Otology & Neurotology最新文献

Hypothesis: Patients with sporadic vestibular schwannoma (VS) have unique plasma protein biomarkers that distinguish them from patients without VS.

Background: No reliable molecular biomarker of VS exists. MRI biomarkers offer limited insight into VS pathophysiology. Identification of plasma biomarkers could enhance disease prognostication and guide treatment decisions.

Methods: A high-throughput DNA aptamer-based proteomic analysis was performed in plasma samples from 12 individuals, 6 with sporadic, non-irradiated and growing VS and 6 age-matched and sex-matched healthy controls (HCs). Dysregulated proteins were identified using a cutoff value of |log 2 foldchange|>1 and padj <0.05. Enriched pathways were determined using Ingenuity Pathway and STRING bioinformatic analysis. Biomarker expression was validated in an established human schwannoma cell line and primary VS culture.

Results: A total of 7310 proteins were profiled. Of 1499 differentially expressed proteins, 152 (10%) were upregulated and 264 (18%) were downregulated in VS. There was an enrichment in cancer proliferation, protein catabolism, and immune cell activation processes. A panel of 40 proteins distinguished VS from HC, accounting for 84% of the variance on principal component analysis. These included members of NF-κB and Wnt signaling pathways (NFKBIA, WNT10A, and WNT16). IGFBP-2 and FCGR3A mRNA expression were significantly elevated in schwannoma cells. Hepcidin (HAMP), a regulator of iron homeostasis that influences tumor growth, was highly expressed in human VS tissue and enriched in primary VS culture secretion.

Conclusions: Proteomic analysis of VS patient plasma identified several disease-classifying biomarkers. Hepcidin warrants further investigation into its role in VS progression.

Objective: This study aimed to investigate the effect of mastoid cavity aeration on recurrent cholesteatoma after canal wall up tympanoplasty with mastoidectomy in patients with cholesteatoma.

Study design: Retrospective study.

Setting: University hospital.

Methods: We analyzed 62 patients who met specific criteria and underwent initial canal wall up tympanoplasty with mastoidectomy for cholesteatoma. Postoperative mastoid cavity aeration was assessed ~1 year after surgery using computed tomography. The relationship between mastoid cavity aeration 1 year after surgery and recurrent cholesteatoma within 3 years post-surgery was explored.

Results: Approximately 1 year post-surgery, 38.7% of the patients showed improved mastoid cavity aeration. The incidence of recurrent cholesteatoma after >3 years was 34.5% in cases without mastoid cavity aeration and 11.1% in cases with aeration.

Conclusions: These findings suggest that the absence of postoperative mastoid cavity aeration is associated with a higher risk of recurrent cholesteatoma. Therefore, the aeration status of the mastoid cavity at 1 year postoperatively seems to be a useful marker for determining surveillance schedule.

Introduction: Cochlear implantation (CI) has been shown to be feasible and beneficial in patients with inner ear malformations (IEMs); however, incomplete partition type III (IP-III) malformations are rarely considered. In this scoping review, we evaluate the benefit of CI in patients with the relatively rare IP-III malformation.

Methods: A review in accordance with PRISMA was performed. A search of MEDLINE, EMBASE, Cochrane CENTRAL, CINAHL, and Web of Science was performed. Cohort studies, case studies, and conference abstracts evaluating complications or outcomes of CI in patients with confirmed IP-III malformation were included.

Results: Our search identified a total of 40 studies after screening 1060 articles. Twenty-five studies (62.5%) reported CSF gusher as an intraoperative complication for a total of 120/122 patients (98.4%) in these studies. The most reported audiologic outcomes were speech perception testing with 10 studies, Categories of Auditory Performance (CAP) scores with 9 studies, and Speech Intelligibility Rating (SIR) with 8 studies. All 4 studies that directly compared IP-III malformation to other malformations using these outcome measures concluded that performance was statistically similar to at least one other malformation (100%). When compared with children with normal ears, only one-sixth of studies (16.7%) reported statistically similar results in IP-III malformation.

Conclusion: CSF gusher is an expected intraoperative complication of CI in patients with IP-III. Expectations regarding performance with CI in the setting of IP-III malformations should be tempered. Nonetheless, CI remains a viable option in patients with IP-III malformations with auditory outcomes comparable to those seen in other IEMs.

Objective: This study evaluates the surgical and audiological outcomes of transcanal endoscopic ear surgery (TEES) in patients with congenital ossicular chain anomalies (COCAs) using the Teunissen-Cremers classification system.

Study design: Multicenter, retrospective clinical study.

Setting: Tertiary referral centers with experienced endoscopic ear surgeons.

Patients: A total of 51 patients were diagnosed with COCAs and treated with TEES. Patients with chronic otitis media, cholesteatoma, tympanosclerosis, otosclerosis, or those requiring microscopic/endaural/retroauricular approaches were excluded.

Intervention: Transcanal endoscopic ossicular chain reconstructions were performed using various techniques, including stapedotomy, partial and total ossicular prosthesis or autologous bone or cartilage graft placements, and bone cement bridging.

Main outcome measures: Air-bone gap (ABG) closure, operation duration, hospital stay, and postoperative complications.

Results: The mean preoperative ABG was 42.5±10.9 dB HL, which improved to a mean postoperative ABG of 20.3±12.3 dB HL, yielding a mean ABG closure of 22.3±12.2 dB HL. Patients classified as Class 1, 2, and 3 demonstrated significantly greater ABG closure rates compared with Class 4 cases ( P <0.059). The mean surgical duration was 72.1±19.9 minutes, and the mean hospital stay was 29.6±15.3 hours. No intraoperative or immediate postoperative complications were observed. Five patients required revision surgery due to recurrent conductive hearing loss.

Conclusions: TEES is a safe and effective technique for managing COCAs, offering significant ABG improvement, particularly in Class 1 to 3 anomalies. The minimally invasive nature of TEES, combined with superior exposure and visualization, results in favorable audiological outcomes with minimal complications and reduced hospital stay.

Background: Foreign bodies (FBs) in the external auditory canal (EAC) are common in children but rarely cause cranial neuropathies. We report the first documented case of vagal and glossopharyngeal nerve palsies secondary to a migratory ear FB penetrating the jugular foramen.

Case presentation: A 2.5-year-old boy presented with a 50-day history of right otalgia, bloody otorrhea, and new-onset dysphonia with aspiration. Initial workup revealed granulation tissue and methicillin-resistant Staphylococcus aureus (MRSA) infection, refractory to antibiotics. Flexible endoscopy demonstrated right vocal cord paralysis as well as diminished pharyngeal sensation and paralysis, suggesting cranial nerve (CN) IX and X dysfunction. Temporal bone imaging initially overlooked a 22 mm glass fragment embedded in the hypotympanum, extending into the jugular foramen. Multidisciplinary review identified the FB, previously misclassified as an artifact. Surgical extraction, performed through mastoidectomy through a post-auricular incision, led to symptom resolution, and confirmed neuropraxia secondary to FB-induced neuritis.

Conclusion: This case highlights a novel mechanism of cranial neuropathy, direct FB migration to the skull base, and underscores the importance of reevaluating imaging in refractory otologic cases. Clinicians should suspect occult FBs in pediatric patients with persistent ear bleeding and cranial nerve deficits, even without a clear history of insertion.

Objective: Automated segmentation models for volumetric measurement of vestibular schwannoma (VS) have been developed for sporadic VS but not for bilateral VS. Automated segmentation would be especially valuable in this setting: patients with neurofibromatosis 2 (NF2) undergo numerous MRI scans, and automated analyses would aid in timely therapeutic decision-making. We developed a computer vision model for the volumetric measurement of bilateral VS.

Patients: Eighty-seven individuals with VS (59 sporadic, 28 NF2) from our institution; 30 patients with sporadic VS from an open-source data set.

Interventions: A nnU-Net was trained on our institutional data augmented with the public data to develop an automated segmentation model for VS on T1-post contrast MRI. The model was tested on a holdout set of sporadic and bilateral VS scans.

Main outcome measures: Dice score to compare pixel-wise agreement, qualitative review.

Results: Median tumor volumes were 0.345 cc for institutional sporadic VS, 2.05 cc and 0.501 cc for the larger and smaller tumors for institutional bilateral VS, and 1.36 cc for the public sporadic VS. There was a high incidence of comorbid intracranial pathology in the NF2 cases, including 39% with meningiomas and 32% with trigeminal schwannomas. The final model achieved a mean Dice score of 0.94 on the internal sporadic VS holdout set, 0.95 on public sporadic VS, and 0.87 on bilateral VS. On qualitative review of the NF2 cases, the model distinguished VS from adjacent non-VS lesions. The model also detected small tumors in cases with significant size asymmetry. The model struggled in cases with noncontiguous segments of tumor, often including one segment but not the other.

Conclusions: This study is the first to report on the automated segmentation of bilateral VS in NF2. Further work is necessary to improve model performance, extend it to the postoperative setting, and apply it to other intracranial tumors.

Objectives: The otologic conditions associated with antiphospholipid syndrome (APS) have yet to be fully categorized. The objective of this study is to investigate the prevalence of otologic diagnoses in both adult-onset and pediatric-onset APS.

Methods: The TriNetX US Collaborative network was utilized to perform a retrospective cohort study. The adult-onset and pediatric-onset APS cohorts included patients with APS diagnosed in adulthood or before 18 years, respectively. Matched comparison cohorts for the general adult and pediatric populations included patients without APS. Outcomes of interest included prevalence of sensorineural hearing loss (SNHL), central and peripheral vertigo, tinnitus, and Ménière's disease.

Results: The adult-onset APS cohort (n=25,981) had a higher risk of SNHL [relative risk (RR): 1.4, 95% CI: 1.4-1.5], central vertigo (RR: 1.9, 95% CI: 1.4-2.7), peripheral vertigo (RR: 1.2, 95% CI: 1.1-1.3), tinnitus (RR: 1.2, 95% CI: 1.1-1.2), and Ménière's disease (RR: 1.8, 95% CI: 1.4-2.3) compared with the matched general adult cohort (n=25,981). The pediatric-onset APS cohort (n=2409) had a higher risk of bilateral sensorineural hearing loss (RR: ≥2.4, 95% CI: 1.2-5.0) compared with the matched general pediatric cohort (n=2409).

Conclusions: Both adult-onset and pediatric-onset APS had a greater prevalence of various otologic diagnoses compared with the general population. More research is needed to evaluate unique patient factors that may contribute to the development of these conditions.

Level of evidence: III.

Objective: To optimize antibiotic regimens in necrotizing otitis externa (NOE) and skull base osteomyelitis (SBO) and evaluate the role of positron emission tomography-computed tomography (PET-CT) in disease monitoring.

Study design: A 2-cycle quality improvement project comparing traditional imaging (CT/MRI) with PET-CT-guided management.

Setting: Tertiary referral center.

Patients: Patients with NOE were retrospectively reviewed in the CT/MRI cohort (2015 to 2018) and prospectively assessed in the PET-CT cohort (2022 to 2024). Nineteen patients were included in the first cycle, and 14 in the second.

Interventions: A revised management pathway incorporating PET-CT for treatment response assessment was implemented. Oral ciprofloxacin regimens were standardized in the PET-CT cohort, with a preference for higher-dose, shorter-duration therapy.

Main outcome measures: Antibiotic duration, relapse rates, and PET-CT efficacy in guiding treatment cessation.

Results: Patients in the PET-CT cohort received significantly shorter oral ciprofloxacin courses (median: 8 vs. 40 wk, P <0.001) without increased relapse rates. PET-CT confirmed resolution in 64% of cases, enabling antibiotic discontinuation. Relapses occurred in 21% of the CT/MRI cohort but were absent in the PET-CT group ( P =0.11). Higher ciprofloxacin doses (750 mg BID) were more frequently used in the PET-CT cohort ( P =0.002), yet this did not increase antibiotic-related complications ( P =1.00). Mortality rates remained comparable between cohorts (7% PET-CT vs. 11% CT/MRI). PET-CT provided superior real-time infection monitoring, preventing unnecessary prolonged antibiotic courses.

Conclusions: PET-CT effectively guided treatment cessation, reducing antibiotic exposure without compromising clinical outcomes. A higher-dose, shorter-duration ciprofloxacin regimen did not increase relapse rates, suggesting that a more intensive yet time-limited antimicrobial approach can be safely implemented. These findings support PET-CT as a valuable tool in NOE/SBO management, reinforcing its role in optimizing antibiotic stewardship.

Objective: To evaluate the ability to distinguish cholesteatoma from surrounding tissues using autofluorescence intraoperatively.

Study design: Prospective cohort study.

Setting: Tertiary referral center.

Patients: Patients with cholesteatoma undergoing middle ear surgery.

Intervention: Autofluorescence imaging with 470 nm excitation light.

Main outcome measure: Compare cholesteatoma autofluorescence with surrounding mucosa, bone, and granulation tissue.

Results: A total of 6 patients undergoing cholesteatoma surgery were prospectively enrolled in the study for imaging. A custom-designed endoscopic and macroscopic imaging set-up utilizing 470 nm excitation was used to image the surgical field in real-time. Intraoperatively, autofluorescence imaging allowed delineation of cholesteatoma from surrounding bone, granulation tissue, and mucosa, even when cholesteatoma identification was difficult with white light alone. The 470 nm excitation was able to target endogenous fluorophores, specifically flavin adenine dinucleotide (FAD) and keratin, which are present within cholesteatoma, but not present in mucosa and granulation tissue.

Conclusion: This study demonstrates the feasibility of autofluorescence-guided identification of cholesteatoma intraoperatively. The results support its potential as an adjunctive tool to surgeons for cholesteatoma identification.

Objective: To characterize patient outcomes after primary stereotactic radiosurgery (SRS) for the management of sporadic facial nerve schwannoma.

Study design: Retrospective cohort study.

Setting: Six tertiary referral centers across the United States and United Kingdom.

Patients: Adults undergoing SRS from 2000 through 2023 for sporadic facial nerve schwannoma along any segment of the facial nerve were included. Patients with NF2-related schwannomatosis were excluded.

Intervention: Stereotactic radiosurgery.

Main outcome measure: Long-term tumor control.

Results: Among 60 patients meeting inclusion, the median age at SRS was 52 years (IQR: 41 to 64) with a median tumor size of 19.5 mm (IQR: 14.7 to 22.8). Tumors commonly involved the internal auditory canal (73%), cisternal (49%), geniculate/labyrinthine (47%), and tympanic segments (22%). Two patients experienced SRS failure and underwent salvage treatment; salvage-free survival rates (95% CI; number still at risk) at 1, 3, 5, and 10 years after SRS were 100% (100 to 100; 55), 100% (100 to 100; 36), 100% (100 to 100; 18), and 87% (72 to 100; 9), respectively. Among 31 (52%) patients with House-Brackmann (HB) grade I facial function at presentation, only 6 demonstrated worse facial function at a median of 3.2 years (IQR: 1.7 to 6.6) after SRS. Of 18 patients with serviceable hearing (AAO-HNS class A/B) at SRS, 13 maintained serviceable hearing at a median of 1.0 years (IQR: 0.5 to 4.9) of post-SRS audiometric follow-up.

Conclusions: Durable tumor control after primary SRS for sporadic facial nerve schwannoma is achieved in most patients. Among those with HB grade I facial function at presentation, treatment with SRS harbors limited additional risk of facial paresis beyond observation alone.