Otology Neurotology 2021-10-01

Admission Rates Following Day-Case Major Otologic Surgery: A Systematic Review

Derks, Laura S. M.; Borgstein, Isabelle L. A.; Lammers, Marc J. W.; Kamalski, Digna M. A.; Thomeer, Hans G. X. M.; Grolman, Wilko; Stokroos, Robert J.; Wegner, Inge

Publication date 01-10-2021


Objective: To assess whether day-case major otologic ear surgery is a safe and feasible alternative to inpatient surgery, while maintaining equal complication rates, and to identify causes of admission after day-case surgery.
Data sources: Pub Med, Embase, and Cochrane.
Review methods: A systematic search was conducted. Studies reporting original data on the effect of day-case ear surgery on admission rate, patient satisfaction, and/or postoperative complications were included. Risk of bias of the selected articles was assessed using criteria based on the Cochrane Collaborations tool for assessing risk of bias.
Results: A total of 1,734 unique studies were retrieved of which 35 articles discussing 34 studies were eligible for data extraction. The admission rates ranged from 0% to 88% following day-case endaural surgery, 0% to 13% following day-case stapes surgery, 0% to 82% following day-case mastoid surgery, and 0% to 15% following day-case cochlear implant surgery. Patient and parent satisfaction regarding day-case surgery ranged from 67% to 99%. Five studies comparing day-case and inpatient otologic surgery showed no difference in hearing outcome, postoperative complications, or patient satisfaction.
Conclusion: The highest pooled admission rate was seen following day-case mastoid surgery. Studies comparing day-case and inpatient care suggest hearing results and postoperative complication rates in day-case otologic surgery are similar to inpatient otologic surgery in both children and adults. Therefore, day-case major otologic surgery seems to be a safe and feasible alternative to inpatient surgery for both children and adults.

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The Effect of CPAP on Middle Ear Pressure: A Systematic Review and Meta-analysis

Ma, Cheng; Yuen, Erick; Nguyen, Shaun A.; Meyer, Ted A.; Lambert, Paul R.

Publication date 01-10-2021


Objective: To assess the effect of short-term and long-term continuous positive airway pressure (CPAP) use on middle ear pressure (MEP) in adult ears.
Databases Reviewed: Pub Med, Scopus, and Embase were searched through October 2020. Search strategies used a combination of subject headings (e.g., MeSH in Pub Med) and keywords for CPAP and middle ear pressure.
Methods: English-language articles studying changes in MEP as measured by tympanometry or tympanograms in patients using CPAP were selected for inclusion. Data extraction from included articles included demographics, baseline, and follow-up data.
Results: A total of 7 articles representing 664 patients with weighted mean age of 42.5 years (range 34.9–57.8 yrs) were included. Meta-analysis of three qualifying studies demonstrated a positive correlation between higher CPAP pressures and degree of MEP rise during CPAP use compared to baseline with mean difference of 25.1 da Pa (95% confidence interval 18.7, 31.4) at 5 cm H2O and 81.9 da Pa (95% confidence interval 60.2, 103.5) at 10 cm H2O. Short-term effects of CPAP use were reviewed in four studies that showed a transient rise in MEP. Three separate studies examining the long-term effects of CPAP use showed resting MEP was higher in patients with severe obstructive sleep apnea (OSA), which might be related to progressive eustachian tube dysfunction as a complication of severe OSA.
Conclusion: Short-term CPAP use is associated with transient elevations of MEP in adult ears. Long-term CPAP use might produce beneficial MEP changes, especially in patients with OSA and eustachian tube dysfunction.

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Sudden Sensorineural Hearing Loss and Metabolic Syndrome: A Systematic Review and Meta-analysis

Lam, Megan; Bao, Yueyang; Hua, Gordon B.; Sommer, Doron D.

Publication date 01-10-2021


Objective: The objective of this systematic review and meta-analysis is to examine the association between sudden sensorineural hearing loss (SSNHL) and risk of metabolic syndrome (MetS), and the association between MetS and prognosis of SSNHL.
Databases Reviewed: We systematically searched MEDLINE, Embase, and Cochrane Central Register electronic databases from their dates of conception to February 4, 2020.
Methods: We included observational studies analyzing 1) the prevalence of MetS among SSNHL patients, or 2) the prognosis of SSNHL patients in MetS patients. A standardized form was completed in duplicate extracting data on study characteristics, participant demographics, and SSNHL outcome or recovery measures. Random-effects meta-analyses were performed pooling odds ratios using the generic inverse method. Risk of bias was assessed using the Newcastle Ottawa Scale.
Results: Three studies examining the prevalence of MetS among patients with SSNHL (11,890 total participants; 3,034 SSNHL participants) yielded a significantly increased risk of MetS among SSNHL, with a pooled odds ratio of 1.88 (95% CI, 1.01–3.50). Three studies examining the association of SSNHL prognosis in patients with MetS (608 SSNHL participants, 234 concomitant SSNHL, and MetS participants) demonstrated that SSNHL patients with MetS were significantly more likely to have poorer recovery compared to SSNHL patients without MetS (pooled odds ratio 2.77; 95% CI, 2.33–3.28).
Conclusion: Our findings suggest an association between prevalence of MetS and SSNHL, as well as poorer prognosis of SSNHL in patients with concomitant MetS.

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Current Evidence for Simulation-Based Training and Assessment of Myringotomy and Ventilation Tube Insertion: A Systematic Review

Hovgaard, Lisette Hvid; Al-Shahrestani, Fahd; Andersen, Steven Arild Wuyts

Publication date 01-10-2021


Objective: Myringotomy and ventilation tube insertion (MT) is a key procedure in otorhinolaryngology and can be trained using simulation models. We aimed to systematically review the literature on models for simulation-based training and assessment of MT and supporting educational evidence.
Databases Reviewed: Pub Med, Embase, Cochrane Library, Web of Science, Directory of Open Access Journals.
Methods: Inclusion criteria were MT training and/or skills assessment using all types of training modalities and learners. Studies were divided into 1) descriptive and 2) educational interventional/observational in the analysis. For descriptive studies, we provide an overview of available models including materials and cost. Educational studies were appraised using Kirkpatricks level of educational outcomes, Messicks framework of validity, and a structured quality assessment tool.
Results: Forty-six studies were included consisting of 21 descriptive studies and 25 educational studies. Thirty-one unique physical and three virtual reality simulation models were identified. The studies report moderate to high realism of the different simulators and trainees and educators perceive them beneficial in training MT skills. Overall, simulation-based training is found to reduce procedure time and errors, and increase performance as measured using different assessment tools. None of the studies used a contemporary validity framework and the current educational evidence is limited.
Conclusion: Numerous simulation models and assessment tools have been described in the literature but educational evidence and systematic implementation into training curricula is scarce. There is especially a need to establish the effect of simulation-based training of MT in transfer to the operating room and on patient outcomes.

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A Novel 3D-Printed Head Holder for Guinea Pig Ear Surgery

Valentini, Chris; Ryu, Young Jae; Szeto, Betsy; Yu, Michelle; Lalwani, Anil K.; Kysar, Jeffrey

Publication date 01-10-2021


Background: Otologic surgery in guinea pig requires head immobilization for microscopic manipulation. Existing commercially available stereotaxic frames are expensive and impede access to the ear as they rely on ear bars or mouthpieces to secure the head.
Method: Prototype head holders were designed using the Solidworks 2019 software and 3D-printed using Formlabs Form 2 Printers with photopolymer resin. The head holder consists of a C-shaped brace with adjustable radial inserts of 1/4-20 UNC standard screws with cone point tips providing head fixation for animals of various sizes. The C-shaped brace is attached to a rod that can be secured to a commercially available micromanipulator. The head holder design was tested during in vivo guinea pig experiments where their head motion with (n = 22) and without the head holder (n = 2) was evaluated visually through a stereotaxic microscope at 24× magnification during surgery.
Results: The head holder design was easy to use and allowed for both nose cone administration of anesthesia and access to the ear for intraoperative auditory testing and manipulation. Functionally, the head holder successfully minimized head movement. Furthermore, harvested round window membranes evaluated at 72 hours following surgery showed precise perforations with the use of head holder.
Conclusion: The novel 3D-printed head holder enables simultaneous access for nose cone administration of anesthesia and surgical manipulation of the ear and brain. Moreover, it provides a modular, intuitive, and economical alternative to commercial stereotaxic devices for minimizing head motion during small animal surgery.

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Association Analysis of Candidate Gene Polymorphisms and Tinnitus in Young Musicians

Bhatt, Ishan Sunilkumar; Dias, Raquel; Torkamani, Ali

Publication date 01-10-2021


Introduction: Subjective tinnitus, a perception of phantom sound, is a common otological condition that affects almost 15% of the general population. It is known that noise-induced hearing loss (NIHL) and tinnitus exhibit a high level of comorbidity in individuals exposed to intense noise and music. However, the influence of genetic variants associated with NIHL on tinnitus remains elusive. We hypothesized that young musicians carrying genetic variants associated with NIHL would exhibit a higher prevalence of tinnitus than their counterparts.
Methods: To test this hypothesis, we analyzed the database by Bhatt et al. (2020) (originally developed by Phillips et al., 2015) that investigated the genetic links to NIHL in young college-aged musicians. The present study identified 186 participants (average age = 20.3 yrs, range = 18–25 yrs) with normal tympanometry and otoscopic findings and with no missing data. We included 19 single nucleotide polymorphisms in 13 cochlear genes that were previously associated with NIHL.
The candidate genes include: KCNE1, KCNQ1, CDH23, GJB2, GJB4, KCNJ10, CAT, HSP70, PCDH70, MYH14, GRM7, PON2, and ESRRB.
Results: We find that individuals with at least one minor allele of rs163171 (C > T) in KCNQ1 exhibit significantly higher odds of reporting tinnitus compared to individuals carrying the major allele of rs163171. KCNE1 rs2070358 revealed a suggestive association (p = 0.049) with tinnitus, but the FDR corrected p-value did not achieve statistical significance (p < 0.05). A history of ear infection and sound level tolerance showed a statistically significant association with tinnitus. Music exposure showed a suggestive association trend with tinnitus. Biological sex revealed a statistically significant association with distortion product otoacoustic emissions SNR measures.
Conclusions: We concluded that KCNQ1/KCNE1 voltage-gated potassium ion channel plays a critical role in the pathogenesis of NIHL and tinnitus. Further research is required to construct clinical tools for identifying genetically predisposed individuals well before they acquire NIHL and tinnitus.

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The Impact of the Frequency-Specific Preoperative Sensorineural Hearing Loss to Postoperative Overclosure of Bone Conduction in Stapedotomy

Fang, Yanqing; Zhang, Kun; Ersbo, Jack H.; Chen, Bing

Publication date 01-10-2021


Objective: To evaluate the frequency-specific relationships between the preoperative sensorineural hearing loss and postoperative overclosure of bone conduction (BC) threshold after stapedotomy.
Methods: We conducted a retrospective analysis of 207 otosclerosis patients who underwent stapedotomy in our hospital. Pre- and postoperative audiometry were collected between patients with or without preoperative sensorineural hearing loss (SNHL) component (i.e., SNHL group and non-SNHL group, respectively). Overclosure rate (OR), deterioration rate (DR), and their significant values (i.e., SOR or SDR) were compared between the sub-groups at each frequency to access the frequency-specific outcomes.
Results: There were 69 patients in non-SNHL group and 138 patients in SNHL group. Postoperative success rate was similar in non-SNHL group and SNHL group. For frequency-specific outcomes, both the OR and SOR were significantly obvious in all SNHL subgroups than those in non-SNHL subgroups (p  0.05). Frequency-specific success rate was similar at each frequency.
Conclusion: Stapedotomy was efficient and safe in patients with or without SNHL component preoperatively. Frequency-specific overclosure effect was more obvious in all tone frequencies in SNHL subgroups than those in non-SNHL subgroups. For frequency-specific outcomes, about 50% BC with preoperative SNHL component at tone frequency meet postoperative significant overclosure effect, which was most obvious at low frequencies.

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Sensorineural Hearing Loss and Tinnitus Characteristics in Patients With Idiopathic Intracranial Hypertension

Shim, Timothy; Chillakuru, Yeshwant; Moncada, Paola; Kim, Sunny; Sabetrasekh, Parisa; Sparks, Andrew; Mulcahy, Collin F.; Monfared, Ashkan

Publication date 01-10-2021


Objectives: To characterize patterns of sensorineural hearing loss (SNHL) and tinnitus in patients with idiopathic intracranial hypertension (IIH).
Study Design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: Adult patients diagnosed with IIH via lumbar puncture (LP) between 2010 and 2020 who had available audiograms. The study included a total of 40 patients; 33 women, and 7 men with a median age of 43.
Interventions: Diagnostic LP and audiogram.
Main Outcome Measures: Otologic symptoms, ophthalmologic signs, hearing thresholds, cerebrospinal fluid opening pressures.
Results: The most commonly reported symptoms were tinnitus in 28 (70%, 23 pulsatile and 5 tonal), aural fullness in 11 (28%), and vertigo in 10 (25%). Twenty-nine patients had ophthalmologic examinations and 18 had evidence of papilledema. Twenty-five (63%) patients had hearing loss in at least one ear at one frequency range. Patients presented with both unilateral and bilateral hearing loss across low, middle, and high frequency ranges. No significant association was observed between hearing loss threshold and LP opening pressure except for 250 Hz in the left ear. After stratification by tinnitus group (pulsatile, tonal, and no tinnitus), no significant difference was found between mean hearing loss threshold at different frequencies. In addition, no significant mean age difference was identified in patients within each tinnitus group.
Conclusions: There was no classic pattern or presentation for hearing loss in our IIH patients. They developed sudden, unilateral, or bilateral SNHL in low, middle, or high frequency range. The degree of hearing loss did not correlate with CSF opening pressure.

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Tinnitus and Subjective Hearing Loss are More Common in Migraine: A Cross-Sectional NHANES Analysis

Goshtasbi, Khodayar; Abouzari, Mehdi; Risbud, Adwight; Mostaghni, Navid; Muhonen, Ethan G.; Martin, Elaine; Djalilian, Hamid R.

Publication date 01-10-2021


Objectives: To investigate whether migraine is independently associated with tinnitus and subjective hearing loss (HL) in a large national database.
Methods: The de-identified 1999 to 2004 National Health and Nutrition Examination Survey database was retrospectively queried for subjects aged 18 to 65. HL and tinnitus were subjectively reported by subjects.
Results: A total of 12,962 subjects (52.9% female) with a mean age of 38.1 ± 14.6 years were included. This consisted of 2,657 (20.5%), 2,344 (18.1%), and 2,582 (19.9%) subjects who had migraine, subjective-HL, and tinnitus, respectively. In patients with tinnitus or subjective-HL, migraine was reported in 35.6% and 24.5%, respectively. Migraineurs were more likely to have subjective-HL (25.0% vs. 16.6%, p < 0.001) and tinnitus (34.6% vs. 16.9%, p < 0.001) compared to the nonmigraineurs. This corresponded to migraine having an odds ratio of 1.5 (95% confidence interval CI 1.3–1.7, p < 0.001) and 2.2 (95% CI 2.0–2.4, p < 0.001) for subjective-HL and tinnitus, respectively. After adjusting for confounders, subjective-HL (odds ratio OR = 1.2, 95% CI 1.1–1.4, p = 0.003), tinnitus (OR = 2.1, 95% CI 1.9–2.3, p < 0.001), and neck pain (OR = 4.0, 95% CI 3.6–4.5, p < 0.001) were more common in migraineurs. Among migraineurs, a higher proportion of those with tinnitus also had subjective-HL compared to those without tinnitus (40.0% vs. 15.3%, p < 0.001), and a higher proportion of those with subjective-HL also had tinnitus compared to those without HL (58.1% vs. 27.3%, p < 0.001).
Conclusions: This study suggests an independent association between migraine with subjective-HL and tinnitus. Otologic migraine, which is the effects of migraine on the ear, may be partly responsible for the link between HL, tinnitus, neck pain, and migraine.

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Otologic Manifestations After COVID-19 Vaccination: The House Ear Clinic Experience

Wichova, Helena; Miller, Mia E.; Derebery, M. Jennifer

Publication date 01-10-2021


Objective: With the increasing numbers of COVID-19 vaccinations available there are some reports of new onset of otologic symptoms. We present our experience in recently vaccinated patients over a 30-day time frame.
Study Design: Retrospective chart review.
Setting: Tertiary otology ambulatory practice.
Patients: All patients with available diagnostic codes, COVID-19 questionnaires and clinical notes.
Interventions: Observational recordings.
Main Outcome Measures: Within the same 30-day time period in 2019, 2020, and 2021, 1.6, 2.4, and 3.8% respectively, of all office visits were for patients with the diagnosis of new onset idiopathic sensorineural hearing loss (SNHL) without other underlying otologic diagnoses. In this time frame in 2021, 30 patients out of the 1,325 clinical visits had new or significantly exacerbated otologic symptoms that began shortly after COVID-19 vaccination. Specifically, 18 patients received Moderna and 12 patients received Pfizer vaccine. Their mean age was 60.9±13.8 years old; 11 were women and 19 men. The mean onset of symptoms was 10.18 ± 9 days post-vaccination. Symptoms included 25 patients (83.3%) with hearing loss, 15 (50%) with tinnitus, eight (26.7%) with dizziness, and five (16.7%) with vertigo. Eleven patients had previous otologic diagnoses, including six patients with Menières disease, two with autoimmune inner ear disease (AIED), and three having both.
Conclusions: There are no definite correlations to the COVID-19 pandemic or vaccination and new or worsened otologic symptoms. Vaccinated patients with new or exacerbated otologic symptoms should be promptly referred for evaluation. Suspected cases of post-vaccination otologic symptoms should be reported to the Center for Disease Control (CDC) vaccine adverse event reporting system (VAERS).

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Magnetic Resonance Imaging in Patients With Hearing Implants – Follow-up on Prevalence and Complications

Loth, Andreas G.; Fischer, Kathrin; Hey, Anna Katharina; Weiß, Roxanne; Leinung, Martin; Burck, Iris; Linke, Albrecht; Kramer, Sabine; Stöver, Timo; Helbig, Silke

Publication date 01-10-2021


Objective: To examine the number of magnetic resonance imaging (MRI) examinations performed in patients with hearing implants and to quantify side effects or complications related to this procedure.
Study Design: Questionnaire.
Setting: Tertiary referral center, academic hospital.
Patients: One thousand four hundred sixty-onepatients with an implanted hearing system.
Intervention: Patients were asked to complete a questionnaire either during a visit to the clinic (304) or by mail contact (1,157) between February 2018 and March 2019.
Main Outcome Measures: Number of examinations by means of MRI per patient and number of side effects or complications.
Results: A total of 711 questionnaires were returned. After excluding nonvalid information on the questionnaire, 12.8% of patients were identified who had undergone an MRI after having received their hearing implant. Within this group of 91 patients, the most common precaution undertaken was a head bandage (69%). Side effects were mainly pain (37%), followed by anxiety (15%) and tinnitus (9%). The MRI had to be aborted in 14% and dislocation of the magnet occurred in 7% of examinations.
Conclusions: Our data indicate that patients undergoing hearing implant surgery need better information about the limitations and requirements of MRI. The occurrence of side effects is likely as only half of the patients in our study group were completely free of symptoms. Dislocation of the implant magnet was observed in several cases, hence patients and physicians need to be educated about this potential complication.

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Digital Live Imaging of Intraoperative Electrocochleography – First Description of Feasibility and Hearing Preservation During Cochlear Implantation

Arweiler-Harbeck, Diana; D’heygere, Victoria; Meyer, Moritz; Hans, Stefan; Waschkies, Laura; Lang, Stephan; Anton, Kristina; Hessel, Horst; Schneider, Armin; Heiler, Thomas; Höing, Benedikt

Publication date 01-10-2021


Introduction: Intraoperative electrocochleography (ECochG) during cochlear implantation is a promising tool to preserve residual hearing. However, the time gap between insertion of the electrode and acoustic feedback from the audiologist to the surgeon can cause delay and subsequently irreparable damage to cochlear structures. In this feasibility study, for the first time, real-time visualization of intraoperative ECochG via digital microscope display directly to the surgeon was successfully performed in four patients.
Materials and methods: Four patients with residual hearing underwent cochlear implantation. Intraoperative electrocochleography responses were collected and direct visualization during the time of electrode insertion into the surgeons field of view in the binoculars using augmented real-time digital imaging was realized. The time of electrode insertion was recorded. Hearing preservation was determined by testing postoperative changes in behavioral thresholds.
Results: Digital live visualization of intraoperative ECochG using image augmentation in a digital microscope was successfully performed in all cases and enabled direct adaptation of the surgeons insertion behavior. Mean time of electrode insertion was 129.8 seconds. Postoperative behavioral thresholds were comparable to preoperative taken thresholds. Preservation of residual hearing in the low frequency range was possible.
Discussion: This study is the first to describe digital visualization of intraoperative electrocochleography as a new method enabling the surgeon to directly react to changes in amplitude of the cochlea microphonics. Our results show that augmentation of the intraoperative live imaging with electrical potentials could add to hearing preservation during cochlear implantation.

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Novel Web-Based Music Re-Engineering Software for Enhancement of Music Enjoyment Among Cochlear Implantees

Hwa, Tiffany Peng; Tian, Lisa Likun; Caruana, Francesco; Chun, Michael; Mancuso, Dean; Cellum, Ilana P.; Lalwani, Anil K.

Publication date 01-10-2021


Objectives: Cochlear implant (CI) listeners experience diminished music perception and enjoyment from a variety of patient-related and implant-related factors. We investigate the hypothesis that patient-directed music re-engineering may enhance music enjoyment with CI.
Study Design: Prospective cohort study.
Setting: Academic cochlear implant center.
Subjects and Methods: A multidisciplinary team of neurotologists, audiologists, and a sound/audio engineer collaborated with a web developer to create a music re-engineering application. Experienced adult CI listeners rated original excerpts from five major genres of music on enjoyment using a visual analog scale (VAS). Subjects were then allowed to re-engineer the original by adjusting treble frequencies, bass frequencies, percussion emphasis, and reverberation and again rated on enjoyment.
Results: Total of 46 subjects, with a mean age of 57.6 years (SD = 16; range, 18–90) participated in the study. User-mixed audio was rated higher across all measures of enjoyment than original recordings (mean difference +0.92; p < 0.05, CI 0.22, 1.62), an effect that was seen across all genres except for country music. Subjects preferred louder bass frequencies (mean difference +7.1 dB; p < 0.01, CI 2.15, 24.3) and more reverberation (mean difference +6.6 ms; p < 0.01, CI 2.85, 10.7). Re-engineered music increased enjoyment in 57%, and 79% reported an interest in being able to mix music of their own choosing.
Conclusion: User-directed music re-engineering increases music enjoyment for CI listeners. The cochlear implantee preferred heightened bass, reverberation, and treble across musical genres. These findings support the implementation of patient-directed music re-engineering to enhance music enjoyment with technology that is readily available today.

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Iatrogenic Third Window After Retrosigmoid Approach to a Vestibular Schwannoma Managed with Cochlear Implantation

Deep, Nicholas L.; Kay-Rivest, Emily; Roland, J. Thomas Jr

Publication date 01-10-2021


Objective: To present a case of an iatrogenic inner ear third window after vestibular schwannoma microsurgery.
Patients: A 42-year-old male presented 9-months after left-sided retrosigmoid approach for an intracanalicular vestibular schwannoma with hearing-preservation attempt performed elsewhere. Immediately postoperatively, he developed the following disabling and persistent symptoms on the ipsilateral side: autophony, pulsatile tinnitus, high-pitched ringing tinnitus, and hearing his footsteps. He denied vertigo. Otoscopy was normal. Tuning fork (512-Hz) lateralized to the left and Rinne was negative on the left. Audiogram demonstrated a severe mixed hearing loss and 10% aided word-recognition score. High-resolution CT demonstrated violation of the common crus and dehiscence of bone along the medial vestibule suggestive of an iatrogenic inner ear third window.
Interventions: Labyrinthectomy and concurrent cochlear implantation.
Main outcome measures: Resolution of third window symptoms, open-set speech recognition, tinnitus suppression.
Results: Patient reported immediate resolution of third window symptoms after labyrinthectomy and cochlear implantation. He demonstrates open-set word recognition of 64% at 1-year postoperatively and tinnitus suppression with his cochlear implant on.
Conclusions: Iatrogenic third window symptoms can occur after hearing-preservation vestibular schwannoma microsurgery. Patients with sufficient hearing preservation who are disabled by third window symptoms yet lack sound clarity and useful hearing may be considered for labyrinthectomy and concurrent cochlear implantation. This intervention effectively extinguishes third window symptoms by destroying residual auditory function and simultaneously provides an opportunity to restore useful hearing and suppress tinnitus, thereby enhancing overall quality of life.

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Estimating the Pose of a Guinea-pig Cochlea Without Medical Imaging

Usevitch, David E.; Park, Albert H.; Scheper, Verena; Abbott, Jake J.

Publication date 01-10-2021


Hypothesis: The pose (i.e., position and orientation) of a guinea-pig cochlea can be accurately estimated using externally observable features, without requiring computed-tomography (CT) scans.
Background: Guinea pigs are frequently used in otologic research as animal models of cochlear-implant surgery. In robot-assisted surgical insertion of cochlear-implant electrode arrays, knowing the cochlea pose is required. A preoperative CT scan of the guinea-pig anatomy can be labeled and registered to the surgical system, however, this process can be expensive and time consuming.
Methods: Anatomical features from both sides of 11 guinea-pig CT scans were labeled and registered, forming sets. Using a groupwise point-set registration algorithm, errors in cochlea position and modiolar-axis orientation were estimated for 11 iterations of registration where each feature set was used as a hold-out set containing a reduced number of features that could all be touched by a motion-tracking probe intraoperatively. The method was validated on 2000 simulated guinea-pig cochleae and six physical guinea-pig-skull cochleae.
Results: Validation on simulated cochleae resulted in cochlea-position estimates with a maximum error of 0.43 mm and modiolar-axis orientation estimates with a maximum error of 8.1 degrees for 96.7% of cochleae. Physical validation resulted in cochlea-position estimates with a maximum error of 0.80 mm and modiolar-axis orientation estimates with a maximum error of 12.4 degrees.
Conclusions: This work enables researchers conducting robot-assisted surgical insertions of cochlear-implant electrode arrays using a guinea-pig animal model to estimate the pose of a guinea-pig cochlea by locating six externally observable features on the guinea pig, without the need for CT scans.

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Cortical Auditory Plasticity Following Cochlear Implantation in Children With Auditory Neuropathy Spectrum Disorder: A Prospective Study

Saki, Nader; Nikakhlagh, Soheila; Moridi, Babak; Karimi, Majid; Aghayi, Azam; Bayat, Arash

Publication date 01-10-2021


Objectives: The cortical auditory evoked potential (CAEP) can provide an insight into the maturation of the central auditory nervous system by recording the auditory cortex responses to speech stimuli. This study aimed to explore the central auditory system development in children with auditory neuropathy spectrum disorder (ANSD) using cochlear implants (CIs) and to find the correlation of CAEP biomarkers with speech perception.
Methods: This study was performed on 23 children with prelingual deafness, diagnosed with ANSD, as well as 23 children with prelingual deafness, without ANSD as the control group. All children underwent unilateral CI before the age of 3 years. Children with ANSD were classified into two groups, based on their CAEP results prior to implantation: children with a clear CAEP response (ANSD-I) and children without an identifiable CAEP (ANSD-II). The P1 component of CAEPs and speech intelligibility rating (SIR) were recorded before the initial device activation (baseline) and at 6, 12, and 24 months postimplantation.
Results: The P1 CAEP responses were present in all children in the control group, while they were recorded in only 52% of ANSD children before the CI surgery. The longitudinal analysis revealed a significant reduction in the P1 latency and a significant improvement in the P1 amplitude across different time points in all study groups. However, the P1 latency and P1 amplitude were significantly shorter and larger in the control group than the ANSD group, respectively. Also, children in the ANSD-I group exhibited a slightly shorter P1 latency, a larger P1 amplitude, and a higher SIR score than the ANSD-II group after 2 years of follow-up. The P1 CAEP latency was significantly correlated with the SIR score.
Conclusion: The CAEP can be used as an objective indicator of auditory cortical maturation and a predictor of speech perception performance in implanted children with ANSD.

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Influence of Cochlear Implant Use on Perceived Listening Effort in Adult and Pediatric Cases of Unilateral and Asymmetric Hearing Loss

Lopez, Erin M.; Dillon, Margaret T.; Park, Lisa R.; Rooth, Meredith A.; Richter, Margaret E.; Thompson, Nicholas J.; O’Connell, Brendan P.; Pillsbury, Harold C.; Brown, Kevin D.

Publication date 01-10-2021


Objective: Assess the influence of cochlear implant (CI) use on the perceived listening effort of adult and pediatric subjects with unilateral hearing loss (UHL) or asymmetric hearing loss (AHL).
Study design: Prospective cohort.
Setting: Tertiary referral center.
Patients: Adults and children with UHL or AHL.
Intervention: Cochlear implantation. Subjects received their CI as part of a clinical trial assessing the effectiveness of cochlear implantation in cases of UHL and AHL.
Main outcome measures: Responses to the Listening Effort pragmatic subscale on the Speech, Spatial, and Qualities of Hearing Scale (SSQ) or SSQ for Children with Impaired Hearing (SSQ-C) were compared over the study period. Subjects or their parents completed the questionnaires preoperatively and at predetermined postactivation intervals. For the adult subjects, responses were compared to word recognition in quiet and sentence recognition in noise.
Results: Forty adult subjects (n = 20 UHL, n = 20 AHL) and 16 pediatric subjects with UHL enrolled and underwent cochlear implantation. Subjects in all three groups reported a significant reduction in perceived listening effort within the initial months of CI use (p < 0.001; η2 ≥ 0.351). The perceived benefit was significantly correlated with speech recognition in noise for the adult subjects with UHL at the 12-month interval (r(20) = .59, p = 0.006).
Conclusions: Adult and pediatric CI recipients with UHL or AHL report a reduction in listening effort with CI use as compared to their preoperative experiences. Use of the SSQ and SSQ-C Listening Effort pragmatic subscale may provide additional information about a CI recipients experience beyond the abilities measured in the sound booth.

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On the Accuracy of Clinical Insertion Angle Predictions With a Surgical Planning Platform for Cochlear Implantation

Avallone, Emilio; Lenarz, Thomas; Timm, Max E.

Publication date 01-10-2021


Hypothesis: Various studies over the last few decades have shown that the cochlea is not a uniform structure, but that its size and shape may vary quite substantially in between subjects. The surgical planning platform enables the user to quickly approximate the size of a cochlea within clinical imaging data by measuring the basal cochlear diameters A and B. It also allows for contact specific insertion angle predictions for MED-EL cochlear implant electrode arrays based on this individual anatomy approximation. The proposed, retrospective study was performed to evaluate the accuracy of these predictions.
Methods: Preoperative CBCT scans of N = 91 MED-EL cochlear implant patients with different types of FLEX electrode arrays (flexible, thin, and straight arrays) were evaluated using a planning module. Both the initial version (based on an equation proposed by Escudé et al.) as well as a novel, recently proposed approach (called elliptic-circular approximation) was employed. All predictions were then compared to the actual insertion angles which were derived from postoperative CBCT images of the same patient.
Results: Most prediction deviations of the investigated cases stayed below 45deg for all electrode arrays and both prediction methods. In general, prediction deviations increased from base to apex were found to be larger for longer electrode arrays. Hardly any significant differences between the two prediction methods were observed. However, particularly large deviations were found for the Escudé method and could be substantially deceased with the updated elliptic-circular approximation approach.
Conclusions: The new platform version with its updated prediction module allows to reliably predict insertion angles even for cochlear anatomies with slightly unusual features and shapes.

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Is There a Role for Intraoperative Navigation During Cochlear Implantation in Patients With Aural Atresia?

Wong, Kevin; Kaul, Vivian F.; Fan, Caleb J.; Schwam, Zachary G.; Cosetti, Maura K.

Publication date 01-10-2021


Objective: Concurrent bilateral congenital aural atresia (CAA) and profound sensorineural hearing loss are rare. While not a contraindication, temporal bone and cochleovestibular abnormalities are an important consideration for cochlear implantation (CI) candidacy. Intraoperative image-guided surgical navigation may play a role during CI surgery in patients with complex anatomy, such as CAA.
Patient: One patient with bilateral CAA, cochlear dysplasia, speech delay, and profound sensorineural hearing loss underwent candidacy evaluation for cochlear implantation.
Interventions: Cochlear implantation using intraoperative image-guided navigation.
Main Outcome Measures: (1) Registration accuracy, (2) surgical outcomes, (3) audiometry.
Results: A four-year-old girl with complete bilateral CAA and profound sensorineural hearing loss successfully underwent a right transmastoid approach for CI using intraoperative image-guided navigation with sticker fiducials. Bony landmarks included the mastoid tip, tympanomastoid suture line, helical root, zygomatic root, and lateral brow. A registration accuracy of 0.9 mm was achieved. There were no intraoperative or immediate postoperative complications. Postoperatively, Neural Response Imaging was confirmed on 9 electrodes and behavioral testing demonstrated Ling-6 access at 30 dB. On most recent follow-up, she has demonstrated gains in language development, vocalizations, and uses total communication in a hearing-impaired educational environment.
Conclusions: Children with CAA and profound sensorineural hearing loss may be candidates for cochlear implantation, with successful outcomes in the setting of complex anatomy. Surgical navigation may play a role corroborating intraoperative landmarks.

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Cochlear Implant Outcomes in Elderly Recipients During the COVID-19 Pandemic

Knickerbocker, Alissa; Bourn, Stephanie; Goldstein, Mary Rose; Jacob, Abraham

Publication date 01-10-2021


Objective: To evaluate the potential significance of social distancing and quarantine precautions for COVID-19 on speech outcomes, missed appointments, wear time, and exposure to various sound environments in the first 6 months following activation for elderly cochlear implant (CI) recipients.
Study Design: Retrospective cohort.
Setting: Tertiary private practice.
Patients: Fifty cochlear implant recipients ≥65 years were evaluated. A Control Group consisted of 26 patients implanted between November 2, 2018 and February 18, 2019 while the Pandemic Group included 24 patients implanted between November 1, 2019 and February 17, 2020.
Intervention: Rehabilitative.
Main Outcome Measures: Preoperative and 6-month postoperative Az Bio sentence scores in quiet were compared between groups along with the number of missed appointments as well as datalogged information regarding average CI wear time and average hours in various sound environments such as quiet, speech, and speech-in-noise.
Results: The Control Group averaged 36.5% improvement for Az Bio in quiet scores while the Pandemic Group averaged only 17.2% improvement, a difference that was both statistically and clinically significant (p = 0.04; g = 0.64). Patients in the Pandemic Group were nearly twice as likely to miss CI programming appointments than the Control Group. The Pandemic Group wore their CI 1.2 less hours per day on average, and while the Pandemic Group spent similar times in quiet and speech environments to the Control Group, the Pandemic Group spent less time in speech with presence of background noise.
Conclusions: While social distancing and quarantine measures are crucial to limiting spread of COVID-19, these precautions may have negatively impacted early speech performance for elderly cochlear implant recipients. Missed CI programming appointments, decreased sound processor wear time, and reduced exposure to complex listening environments such as speech in the presence of background noise were more common in the Pandemic Group than in the Control Group operated the year prior.

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Opioids Are Infrequently Required following Ambulatory Otologic Surgery

Mavrommatis, Maria A.; Fan, Caleb J.; Villavisanis, Dillan F.; Kaul, Vivian F.; Schwam, Zachary G.; Wong, Kevin; Perez, Enrique; Wanna, George B.; Cosetti, Maura K.

Publication date 01-10-2021


Objective: To determine the frequency with which postoperative opioid prescriptions are required after ambulatory otologic surgery.
Study Design: Retrospective chart review.
Setting: Tertiary otology–neurotology practice.
Patients: Patients (n = 447) given over-the-counter acetaminophen and ibuprofen following ambulatory otologic surgery between July 1, 2018 and June 30, 2020.
Intervention: Opioid prescription upon request.
Main Outcome Measures: Patient, disease, and surgical variables such as age, sex, past medical history, chronic pain condition, surgical procedure, primary versus (vs.) revision surgery, and endoscopic vs. microscopic approach were examined for relationship to ad hoc opioid prescription rate.
Results: Of 370 adult patients (mean age 49.0 yrs, range 18.0–88.5 yrs), 75 (20.3%) were prescribed opioids for postoperative pain, most commonly oxycodone-acetaminophen 5/325 mg. Of 77 pediatric patients (mean age 8.8 yrs, range 0.7–17.9 yrs), 5 (6.5%) were prescribed postoperative opioid analgesia. In the adult population, chronic pain condition, pain medication use at baseline, canal wall up mastoidectomy, tympanoplasty, tympanomeatal flap, bone removal of the mastoid, postauricular incision, and intraoperative microscopy were independent predictors of opioid pain prescription. When controlling for all significant variables, only chronic pain condition remained significant (odds ratio = 3.94; p = 0.0007). In the pediatric population, atresiaplasty, meatoplasty, and conchal cartilage removal were independently associated with opioid prescription, but none remained significant when analyzed in a multivariate linear model.
Conclusions: Pain following ambulatory otologic surgery may be adequately managed with over-the-counter pain medications in the majority of cases. Opioids may be necessary in adults with preexisting pain conditions.

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Treatment Outcome of the Bony Obliteration Tympanoplasty Versus Nonobliterative Techniques in Cholesteatoma Surgery: A Retrospective Analysis

van der Toom, Hylke F. E.; van der Schroeff, Marc P.; Metselaar, Mick; van Linge, Anne; Vroegop, Jantien L.; Pauw, Robert J.

Publication date 01-10-2021


Objective: In cholesteatoma surgery, obliteration of the mastoid and epitympanic space (bony obliteration tympanoplasty, BOT) is an increasingly used technique with low recurrent and residual cholesteatoma rates. While factors as the postoperative hearing level and infection rate are important for the patient as well, these outcome parameters are not frequently reported on in current literature. The objective of this study is to evaluate the recurrent and residual cholesteatoma rates of the BOT technique and nonobliterative canal wall up (CWU) and canal wall down (CWD) mastoidectomy in a large patient cohort. Secondary objectives were to evaluate the infection rate and hearing outcome for all three techniques.
Design: Retrospective cohort study.
Setting: Single-center study.
Patients: All 337 adult patients (≥18 yrs) who underwent primary or revision cholesteatoma surgery between January 2013 and March 2019 were included.
Main Outcome and Measures: Recurrent cholesteatoma rates, residual cholesteatoma rates, postoperative infections and other complications, hearing outcome.
Results: The estimated combined rate of recurrent and residual cholesteatoma at 5 years follow-up was 7.6% in the BOT group, 34.9% in the CWU group, and 17.9% in the CWD group. The postoperative infection rate in the different groups ranged from 4.3% to 4.9%. The median gain in AC threshold level varied from 0.0 dB in the BOT and CWD group to 3.8 dB in the CWU group.
Conclusions: We show that cholesteatoma recurrence rates after the BOT technique in our clinic are significantly lower compared to CWU surgery. There were no differences in infection rate and no clinically relevant differences in postoperative hearing between the BOT, CWU, and CWD technique.

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Topical Therapy Failure in Chronic Suppurative Otitis Media is Due to Persister Cells in Biofilms

Santa Maria, Peter L.; Kaufman, Adam C.; Bacacao, Brian; Thai, Anthony; Chen, Xiaohua; Xia, Anping; Cao, Zhixin; Fouad, Ayman; Bekale, Laurent A.

Publication date 01-10-2021


Objective: Chronic suppurative otitis media (CSOM) is characterized by a chronically draining middle ear. CSOM is typically treated with multiple courses of antibiotics or antiseptics which are successful in achieving quiescence; however, the disease is prone to relapse. Understanding why these treatment failures occur is essential.
Study Design: The minimum inhibitory concentration (MIC), minimal biofilm eradication concentration, and the inhibitory zone were determined for ototopicals and ofloxacin for the laboratory strains and CSOM-derived isolates. The percentage of persister cells and bacterial biofilm formation were measured. Disease eradication was tested in a validated in-vivo model of CSOM after treatment with ofloxacin.
Setting: Microbiology Laboratory.
Methods: Basic science experiments were performed to measure the effectiveness of a number of compounds against CSOM bacteria in a number of distinct settings.
Results: The minimal biofilm eradication concentration is higher than is physiologically achievable with commercial preparations, except for povo-iodine. Clincial isolates of CSOM have equivalent biofilm-forming ability but increased proportions of persister cells. Ofloxacin can convert to inactive disease temporarily but fails to eradicate disease in an in-vivo model.
Conclusions: Higher percentages of persister cells in clinical CSOM isolates are associated with resistance to ototopicals. Current ototopicals, except povo-iodine, have limited clinical effectiveness; however, it is unknown what the maximum achievable concentration is and there are ototoxicity concerns. Fluoroquinolones, while successful in producing inactive disease in the short term, have the potential to encourage antimicrobial resistance and disease recalcitrance and do not achieve a permanent remission. Given these limitations, clinicians should consider surgery earlier or use of clinically safe concentrations of povo-iodine earlier into the treatment algorithm.

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Transtympanic Tripod-shaped Angiocatheter Insertion for Patients With Intractable Patulous Eustachian Tube

Park, Jung Mee; Han, Jae Sang; Park, So Young; Park, Shi Nae

Publication date 01-10-2021


Objective: Despite the efforts to treat patients with patulous Eustachian tube (PET), intractable symptoms of PET may require surgical intervention. In this study, we introduce our surgical technique of “transtympanic tripod-shaped angiocatheter” (TTA) insertion and evaluate the safety and efficacy of the procedure in patients with intractable PET.
Study design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: Thirty-two cases (26 patients) diagnosed with intractable PET between the years 2011 and 2019 were included in this study.
Intervention: All cases were surgically treated with TTA insertion.
Main outcome measures: The clinical characteristics, surgical results, complication rates, and the level of satisfaction by questionnaires were analyzed for evaluation.
Results: The mean age of enrolled patients was 40.9 ± 19.5 years, with slight male gender predominance (59.4% vs. 40.6%). Both ears were operated in six patients, simultaneously in three. All patients successfully received the insertion of TTA, with no immediate complication. Eleven cases were re-inserted after the first insertion due to the recurrence of symptoms or spontaneous extrusion of TTA. No major complications were observed during the follow-up period of 1 to 115 months (mean 37.7 mo). The average Visual Analogue Scale score of PET symptoms was significantly decreased (p < 0.05), along with 92% of patients with relief from autophony symptoms.
Conclusions: TTA insertion is a relatively simple procedure that can be performed in a short period under local anesthesia. Surgical intervention of TTA insertion for patients with intractable PET symptoms seems promising with a high success rate.

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The Role of Biofilms in Chronic Otitis Media-Active Squamosal Disease: An Evaluative Study

Singh, Gautam Bir; Malhotra, Sonali; Yadav, Subhash Chandra; Kaur, Ravinder; Kwatra, Devanshu; Kumar, Sunil

Publication date 01-10-2021


Objectives: To study the presence of biofilms in patients of chronic otitis media (COM)-active squamosal disease and to evaluate the microflora and clinical impact of biofilms.
Methods: A total of 35 patients suffering from COM – active squamosal disease was studied. Cholesteatoma sample was collected at the time of mastoid surgery and the same was used to image for biofilms by scanning electron microscope. The said sample was also analyzed microbiologically.
Results: Biofilms were present in 25 (72%) patients including 6 cases of fungal biofilms (24%). Biofilms were detected irrespective of the micro-organism growth. No statistically significant relationship was observed between biofilms and discharging ear and complications of COM. Though univariate analysis of our data found some correlation between duration of disease, the extent of cholesteatoma and ossicular necrosis but multivariate analysis revealed no such associations, thereby implying a doubtful association between these factors and biofilm.
Conclusions: Biofilms are an integral part of cholesteatoma ear disease and sometimes harbors fungal isolates.

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Machine Learning Technique Reveals Prognostic Factors of Vibrant Soundbridge for Conductive or Mixed Hearing Loss Patients

Koyama, Hajime; Mori, Anjin; Nagatomi, Daisuke; Fujita, Takeshi; Saito, Kazuya; Osaki, Yasuhiro; Yamasoba, Tatsuya; Doi, Katsumi

Publication date 01-10-2021


Objectives: Vibrant Soundbridge (VSB) was developed for treatment of hearing loss, but clinical outcomes vary and prognostic factors predicting the success of the treatment remain unknown. We examined clinical outcomes of VSB for conductive or mixed hearing loss, prognostic factors by analyzing prediction models, and cut-off values to predict the outcomes.
Study design: Retrospective chart review.
Setting: Tertiary care hospital.
Patients: Thirty patients who underwent VSB surgery from January 2017 to December 2019 at our hospital.
Intervention: Audiological tests were performed prior to and 3 months after surgery; patients completed questionnaires 3 months after surgery.
Main outcome measures: We used a multiregression and the random forest algorithm for predictions. Mean absolute errors and coefficient of determinations were calculated to estimate prediction accuracies. Coefficient values in the multiregression model and the importance of features in the random forest model were calculated to clarify prognostic factors. Receiver operation characteristic curves were plotted.
Results: All audiological outcomes improved after surgery. The random forest model (mean absolute error: 0.06) recorded more accuracy than the multiregression model (mean absolute error: 0.12). Speech discrimination score in a silent context in patients with hearing aids was the most influential factor (coefficient value: 0.51, featured value: 0.71). The candidate cut-off value was 36% (sensitivity: 89%, specificity: 75%).
Conclusions: VSB is an effective treatment for conductive or mixed hearing loss. Machine learning demonstrated more precise predictions, and speech discrimination scores in a silent context in patients with hearing aids were the most important factor in predicting clinical outcomes.

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Trends and Healthcare Use Following Different Cholesteatoma Surgery Types in a National Cohort, 2003–2019

Qian, Zhen Jason; Tran, Emma D.; Alyono, Jennifer C.; Cheng, Alan G.; Ahmad, Iram N.; Chang, Kay W.

Publication date 01-10-2021


Objective: To describe national trends in cholesteatoma management.
Study Design and Setting: Retrospective analysis Optum Clinformatics® Database from 2003 to 2019.
Patients: 16,179 unique adult and pediatric patients who received cholesteatoma surgery.
Interventions and Main Outcome Measures: Patients were categorized into three groups by initial surgical modality: canal wall down (CWD), canal wall up (CWU), and tympanoplasty without mastoidectomy (TnoM).
Three major comparisons between groups were performed: 1) temporal trends, 2) clinical and sociodemographic determinants, and 3) healthcare use in terms of total costs and incidence of postoperative imaging and subsequent surgery.
Results: Overall, 23.2% received initial CWD surgery, 44.3% CWU, and 32.5% TnoM. 1) The incidence of initial CWD surgery decreased (odds ratios OR = 0.98, 95% confidence intervals CI 0.97,0.99), while CWU increased (OR = 1.02, 95% CI 1.01,1.03), and TnoM remained stable over the study period (OR = 0.99, 95% CI 0.98,1.00). 2) Relative to CWU, TnoM surgery was less likely in adults, patients with prior complications, and non-White patients, while being more likely in patients with higher household income. CWD was more likely than CWU in adults, patients with prior complications, and non-White patients, while income had no effect. 3) Postoperative costs for CWU and CWD were similar. In 2 years following initial surgery, postoperative imaging and/or subsequent surgery was performed in 45.48% of CWD, 57.42% of CWU, and 41.62% of TnoM patients.
Conclusions: Incidence of initial CWD surgery decreased and social disparities in cholesteatoma management were observed. Postoperative imaging or second-look surgery were performed in less than 60% of patients with initial CWU surgery and over 40% of patients with initial CWD.

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Congenital Unilateral Hearing Loss: Characteristics and Etiological Analysis in 121 Patients

Acke, Frederic R. E.; Van Hoecke, Helen; De Leenheer, Els M. R.

Publication date 01-10-2021


Objective: To describe the characteristics and etiological analysis in patients with congenital unilateral hearing loss.
Study design: Retrospective cohort analysis.
Setting: Tertiary referral center.
Patients: Children with permanent congenital unilateral hearing loss born between 2007 and 2018. Patients were referred after universal newborn hearing screening or by a colleague to confirm the diagnosis and perform etiological examinations.
Main outcome measures: Hearing loss type, severity, and evolution linked with the results of etiological testing.
Results: In the 121 included children, aural atresia is the leading cause of congenital unilateral hearing loss (32%), followed by structural anomalies (19%) and cCMV (13%), whereas 24% remained idiopathic after etiological work-up. Severity is mainly moderately severe (33% with 56–70 dB hearing loss, majority aural atresia) or profound (31% with > 90 dB hearing loss, predominantly cochlear nerve deficiency). Syndromic features were present in 26%. Although discussed with all parents, only 26% of the children regularly used hearing amplification.
Conclusions: Congenital conductive unilateral hearing loss is mainly caused by aural atresia, which proportion in congenital unilateral hearing loss proved higher than previously reported. Cochlear nerve deficiency and cCMV are the predominant etiologies of congenital unilateral sensorineural hearing loss. Etiological work-up in affected patients is mandatory as it might impact the approach, and syndromic features should be actively searched for.

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Fatigue in Children With Unilateral and Bilateral Hearing Loss

Sindhar, Sampat; Friesen, Tzyynong L.; Carpenter, Delaney; Kesser, Bradley; Lieu, Judith E. C.

Publication date 01-10-2021


Objective: To determine whether children with unilateral hearing loss (UHL) experience similar levels fatigue as children with bilateral hearing loss (BHL) or normal-hearing (NH).
Design: Cross-sectional study.
Setting: Two tertiary care otolaryngology practices.
Participants: Children, 5 to 18 years old, with UHL or BHL and their parents.
Main Outcome Measures: PedsQL Multidimensional Fatigue Scale (MFS) survey.
Results: Overall response rate was 90/384 (23%). Mean age of child participants was 10.7 years old (standard deviations SD 3.1); 38 (42%) were men and 52 (58%) were women. Sixty-nine (77%) children had UHL, 21 (23%) had BHL. Children with BHL (mean 65, SD 21) and UHL (mean 75, SD 17) reported greater levels of fatigue than children with NH (BHL difference –15, 95% confidence interval CI –25 to –5; UHL difference –6, 95% CI –13–1.2). Parent-proxy reports for children with BHL (mean 67, SD 20) and UHL (mean 76, SD 20) reported more fatigue than NH (BHL difference –22, 95% CI –33 to –12; UHL difference –14; 95% CI –20 to –8). Sub-section scores for general, sleep, and cognitive fatigue were higher for children with BHL and UHL than NH.
Conclusion and Relevance: Children with UHL and BHL reported significantly more fatigue than children with NH, and children with BHL reported more fatigue than UHL. These findings underscore the need to increase auditory rehabilitation and educational resources for children with UHL and support the use of the PedsQL MFS questionnaire as a measure to follow disability experienced by children with HL as they undergo hearing rehabilitation.

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Hearing Outcomes with Percutaneous and Transcutaneous BAHA® Technology in Conductive and Mixed Hearing Loss

Tobia, Amjad; Yehudai, Noam; Khnifes, Riad; Shpak, Talma; Roth, Osnat; Khayr, Ranin; Luntz, Michal

Publication date 01-10-2021


Objectives: To evaluate outcomes of BAHA Connect® and BAHA Attract® implantations, and to examine the prognostic utility of a preimplantation Softband®-attached processor trial.
Study Design: Retrospective case review Setting: Tertiary referral center.
Patients: Patients who underwent Connect® (19 ears) and Attract® (25 ears) implantation between 2007 and 2017.
Intervention: BAHA® implantation.
Main Outcome Measures: Unaided air conduction (AC), bone conduction (BC), and speech reception thresholds (SRTs), as well as free field (FF) aided with Softband®-attached processor and with implant-attached processor thresholds.
Results: Serviceable implant-attached processor PTA0.5,1,2 k Hz (≤35 dB HL) was achieved in 89 and 88% of the Connect® and the Attract® ears, respectively, while at 4 k Hz this was achieved in 68 and 32% of the Connect® and the Attract® ears, respectively (p  =  0.032). Significantly more Connect® ears showed alignment between FF aided with implant-attached processors thresholds and BC thresholds. The alignment between the Softband®-attached processors thresholds and implant-attached processors thresholds was similar in the two groups.
Both groups exhibited similar positive improvement in the quality of life questionnaires Conclusions: Accessibility to sound with the implant-attached processor is well predicted by the pre-implantation Softband® trial, both in the BAHA Connect® and in the BAHA Attract® ears. Hearing rehabilitation targets at 0.5, 1, and 2 k Hz are met by most Connect® and Attract® ears, while at 4 k Hz the outcome with Attract® is poorer. This information should be presented to the patient during consultation prior to a decision as to the type of BAHA® device to be implanted.

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Transcutaneous Osseo-integrated Auditory Devices: Analysis of Two Different Implants in Adults With Different Audiological Inclusion Criteria

Plasencia, Daniel Perez; Suárez, Antonia Alemán; Barreiro, Silvia Borkoski; de Miguel, Angel Ramos; Macias, Angel Ramos

Publication date 01-10-2021


Objective: To determine and compare audiological and speech/language discrimination results in patients with osteo-integrated auditory devices, by comparing two different systems including different audiological indications in adult population.
Study Design: Descriptive and analytic, cross-sectional, cohort, and retrospective case review.
Setting: Tertiary referral center.
Materials and Methods: Fifty patients >14 years old, with conductive, mixed, or unilateral sensorineural hearing loss were compared.
The variables studied were the following: pure tone audiometry results, air-bone gap, and the percentage of speech/language discrimination using Disyllabic Word Test. The patients were tested preoperative and 12 months after surgical procedure. Subsequently, a comparative analysis of the both systems was carried out.
Results: With both implants, pure tone audiometry results and the difference in auditory thresholds and between the air-bone gap improved significantly. Speech discrimination increased significantly after implantation too. A significant difference was observed after implantation of both devices. Patients implanted with one of the implants showed better audiological results, but no significant differences were found with the other device. So, it was shown that the type of device and the type of hearing loss have no influence on the audiological results or complications.
Conclusion: Both implants represent a good choice for audiological rehabilitation in patients with transmissive hearing loss, mixed hearing loss, or unilateral sensorineural hearing loss with a high success and low complication rates.

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"Rupture-Like Shrinkage and Regrowth of Endolymphatic Hydrops in Ménières Disease During Remission"

Fukushima, Munehisa; Suekata, Yu; Kusumoto, Takuya; Akahani, Shiro; Okamoto, Hidehiko; Inohara, Hidenori; Takeda, Noriaki

Publication date 01-10-2021


Objective: To clarify pathophysiological characteristics of Ménières disease during the remission phase.
Patients: Two Ménières disease patients with different disease durations, whose endolymphatic hydrops was longitudinally observed using 3-T magnetic resonance imaging.
Interventions: Diagnostic.
Main Outcomes and Measures: Morphological changes of endolymphatic hydrops visualized using 3-T magnetic resonance imaging.
Results: Rupture-like shrinkage and regrowth of endolymphatic hydrops during the remission phase was observed in both patients.
Conclusion: The remission phase with hearing improvement could be as important as vertigo attacks in Ménières disease.

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Morphological Effect of Vitamin D Deficiency on Globular Substances in Mice

Song, Penglong; Zhao, Xianshu; Xu, Yanjun; Zhao, Zhigang; Liu, Yang; Gao, Qian

Publication date 01-10-2021


Objective: Many authors, including us, elucidated that vitamin D deficiency was a risk factor for benign paroxysmal position vertigo. We speculated vitamin D deficiency was likely to intervene otoconia formation by globular substance (GS).
Methods: Kunming mice were randomly divided into three groups: vitamin D sufficient group (12-wk standard control diet), vitamin D deficiency group (16-wk vitamin D deficiency diet), and vitamin D supplement group (16-wk vitamin D deficiency diet and followed 8-wk standard control diet). At 12, 16, and 24 weeks, blood was collected for measuring vitamin D and macula utriculi were obtained for research under scanning electron microscope (SEM). We randomly selected 10 SEM photographs of macula utriculi in each mouse, counted cilium and GS, and measured diameters of counted GS. The ratio of the number of GS to cilium in each SEM photograph was defined as density of GS.
Results: The diameter and density of GS were larger and higher in vitamin D deficiency group than sufficient group (p < 0.05; p < 0.05). There was no significant difference in density and diameters of GS between vitamin D deficiency and supplement group. The rough and grainy surface became smoother and smoother along with vitamin D deficiency, and reappeared after vitamin D supplement for 8 weeks.
Conclusion: GS secreted as a precursor of mature otoconia is affected by vitamin D deficiency and vitamin D supplementation can mitigate the effects in mice. The density of GS, a quantitative method we designed, can quantify GS well.

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Spectral Composition of Body Sway in Persistent Postural-Perceptual Dizziness

Anagnostou, Evangelos; Stavropoulou, Georgia; Zachou, Athena; Kararizou, Evangelia

Publication date 01-10-2021


Objective: Previous studies in phobic postural vertigo patients showed characteristic frequency changes in body sway fluctuations, raising the question whether similar spectral changes can be also observed in the recently defined syndrome of persistent postural-perceptual dizziness (PPPD).
Study design: Cross-sectional prospective study.
Setting: Tertiary referral center.
Subjects: Sixty-one PPPD patients and 41 healthy controls.
Interventions: Static balance was assessed while standing on firm surface with eyes open or closed (conditions 1 and 2) and while standing on foam with eyes open or closed (conditions 3 and 4). Postural sway was analyzed by means of time (sway area and standard deviation) and frequency domain metrics.
The latter was based on comparisons of the percentage of energy in each of three frequency bands: low (0–0.5 Hz), middle (0.05–2 Hz), and high frequency (2–20 Hz).
Main outcome measure: Stabilometric time and frequency domain parameters.
Results: Time domain metrics deteriorated significantly from conditions 1 through condition 4 in patients and controls. Spectral changes, however, were more abundant in PPPD subjects than in controls. Patients showed increased low frequency, but decreased high frequency spectral power in condition 3 as compared to condition 2. Dizziness Handicap Inventory score was positively correlated with middle frequency and negatively correlated with low frequency fluctuations.
Conclusions: We conclude that PPPD patients exhibit a time domain sway pattern in different conditions which is grossly similar to that of controls. However, sensory feedback conditions with equal sway area show unique differences in their spectral content in PPPD patients. Moreover, perceived severity of dizziness is associated with greater body oscillations in the middle frequency band.

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Vestibular Function in Pendred Syndrome: Intact High Frequency VOR and Saccular Hypersensitivity

West, Niels Cramer; Ryberg, Alexander Christian; Cayé-Thomasen, Per

Publication date 01-10-2021


Objective: Although Pendred syndrome involves anatomical abnormalities in the vestibular system and patient-perceived dizziness, the literature on vestibular function is scarce. The objective was to evaluate objective vestibular function in patients with PS using the video head impulse test (VHIT) and the cervical vestibular evoked myogenic potential (cVEMP) test, assessing the high frequency vestibulo-ocular reflex (VOR) and the saccular function, respectively.
Methods: Twenty-six subjects (52 ears) with Pendred Syndrome and severe-to-profound hearing loss (cochlear implant candidacy) were studied retrospectively. Main outcome measures were VHIT VOR gain, refixation saccades, cVEMP stimulus thresholds and amplitudes.
Results: In total, 4 of 52 ears (8%) had pathological VHIT outcomes (2 ears with low VHIT gain and 2 ears with saccades). The mean VHIT gain value was 0.96 (range 0.63–2.02). No patients had complete VOR loss. CVEMP responses were present in 76% of tested ears and absent in 24%. The mean cVEMP amplitude value was 192 μV. Absence of cVEMP response was associated with the presence of VHIT saccades (p = 0.038) and advanced age (rs = −0.34; p < 0.05). cVEMP amplitude was correlated with VHIT gain (rs = 0.46; p < 0.001).
Conclusion: Despite dysmorphic vestibular anatomy and severe loss of hearing, the VHIT VOR function is normal in patients with Pendred Syndrome. However, saccular function appears to be abnormally sensitive, as documented by low cVEMP thresholds and high amplitudes, which agrees with a “third window” effect secondary to the large vestibular aqueduct.

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Higher Readmission Rates After Hip Fracture Among Patients With Vestibular Disorders

Curry, Steven D.; Carotenuto, Alessandro; DeLuna, Devin A.; Maar, Dennis J. II; Huang, Ye; Samson, Kaeli K.; Siebler, Justin C.; Hatch, Jonathan L.

Publication date 01-10-2021


Objective: Falls in older adults are associated with high morbidity and mortality. Patients with vestibular disorders may have an increased risk. The purpose of this study was to examine the outcomes among patients with underlying vestibular disorders who have hip fractures and identify predictors of increased morbidity and mortality.
Study design: Retrospective cohort study.
Setting: Tertiary care academic medical center.
Patients: Two hundred one adults diagnosed with a vestibular disorder and hip fracture due to a ground-level fall were compared to 327 age- and sex-matched controls with fractures due to ground-level falls without vestibular diagnoses. Patients were treated between 2013 and 2019.
Main outcome measures: Length of hospital stay, 30-day readmission rate, and 30-day mortality rate.
Results: Thirty-day readmission rate after hip fracture was significantly increased in patients with vestibular disorders compared to matched controls (p < 0.001), odds ratio 3.12 (95% confidence interval 1.84–5.39). Reasons for readmission in the vestibular patient group included higher rates of repeat falls, infections, and recurrent vestibular symptoms. Use of medication classes associated with falls or hip fractures was not significantly different between groups, except for lower rates of antihypertensive use in the vestibular group (54.0% vs. 67.7%, p = 0.002). No significant difference was found for length of hospital stay (7.34 ± 4.95 vs. 8.14 ± 20.50 days, p = 0.51) or 30-day mortality rate (5.0% vs. 4.6%, p = 0.99). No significant differences were found between groups for age, sex, race, rate of surgical treatment for hip fracture, or disposition at discharge.
Conclusions: Patients with vestibular disorders are at a significantly higher risk of hospital readmission within 30 days after discharge for treatment for hip fracture.

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Matched Cohort Analysis of the Effect of the Facial Recess Approach on Cerebrospinal Fluid Leak After Translabyrinthine Surgery for Schwannoma

Christopher, Laura; Slattery, William; Lekovic, Gregory P.; Mehta, Gautam U.; Miller, Mia

Publication date 01-10-2021


Objective: The facial recess approach during translabyrinthine surgery has been used to expose the eustachian tube (ET) for packing. We sought to determine the effect of this technique on the development of postoperative nasopharyngeal cerebrospinal fluid (CSF) leaks.
Patients: Cohorts of patients with cerebellopontine angle schwannomas who underwent a facial recess approach or no facial recess approach were matched based on tumor size.
Interventions: Translabyrinthine surgery for tumor resection.
Main Outcome Measures: Postoperative CSF leaks were recorded and nasopharyngeal CSF leaks were utilized as the primary outcome measure.
Results: Using an exact matching protocol based on tumor size, 102 patients were included in each group (204 total, 111 female, 93 male). Overall, 9 patients (4.4%) demonstrated a postoperative nasopharyngeal CSF leak. Postoperative CSF rhinorrhea was noted in 3.9% of the group who underwent a facial recess approach for packing of the ET and 4.9% of the group who did not undergo a facial recess approach. This rate was not significantly different between groups (p = 0.99, Odds ratio: 0.79, 95% CI: 0.15–3.8). Secondary variables including age, tumor size, a diagnosis of NF2, and the packing material used were not significant predictors of nasopharyngeal CSF leaks.
Conclusions: CSF rhinorrhea is infrequent after translabyrinthine surgery. The incidence of this complication is not affected by whether or not a facial recess approach is performed during surgery to pack the ET. Based on these data, use of this technique should be based on surgeon comfort and preference.

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Cochlear T2 Signal May Predict Hearing Outcomes After Resection of Acoustic Neuroma

Tawfik, Kareem O.; McDonald, Marin; Ren, Yin; Moshtaghi, Omid; Schwartz, Marc S.; Friedman, Rick A.

Publication date 01-10-2021


Objective: Examine the impact of preoperative cochlear Fast Imaging Employing Steady-state Acquisition (FIESTA) and Constructive Interference in Steady State (CISS) signal intensity on hearing outcomes after middle cranial fossa (MCF) resection of acoustic neuroma (AN).
Methods: Adult patients (≥18 years) who underwent MCF AN resection for hearing preservation (HP) between November 2017 and September 2019 were retrospectively reviewed. All patients had preoperative word recognition score (WRS) ≥50%. HP was defined as postoperative WRS ≥50%. A neuroradiologist blinded to patients’ clinical and audiometric outcomes reviewed patients’ preoperative magnetic resonance images. Ipsilateral-to-contralateral cochlear FIESTA/CISS signal intensity ratios were determined using hand-drawn regions of interest focused on the cochlear fluid. Preoperative and postoperative pure tone average (PTA) and WRS were reviewed.
Results: Fifty-one patients were reviewed (60.8% female). Mean age was 47 years and mean tumor size 9.2 mm (±3.8). Hearing was preserved in 56.9% (n = 29). FIESTA/CISS signal ratios did not significantly differ between patients with and without HP. Lower FIESTA/CISS signal ratios correlated with greater declines in hearing (r = 0.322, p = 0.011 for PTA; and r = 0.384, p = 0.004 for WRS). On multivariate analysis accounting for tumor size and preoperative PTA/WRS, decreases in FIESTA/CISS signal ratios independently predicted greater declines in hearing by PTA (b = −100.9, p = 0.012) and WRS (b = 76.208), although the latter result was not statistically significant (p = 0.078).
Conclusions: Cochlear FIESTA/CISS signal intensity may be a predictor of postoperative hearing loss after MCF AN resection. In this cohort, degraded preoperative cochlear FIESTA/CISS signal strongly predicted postoperative hearing loss.
Level of Evidence: IV.

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Outcomes of Initial Observation Versus Upfront Microsurgical Resection for Small to Medium-sized Vestibular Schwannomas

Patro, Ankita; Totten, Douglas J.; Sherry, Alexander D.; Manzoor, Nauman F.; Cass, Nathan D.; Tawfik, Kareem; Bennett, Marc L.; O’Malley, Matthew R.; Haynes, David S.; Perkins, Elizabeth L.

Publication date 01-10-2021


Objective: To assess postoperative outcomes and predictive factors of patients observed prior to microsurgery and those undergoing upfront resection for small and medium-sized VS.
Study design: Retrospective cohort.
Setting: Tertiary referral center.
Patients: VS patients who had microsurgery from 2003 to 2018 for tumors up to 2.5 cm.
Main outcome measures: Postoperative outcomes including facial nerve function and interventions, complications, extent of resection, and salvage therapy.
Results: Of 220 patients, 120 were initially observed, and 100 pursued upfront microsurgery. There was no significant association between initial observation and upfront microsurgery for postoperative facial nerve function at 2 to 3 weeks (p = 0.18) or 12 months (p = 0.5), facial nerve intervention (p = 0.5), major/minor complications (p = 0.48/0.63), recurrence (p = 0.8), subtotal resection (p = 0.6), or salvage therapy (p = 0.9). Time from initial consultation to surgery did not significantly impact outcomes. Intrameatal tumors were more likely to be observed (odds ratios OR 2.93; 95% CI 1.53–5.63; p = 0.001). Patients with larger tumor volume (OR 0.52; 95% CI 0.37–0.72; p < 0.0001), brainstem compression (OR 0.28; 95% CI 0.09–0.91; p = 0.03), or higher PTA were less likely to undergo observation (OR 0.99; 95% CI 0.97–0.997; p = 0.02). On multivariable analysis, predictive factors for observation were smaller tumor volume (OR 0.53; 95% CI 0.38–0.75; p < 0.001), lower PTA (OR 0.99; 95% CI 0.98–0.999; p = 0.04), and diabetes (OR 2.54; 95% CI 0.95–6.83; p = 0.06).
Conclusions: Patients with worse hearing, larger tumor volume, and brainstem compression were more likely to pursue upfront microsurgery. A watchful waiting period does not appear to worsen outcomes and can be considered for patients with better hearing and smaller tumors without brainstem compression.

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Simultaneous Cochlear Implantation After Translabyrinthine Vestibular Schwannoma Resection: A Report of 41 Cases

Sanna, Mario; Piccirillo, Enrico; Kihlgren, Caterina; Cagliero, Gloria; Guidi, Mariapaola; Saleh, Essam

Publication date 01-10-2021


Objective: To study the auditory outcome of simultaneous translabyrinthine vestibular schwannoma (VS) resection and cochlear implantation (CI) after successful cochlear nerve preservation.
Study Design: A retrospective case series and patient questionnaire.
Setting: Quaternary referral center for skull base pathologies.
Patients: Patients with small (<2 cm) sporadic or neurofibromatosis 2 related tumors were included in this study.
Intervention: Vestibular schwannoma resection + simultaneous cochlear implantation.
Main Outcome Measure: Audiological performance postimplantation and perceived patients’ benefits.
Results: Forty-one patients were included. Thirty-three were sporadic VS and eight were neurofibromatosis 2. Auditory perception postimplantation was achieved in 33 patients (80.5%). At the last follow-up, 20 patients (48.8%) were users and 21 (51.2%) were nonusers. In the users’ group, and after 1 year of implant activation, vowel identification was 75.3%, disyllabic word recognition 54%, sentence recognition 60.7%, and common phrase comprehension 61%, whereas in the nonusers’ group and after 1 year of implant activation, vowel identification was 22.9%, disyllabic word recognition 14.8%, sentence recognition 15.3%, and common phrase comprehension 14%. Sixteen users were classified into 10 high performers, three intermediate performers, and three poor performers. In the user’ group, the mean postimplantation pure tone average was 63.4 dB and the mean speech discrimination score was 63.7%.
Conclusions: Simultaneous CI and VS resection is a viable option with many patients achieving auditory perception and nearly half the patients are CI users at long follow-up.

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Diameter-Based Volumetric Models May Inadequately Calculate Jugular Paraganglioma Volume Following Sub-Total Resection

Totten, Douglas J.; Sherry, Alexander D.; Manzoor, Nauman F.; Perkins, Elizabeth L.; Cass, Nathan D.; Khattab, Mohamed H.; Cmelak, Anthony J.; Haynes, David S.; Aulino, Joseph M.

Publication date 01-10-2021


Background: As gross total resection of jugular paragangliomas (JPs) may result in cranial nerve deficits, JPs are increasingly managed with subtotal resection (STR) with postoperative radiological monitoring. However, the validity of commonly used diameter-based models that calculate postoperative volume to determine residual tumor growth is dubious. The purpose of this study was to assess the accuracy of these models compared to manual volumetric slice-by-slice segmentation.
Methods: A senior neuroradiologist measured volumes via slice-by-slice segmentation of JPs pre- and postoperatively from patients who underwent STR from 2007 to 2019. Volumes from three linear-based models were calculated. Models with absolute percent error (APE) > 20% were considered unsatisfactory based on a common volumetric definition for residual growth. Bland-Altman plots were used to evaluate reproducibility, and Wilcoxon matched-pairs signed rank test evaluated model bias.
Results: Twenty-one patients were included. Median postoperative APE exceeded the established 20% threshold for each of the volumetric models as cuboidal, ellipsoidal, and spherical model APE were 63%, 28%, and 27%, respectively. The postoperative cuboidal model had significant systematic bias overestimating volume (p = 0.002) whereas the postoperative ellipsoidal and spherical models lacked systematic bias (p = 0.11 and p = 0.82).
Conclusion: Cuboidal, ellipsoidal, and spherical models do not provide accurate assessments of postoperative JP tumor volume and may result in salvage therapies that are unnecessary or inappropriately withheld due to inaccurate assessment of residual tumor growth. While more time-consuming, slice-by-slice segmentation by an experienced neuroradiologist provides a substantially more accurate and precise measurement of tumor volume that may optimize clinical management.

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The Influence of Extent of Resection and Tumor Morphology on Facial Nerve Outcomes Following Vestibular Schwannoma Surgery

Perkins, Elizabeth L.; Manzoor, Nauman F.; Totten, Douglas J.; Sherry, Alexander D.; Cass, Nathan; Thompson, Reid; Tawfik, Kareem; O’Malley, Matthew; Bennett, Marc; Haynes, David S.

Publication date 01-10-2021


Objective: To determine the influence extent of resection and tumor characteristics on facial nerve (FN) outcomes following microsurgical resection of vestibular schwannoma (VS).
Study Design: Retrospective chart review.
Setting: Tertiary referral center.
Patients: Three hundred eighty-five patients who underwent VS microsurgical resection.
Interventions: Microsurgical VS resection.
Main Outcome Measures: House-Brackmann (HB) scores postoperatively. Good FN function was defined as HB grade I and II and poor FN function was defined as HB grade III and VI. Gross total resection (GTR) versus subtotal resection (STR). Propensity-score matching was used in subset analysis to balance tumor volume between the surgical cohorts, followed by multivariable analysis.
Results: Seventy-one patients (18%) underwent STR and 314 patients (82%) underwent GTR. Two hundred fourteen patients (63%) had good FN function at 2 to 3 weeks postoperatively, and 80% had good FN function at 1 year. In single predictor analysis, STR did not influence FN function at 2 to 3 weeks (p = 0.65). In propensity-score matched subset analysis (N = 178), patients with STR were less likely to have poor FN function at 2 to 3 weeks (p = 0.02) independent of tumor volume (p = 0.004), but there was no correlation between STR and FN function at 1 year (p = 0.09). Ventral extension of tumor relative to the internal auditory canal plane was associated with poor FN outcomes at 2 to 3 weeks (p = 0.0001) and 1-year postop (p = 0.002).
Conclusions: When accounting for tumor volume, STR is protective in immediate postoperative FN function compared to GTR. Ventral extension of the tumor is a clinical predictor of long-term FN outcomes.

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A Rare Presentation of Primary Cutaneous Low-Grade Neuroendocrine Tumor of the External Auditory Canal

Bhardwaj, Priya; Tanenbaum, Zachary G.; Nguy, Peter L.; Jay, Ann K.; Dimopoulos, Yiannis Petros; Hoa, Michael

Publication date 01-10-2021


Objective: To describe the first case of a primary cutaneous low-grade neuroendocrine tumor (cLGNET) originating from the external auditory canal as well as our teams surgical management.
Patient: A healthy 34-year-old female presented with a low-grade neuroendocrine tumor of her right external auditory canal (EAC) which extended from the posterior–superior aspect of the EAC into the middle ear.
Intervention: A complete otological examination was performed in addition to CT and MRI imaging. The low-grade neuroendocrine tumor was surgically biopsied and further surgery was recommended for complete resection.
Results: Audiogram revealed profound right sensorineural hearing loss. CT scan demonstrated complete opacification of the right EAC, middle ear, and mastoid air cells, dystrophic calcification in the mesotympanum overlying the cochlear promontory, and no associated osseous erosion. MRI revealed abnormal FLAIR hyperintensity and enhancement of the labyrinthine segment of the right facial nerve, cochlea, and horizontal and posterior semicircular canals. An enhancing mass opacifying the right EAC demonstrating restricted diffusion on diffusion-weighted image was also evident. Pathologic examination and immunohistochemical staining confirmed a diagnosis of primary cLGNET of the EAC.
Conclusion: Primary cLGNETs of the external ear are exceedingly rare but should be considered if an adult patient presents with a mass in the EAC. Management should include early biopsy and surgical excision followed by histological and immunohistochemical confirmation.

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Surgical Outcomes After Conservative Resection of Vestibular Schwannoma in the Elderly

Luryi, Alexander L.; Babu, Seilesh; Bojrab, Dennis I.; Kveton, John F.; Schutt, Christopher A.

Publication date 01-10-2021


Objective: To assess outcomes after surgery for vestibular schwannoma in patients over 70 years of age.
Study Design: Retrospective chart review.
Setting: Two tertiary otology and neurotology centers.
Patients and Interventions: Patients undergoing primary surgery for vestibular schwannoma between 2007 and 2018.
Main Outcome Measures: Postoperative complications and surgical outcomes.
Results: A total of 452 patients met inclusion criteria, 31 of whom (6.9%) were over 70 years of age. Age ranged from 18 to 90 years with a mean of 53 years. Elderly patients were more likely to have pre-existing hypertension (58.1% versus 34.0%, p = 0.007) and diabetes mellitus (19.4% versus 7.4%, p = 0.02). Elderly patients were less likely to undergo gross total resections of their tumors (35.5% versus 60.6%, p = 0.05) although they were not statistically significantly more likely to undergo subtotal ( 0.05). Elderly patients were also less likely to undergo second stage procedures (0% versus 9.5%, p = 0.04). There were no significant differences between elderly and non-elderly patients in the rates of any complications, ultimate facial nerve function, or duration of surgery. No patients over 70 years of age expired within 1 year of surgery.
Conclusions: Conservative surgery for vestibular schwannoma in appropriately selected elderly patients is appropriate and safe, given adequate consideration to risk-benefit analysis and goals of care.

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Risk Factors for Complications Following Lateral Skull Base Surgery and the Utility of ICU Monitoring

Sioshansi, Pedrom C.; Conway, Robert M.; Anderson, Brian; Minutello, Katrina; Bojrab, Dennis I.; Hong, Robert S.; Sargent, Eric W.; Schutt, Christopher A.; Zappia, John J.; Babu, Seilesh C.

Publication date 01-10-2021


Objective: To examine the role of intensive care unit (ICU) management following lateral skull base surgery for vestibular schwannoma and identify risk factors for complications warranting admission to the ICU.
Study Design: Retrospective review.
Setting: Tertiary referral center.
Patients: Two hundred consecutive patients undergoing lateral skull base surgery for vestibular schwannomas.
Intervention: Lateral skull base approach for resection of vestibular schwannoma and postoperative monitoring.
Main Outcome Measures: Patients were grouped if they sustained an ICU complication, a non-ICU complication, or no complication. Analysis was performed to determine patient or treatment factors that may be associated with ICU complications. Multivariate and three-way analysis of variance compared groups, and multivariate logistic regression determined adjusted odds ratios (aOR) for analyzed factors.
Results: Seventeen of 200 patients sustained ICU complications (8.5%), most commonly hypertensive urgency (n = 15). Forty-six (23%) sustained non-ICU complications, and 137 (68.5%) had no complications. When controlling for age, sex, obesity, and other comorbidities, only hypertension (aOR 5.43, 95% confidence interval (CI) 1.35–21.73, p = 0.017) and tumor volume (aOR 3.29, 95% CI 1.09–9.96, p = 0.035) were independently associated with increased risk of ICU complications.
Conclusions: The necessity of intensive care following lateral skull base surgery is rare, with the primary ICU complication being hypertensive urgency. Preoperative hypertension and large tumor volume (>4500 mm3) were independently associated with increased risk for ICU complications. These findings may allow for risk stratification of patients appropriate for admission to stepdown units following resection of vestibular schwannomas. Further prospective, multi-center, randomized studies are necessary to validate these findings before systematic changes to current postoperative care practices.

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Lifetime Cost and Quality-Adjusted Life-Years Across Management Options for Small- and Medium-Sized Sporadic Vestibular Schwannoma

Macielak, Robert J.; Thao, Viengneesee; Borah, Bijan J.; Moriarty, James P.; Marinelli, John P.; Van Gompel, Jamie J.; Carlson, Matthew L.

Publication date 01-10-2021


Objective: Despite the growing emphasis on healthcare costs, limited data address this aspect of care within the vestibular schwannoma (VS) literature. We sought to determine which strategy confers the lowest lifetime cost and greatest quality-adjusted life-years (QALYs) for patients with small- to medium-sized sporadic VS tumors.
Study Design: A Markov model was created to determine the most cost-effective management algorithm. Tumor characteristics, magnetic resonance imaging surveillance schedule, treatment outcomes, and health-related quality of life values were derived from previously published data. Cost estimates were based on CMS Fee Schedule reimbursement rates.
Setting: Economic Evaluation Service within the Kern Center for the Science of Healthcare Delivery.
Patients: Patients diagnosed with small- to medium-sized sporadic VS.
Interventions: Upfront microsurgery following diagnosis, upfront radiosurgery following diagnosis, observation with microsurgery reserved for observed tumor growth, and observation with radiosurgery reserved for observed tumor growth.
Results: Across patient ages at time of diagnosis ranging from 18 to 70 years, observation with subsequent radiosurgery used for tumor growth was the most cost-effective management algorithm while upfront microsurgery was the least. When presented with a hypothetical 50-year-old patient, the strategy with the lowest lifetime cost and highest QALYs was observation with subsequent radiosurgery reserved for tumor growth ($32,161, 14.11 QALY), followed by observation with microsurgery reserved for tumor growth ($34,503, 13.94 QALY), upfront radiosurgery ($43,456, 14.02 QALY), and lastly, upfront microsurgery ($47,252, 13.60 QALY). Sensitivity analyses varying mortality rates, estimated costs, health-related quality of life, and progression to nonserviceable hearing demonstrated consistent ranking among treatments.
Conclusions: When considering initial management of small- and medium-sized sporadic VSs, neither lifetime cost nor QALYs support upfront microsurgery or radiosurgery, even for younger patients. Initial observation with serial imaging, reserving radiosurgery or microsurgery for patients exhibiting tumor growth, confers the greatest potential for optimized lifetime healthcare cost and QALY outcomes.

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Intratympanic Insulin-like Growth Factor-1 Administration Via the Otic Bulla in a Severe Facial Paralysis Model

Kimura, Takuya; Yamada, Hiroyuki; Teraoka, Masato; Joko, Tomonori; Iwata, Shinji; Tabata, Yasuhiro; Wakisaka, Hiroyuki; Hato, Naohito

Publication date 01-10-2021


Hypothesis: We investigated the treatment effect of intratympanic insulin-like growth factor-1 (IGF-1) on severe facial paralysis in guinea pigs.
Background: The use of regenerative medicine involving growth factors has been reported in the treatment of peripheral nerve diseases. IGF-1 plays a crucial role in nerve regeneration.
Methods: We performed the following procedures on guinea pigs. In the normal group (n = 7), no procedure was performed. In the saline (n = 7) and IGF-1 (n = 7) groups, facial paralysis was induced by freezing of the facial canal. Subsequently, in the saline and IGF-1 groups, a gelatin hydrogel impregnated with 100 μL saline and 400 μg/100 μL IGF-1, respectively, was placed in the facial canal.
Facial nerve functions were evaluated using three test batteries: facial movement observation, electrophysiological testing, and histological assessment.
Results: At 10 weeks postoperatively, the facial movement scores for the IGF-1 group were improved compared to those in the saline group. The conductive velocity was significantly faster in the IGF-1 group than in the saline group. There was a significant between-group difference in the nerve fiber number and myelin thickness.
Conclusion: Intratympanic IGF-1 administration improved facial nerve regeneration. This novel method could provide prompt ambulatory regenerative treatment and reduce the incidence of poor recovery in patients with severe facial paralysis.

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The Correlation of Clinical Corticosteroid Responsiveness With Expression of IL-6 in Peripheral Blood Immune Cells (PBMC) in Patients With Autoimmune Inner Ear Disease (AIED)

Gorthey, Scott W.; Pathak, Shresh; Vambutas, Andrea

Publication date 01-10-2021


Hypothesis: Autoimmune inner ear disease (AIED) patients will differentially express interleukin (IL)-6 based on corticosteroid responsiveness.
Background: AIED is characterized by periods of acute sensorineural hearing loss (SNHL). In a majority of patients corticosteroid responsiveness is lost over time. The mechanisms that control corticosteroid responsiveness have not been fully elucidated.
Methods: Thirty-five AIED patients and 13 age-matched control subjects were enrolled in this study. Steroid responsive (n = 15) and steroid nonresponsive AIED patients (n = 20) were characterized based on audiometry before and after treatment for acute SNHL. Plasma and peripheral blood mononuclear cells (PBMC) were obtained at the time of acute SNHL to quantify plasma IL-6, soluble IL-6 receptor (sIL-6R), and C-C Motif Chemokine Ligand 3 (CCL3). PBMCs were stimulated with dexamethasone and release of soluble IL-6, sIL-6R, and CCL3 protein into conditioned supernatants was measured. Plasma IL-6 was also correlated to serum c-reactive protein (CRP), cardiac CRP, erythrocyte sedimentation rate.
Results: Statistically significant differences were observed in the plasma IL-6 between AIED patients and controls (2.37 versus 2.03 pg/ml, p < 0.01), plasma IL-6, and CCL3 between responders and nonresponders (0.136 versus 3.84 pg/ml, p < 0.005; 30.5 versus 32.4, p < 0.05) and released IL-6 from dexamethasone stimulated PBMC in AIED patients compared with controls (0.54 versus 1.12 pg/ml, p < 0.001). There was a correlation between plasma IL-6 levels of AIED patients to both serum CRP and cardiac CRP (R2 = 0.83, R2 = 0.88).
Conclusions: We observed AIED patients, specifically nonresponders expressed greater levels of IL-6. Elevated IL-6 levels in AIED patients correlated with CRP levels, providing a commonly available laboratory test that may aid in rapid clinical decision-making in these patients.

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A Web-Based Deep Learning Model for Automated Diagnosis of Otoscopic Images

Tsutsumi, Kotaro; Goshtasbi, Khodayar; Risbud, Adwight; Khosravi, Pooya; Pang, Jonathan C.; Lin, Harrison W.; Djalilian, Hamid R.; Abouzari, Mehdi

Publication date 01-10-2021


Objectives: To develop a multiclass-classifier deep learning model and website for distinguishing tympanic membrane (TM) pathologies based on otoscopic images.
Methods: An otoscopic image database developed by utilizing publicly available online images and open databases was assessed by convolutional neural network (CNN) models including Res Net-50, Inception-V3, Inception-Resnet-V2, and Mobile NetV2. Training and testing were conducted with a 75:25 breakdown. Area under the curve of receiver operating characteristics (AUC-ROC), accuracy, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were used to compare different CNN models’ performances in classifying TM images.
Results: Our database included 400 images, organized into normal (n = 196) and abnormal classes (n = 204), including acute otitis media (n = 116), otitis externa (n = 44), chronic suppurative otitis media (n = 23), and cerumen impaction (n = 21). For binary classification between normal versus abnormal TM, the best performing model had average AUC-ROC of 0.902 (Mobile NetV2), followed by 0.745 (Inception-Resnet-V2), 0.731 (Res Net-50), and 0.636 (Inception-V3). Accuracy ranged between 0.73–0.77, sensitivity 0.72–0.88, specificity 0.58–0.84, PPV 0.68–0.81, and NPV 0.73–0.83. Macro-AUC-ROC for Mobile NetV2 based multiclass-classifier was 0.91, with accuracy of 66%. Binary and multiclass-classifier models based on Mobile NetV2 were loaded onto a publicly accessible and user-friendly website (https://headneckml.com/tympanic). This allows the readership to upload TM images for real-time predictions using the developed algorithms.
Conclusions: Novel CNN algorithms were developed with high AUC-ROCs for differentiating between various TM pathologies. This was further deployed as a proof-of-concept publicly accessible website for real-time predictions.

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Notch Signaling in Acquired Middle Ear Cholesteatoma

Fukuda, Atsushi; Kano, Satoshi; Nakamaru, Yuji; Morita, Shinya; Hoshino, Kimiko; Fujiwara, Keishi; Homma, Akihiro

Publication date 01-10-2021


Hypothesis: We hypothesized that an anomalous change of Notch signaling might be involved in the pathophysiology of cholesteatoma.
Background: The Notch signaling pathway regulates integrated growth and differentiation control of keratinocytes. Its involvement in cholesteatoma proliferation has not been elucidated.
Methods: We obtained cholesteatoma and external auditory canal (EAC) skin samples from patients with middle ear cholesteatoma who underwent tympanomastoid surgery. We performed polymerase chain reaction using the RT2 Profiler™ PCR Array Human Notch Signaling Pathway (Qiagen) in the cholesteatoma and EAC skin samples (n = 6 each). This was followed by immunohistochemical staining of Notch1, enhancer of split-1 (HES1), and p53 in 41 and 8 cholesteatoma and EAC skin samples, respectively.
Results: The fold change of Notch1 gene expression was lowest in cholesteatoma, with a statistically significant difference (p = 0.0424). Moreover, the fold change of HES1 expression decreased (p = 0.272). The positive rates of Notch1 and HES1 protein expressions in the cholesteatoma (48.5 ± 32.4% and 44.9 ± 17.8%, respectively) were significantly lower than in the EAC skin (83.4 ± 17.5% and 55.7 ± 7.1%, respectively) (p < 0.001 and p < 0.01). In contrast, the positive rate of p53 expression in the cholesteatoma (8.5 ± 11.4%) was significantly higher than in the EAC skin (0.5 ± 0.7%) (p < 0.001).
Conclusion: The decreases in Notch1 and HES1 protein expression might play an important role in the hyperproliferative character of the keratinizing squamous epithelium in cholesteatoma. An increase in p53 might reflect the reaction to cellular hyperproliferation.

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Cannabinoid Receptor 2 Agonism is Capable of Preventing Lipopolysaccharide Induced Decreases of Cochlear Microcirculation – A Potential Approach for Inner Ear Pathologies

Weiss, Bernhard G.; Freytag, Saskia; Kloos, Benedikt; Haubner, Frank; Sharaf, Kariem; Spiegel, Jennifer Lee; Canis, Martin; Ihler, Friedrich; Bertlich, Mattis

Publication date 01-10-2021


Hypothesis: The ability of JWH-133, an agonist at the cannabinoid receptor 2, to abrogate the effects of lipopolysaccharide on cochlear microcirculation was investigated.
Background: Cochlear inflammation and subsequent impairment of microcirculation is part of numerous pathologies affecting inner ear function, including suppurative labyrinthitis, noise trauma, and sudden sensorineural hearing loss. One way of causing cochlear inflammation is exposing the cochlea to lipopolysaccharide, a bacterial endotoxin.
Methods: Twenty Dunkin-hartley guinea pigs were divided into four groups of five animals each. Two groups received topic treatment with JWH-133 and two received treatment with placebo. One group that had been treated with JWH-133 and one with placebo were then exposed to lipopolysaccharide or placebo, respectively. Cochlear microcirculation was quantified before, in between and after treatments by in vivo fluorescence microscopy.
Results: Significantly different changes in cochlear blood flow were only seen in the group that was treated with placebo and subsequently lipopolysaccharide. Every other group showed no significant change in cochlear blood flow.
Conclusion: JWH-133 is capable of abrogating the effects of lipopolysaccharide on cochlear microcirculation. It may therefore be clinical interest in treating numerous inflammation associated cochlear pathologies.

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A Cohort Study Comparing Importance of Clinical Factors in Determining Diagnosis and Treatment for Superior Semicircular Canal Dehiscence Syndrome

Zhang, Lisa; Creighton, Francis X. Jr; Carey, John P.

Publication date 01-10-2021


Objective: To determine which clinical factors have the strongest impact on determining diagnosis and decision for surgical repair for superior semicircular canal dehiscence syndrome (SCDS).
Patients: A total of 81 patients presented with a suspicion for SCDS between October 2017 and October 2018. 38 patients ultimately were diagnosed with SCDS, and 28 chose surgical repair.
Methods: Clinical factors of subjective patient symptoms (autophony, sensitivity to loud sounds, vertigo induced by loud sounds, dizziness, hearing their own heartbeats or other visceral organs) and audiogram findings (low frequency conductive hearing loss CHL, bone-conduction hyperacusis, low cVEMP threshold 17 μV amplitudes) were analyzed with logistic regression to identify factors that predicted those who would ultimately be diagnosed with SCDS and those who would have surgical repair. Preoperative high-resolution CT imaging was performed on all patients.
Results: Significant predictors of presence of SCDS from logistic regression included low frequency CHL and increased oVEMP amplitude (p = 0.002 OR 40, 95%CI 3.8-413, p = 0.001 OR 93, 95%CI 6.8-1267, respectively). These were also significant predictors of individuals who chose to have surgical repair (p = 0.004 OR 47, 95%CI 3.5-620, p = 0.001 OR 126, 95%CI 8.2-1941, respectively). Area under the receiver operating characteristic curve (ROC curve) for these two variables are 93% for diagnosing SCDS and 94% for predicting choice for surgical repair.
Conclusions: Low frequency CHL and increased oVEMP amplitude are the strongest predictive factors for making a diagnosis of SCDS and for choosing surgical repair.

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From Galen to Eustachio: Discovering the Anatomy of the Facial Nerve

Maoz, Sabrina Leah Levy; Canalis, Rinaldo Fernando

Publication date 01-10-2021


Objective: To discuss the historical relevance of the original illustrations and descriptions of the facial nerve during the Italian Renaissance (ca. late 14th to early 17th centuries).
Material and Methods: Graphic and textual information related to facial nerve discoveries were harvested from the works of the study periods anatomists, with special attention to those of Leonardo da Vinci, Andreas Vesalius, Gabrielle Falloppio, and Bartolomeo Eustachio. The importance of Galens anatomical works as a guide to these discoveries is discussed.
Conclusion: The reviewed texts and the illustrations therein contained provided sufficient information to establish a sequence of discoveries that resulted in a near-modern knowledge of the anatomy of the facial nerve and the basis to comprehend its function.

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Postinfective Modiolus Erosion after Cochlear Implantation

Bertoli, Giulia; Guida, Maurizio; Falcioni, Maurizio

Publication date 01-10-2021


No abstract available

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A Rare Case of Myopericytoma in the Mastoid

Sawaf, Tuleen; Wasman, Jay; Mowry, Sarah E.

Publication date 01-10-2021


No abstract available

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Leiomyoma of the External Auditory Channel: Unusual Symptomatology

Rossetti, Valeria; Pace, Annalisa; Corsi, Alessandro; Magliulo, Giuseppe

Publication date 01-10-2021


No abstract available

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Cadaveric Feasibility Study of Exoscope-Assisted Transcanal Transpromontorial Approach to the Internal Auditory Canal

Kaul, Vivian F.; Schwam, Zachary G.; Filip, Peter; Laitman, Jeffrey; Wanna, George B.

Publication date 01-10-2021


No abstract available

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