounding VS likely suffers from referral bias, whereby disease characteristics and management decisions are distinct from that of the general VS patient population.

Postoperative MRI Surveillance of Vestibular Schwannomas: Is There a Standard of Care?

01-02-2020 – Bukoski, Ronald S.; Appelbaum, Eric N.; Coelho, Daniel H.

Journal Article

Objective: To examine current practices for postoperative imaging surveillance following vestibular schwannoma resection.
Study Design: Cross-sectional survey of practicing neurotologists.
Setting: Tertiary referral centers.
Patients: Not applicable.
Intervention: Two hundred seventy-six members of the American Neurotology Society were invited to participate. Using a web-based format, respondents self-reported demographic and practice details. Case scenarios were presented. For each scenario, both quantitative and qualitative data were recorded.
Main Outcome Measures: Timing, frequency, duration, and modality of postoperative imaging.
Results: For all scenarios, responses were widely disparate with respect to timing, frequency, number, and duration of follow-up imaging. Following gross total resection, respondents most commonly (46.5%) obtain the first magnetic resonance imaging 1 year after surgery, with the most common endpoint to discontinue surveillance imaging at 10 years (34.9%). Tumor beds with postoperative enhancement were generally followed longer than those without enhancement, but with wide variability in practice patterns. The majority of neurotologists do not change surveillance patterns regarding tumor size or completeness of initial resection. Lower volume surgeons appear to be more aggressive with initial surveillance postoperatively than higher volume surgeons.
Conclusion: Wide variability exists amongst neurotologists concerning postsurgical tumor surveillance imaging. Despite recent data to suggest more standardized protocols, disparate practice patterns persist. Additional research is needed to this end, as the subsequent establishment of such evidence-based protocols could obviate substantial medical, medico-legal, and economic burdens concerning this patient population.

Quality of Life in Vestibular Schwannoma Patients: A Longitudinal Study

01-02-2020 – Miller, Lauren E.; Brant, Jason A.; Naples, James G.; Bigelow, Douglas C.; Lee, John Y. K.; Ruckenstein, Michael J.

Journal Article

Objective: To examine differences in quality of life in patients with vestibular schwannoma following a single treatment modality: observation, stereotactic radiosurgery (SRS), or microsurgery.
Study design: Retrospective review.
Setting: Tertiary academic medical center.
Patients: Patients diagnosed with sporadic vestibular schwannoma who had completed at least 2 Penn Acoustic Neuroma Quality of Life (PANQOL) surveys.
Interventions: Treatment via SRS, microsurgery, or observation.
Main Outcome Measures: PANQOL total survey and subdomain scores.
Results: One hundred and thirty-four patients (94 observations, 24 SRS, 16 microsurgeries) were included. The mean number of PANQOL surveys completed was 2.8 (range 2–5). The total PANQOL scores were significantly lower in the SRS group, but not the microsurgery group, compared with observation at the time of diagnosis (observation 550.4 ± 58.4; SRS 471.4 ± 37.4; microsurgery 492.6 ± 40.7; p = 0.03). Over time, there were no significant differences in the change of PANQOL scores across the 3 groups (SRS PANQOL score worsened 6.8/year compared with observation, p = 0.3; microsurgery PANQOL score worsened 7.8/year compared with observation, p = 0.5). Anxiety was the only subdomain that significantly worsened over time in the microsurgery group (microsurgery PANQOL score worsened 3.8/year compared with observation; p = 0.009).
Conclusion: Despite differences in PANQOL scores at baseline, changes in total PANQOL score over time were not found to be statistically significant, regardless of the treatment group chosen. Overall, these results hold implications for patient counseling when considering treatment choice and quality of life predictions.

Current Volumetric Models Overestimate Vestibular Schwannoma Size Following Stereotactic Radiosurgery

01-02-2020 – Sherry, Alexander D.; Khattab, Mohamed H.; Totten, Douglas J.; Wharton, David M.; Luo, Guozhen; Manzoor, Nauman F.; Rivas, Alejandro; Chambless, Lola B.; Davis, Larry T.; Attia, Albert; Cmelak, Anthony J.

Journal Article

Objective: Accurate volume assessment is essential for the management of vestibular schwannoma after stereotactic radiosurgery (SRS). A cuboidal approximation for volume is the standard surveillance method; however, this may overestimate tumor volume. We sought to evaluate several volumetric models and their suitability for post-SRS surveillance.
Study Design: Retrospective cohort study.
Setting: Tertiary referral center.
Patients: We evaluated 54 patients with vestibular schwannoma before and after SRS.
Intervention(s): Gold-standard volumes were obtained by a radiation oncologist using contouring software. Volume was also calculated by cuboidal, ellipsoidal, and spherical formulae using tumor diameters obtained by a neuroradiologist.
Main Outcome Measure(s): Percent error (PE) and absolute percent error (APE) were calculated. Paired t test evaluated bias, and the Bland–Altman method evaluated reproducibility. Linear regression evaluated predictors of model error.
Results: All models overestimated volume compared with the gold standard. The cuboidal model was not reproducible before SRS (p < 0.001), and no model was reproducible after SRS (cuboidal p < 0.001; ellipsoidal p = 0.02; spherical p = 0.02). Significant bias was present before SRS for the cuboidal model (p < 0.001), and post-SRS for all models cuboidal (p < 0.001), ellipsoidal (p < 0.02), and spherical (p = 0.005). Model error was negatively associated with pretreatment volume for the cuboidal (PE p = 0.03; APE p = 0.03), ellipsoidal (PE p = 0.03; APE p = 0.04), and spherical (PE p = 0.02; APE p = 0.03) methods and lost linearity post-SRS.
Conclusions: The standard cuboidal practice for following vestibular schwannoma tumor volume after SRS overestimates size. Ellipsoidal and spherical estimations have improved performance but also overestimate volume and lack reliability post-SRS. The development of other volumetric models or application of contouring software should be investigated.

Is Longer Surgery More Dangerous? Operative Duration Not Associated With Complications After Vestibular Schwannoma Resection

01-02-2020 – Raghavan, Arun M.; Lipschitz, Noga; Kohlberg, Gavriel D.; Samy, Ravi N.; Zuccarello, Mario; Pensak, Myles L.; Breen, Joseph T.

Journal Article

Objective: To examine the association between operative duration and complications after vestibular schwannoma (VS) surgery.
Study Design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: One hundred forty-eight patients undergoing vestibular schwannoma resection in a single institution.
Intervention: Vestibular schwannoma resection.
Main Outcome Measures: Operative duration, surgical approach, tumor size, and postoperative complications.
Results: Forty-one patients underwent middle cranial fossa (MCF) approach, 46 underwent translabyrinthine (TL) approach, and 61 underwent retrosigmoid (RS) approach. The mean operative duration overall was 407 minutes (MCF—339 min, TL—450 min, RS 420 min). When controlling for tumor size, there was no difference in procedure duration by approach (OR 0.92, CI 0.82–1.02, p=0.11).
When controlling for approach, there was a significant increase in procedure duration by tumor size (OR 1.36, CI 1.23–1.50, p < 0.0001). Increased procedure duration was not associated with 30-day readmission (p = 0.82), cerebrospinal fluid leak (CSF) (p = 0.84), return to the operating room (p = 0.75), postoperative deep vein thrombosis (p = 1.0), postoperative stroke (p = 0.23), or postoperative wound complications (p = 0.70). Longer operative time was associated with increased hospital length of stay (p = 0.04). However, when controlling for tumor size and surgical approach, hospital length of stay was no longer associated with increased procedure duration (OR 1.15, CI 0.98–1.33, p = 0.3).
Conclusion: Increased operative duration was associated with larger tumor size; however contrary to previous reports, increased operative duration was not associated with postoperative complications.

Cerebral Venous Sinus Thrombosis After Translabyrinthine Vestibular Schwannoma—A Prospective Study and Suggested Management Paradigm

01-02-2020 – Guazzo, Emily; Panizza, Benedict; Lomas, Andrew; Wood, Martin; Amato, Damien; Alalade, Andrew; Gandhi, Mitesh; Bowman, James

Journal Article

Objective: To prospectively evaluate the incidence, risk factors and natural history of postoperative cerebral venous sinus thrombosis (p
CVST) in translabyrinthine vestibular schwannoma microsurgical resection and propose a potential management paradigm.
Study Design: Prospective, single cohort, multicenter study.
Setting: State-wide academic tertiary referral centers.
Patients: Fifty-four consecutive patients who underwent translabyrinthine vestibular schwannoma resection.
Main Outcome Measures: Incidence of p
CVST on postoperative imaging on Day 7, Day 28, and 12 months postoperatively. Patients and tumor characteristics, risk factors, length of stay, intraoperative parameters, complications, and follow-up were analyzed.
Results: p
CVST was demonstrated in 21 patients (38.9%) on postoperative imaging. All patients with p
CVST were treated conservatively and remained asymptomatic in the immediate postoperative period and long-term follow-up. There were no instances of venous infarction, intracranial hemorrhage, or neurological deficits. A majority (61.1%) of p
CVST recannalised on long-term follow up with conservative management. There was a statistical association with p
CVST and surgery on the side of the non-dominant cerebral venous drainage (n = 17, 80.1%, p = 0.034). Patients with p
CVST were significantly more likely to have a postoperative cerebrospinal fluid (CSF) leak (n = 5, 23.8%, p = 0.017).
Conclusion: The incidence of p
CVST following translabyrinthine vestibular schwannoma resection is much higher than previously recognized. p
CVST is more likely to occur when surgery is performed on tumors situated on the side of non-dominant cerebral venous drainage. Despite the high prevalence of this iatrogenic phenomenon, all patients were asymptomatic and a majority resolved, thereby suggesting that conservative management is safe. Correlation between p
CVST and increased incidence of CSF leak requires further investigation.

“The Physiologic Role of Corticosteroids in Menières Disease: An Update on Glucocorticoid-mediated Pathophysiology and Corticosteroid Inner Ear Distribution”

01-02-2020 – Froehlich, Michael H.; Lambert, Paul R.

Journal Article

There are multiple treatment options for Ménières disease (MD), including dietary modifications, aminoglycoside therapy, and surgery. All have limitations, ranging from limited effectiveness to permanent hearing loss. Corticosteroids have long been used to manage MD due to their relative efficacy and tolerability, but the exact mechanism for disease alleviation is uncertain. Until recently, the precise distribution and role that glucocorticoid receptors play in inner ear diseases have remained largely uninvestigated. Several studies propose they influence mechanisms of fluid regulation through ion and water homeostasis. This review will provide an update on the basic science literature describing the activity of endogenous glucocorticoids and exogenous corticosteroids in the inner ear and the relevance to MD, as well as early clinical trial data pertaining to the application of novel technologies for more effective administration of corticosteroids for the treatment of MD.

3D-Printed Microneedles Create Precise Perforations in Human Round Window Membrane in Situ

01-02-2020 – Chiang, Harry; Yu, Michelle; Aksit, Aykut; Wang, Wenbin; Stern-Shavit, Sagit; Kysar, Jeffrey W.; Lalwani, Anil K.

Journal Article

Hypothesis: Three-dimensional (3D)-printed microneedles can create precise holes on the scale of micrometers in the human round window membrane (HRWM).
Background: An intact round window membrane is a barrier to delivery of therapeutic and diagnostic agents into the inner ear. Microperforation of the guinea pig round window membrane has been shown to overcome this barrier by enhancing diffusion 35-fold. In humans, the challenge is to design a microneedle that can precisely perforate the thicker HRWM without damage.
Methods: Based on the thickness and mechanical properties of the HRWM, two microneedle designs were 3D-printed to perforate the HRWM from fresh frozen temporal bones in situ (n = 18 total perforations), simultaneously measuring force and displacement. Perforations were analyzed using confocal microscopy; microneedles were examined for deformity using scanning electron microscopy.
Results: HRWM thickness was determined to be 60.1 ± 14.6 (SD) μm. Microneedles separated the collagen fibers and created slit-shaped perforations with the major axis equal to the microneedle shaft diameter. Microneedles needed to be displaced only minimally after making initial contact with the RWM to create a complete perforation, thus avoiding damage to intracochlear structures. The microneedles were durable and intact after use.
Conclusion: 3D-printed microneedles can create precise perforations in the HRWM without damaging intracochlear structures. As such, they have many potential applications ranging from aspiration of cochlear fluids using a lumenized needle for diagnosis and creating portals for therapeutic delivery into the inner ear.

Anatomical and Functional Consequences of Microneedle Perforation of Round Window Membrane

01-02-2020 – Yu, Michelle; Arteaga, Daniel N.; Aksit, Aykut; Chiang, Harry; Olson, Elizabeth S.; Kysar, Jeffrey W.; Lalwani, Anil K.

Journal Article

Hypothesis: Microneedles can create microperforations in the round window membrane (RWM) without causing anatomic or physiologic damage.
Background: Reliable delivery of agents into the inner ear for therapeutic and diagnostic purposes remains a challenge. Our novel approach employs microneedles to facilitate intracochlear access via the RWM. This study investigates the anatomical and functional consequences of microneedle perforations in guinea pig RWMs in vivo.
Methods: Single three-dimensional-printed, 100 μm diameter microneedles were used to perforate the guinea pig RWM via the postauricular sulcus. Hearing was assessed both before and after microneedle perforation using compound action potential and distortion product otoacoustic emissions. Confocal microscopy was used ex vivo to examine harvested RWMs, measuring the size, shape, and location of perforations and documenting healing at 0 hours (n = 7), 24 hours (n = 6), 48 hours (n = 6), and 1 week (n = 6).
Results: Microneedles create precise and accurate perforations measuring 93.1 ± 29.0 μm by 34.5 ± 16.8 μm and produce a high-frequency threshold shift that disappears after 24 hours. Examination of perforations over time demonstrates healing progression over 24 to 48 hours and complete perforation closure by 1 week.
Conclusion: Microneedles can create a temporary microperforation in the RWM without causing significant anatomic or physiologic dysfunction. Microneedles have the potential to mediate safe and effective intracochlear access for diagnosis and treatment of inner ear disease.

International Collaborative Assessment of the Validity of the EAONO-JOS Cholesteatoma Staging System: Methodological Issues on Validity and Reliability

01-02-2020 – Sabour, Siamak

Journal Article

No abstract available

International Collaborative Assessment of the Validity of the EAONO-JOS Cholesteatoma Staging System: Confirmation of Validity and Reliability

01-02-2020 – James, Adrian L.; Siu, Jennifer; Borkhoff, Cornelia M.; Yung, Matthew; Stephens, Derek

Journal Article

No abstract available

Bilateral Persistent Stapedial Arteries Associated With Abnormal Lower Extremity Bone Growth

01-02-2020 – Espahbodi, Mana; Michel, Michelle A.; Harris, Michael S.

Journal Article

No abstract available

Carotid Artery-Cochlear Dehiscence

01-02-2020 – Oliver, Jamie R.; Chen, David S.; Pearl, Monica S.; Carey, John P.; Sun, Daniel Q.

Journal Article

No abstract available

Cochlear–Facial Dehiscence Detected After Cochlear Implant

01-02-2020 – Camerin, Gabriela R.; Passos, Ula Lindoso; da Costa, Sady Selaimen; Gebrim, Eloisa Maria Mello Santiago; Cruz, Oswaldo Laércio Mendonça

Journal Article

No abstract available