On the Difficulty Predicting Word Recognition Performance After Cochlear Implantation.

HYPOTHESIS: Preimplantation word scores cannot reliably predict postimplantation outcomes. BACKGROUND: To date, there is no model based on preoperative data that can reliably predict the postoperative outcomes of cochlear implantation in the postlingually deafened adult patient. METHODS: In a group of 228 patients who received a cochlear implant between 2002 and 2021, we tested the predictive power of nine variables (age, etiology, sex, laterality of implantation, preimplantation thresholds and word scores, as well as the design, insertion approach, and angular insertion depth of the electrode array) on postimplantation outcomes. Results of multivariable linear regression analyses were then interpreted in light of data obtained from histopathological analyses of human temporal bones. RESULTS: Age and etiology were the only significant predictors of postimplantation outcomes. In agreement with many investigations, preimplantation word scores failed to significantly predict postimplantation outcomes. Analysis of temporal bone histopathology suggests that neuronal survival must fall below 40% before word scores in quiet begin to drop. Scores fall steeply with further neurodegeneration, such that only 20% survival can support acoustically driven word scores of 50%. Because almost all cochlear implant implantees have at least 20% of their spiral ganglion neurons (SGNs) surviving, it is expected that most cochlear implant users on average should improve to at least 50% word recognition score, as we observed, even if their preimplantation score was near zero as a result of widespread hair cell damage and the fact that ~50% of their SGNs have likely lost their peripheral axons. These "disconnected" SGNs would not contribute to acoustic hearing but likely remain electrically excitable. CONCLUSION: The relationship between preimplantation word scores and data describing the survival of SGNs in humans can explain why preimplantation word scores obtained in unaided conditions fail to predict postimplantation outcomes.

Diagnostic Yield of Patients Undergoing Evaluation of Possible Superior Canal Dehiscence.

OBJECTIVE: To characterize the diagnostic yield of patients undergoing evaluation for superior canal dehiscence syndrome (SCDS), and identify alternative conditions diagnosed in patients suspected of, but not ultimately diagnosed with, SCDS. METHODS: Diagnostically undifferentiated adult patients suspected of having SCDS were identified between 2016 and 2021 at a tertiary academic medical system. Patients were categorized by diagnostic testing, radiographic superior semicircular canal (SSC) abnormality, symptoms, evaluating clinician specialty, operative intervention, and diagnosis. Differences among groups were assessed for statistical significance. RESULTS: Of 1242 candidate patients, 477 met inclusion criteria-evaluation by a clinician with SCDS on their differential diagnosis prior to diagnostic imaging. The mean (SD) age was 53.0 (15.0) years and 70.6% were female. A total of 364 patients underwent subsequent diagnostic imaging, and among these, 164 (45.1%) had a radiographic SSC abnormality with 99 (27.2%) receiving a diagnosis of SCDS (two cases of "near dehiscence syndrome"). One third (33.3%) of patients with SCDS underwent operative repair. Most clinicians with the initial suspicion for SCDS were otolaryngologists (90.6%), who had greater diagnostic yield than clinicians from other specialties (22.2% vs. 6.7%, p = 0.012). Patients not diagnosed with SCDS alternatively received 21 unique diagnoses and 52.1% (138/265) were not definitively diagnosed with any condition. CONCLUSIONS: This study characterizes the diagnostic incidence, or yield, of newly identified radiographic SSC abnormalities (45.1%) and SCDS (27.2%) among people suspected of having SCDS. Considerable overlap in presentation between SCDS and other conditions exists, and there is need for improvement in efficiently diagnosing patients with SCDS and audio-vestibular complaints in general. LEVEL OF EVIDENCE: III Laryngoscope, 2024.

Surgical Navigation in the Anterior Skull Base Using 3-Dimensional Endoscopy and Surface Reconstruction.

Importance: Image guidance is an important adjunct for endoscopic sinus and skull base surgery. However, current systems require bulky external tracking equipment, and their use can interrupt efficient surgical workflow. Objective: To evaluate a trackerless surgical navigation system using 3-dimensional (3D) endoscopy and simultaneous localization and mapping (SLAM) algorithms in the anterior skull base. Design, Setting, and Participants: This interventional deceased donor cohort study and retrospective clinical case study was conducted at a tertiary academic medical center with human deceased donor specimens and a patient with anterior skull base pathology. Exposures: Participants underwent endoscopic endonasal transsphenoidal dissection and surface model reconstruction from stereoscopic video with registration to volumetric models segmented from computed tomography (CT) and magnetic resonance imaging. Main Outcomes and Measures: To assess the fidelity of surface model reconstruction and accuracy of surgical navigation and surface-CT model coregistration, 3 metrics were calculated: reconstruction error, registration error, and localization error. Results: In deceased donor models (n = 9), high-fidelity surface models of the posterior wall of the sphenoid sinus were reconstructed from stereoscopic video and coregistered to corresponding volumetric CT models. The mean (SD; range) reconstruction, registration, and localization errors were 0.60 (0.24; 0.36-0.93), 1.11 (0.49; 0.71-1.56) and 1.01 (0.17; 0.78-1.25) mm, respectively. In a clinical case study of a patient who underwent a 3D endoscopic endonasal transsphenoidal resection of a tubercular meningioma, a high-fidelity surface model of the posterior wall of the sphenoid was reconstructed from intraoperative stereoscopic video and coregistered to a volumetric preoperative fused CT magnetic resonance imaging model with a root-mean-square error of 1.38 mm. Conclusions and Relevance: The results of this study suggest that SLAM algorithm-based endoscopic endonasal surgery navigation is a novel, accurate, and trackerless approach to surgical navigation that uses 3D endoscopy and SLAM-based algorithms in lieu of conventional optical or electromagnetic tracking. While multiple challenges remain before clinical readiness, a SLAM algorithm-based endoscopic endonasal surgery navigation system has the potential to improve surgical efficiency, economy of motion, and safety.

An External Validation Study for Automated Segmentation of Vestibular Schwannoma.

OBJECTIVE: To validate how an automated model for vestibular schwannoma (VS) segmentation developed on an external homogeneous dataset performs when applied to internal heterogeneous data. PATIENTS: The external dataset comprised 242 patients with previously untreated, sporadic unilateral VS undergoing Gamma Knife radiosurgery, with homogeneous magnetic resonance imaging (MRI) scans. The internal dataset comprised 10 patients from our institution, with heterogeneous MRI scans. INTERVENTIONS: An automated VS segmentation model was developed on the external dataset. The model was tested on the internal dataset. MAIN OUTCOME MEASURE: Dice score, which measures agreement between ground truth and predicted segmentations. RESULTS: When applied to the internal patient scans, the automated model achieved a mean Dice score of 61% across all 10 images. There were three tumors that were not detected. These tumors were 0.01 ml on average (SD = 0.00 ml). The mean Dice score for the seven tumors that were detected was 87% (SD = 14%). There was one outlier with Dice of 55%-on further review of this scan, it was discovered that hyperintense petrous bone had been included in the tumor segmentation. CONCLUSIONS: We show that an automated segmentation model developed using a restrictive set of siloed institutional data can be successfully adapted for data from different imaging systems and patient populations. This is an important step toward the validation of automated VS segmentation. However, there are significant shortcomings that likely reflect limitations of the data used to train the model. Further validation is needed to make automated segmentation for VS generalizable.

Auditory and Vestibular Symptom Improvement With Surgery for Superior Canal Dehiscence Syndrome.

OBJECTIVE: (1) To measure the change in auditory and vestibular symptoms following superior canal dehiscence (SCD) surgery, and (2) to determine differences in clinical features and surgical outcomes between superior canal dehiscence syndrome (SCDS) patients with primarily auditory or vestibular complaints. STUDY DESIGN: Retrospective cohort study. SETTING: Single surgeon series at the tertiary academic medical center from 2002 to 2021. METHODS: Retrospective review of SCDS patients who underwent surgical repair. (1) Patients were administered a standardized symptom questionnaire at preoperative and follow-up visits, and results were compared with paired statistical testing. (2) Patients were divided into 2 cohorts based on either auditory or vestibular chief complaint and differences in demographic, clinical, and outcome variables were examined. RESULTS: Our study included 113 patients with 118 operated ears. Twenty-seven patients (24%) had radiographic bilateral dehiscence. 10/11 auditory symptoms (91%) and 5/8 vestibular symptoms (63%) solicited on the questionnaire improved significantly with surgery, except for nonpulsatile tinnitus, sense of imbalance, positional dizziness, and oscillopsia. Analyses stratified by chief complaint (auditory vs vestibular) revealed overall similar characteristics and surgical outcomes. Patients with chief vestibular complaints underwent surgery at an earlier age (45.5 vs 53.9 years, p < 0.05). CONCLUSION: SCD surgery alleviates a wide range of auditory and vestibular symptoms. Overall, we did not find significant differences between patients with chief auditory versus vestibular complaints, and both groups benefited from surgery. Symptoms are not directly linked to third-window physiology and certain vestibular symptoms may be more likely to persist. Bilateral dehiscence may play an important role in persistent symptoms as well.

Otolaryngology Training in Uganda: The Mbarara University of Science and Technology Experience.

Sub-Saharan Africa has a high otolaryngologic disease burden exacerbated by an inadequate number of otolaryngologists. The Otolaryngology department at Mbarara University of Science & Technology in Uganda is addressing this problem by having created Uganda's second national residency training program in 2010. We chronicled an early period in the program's development by reporting surgical case quantity and complexity, as defined by "key indicator procedure" classification per the United States Accreditation Council for Graduate Medical Education, and interpreting it with respect to a timeline of significant events. Procedure complexity, but not total number per year, increased over the study period-KIPs increased from 3% in 2012 (6 of 175 total procedures) to 29% in 2016 (35 of 135 total procedures). During this period of complexity increase, operating room capacity expanded, faculty received advanced training and increased in number, and operative equipment improved.

Making Use of Artificial Intelligence-Generated Synthetic Tympanic Membrane Images.

This diagnostic study examines the application of generative artificial intelligence in clinical tool research and development.

Hospital-Negotiated Pricing of Cochlear Implants.

Access to cochlear implantation can be restricted by financial burden, and little is known about the extent to which cochlear implant (CI) devices prices may vary between hospitals or manufacturers. We performed a cross-sectional analysis of private payer-negotiated prices for CI devices. In total, 161 hospitals were analyzed. Prices varied widely across hospitals and between payers. Despite clinical equipoise between manufacturers with regards to CI user outcomes, significant differences in prices were identified, with higher prices for MED-EL ($38,478 ± 2633) than for Cochlear Ltd. ($34,150 ± 2418, p < .001). Markup analysis for Cochlear Ltd. devices revealed a mean 58.5% ± 7.4% markup in excess of the company's average sales price ($24,649). Negotiated prices were also at least $5000 lower when all 3 brands were offered at the same center (p < .05). Further research examining the influence of patient outcomes and hospital volume on prices are needed.

Generation of synthetic tympanic membrane images: Development, human validation, and clinical implications of synthetic data.

Synthetic clinical images could augment real medical image datasets, a novel approach in otolaryngology-head and neck surgery (OHNS). Our objective was to develop a generative adversarial network (GAN) for tympanic membrane images and to validate the quality of synthetic images with human reviewers. Our model was developed using a state-of-the-art GAN architecture, StyleGAN2-ADA. The network was trained on intraoperative high-definition (HD) endoscopic images of tympanic membranes collected from pediatric patients undergoing myringotomy with possible tympanostomy tube placement. A human validation survey was administered to a cohort of OHNS and pediatrics trainees at our institution. The primary measure of model quality was the Frechet Inception Distance (FID), a metric comparing the distribution of generated images with the distribution of real images. The measures used for human reviewer validation were the sensitivity, specificity, and area under the curve (AUC) for humans' ability to discern synthetic from real images. Our dataset comprised 202 images. The best GAN was trained at 512x512 image resolution with a FID of 47.0. The progression of images through training showed stepwise "learning" of the anatomic features of a tympanic membrane. The validation survey was taken by 65 persons who reviewed 925 images. Human reviewers demonstrated a sensitivity of 66%, specificity of 73%, and AUC of 0.69 for the detection of synthetic images. In summary, we successfully developed a GAN to produce synthetic tympanic membrane images and validated this with human reviewers. These images could be used to bolster real datasets with various pathologies and develop more robust deep learning models such as those used for diagnostic predictions from otoscopic images. However, caution should be exercised with the use of synthetic data given issues regarding data diversity and performance validation. Any model trained using synthetic data will require robust external validation to ensure validity and generalizability.

Lower Eyelid Sling for Primary and Revision Correction of Paralytic Lagophthalmos.

Background: Lateral tarsal techniques alone for lower eyelid correction in paralytic lagophthalmos may yield suboptimal outcomes. Objective: To describe a lower eyelid sling technique for primary and revision correction of lower eyelid ptosis and ectropion and evaluate outcomes as measured by margin reflex distance 2 (MRD2). Methods: A retrospective review of patients with long-standing unilateral paralytic lagophthalmos who underwent primary or revision lower eyelid ptosis correction by sling suspension between January 2016 and August 2020 at a tertiary medical center was performed. Surgical technique is illustrated with video and technical considerations are discussed. Pre- and postoperative MRD2 values were quantified from databased photographs. Results: Thirty-eight patients were included. Eighteen patients had undergone prior procedures for ptosis correction. Lower eyelid symmetry and paralyzed side MRD2 significantly improved after lower lid sling for primary and revision cases (p < 0.05), and improvement was sustained over the study period (mean follow-up duration 13.3 months, range 1-33 months). No postoperative complications occurred. Conclusion: Lower eyelid sling yielded safe, effective, and durable correction of lower eyelid position in a cohort of patients with paralytic lagophthalmos.

A Rare Complication of Chronic Otitis Media: Central Skull Base Osteomyelitis Managed With Combined Endoscopic Transmastoid and Transsphenoidal Debridement.

OBJECTIVE: This report describes a case of otogenic central skull base osteomyelitis (CSBO) requiring complex surgical intervention and reviews the literature on management of this entity. PATIENT: A 76-year-old man presented with a nearly 20-year history of chronic otomastoiditis and cholesteatoma with ultimate progression to severe CSBO with involvement of the petrous apex, clivus, and craniocervical junction. INTERVENTIONS: CSBO was managed with culture-directed antibiotic therapy, hyperbaric oxygen, and surgical intervention including serial combined endoscopic transmastoid and transsphenoidal debridements. MAIN OUTCOME MEASURES: Symptom resolution, antibiotic holiday, and stable disease on surveillance imaging. RESULTS: With antibiotic treatment and surgical debridement including creation of a drainage pathway from the skull base to the sphenoid sinus, intermittent stretches of disease quiescence were realized over the course of nearly a decade. Despite extensive debridement and skull base reconstruction, the patient ultimately succumbed to the disease process. CONCLUSIONS: CSBO poses significant management challenges to the otologist. Herein, we present a rare case of CSBO managed over a prolonged period of time with antibiotics and combined anterior and lateral skull base debridement.

Hypothalamic-Extended Amygdala Circuit Regulates Temporal Discounting.

Choice behavior is characterized by temporal discounting, i.e., preference for immediate rewards given a choice between immediate and delayed rewards. Agouti-related peptide (AgRP)-expressing neurons located in the arcuate nucleus of the hypothalamus (ARC) regulate food intake and energy homeostasis, yet whether AgRP neurons influence choice behavior and temporal discounting is unknown. Here, we demonstrate that motivational state potently modulates temporal discounting. Hungry mice (both male and female) strongly preferred immediate food rewards, yet sated mice were largely indifferent to reward delay. More importantly, selective optogenetic activation of AgRP-expressing neurons or their axon terminals within the posterior bed nucleus of stria terminalis (BNST) produced temporal discounting in sated mice. Furthermore, activation of neuropeptide Y (NPY) type 1 receptors (Y1Rs) within the BNST is sufficient to produce temporal discounting. These results demonstrate a profound influence of hypothalamic signaling on temporal discounting for food rewards and reveal a novel circuit that determine choice behavior.SIGNIFICANCE STATEMENT Temporal discounting is a universal phenomenon found in many species, yet the underlying neurocircuit mechanisms are still poorly understood. Our results revealed a novel neural pathway from agouti-related peptide (AgRP) neurons in the hypothalamus to the bed nucleus of stria terminalis (BNST) that regulates temporal discounting in decision-making.

Long-term cochlear implantation outcomes in patients following head injury.

OBJECTIVE: In cases of a severe to profound sensorineural hearing loss following head injury, the cochlear implant (CI) is the primary option for auditory rehabilitation. Few studies, however, have investigated long-term CI outcomes in patients following head trauma, including those without temporal bone fracture (TBF). Herein, the aim of this study is to examine CI outcomes following cases of head injury with and without TBF. METHODS: Audiometric outcomes of patients who received a CI due to a head injury resulting in severe to profound hearing loss at two tertiary care hospitals were analyzed. Patients were divided into those who received a CI in a fractured temporal bone (group A, n = 11 patients corresponding to 15 ears) and those who received a CI in a non-fractured temporal bone (group B, n = 8 patients corresponding to nine ears). Primary outcomes included duration of deafness prior to CI and postoperative consonant-nucleus-constant whole word (CNC) scores. RESULTS: Nineteen patients (84% male), corresponding to 24 CIs, were identified. Fifteen CI were performed on ears with TBF (group A), and nine CI were performed on ears without TBF (group B). No patients had an enlarged vestibular aqueduct (EVA). The mean duration of deafness was 5.7 and 11.3 years in group A and group B, respectively. The mean duration of CI follow-up (CI experience) was 6.5 years in group A and 2.1 years in group B. The overall mean postoperative CNC score for all subjects was 68.6% (±21.2%, n = 19 with CNC testing). There was no difference in CNC score between group A and group B (69.8% and 66% respectively, P = .639). CONCLUSION: The study is among the largest series examining long-term outcomes of CI after head injury. CI is an effective method for auditory rehabilitation in patients after head injury. LEVEL OF EVIDENCE: IV.

Labyrinthine concussion: Historic otopathologic antecedents of a challenging diagnosis.

OBJECTIVE: The term "labyrinthine concussion" has evolved to mean audiovestibular dysfunction in the absence of a temporal bone fracture (TBF). Despite a multitude of case descriptions of labyrinthine concussion, the precise pathophysiology remains poorly understood. Herein, we explore the historical otopathologic underpinnings of the diagnosis of labyrinthine concussion with a focus on the auditory pathway during the late 19th to the mid-20th centuries and conclude with a discussion of its contemporary relevance. METHODS AND DATA SOURCES: A review of primary and secondary medical sources written in English, German, and French on otopathology labyrinthine concussion studies from the late-19th to the mid-20th centuries. RESULTS: Around the turn of the 20th century, otopathologists identified histologic changes in the temporal bones of individuals that sustained head injury without TBFs. Based on these otopathologic findings in humans, early experiments investigating the pathophysiology of labyrinthine concussion were performed in animals through either the delivery of blows to the head or direct introduction of a pressure wave into the labyrinthine fluid. Collectively, otopathologists hypothesized that predominant mechanisms for labyrinthine concussion included inner ear hemorrhage, cochleovestibular nerve traction injury, direct damage from a labyrinthine fluid pressure wave, or vasomotor dysfunction. CONCLUSION: Historical study shows a variety of inner ear pathologies potentially responsible for auditory dysfunction following head injury. Understanding the history and otopathology of labyrinthine concussion may help clinicians focus on new pathways toward novel research and improved patient care.

Prevalence and significance of cranial nerve imaging abnormalities in patients with hereditary neuropathies: Clinical implications at the skull base.

OBJECTIVE: To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications. METHODS: We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy diagnoses who had undergone gadolinium-enhanced magnetic resonance imaging (MRI) of the brain or skull base between 2004 and 2018. MRI scans, as well as computed tomography imaging when available, were reviewed and bivariable analysis was performed to identify predictors of CN abnormalities on imaging. RESULTS: Among 39 patients meeting study criteria, 11 had clinical CN deficits (28%) and 8 had CN abnormalities on imaging (21%). Of the patients with CN abnormalities on imaging, half had CN deficits (4/8) and only a quarter had imaging abnormalities of the CNs with the deficits (2/8). Imaging abnormalities were found in varied CNs, including CNs III, V, VII, and the VII/VIII complex in the internal auditory canal. MRI obtained for the purpose of evaluating CN deficits had a statistically significant increased likelihood of containing CN imaging abnormalities. However, CN deficits themselves were not predictive of imaging abnormalities. CONCLUSION: Thickening and enhancement of CNs on MRI may be found in approximately 1/5 of patients with hereditary neuropathies and are inconsistently associated with clinical deficits. These imaging findings should not be mistaken for neoplastic and infectious processes as they may be manifestations of the patients' underlying genetic neuropathy. LEVEL OF EVIDENCE: 4.

Iatrogenic Inner Ear Dehiscence After Lateral Skull Base Surgery: Therapeutic Dilemma and Treatment Options.

OBJECTIVE: To describe a series of patients with iatrogenic inner ear dehiscence after lateral skull base surgery and discuss treatment options. PATIENTS: Three patients with history of recent lateral skull base surgery, symptoms consistent with inner ear dehiscence, and radiographically proven bony dehiscence on postoperative imaging. INTERVENTIONS: All patients were initially managed conservatively with serial outpatient visits. Two patients with large cochlear and vestibular dehiscences had round window reinforcement procedures. One patient had transmastoid resurfacing for repair of an iatrogenic posterior semicircular canal dehiscence. MAIN OUTCOME MEASURES: Anatomical location of dehiscences; treatment options; subjective auditory and vestibular symptoms pre-dehiscence, post-dehiscence and after dehiscence repair; pre- and post-audiogram when available. RESULTS: Patient ages were 46, 52, and 60 with two of three being women. None of the patients had subjective auditory or vestibular symptoms of inner ear dehiscence before initial skull base surgery, but they all had development of these symptoms afterwards. All patients were initially managed conservatively, but all ultimately required a surgical procedure. The two patients who elected for round window reinforcements, and the one patient who required transmastoid resurfacing, had significant improvement of symptoms. CONCLUSIONS: Iatrogenic inner ear dehiscence after skull base surgery is best dealt with and repaired intraoperatively. Should intraoperative repair not be possible, transcanal round window reinforcement is a minimally invasive option for medial otic capsule dehiscence, although long-term outcomes are unclear. For lateral otic capsule dehiscence, a transmastoid approach is recommended. LEVEL OF EVIDENCE: IV.

Opioid prescription patterns and use among patients undergoing endoscopic sinus surgery.

OBJECTIVES/HYPOTHESIS: Opioid-related deaths in the United States have increased 200% since 2000, in part due to prescription diversion from patients who had a surgical procedure. The purpose of this study was to characterize provider prescription patterns and assess patient-reported opioid use after endoscopic sinus surgery (ESS). STUDY DESIGN: Retrospective chart review. METHODS: Patients who underwent ESS between May 2017 and May 2018 were included. Opioid prescription, operative details, and postoperative opioid use data were extracted. The Massachusetts Prescription Awareness Tool (MassPAT) was queried to determine if patients filled their prescription. RESULTS: One hundred fifty-five patients were included. Nearly all patients received an opioid prescription (94.8%). An average of 15.6 tablets was prescribed per patient. Among 116 patients with MassPAT data, 91.4% filled their prescription. Among 67 patients who reported the number of tablets they had used at the time of first follow-up appointment, 73.1% reported taking no opioids. Mean number of tablets prescribed was significantly greater among patients who underwent primary versus revision surgery (16.5 vs. 13.5, P = .0111) and those who had splints placed (21.5 vs. 15.1, P = .0037). Predictors of opioid use included concurrent turbinate reduction (58.3% vs. 14.3%, P < .0001) and concurrent septoplasty (45.5% vs. 21.6%, P = .039). CONCLUSIONS: Nearly all patients who underwent ESS were prescribed an opioid, and nearly all patients filled their prescription. However, the vast majority of patients did not require any opioid medication for postoperative pain control. As the opioid epidemic continues to persist, these findings have immediate relevance to current prescribing patterns and pain management practices. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1046-1052, 2019.