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Neuro-oncology Jul 2022
Topics: Glioma; Humans; Neuroma, Acoustic
PubMed: 35323960
DOI: 10.1093/neuonc/noac078 -
Journal of Neurological Surgery. Part... Jun 2022While postoperative outcomes of acoustic neuroma (AN) resection commonly consider hearing preservation and facial function, headache is a critical quality of life...
While postoperative outcomes of acoustic neuroma (AN) resection commonly consider hearing preservation and facial function, headache is a critical quality of life factor. Postoperative headache is described in the literature; however, there is limited discussion specific to occipital neuralgia (ON) following AN resection. The aim of this study is to investigate the effectiveness of conservative management and surgery. We conducted a retrospective review of 872 AN patients who underwent resection at our institution between 1988 and 2017 and identified 15 patients (1.9%) that met International Classification of Headache Disorders criteria for ON. Of the 15 ON patients, surgical approaches included 13 (87%) retrosigmoid (RS), one (7%) translabyrinthine (TL), and one (7%) combined RS + TL. Mean clinical follow-up was 119 months (11-263). Six (40%) patients obtained pain relief through conservative management, while the remaining nine (60%) underwent surgery or ablative procedure. Three (38%) patients received an external neurolysis, four (50%) received a neurectomy, one (13%) had both procedures, and one (13%) received two C2 to 3 radio frequency ablations. Of the nine patients who underwent procedural ON treatment, seven (78%) patients achieved pain relief, one patient (11%) continued to have pain, and one patient (11%) was lost to follow-up. Of the six patients whose pain was controlled with conservative management and nerve blocks, five (83%) found relief by using neuropathic pain medication and one (17%) found relief on nonsteroidal anti-inflammatory drug. Our series demonstrates success with conservative management in some, but overall a minority (40%) of patients, reserving decompression only for refractory cases.
PubMed: 35832990
DOI: 10.1055/s-0041-1722934 -
Ugeskrift For Laeger Jan 2021In this review, we discuss the auditory brainstem implant (ABI), which is a neuroprosthetic device being an advanced hearing aid in cases of bilateral, profound or... (Review)
Review
In this review, we discuss the auditory brainstem implant (ABI), which is a neuroprosthetic device being an advanced hearing aid in cases of bilateral, profound or complete hearing impairment due to a non-functional or absent cochlear nerve, or an inner ear malformation precluding cochlear implantation. Originally indicated in patients with bilateral vestibular schwannomas caused by neurofibromatosis type 2, the ABI has in recent years seen an increase in the aural rehabilitation of congenitally deaf children. Outcomes for patients are mixed, but generally the device leads to a reasonable improvement in speech perception.
Topics: Auditory Brain Stem Implantation; Auditory Brain Stem Implants; Child; Cochlear Implantation; Humans; Neurofibromatosis 2; Treatment Outcome
PubMed: 33491645
DOI: No ID Found -
Wiener Medizinische Wochenschrift (1946) Feb 2022Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in...
Vestibular schwannomas can severely impair the quality of life of patients. Next to impaired hearing function, facial palsy is perceived as particularly disturbing in this context. Varying growth rates of these benign tumors complicate a prediction of functional impairment of cranial nerves. Therefore, a regular update on current therapeutic strategies and alternative treatment options is relevant for both physicians and patients.
Topics: Aftercare; Humans; Neuroma, Acoustic; Quality of Life
PubMed: 33439379
DOI: 10.1007/s10354-020-00800-y -
Cancer Cell International May 2023Neurofibromatosis type 2 (NF2) is a genetic condition marked by the development of multiple benign tumors in the nervous system. The most common tumors associated with... (Review)
Review
Neurofibromatosis type 2 (NF2) is a genetic condition marked by the development of multiple benign tumors in the nervous system. The most common tumors associated with NF2 are bilateral vestibular schwannoma, meningioma, and ependymoma. The clinical manifestations of NF2 depend on the site of involvement. Vestibular schwannoma can present with hearing loss, dizziness, and tinnitus, while spinal tumor leads to debilitating pain, muscle weakness, or paresthesias. Clinical diagnosis of NF2 is based on the Manchester criteria, which have been updated in the last decade. NF2 is caused by loss-of-function mutations in the NF2 gene on chromosome 22, leading the merlin protein to malfunction. Over half of NF2 patients have de novo mutations, and half of this group are mosaic. NF2 can be managed by surgery, stereotactic radiosurgery, monoclonal antibody bevacizumab, and close observation. However, the nature of multiple tumors and the necessity of multiple surgeries over the lifetime, inoperable tumors like meningiomatosis with infiltration of the sinus or in the area of the lower cranial nerves, the complications caused by the operation, the malignancies induced by radiotherapy, and inefficiency of cytotoxic chemotherapy due to the benign nature of NF-related tumors have led a march toward exploring targeted therapies. Recent advances in genetics and molecular biology have allowed identifying and targeting of underlying pathways in the pathogenesis of NF2. In this review, we explain the clinicopathological characteristics of NF2, its genetic and molecular background, and the current knowledge and challenges of implementing genetics to develop efficient therapies.
PubMed: 37217995
DOI: 10.1186/s12935-023-02940-8 -
Cell Death & Disease Sep 2023Hearing loss (HL) is the most common and heterogeneous disorder of the sensory system, with a large morbidity in the worldwide population. Among cells of the acoustic... (Review)
Review
Hearing loss (HL) is the most common and heterogeneous disorder of the sensory system, with a large morbidity in the worldwide population. Among cells of the acoustic nerve (VIII cranial nerve), in the cochlea are present the hair cells, the spiral ganglion neurons, the glia-like supporting cells, and the Schwann cells (SCs), which alterations have been considered cause of HL. Notably, a benign SC-derived tumor of the acoustic nerve, named vestibular schwannoma (VS), has been indicated as cause of HL. Importantly, SCs are the main glial cells ensheathing axons and forming myelin in the peripheral nerves. Following an injury, the SCs reprogram, expressing some stemness features. Despite the mechanisms and factors controlling their biological processes (i.e., proliferation, migration, differentiation, and myelination) have been largely unveiled, their role in VS and HL was poorly investigated. In this review, we enlighten some of the mechanisms at the base of SCs transformation, VS development, and progression, likely leading to HL, and we pose great attention on the environmental factors that, in principle, could contribute to HL onset or progression. Combining the biomolecular bench-side approach to the clinical bedside practice may be helpful for the diagnosis, prediction, and therapeutic approach in otology.
Topics: Humans; Neuroma, Acoustic; Hearing Loss; Deafness; Schwann Cells; Neuroglia
PubMed: 37741837
DOI: 10.1038/s41419-023-06141-z -
Neurology India 2023Gamma Knife Radiosurgery (GKRS) is now an established standard of treatment for the small-sized arteriovenous malformations (AVMs), meningiomas, schwannomas, metastasis,... (Review)
Review
Gamma Knife Radiosurgery (GKRS) is now an established standard of treatment for the small-sized arteriovenous malformations (AVMs), meningiomas, schwannomas, metastasis, and other benign diseases. With an exponential rise in the indications for GKRS, we have witnessed an increase in the adverse radiation effects (ARE) following GKRS. The common AREs and associated risk factors following GKRS have been described for pathologies including vestibular schwannomas, arteriovenous malformations, meningiomas, and metastases based on the authors' experience, and a simplified management protocol has been provided for radiation-induced changes based on clinical and radiologic parameters. The dose, volume, location, and repeat stereotactic radiosurgery (SRS) are implied as the risk factors for ARE. Clinically symptomatic AREs require oral steroids for weeks to alleviate symptoms. In refractory cases, bevacizumab and surgical resection can be offered as a treatment modality. Appropriate dose planning strategy and hypofractionation for larger lesions help in mitigating the AREs.
Topics: Humans; Meningioma; Radiosurgery; Neuroma, Acoustic; Intracranial Arteriovenous Malformations; Meningeal Neoplasms; Treatment Outcome; Retrospective Studies; Follow-Up Studies
PubMed: 37026335
DOI: 10.4103/0028-3886.373645 -
Neuro-oncology Dec 2023Schwannomas are common peripheral nerve sheath tumors that can cause severe morbidity given their stereotypic intracranial and paraspinal locations. Similar to many...
BACKGROUND
Schwannomas are common peripheral nerve sheath tumors that can cause severe morbidity given their stereotypic intracranial and paraspinal locations. Similar to many solid tumors, schwannomas and other nerve sheath tumors are primarily thought to arise due to aberrant hyperactivation of the RAS growth factor signaling pathway. Here, we sought to further define the molecular pathogenesis of schwannomas.
METHODS
We performed comprehensive genomic profiling on a cohort of 96 human schwannomas, as well as DNA methylation profiling on a subset. Functional studies including RNA sequencing, chromatin immunoprecipitation-DNA sequencing, electrophoretic mobility shift assay, and luciferase reporter assays were performed in a fetal glial cell model following transduction with wildtype and tumor-derived mutant isoforms of SOX10.
RESULTS
We identified that nearly one-third of sporadic schwannomas lack alterations in known nerve sheath tumor genes and instead harbor novel recurrent in-frame insertion/deletion mutations in SOX10, which encodes a transcription factor responsible for controlling Schwann cell differentiation and myelination. SOX10 indel mutations were highly enriched in schwannomas arising from nonvestibular cranial nerves (eg facial, trigeminal, vagus) and were absent from vestibular nerve schwannomas driven by NF2 mutation. Functional studies revealed these SOX10 indel mutations have retained DNA binding capacity but impaired transactivation of glial differentiation and myelination gene programs.
CONCLUSIONS
We thus speculate that SOX10 indel mutations drive a unique subtype of schwannomas by impeding proper differentiation of immature Schwann cells.
Topics: Humans; INDEL Mutation; Transcriptional Activation; Neurilemmoma; Neuroma, Acoustic; Mutation; Nerve Sheath Neoplasms; SOXE Transcription Factors
PubMed: 37436963
DOI: 10.1093/neuonc/noad121 -
World Neurosurgery Sep 2022Vestibular schwannomas are benign, slow-growing tumors that often reduce patient quality of life by compressing nearby nerves. Neurological function preservation is one... (Review)
Review
OBJECTIVE
Vestibular schwannomas are benign, slow-growing tumors that often reduce patient quality of life by compressing nearby nerves. Neurological function preservation is one of the indicators of treatment success, with hearing preservation being the most difficult to obtain. This paper provides a bibliometric analysis of hearing preservation in treating acoustic neuromas and a greater understanding of the most highly cited articles, which have enhanced our understanding of this topic.
METHODS
Key terms of "acoustic neuroma," "vestibular schwannoma," and "hearing preservation" were queried through Web of Science. Articles were sorted by citation frequency, and the top 100 articles were recorded for title, name of first author, journal title, year of publication, total number of citations (and associated rank), average number of citations per year, country of the first author's associated institution, and type of study.
RESULTS
The top 100 cited articles were published from 1980 to 2014. The United States had the highest involvement as a country (55%), the University of Pittsburgh as an institution (13%), and The Journal of Neurosurgery as a publishing source (27%). Fourteen were reviews, and 86 were clinical papers. Of the 86, 73 were retrospective studies.
CONCLUSION
Bibliometric analyses summarize and assess potential areas of strength and knowledge gaps within the literature. Studies on hearing preservation in vestibular schwannomas mostly consist of retrospective reviews that assess postoperative outcomes of microsurgery and radiosurgery. Prospective studies and novel treatment options for hearing preservation in vestibular schwannomas are needed to increase current literature diversity.
Topics: Hearing; Humans; Neuroma, Acoustic; Prospective Studies; Quality of Life; Radiosurgery; Retrospective Studies
PubMed: 35779753
DOI: 10.1016/j.wneu.2022.06.120 -
Clinical Neurology and Neurosurgery May 2023Secondary trigeminal neuralgia is a facial pain in trigeminal nerve dermatome caused by an underlying disease, such as cerebellopontine angle tumours. Treatment options... (Review)
Review
BACKGROUND
Secondary trigeminal neuralgia is a facial pain in trigeminal nerve dermatome caused by an underlying disease, such as cerebellopontine angle tumours. Treatment options to relieve the pains were surgical tumour resection and stereotactic radiosurgery of the tumour or trigeminal nerve. This study aims to review the efficacy of open surgery and stereotactic radiosurgery and recommend the treatment of choice for secondary trigeminal neuralgia due to cerebellopontine angle tumours.
METHOD
The inclusion criteria were studies covering patients with trigeminal neuralgia associated with cerebellopontine angle tumours that were treated with either open surgery or stereotactic radiosurgery and reported pain outcomes after treatment. Non-English articles or studies with a population of less than five were excluded. We systematically searched studies from PubMed, Ebscohost, and Cochrane Library from inception until December 20, 2021. Several works of literature from manual search were also added. Selected articles were appraised using a critical appraisal tool for prognostic studies.
RESULT
Included articles were 26 retrospective studies and one prospective study comprising 517 patients. Of 127 schwannomas, 226 epidermoids, 154 meningiomas, and ten other tumours, 320 cases received surgical tumour excision with or without MVD, 196 had tumour-targeted radiosurgery, and 22 underwent nerve-targeted radiosurgery. In surgical series, 92.2 % gained pain improvement, 2.8 % were unchanged, and 4.5 % had recurrence; none of the patients had worsened outcomes. In cases treated with tumour-targeted radiosurgery, the improvement rate was 79.1 %, unchanged at 14.3 %, recurrence at 26.5 %, and worse symptoms rate after the intervention was 6.6 %. Six patients with recurrent pain after tumour-targeted radiosurgery received secondary nerve-targeted radiosurgery with improved outcomes. Only one patient in our review underwent primary nerve-targeted radiosurgery, and the result was satisfactory. One study treated 15 patients with a single session of tumour-targeted and nerve-targeted radiosurgery, with an improvement rate of 93.3 % and a recurrence rate of 21.4 %.
CONCLUSION
Open surgery releasing the nerve root from compressive lesions is advocated to be the first-line treatment to gain satisfactory outcomes. Total removal surgery is recommended if possible. Nerve-targeted radiosurgery should be reserved as a secondary treatment for recurrent cases.
Topics: Humans; Trigeminal Neuralgia; Neuroma, Acoustic; Radiosurgery; Treatment Outcome; Retrospective Studies; Prospective Studies; Facial Pain; Meningeal Neoplasms
PubMed: 37001475
DOI: 10.1016/j.clineuro.2023.107683