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Otolaryngology--head and Neck Surgery :... Jul 2024To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma.
OBJECTIVE
To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma.
STUDY DESIGN
Retrospective study.
SETTING
Tertiary general hospital.
METHODS
Twenty-two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow-up were included. Patients were categorized as "users" or "nonusers" of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip-reading was assessed in quiet conditions 1-year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (≥40% or <40%). Demographic data, treatment options, and tumor size were also recorded.
RESULTS
One year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility ≥40% (mean: 74 ± 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow-up: 6 ± 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility ≥40%. No predictive factors for good performance at 1 year or performance stability were identified.
CONCLUSION
Neurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case-by-case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors.
Topics: Humans; Neurofibromatosis 2; Male; Retrospective Studies; Female; Cochlear Implantation; Middle Aged; Adult; Neurofibromatoses; Speech Perception; Treatment Outcome; Skin Neoplasms; Aged; Neurilemmoma; Neuroma, Acoustic; Speech Intelligibility; Follow-Up Studies
PubMed: 38482961
DOI: 10.1002/ohn.702 -
British Journal of Cancer Jun 2024Vestibular schwannomas (VSs) remain a challenge due to their anatomical location and propensity to growth. Macrophages are present in VS but their roles in VS...
BACKGROUND
Vestibular schwannomas (VSs) remain a challenge due to their anatomical location and propensity to growth. Macrophages are present in VS but their roles in VS pathogenesis remains unknown.
OBJECTIVES
The objective was to assess phenotypic and functional profile of macrophages in VS with single-cell RNA sequencing (scRNAseq).
METHODS
scRNAseq was carried out in three VS samples to examine characteristics of macrophages in the tumour. RT-qPCR was carried out on 10 VS samples for CD14, CD68 and CD163 and a panel of macrophage-associated molecules.
RESULTS
scRNAseq revealed macrophages to be a major constituent of VS microenvironment with three distinct subclusters based on gene expression. The subclusters were also defined by expression of CD163, CD68 and IL-1β. AREG and PLAUR were expressed in the CD68+CD163+IL-1β+ subcluster, PLCG2 and NCKAP5 were expressed in CD68+CD163+IL-1β- subcluster and AUTS2 and SPP1 were expressed in the CD68+CD163-IL-1β+ subcluster. RT-qPCR showed expression of several macrophage markers in VS of which CD14, ALOX15, Interleukin-1β, INHBA and Colony Stimulating Factor-1R were found to have a high correlation with tumour volume.
CONCLUSIONS
Macrophages form an important component of VS stroma. scRNAseq reveals three distinct subsets of macrophages in the VS tissue which may have differing roles in the pathogenesis of VS.
Topics: Humans; Neuroma, Acoustic; Single-Cell Analysis; Macrophages; Sequence Analysis, RNA; Tumor Microenvironment; Female; Male; Middle Aged; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Interleukin-1beta
PubMed: 38480935
DOI: 10.1038/s41416-024-02646-2 -
Neuroradiology Jul 2024We aimed to evaluate the effect of deep learning-based reconstruction (DLR) on high-spatial-resolution three-dimensional T2-weighted fast asymmetric spin-echo (HR-3D...
Effect of deep learning-based reconstruction on high-resolution three-dimensional T2-weighted fast asymmetric spin-echo imaging in the preoperative evaluation of cerebellopontine angle tumors.
PURPOSE
We aimed to evaluate the effect of deep learning-based reconstruction (DLR) on high-spatial-resolution three-dimensional T2-weighted fast asymmetric spin-echo (HR-3D T2-FASE) imaging in the preoperative evaluation of cerebellopontine angle (CPA) tumors.
METHODS
This study included 13 consecutive patients who underwent preoperative HR-3D T2-FASE imaging using a 3 T MRI scanner. The reconstruction voxel size of HR-3D T2-FASE imaging was 0.23 × 0.23 × 0.5 mm. The contrast-to-noise ratios (CNRs) of the structures were compared between HR-3D T2-FASE images with and without DLR. The observers' preferences based on four categories on the tumor side on HR-3D T2-FASE images were evaluated. The facial nerve in relation to the tumor on HR-3D T2-FASE images was assessed with reference to intraoperative findings.
RESULTS
The mean CNR between the tumor and trigeminal nerve and between the cerebrospinal fluid and trigeminal nerve was significantly higher for DLR images than non-DLR-based images (14.3 ± 8.9 vs. 12.0 ± 7.6, and 66.4 ± 12.0 vs. 53.9 ± 8.5, P < 0.001, respectively). The observer's preference for the depiction and delineation of the tumor, cranial nerves, vessels, and location relation on DLR HR-3D T2FASE images was superior to that on non-DLR HR-3D T2FASE images in 7 (54%), 6 (46%), 6 (46%), and 6 (46%) of 13 cases, respectively. The facial nerves around the tumor on HR-3D T2-FASE images were visualized accurately in five (38%) cases with DLR and in four (31%) without DLR.
CONCLUSION
DLR HR-3D T2-FASE imaging is useful for the preoperative assessment of CPA tumors.
Topics: Humans; Female; Male; Middle Aged; Adult; Deep Learning; Imaging, Three-Dimensional; Magnetic Resonance Imaging; Preoperative Care; Aged; Cerebellopontine Angle; Cerebellar Neoplasms; Image Interpretation, Computer-Assisted; Retrospective Studies; Neuroma, Acoustic
PubMed: 38480538
DOI: 10.1007/s00234-024-03328-9 -
Journal of Medical Imaging and... Jun 2024Vestibular schwannomas (VSs) are rare, benign intracranial tumours that have prompted clinical practice guideline (CPG) creation given their complex management. Our aim...
INTRODUCTION
Vestibular schwannomas (VSs) are rare, benign intracranial tumours that have prompted clinical practice guideline (CPG) creation given their complex management. Our aim was to utilize the Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument to assess if such CPGs on the management of VSs with radiosurgery and radiotherapy are of acceptable quality.
METHODS
Relevant CPGs were identified following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocols. Experienced reviewers then extracted general CPG properties and rated their quality via the AGREE II instrument. Intraclass correlation coefficients (ICCs) were quantified to assess interrater reliability.
RESULTS
Nine CPGs on the management of VSs with radiosurgery and radiotherapy were identified. All CPGs were created in the past six years and developed recommendations based on literature review and expert consensus. One guideline was deemed as high quality with seven others being moderate and one being low in quality. The clarity of the presentation domain had the highest mean scaled domain score of 96.0%. The domains of stakeholder involvement and applicability had the lowest means of 49.2% and 47.2%, respectively. ICCs were either good or excellent across all domains.
CONCLUSION
Current CPGs on the management of VSs with radiosurgery and radiotherapy are of acceptable quality but would greatly benefit from improvements in applicability, stakeholder involvement, editorial independence and rigour of development. We recommend CPG authors reference the European Association of Neuro-Oncology (EANO) guideline as a developmental framework with the Congress of Neurological Surgeons/American Association of Neurological Surgeons (CNS/AANS) CPG being a valid alternative.
Topics: Humans; Radiosurgery; Neuroma, Acoustic; Practice Guidelines as Topic
PubMed: 38477433
DOI: 10.1111/1754-9485.13640 -
Acta Oto-laryngologica Jan 2024There is no comprehensive and up-to-date overview of audiovestibular approach to the posterior fossa tumors in the literature. (Review)
Review
BACKGROUND
There is no comprehensive and up-to-date overview of audiovestibular approach to the posterior fossa tumors in the literature.
OBJECTIVE
This paper reviewed the literature relating to tumors at the posterior cranial fossa to find red flags alerting a posterior fossa lesion from audiovestibular perspectives.
METHODS
This review was developed from articles published in those journals listed on the journal citation reports. Through the PubMed database, Embase, Google Scholar, and Cochrane library, 60 articles were finally obtained based on the PRISMA guidelines for reporting reviews.
RESULTS
The presence of one red flag indicates a positive predictive value of 33% for detecting a posterior fossa lesion. Clinical features, namely, 1) mid-frequency sudden sensorineural hearing loss (SNHL), 2) bilateral sudden SNHL, and 3) rebound nystagmus may indicate a posterior fossa lesion, representing one, two, and three red flags, respectively.
CONCLUSION
Those with 1) mid-frequency sudden SNHL, 2) bilateral sudden SNHL, and 3) rebound nystagmus trigger one, two, and three red flags, respectively, alerting clinicians the possibility of a posterior fossa lesion, which warrant MR imaging to exclude life-threatening or treatable conditions.
SIGNIFICANCE
Patients with posterior fossa tumors may have potential life-threatening outcome.
Topics: Humans; Hearing Loss, Sensorineural; Nystagmus, Pathologic; Infratentorial Neoplasms; Cranial Fossa, Posterior; Hearing Loss, Sudden
PubMed: 38461404
DOI: 10.1080/00016489.2024.2316262 -
Environment International Mar 2024Each new generation of mobile phone technology has triggered discussions about potential carcinogenicity from exposure to radiofrequency electromagnetic fields (RF-EMF)....
BACKGROUND
Each new generation of mobile phone technology has triggered discussions about potential carcinogenicity from exposure to radiofrequency electromagnetic fields (RF-EMF). Available evidence has been insufficient to conclude about long-term and heavy mobile phone use, limited by differential recall and selection bias, or crude exposure assessment. The Cohort Study on Mobile Phones and Health (COSMOS) was specifically designed to overcome these shortcomings.
METHODS
We recruited participants in Denmark, Finland, the Netherlands, Sweden, and the UK 2007-2012. The baseline questionnaire assessed lifetime history of mobile phone use. Participants were followed through population-based cancer registers to identify glioma, meningioma, and acoustic neuroma cases during follow-up. Non-differential exposure misclassification was reduced by adjusting estimates of mobile phone call-time through regression calibration methods based on self-reported data and objective operator-recorded information at baseline. Hazard ratios (HR) and 95% confidence intervals (CI) for glioma, meningioma, and acoustic neuroma in relation to lifetime history of mobile phone use were estimated with Cox regression models with attained age as the underlying time-scale, adjusted for country, sex, educational level, and marital status.
RESULTS
264,574 participants accrued 1,836,479 person-years. During a median follow-up of 7.12 years, 149 glioma, 89 meningioma, and 29 incident cases of acoustic neuroma were diagnosed. The adjusted HR per 100 regression-calibrated cumulative hours of mobile phone call-time was 1.00 (95 % CI 0.98-1.02) for glioma, 1.01 (95 % CI 0.96-1.06) for meningioma, and 1.02 (95 % CI 0.99-1.06) for acoustic neuroma. For glioma, the HR for ≥ 1908 regression-calibrated cumulative hours (90th percentile cut-point) was 1.07 (95 % CI 0.62-1.86). Over 15 years of mobile phone use was not associated with an increased tumour risk; for glioma the HR was 0.97 (95 % CI 0.62-1.52).
CONCLUSIONS
Our findings suggest that the cumulative amount of mobile phone use is not associated with the risk of developing glioma, meningioma, or acoustic neuroma.
Topics: Humans; Meningioma; Cohort Studies; Neuroma, Acoustic; Prospective Studies; Cell Phone Use; Brain Neoplasms; Glioma; Electromagnetic Fields; Cell Phone; Surveys and Questionnaires; Meningeal Neoplasms; Case-Control Studies
PubMed: 38458118
DOI: 10.1016/j.envint.2024.108552 -
The Journal of International Advanced... Jan 2024The precise treatment of iatrogenic cerebrospinal fluid (CSF) otorhinorrhea has been poorly studied. The purpose of the study was to investigate the clinical...
BACKGROUND
The precise treatment of iatrogenic cerebrospinal fluid (CSF) otorhinorrhea has been poorly studied. The purpose of the study was to investigate the clinical manifestation, surgical results, and management of CSF leak.
METHODS
Electronic medical record database of iatrogenic CSF leaks after erebellopontine angle(CPA) surgery from 2019 to 2022 was retrospectively analyzed. Three patients returned to the hospital with the complication of CSF leak. After failed attempts of conservative strategies or reverse surgical repair, adipose tissue was applied to the mastoid cracks repair.
RESULTS
With the techniques described above, the CSF leaks were successfully settled. The identified patients were observed for at least 10 months. and there was no recurrence or other complications.
CONCLUSION
Conservative treatment and initial surgical methods for occult postoperative CSF leaks are prone to delay effective results, particularly in patients with well-evaporated temporal bone. This complication can be minimized with transmastoid closure utilizing autologous fat.
Topics: Humans; Neuroma, Acoustic; Retrospective Studies; Cerebrospinal Fluid Leak; Temporal Bone; Postoperative Complications; Iatrogenic Disease; Treatment Outcome
PubMed: 38454285
DOI: 10.5152/iao.2024.231084 -
Child's Nervous System : ChNS :... Jul 2024NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case...
NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features.
Topics: Humans; Male; Meningioma; Child; Meningeal Neoplasms; Neurofibromatoses; Neurofibromatosis 2; Neurilemmoma; Skin Neoplasms; Magnetic Resonance Imaging
PubMed: 38451298
DOI: 10.1007/s00381-024-06350-7 -
Child's Nervous System : ChNS :... Jun 2024Tethered spinal cord syndrome (TCS) is characterized by cutaneous attachments on the filum terminale that stretch the spinal cord, leading to musculoskeletal and...
PURPOSE
Tethered spinal cord syndrome (TCS) is characterized by cutaneous attachments on the filum terminale that stretch the spinal cord, leading to musculoskeletal and urogenital sequelae. While the neurocutaneous associations with TCS remain undefined, a recent study reports a high incidence of TCS among a pediatric neurofibromatosis (NF) cohort. This present study utilizes a population-level database to estimate TCS incidence among pediatric patients with neurofibromatosis types 1 and 2 (NF1, NF2).
METHODS
The TriNetX Research Network was queried to identify patients diagnosed with NF and/or TCS before the age of 21. Symptomatic TCS requiring surgical intervention was identified using corresponding procedural codes within 12 months following TCS diagnosis. Odds ratios (OR) were calculated to measure the associations of NF1/NF2 with TCS.
RESULTS
19,426 pediatric NF patients were evaluated (NF1: 18,383, NF2: 1042). The average ages of TCS diagnosis among NF1, NF2, and non-NF patients were 12, 16, and 9 years, respectively. The incidence of TCS was 1.2% in NF1 patients and 7.3% in NF2 patients, compared to 0.074% in the general population. The associations of NF incidence with TCS were significantly increased in both NF1 (OR 16.42; 14.38-18.76) and NF2 (OR 105.58; 83.56-133.40) patients compared to the general population. Symptomatic TCS requiring surgical intervention was not significantly associated with NF1/NF2 patients compared to the general TCS population.
CONCLUSION
This analysis demonstrates a high incidence of TCS but delayed intervention in pediatric NF patients. Considering TCS counseling, spinal MRI, and earlier intervention may be warranted for NF patients experiencing musculoskeletal symptomatology.
Topics: Humans; Neurofibromatosis 1; Child; Male; Female; Incidence; Adolescent; Neural Tube Defects; Neurofibromatosis 2; Child, Preschool; Infant; Young Adult
PubMed: 38451296
DOI: 10.1007/s00381-024-06325-8 -
World Neurosurgery: X Apr 2024While previous studies have assessed patient reported quality of life (QOL) of various vestibular schwannoma (VS) treatment modalities, few studies have assessed QOL as...
BACKGROUND
While previous studies have assessed patient reported quality of life (QOL) of various vestibular schwannoma (VS) treatment modalities, few studies have assessed QOL as related to the amount of residual tumor and need for retreatment in a large series of patients. Objective: To assess patient reported QOL outcomes following VS resection with a focus on extent of resection and retreatment.
METHODS
A retrospective chart review was performed using single-center institutional data of adult patients who underwent VS resection by the senior authors between 1989-2018 at Loyola University Medical Center. The Penn Acoustic Neuroma Quality of Life (PANQOL) survey was sent to all patients via postal mail.
RESULTS
Fifty-five percent of 367 total patients were female with a mean age of 61.6 years (SD 12.63). The mean period between surgery and PANQOL response was 11.4 years (IQR: 4.74-7.37). The median tumor size was 2 cm (IQR: 1.5-2.8). The mean total PANQOL score was 70 (SD 19). Patients who required retreatment reported lower overall scores (μdiff = -10.11, 95% CI: -19.48 to -0.74; p = 0.03) and face domain scores (μdiff = -20.34, 95% CI: -29.78 to -10.91; p < .001). There was no association between extent of resection and PANQOL scores in any domain.
CONCLUSION
In an analysis of 367 patients who underwent microsurgical resection of VS, extent of resection did not affect PANQOL scores in contrast to previous reports in the literature, while the need for retreatment and facial function had a significant impact on patient-reported outcomes.
PubMed: 38450247
DOI: 10.1016/j.wnsx.2024.100294