Materiały źródłowe

• #1 Acoustic Neuroma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470177/

  Acoustic neuromas are tumors that develop from the sheath of Schwann cells. They tend to occupy the cerebellopontine angle and are usually found adjacent to the cochlear or vestibular nerve, either intracranially or extra-axially. […] Acoustic neuroma is also known by other names such as vestibular schwannoma (VS), acoustic neurinoma, vestibular neuroma, and acoustic neurofibroma. These tumors originate from the Schwann cells in the sheath surrounding the vestibular and cochlear nerves. Acoustic neuroma can occur either intracranially or extra-axially. […] Bilateral acoustic neuromas can be associated with neurofibromatosis type 2, which is caused by a defect on chromosome 22q12.2 at the location of the neurofibromin 2 gene, responsible for encoding the merlin protein. Studies have indicated a predisposing mutation for the development of acoustic neuroma. Additionally, radiation exposure may increase the likelihood of developing this condition.

• #2 Vestibular Schwannoma (Acoustic Neuroma) – ENT Health

  https://www.enthealth.org/conditions/vestibular-schwannoma-acoustic-neuroma/

  Vestibular schwannoma, also called an acoustic neuroma, is a benign (non-cancerous) tumor that typically grows on the balance portion of the eighth cranial nerve. […] The exact cause of most vestibular schwannomas is unknown. Ninety-five percent of tumors occur spontaneously and are found on one side (unilateral) only. A small subset of vestibular schwannomas is associated with a genetic condition called Neurofibromatosis Type 2 (NF2). NF2 is rare and results in bilateral vestibular schwannomas. Regardless of the cause, the tumor originates from Schwann cells, which insulate nerves allowing them to transmit their neural signal quickly. When a tumor develops, the Schwann cells grow too quickly and can damage the nerve. In general, vestibular schwannomas grow slowly with an average growth rate of one to two millimeters per year. However, some tumors do not grow for several years and others grow rapidly.

• #3 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic neuromas (also known as vestibular schwannomas) are non-cancerous intracranial, extra-axial tumors that arise from Schwann cells. Schwann cells myelinate and insulate peripheral nerves, aiding in both nerve conduction and axonal regeneration. […] In the case of acoustic neuromas/vestibular schwannomas, a benign tumor arises from the Schwann cells surrounding one of the vestibular nerves or, less commonly, the cochlear nerve. […] The vast majority of acoustic neuromas develop from the Schwann cells investing the vestibular portion of the vestibulocochlear nerve. Less than 5% of tumors arise from the cochlear nerve. The superior and inferior vestibular nerves appear to be the nerves of origin with about equal frequency. Overall, three separate growth patterns can be distinguished within acoustic tumors, as follows: (1) no growth or very slow growth, (2) slow growth (ie, 2 mm/y), and (3) fast growth (ie, 1.0 cm/y on imaging studies). Although most acoustic neuromas grow slowly, rarely, a tumor may grow quickly and may double in volume within 6 months to a year.

• #4 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?lang=us

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumors that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumors (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumors arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] A number of other possibilities have been proposed including perilymphatic CSF flow dysfunction, neurovascular compromise, or altered blood-labyrinth barrier permeability. […] Immunohistochemical staining is usually positive for S-100 protein.

• #5 Acoustic Neuroma Symptoms, Surgery and Treatment | UPMC

  https://www.upmc.com/services/neurosurgery/brain/conditions/brain-tumors/acoustic-neuroma

  An acoustic neuroma (vestibular schwannoma) is a benign, usually slow-growing tumor that develops from the balance and hearing nerves supplying the inner ears. […] The more precise term for this tumor is vestibular schwannoma, because it involves the vestibular portion of the nerve, not the acoustic (cochlear) portion, and it arises from Schwann cells rather than from neurons. […] The cause of most acoustic neuromas is not known. In a minority of cases, acoustic neuroma may be caused by a rare genetic disorder, called neurofibromatosis type 2, which produces benign tumors of the nervous system. […] There are no known risk factors for acoustic neuroma.

• #6 Acoustic Neuroma (Vestibular Schwannoma) | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/acoustic-neuroma

  Acoustic neuroma, also called vestibular schwannoma, is a type of benign (noncancerous) tumor. It starts in the cells that wrap around the hearing and balance nerve that connects your ear to your brain. […] Most acoustic neuromas grow very slowly. As this happens, the tumor begins to press on nearby nerves, blood vessels, and the surface of the brain stem and cerebellum (the base of the brain). The pressure from a growing acoustic neuroma is what leads to the development of symptoms. […] Acoustic neuroma tumors begin in what are called Schwann cells. These cells make myelin, which is the material that insulates and protects the nerves throughout your body. […] Most acoustic neuromas begin in the vestibular nerve, which helps you keep your balance. Tumor growth leads to hearing loss and balance disorders.

• #7 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor originates when Schwann cells that form the insulating myelin sheath on the nerve malfunction. Normally, Schwann cells function beneficially to protect the nerves which transmit balance and sound information to the brain. However, sometimes a mutation in the tumor suppressor gene, NF2, located on chromosome 22, results in abnormal production of the cell protein named Merlin, and Schwann cells multiply to form a tumor. The tumor originates mostly on the vestibular division of the nerve rather than the cochlear division, but hearing as well as balance will be affected as the tumor enlarges. […] Variations in the mutation determine the nature of the tumor’s development. The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head.

• #8 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  The vestibular schwannoma can arise anywhere along the course of the vestibular nerve, running from the cistern to inside the vestibular organ. […] Vestibular schwannomas are clinically and oncologically regarded as benign tumors. […] Despite this, the management of vestibular schwannoma is difficult and controversial because vestibular schwannoma interventions can cause complications. […] Improving vestibular schwannoma management to prevent complications such as hearing loss and facial nerve palsy is desirable. […] With recent advances in surgical equipment systems and the molecular understanding of the disease, vestibular schwannoma treatment has changed and improved. […] This review summarizes the recent advances in vestibular schwannoma management and treatment. […] The molecular mechanisms underlying tumorigenesis need to be investigated in these cases.

• #9 Acoustic Neuroma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470177/

  While there have been concerns regarding mobile phone radiation, several studies have failed to establish a direct causal relationship between mobile phone radiation and the development of vestibular schwannomas. […] Schwannomas account for approximately 8% of all clinically manifested intracranial tumors. Most acoustic neuromas are unilateral and occur sporadically. However, genetic factors contribute to developing bilateral acoustic neuromas, constituting less than 5% of all schwannomas. […] Generally, acoustic neuromas are diagnosed between the fourth to sixth decades of life. However, individuals with neurofibromatosis type 2 (NF II) tend to present earlier, with the peak incidence occurring in the third decade of life. […] Acoustic neuromas originate near the porus acusticus, where the transition between glial and Schwann cells, known as the Obersteiner-Redlich zone, can be observed.

• #10 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.icoradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=gb

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumours that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumours (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumours arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] They can display two types of growth patterns: Antoni A and Antoni B. […] Immunohistochemical staining is usually positive for S-100 protein.

• #11 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?lang=us

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumors that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumors (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumors arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] A number of other possibilities have been proposed including perilymphatic CSF flow dysfunction, neurovascular compromise, or altered blood-labyrinth barrier permeability. […] Immunohistochemical staining is usually positive for S-100 protein.

• #12 Vestibular Schwannoma (Acoustic Neuroma) – ENT Health

  https://www.enthealth.org/conditions/vestibular-schwannoma-acoustic-neuroma/

  Vestibular schwannoma, also called an acoustic neuroma, is a benign (non-cancerous) tumor that typically grows on the balance portion of the eighth cranial nerve. […] The exact cause of most vestibular schwannomas is unknown. Ninety-five percent of tumors occur spontaneously and are found on one side (unilateral) only. A small subset of vestibular schwannomas is associated with a genetic condition called Neurofibromatosis Type 2 (NF2). NF2 is rare and results in bilateral vestibular schwannomas. Regardless of the cause, the tumor originates from Schwann cells, which insulate nerves allowing them to transmit their neural signal quickly. When a tumor develops, the Schwann cells grow too quickly and can damage the nerve. In general, vestibular schwannomas grow slowly with an average growth rate of one to two millimeters per year. However, some tumors do not grow for several years and others grow rapidly.

• #13 Acoustic Neuroma | Vestibular Schwannoma | Dr LM Hofmeyr

  https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/acoustic-neuroma/

  An acoustic neuroma, or vestibular schwannoma, is a benign (non-cancerous), usually slow-growing tumour originating on the nerve for balance and hearing (cochleovestibular nerve). […] The cause of vestibular schwannoma is unknown in 95% of cases. It has a genetic origin in 5 % of cases, most often related to neurofibromatosis type 2 (NF2). […] A vestibular schwannoma originates from the Schwann sheath of the cochleovestibular nerve. Schwann cells are specialized cells that form the myelin sheath around nerves to provide insulation and support. When these cells grow uncontrollably, they can create a tumour known as a schwannoma. […] The majority of vestibular schwannoma will continue to grow after diagnosis. The growth pattern is irregular, with some vestibular schwannoma continuously growing and others on growth spurts with stationary periods in between. Some vestibular schwannomas may not grow, and small vestibular schwannomas may even disappear in a few cases.

• #14 Acoustic Neuroma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470177/

  Acoustic neuromas are tumors that develop from the sheath of Schwann cells. They tend to occupy the cerebellopontine angle and are usually found adjacent to the cochlear or vestibular nerve, either intracranially or extra-axially. […] Acoustic neuroma is also known by other names such as vestibular schwannoma (VS), acoustic neurinoma, vestibular neuroma, and acoustic neurofibroma. These tumors originate from the Schwann cells in the sheath surrounding the vestibular and cochlear nerves. Acoustic neuroma can occur either intracranially or extra-axially. […] Bilateral acoustic neuromas can be associated with neurofibromatosis type 2, which is caused by a defect on chromosome 22q12.2 at the location of the neurofibromin 2 gene, responsible for encoding the merlin protein. Studies have indicated a predisposing mutation for the development of acoustic neuroma. Additionally, radiation exposure may increase the likelihood of developing this condition.

• #15 Vestibular Schwannoma: What We Know and Where We are Heading

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7669921/

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. […] Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. […] Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. […] This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #16 Acoustic neuroma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/acoustic-neuroma/symptoms-causes/syc-20356127

  An acoustic neuroma develops from the Schwann cells covering the vestibular nerve. […] The cause of acoustic neuromas can sometimes be linked to a problem with a gene on chromosome 22. Typically, this gene produces a tumor suppressor protein that helps control the growth of Schwann cells covering the nerves. […] This gene change is inherited in people with a rare disorder called neurofibromatosis type 2.

• #17 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor originates when Schwann cells that form the insulating myelin sheath on the nerve malfunction. Normally, Schwann cells function beneficially to protect the nerves which transmit balance and sound information to the brain. However, sometimes a mutation in the tumor suppressor gene, NF2, located on chromosome 22, results in abnormal production of the cell protein named Merlin, and Schwann cells multiply to form a tumor. The tumor originates mostly on the vestibular division of the nerve rather than the cochlear division, but hearing as well as balance will be affected as the tumor enlarges. […] Variations in the mutation determine the nature of the tumor’s development. The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head.

• #18

  https://link.springer.com/article/10.1007/s12105-020-01155-x

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #19 The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies | Acoustic Neuroma Association of Canada

  https://www.anac.ca/the-current-landscape-of-vestibular-schwannoma-therapy-development-of-novel-targeted-therapies

  An acoustic neuroma, also known as a vestibular schwannoma, is a benign tumour that arises from Schwann cells of the vestibulocochlear nerve. […] The loss of the NF2 gene is linked to pathogenesis of acoustic neuromas, both in sporadic and genetic conditions. About 60% of sporadic unilateral acoustic neuromas have mutations in the NF2 gene. Mutations of the NF2 gene lead to deregulation of important pathways that regulate tumour growth such as RAS and mTORC1 pathway. […] Further work is needed to understand the biological alterations that are driving the tumour development, in order to better develop targeted therapies that will effectively treat this disease process.

• #20 The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies | Acoustic Neuroma Association of Canada

  https://www.anac.ca/the-current-landscape-of-vestibular-schwannoma-therapy-development-of-novel-targeted-therapies

  An acoustic neuroma, also known as a vestibular schwannoma, is a benign tumour that arises from Schwann cells of the vestibulocochlear nerve. […] The loss of the NF2 gene is linked to pathogenesis of acoustic neuromas, both in sporadic and genetic conditions. About 60% of sporadic unilateral acoustic neuromas have mutations in the NF2 gene. Mutations of the NF2 gene lead to deregulation of important pathways that regulate tumour growth such as RAS and mTORC1 pathway. […] Further work is needed to understand the biological alterations that are driving the tumour development, in order to better develop targeted therapies that will effectively treat this disease process.

• #21

  https://step2.medbullets.com/oncology/120406/vestibular-schwannoma-acoustic-neuroma

  a Schwann cell-derived tumor arising from the 8th cranial nerve […] typically the vestibular portion […] usually unilateral (~90% of cases) […] neurofibromatosis type 2 […] characteristically presents as bilateral acoustina neuromas […] secondary to a mutation in merlin in chromosome 22

• #22 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Vestibular schwannomas (VS) are benign tumors of the nerve sheath and the most common tumor in the cerebellopontine angle, accounting for 6-8% of all intracranial tumors and 80% of cerebellopontine angle (CPA) tumors, with an estimated prevalence of 0.02% and mean age of diagnosis at 58 years. The sporadic form of VS makes up 90% of cases; there is no predominance for the left or right side. The remainder predominantly occur in neurofibromatosis type 2 (NF2); one of the diagnostic criteria for which is bilateral VS. NF2 is a rare autosomal dominant multiple neoplasia syndrome, occurring due to mutations in the NF2 gene located on chromosome 22q12, coding for the tumor suppressor Merlin. Genetic studies have demonstrated loss of both wild-type copies in the NF2 tumor suppressor gene as a common finding in sporadic VS as well NF2.

• #23 Acoustic Neuroma (Vestibular Schwannoma): Diagnosis, TreatmentSecond Opinion IconGroup 5

  https://www.barrowneuro.org/condition/acoustic-neuroma/

  Acoustic neuromas have been linked to a genetic disorder called neurofibromatosis type 2, but 95 percent of these tumors occur spontaneously without any evidence of family history. […] A vestibular schwannoma diagnosis can be difficult to detect early on because symptoms usually develop gradually and can resemble those of other middle and inner ear problems.

• #24 Vestibular Schwannoma: What We Know and Where We are Heading

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7669921/

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. […] Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. […] Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. […] This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #25

  https://link.springer.com/article/10.1007/s12105-020-01155-x

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #26 Vestibular Schwannoma (Acoustic Neuroma) – ENT Health

  https://www.enthealth.org/conditions/vestibular-schwannoma-acoustic-neuroma/

  Vestibular schwannoma, also called an acoustic neuroma, is a benign (non-cancerous) tumor that typically grows on the balance portion of the eighth cranial nerve. […] The exact cause of most vestibular schwannomas is unknown. Ninety-five percent of tumors occur spontaneously and are found on one side (unilateral) only. A small subset of vestibular schwannomas is associated with a genetic condition called Neurofibromatosis Type 2 (NF2). NF2 is rare and results in bilateral vestibular schwannomas. Regardless of the cause, the tumor originates from Schwann cells, which insulate nerves allowing them to transmit their neural signal quickly. When a tumor develops, the Schwann cells grow too quickly and can damage the nerve. In general, vestibular schwannomas grow slowly with an average growth rate of one to two millimeters per year. However, some tumors do not grow for several years and others grow rapidly.

• #27

  https://www.aurorahealthcare.org/services/neuroscience/brain-skull-base-care/vestibular-schwannoma

  A vestibular schwannoma develops in Schwann cells, which are the basis of myelin, a protective layer that grows around nerves. […] We dont know what causes vestibular schwannomas. However, almost all of these tumors have mutations related to a gene located on chromosome 22. Researchers continue to look for more information.

• #28 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor originates when Schwann cells that form the insulating myelin sheath on the nerve malfunction. Normally, Schwann cells function beneficially to protect the nerves which transmit balance and sound information to the brain. However, sometimes a mutation in the tumor suppressor gene, NF2, located on chromosome 22, results in abnormal production of the cell protein named Merlin, and Schwann cells multiply to form a tumor. The tumor originates mostly on the vestibular division of the nerve rather than the cochlear division, but hearing as well as balance will be affected as the tumor enlarges. […] Variations in the mutation determine the nature of the tumor’s development. The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head.

• #29 Acoustic Neuroma (Vestibular Schwannoma) – UC Health

  https://www.uchealth.com/en/conditions/acoustic-neuroma

  Acoustic neuromas arise from the Schwann cells covering the nerves and typically grow slowly. Rarely, these tumors may grow rapidly and become large enough to press against the brain and interfere with vital functions. […] The cause of acoustic neuromas is not well understood. For most acoustic neuromas, the cause at the level of the cellular machinery is the failure of a governor gene to exert its effect in suppressing the growth of Schwann cells those cells responsible for coating nerve fibers with insulation. The result is wart-like growth of these cells to produce the neuroma (or more precisely, schwannoma). […] The only environmental exposure that has been definitively associated with an increased incidence is radiation exposure to the head. […] For the most part it is not an inherited disease; however, 5% of cases are associated with a genetic disorder called neurofibromatosis type 2. These individuals demonstrate two-sided (I.e., bilateral) vestibular tumors often associated with other tumors around the brain and/or in the spine. The vast majority of acoustic neuromas are unilateral (one-sided) and sporadic (nonhereditary).

• #30 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Concern has risen in recent years about the potential role of microwave radiation from cellular telephones in the genesis of brain tumors, including vestibular schwannomas. […] Some studies have implicated long-term cellular phone use with an increased risk of vestibular schwannomas or other cranial tumors, especially with long latency and onset of use before the age of 20. […] Kundi reviewed 33 published studies and concluded that, although methodological flaws were found in every study, the weight of the evidence favored an increased risk for ipsilateral vestibular schwannomas with increasing duration of cellular phone usage, but the magnitude of that risk could not be assessed. […] A similar conclusion was reached in a meta-analysis by Hardell in 2008 for ipsilateral glioma and vestibular schwannomas.

• #31 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Concern has risen in recent years about the potential role of microwave radiation from cellular telephones in the genesis of brain tumors, including vestibular schwannomas. […] Some studies have implicated long-term cellular phone use with an increased risk of vestibular schwannomas or other cranial tumors, especially with long latency and onset of use before the age of 20. […] Kundi reviewed 33 published studies and concluded that, although methodological flaws were found in every study, the weight of the evidence favored an increased risk for ipsilateral vestibular schwannomas with increasing duration of cellular phone usage, but the magnitude of that risk could not be assessed. […] A similar conclusion was reached in a meta-analysis by Hardell in 2008 for ipsilateral glioma and vestibular schwannomas.

• #32 Vestibular Schwannoma (Acoustic Neuroma) – ENT Health

  https://www.enthealth.org/conditions/vestibular-schwannoma-acoustic-neuroma/

  Researchers continue to look for potential causes of vestibular schwannomas. High-dose therapeutic radiation to the head may increase the risk. Overall, there is no clear evidence that environmental factors, such as cell phones, cause these tumors. The rising incidence of vestibular schwannomas likely relates to improvements in magnetic resonance imaging (MRI) and increased screening for concerning symptoms.

• #33 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Studies in rats and mice suggest a carcinogenic effect of whole body exposure to radiofrequency radiation. […] Cell cultures of human Schwann cells exposed to electromagnetic fields showed alterations in proliferation, intracellular signaling, metabolic pathways, and in the expression of genes related to hearing loss. […] In 2013 and 2019, Hardell and Carlberg concluded that emissions from wireless phones should be regarded as group 1 carcinogens. […] Some are skeptical of this interpretation, citing the fact that cell phone emissions are not energetic enough to break molecular bonds within cells, a necessary step toward oncogenesis. […] At least two studies have implicated loud noise exposure in the genesis of vestibular schwannoma, but others have shown no increased risk or an indeterminate risk.

• #34 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-a-Vestibular-Schwannoma.aspx

  Vestibular schwannoma is associated with long-term exposure to noise including loud music or workplace noise. […] Radiation to the neck or face is also linked to vestibular schwannoma, though after many years. […] NF2, which may run in families, is another risk factor. […] Some scientists think that both types are caused by defects in the same gene which causes Schwann cells to function. […] In the case of unilateral vestibular schwannomas, the mutation is acquired, whereas it is inherited in the case of bilateral tumors.

• #35 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Historically known as acoustic neuromas, these tumors have been demonstrated not to involve the acoustic (cochlear) nerve in most cases, nor to be of neuroglial origin. Instead, they originate from Schwann cells, and they can occur anywhere lateral to the glial-schwannian junction. […] Two different tissue types may be present in vestibular schwannomas: Antoni type A tissue, which is compact and ordered with a palisading architecture, and Antoni type B tissue, which is myxoid and loose. Histological studies have shown no correlation between dominance of Antoni A or B tissue in either cystic or solid vestibular schwannomas. […] The rapid growth of cystic VS is attributed to expansion of the cystic component. On histological studies, the Ki-67 index of cystic VS is either lower or not significantly different to that of solid VS. Several mechanisms by which the cystic component grows have been proposed: extravasation of serum proteins from impaired blood-tumor barrier; protein secretion from tumor cells; isolated or repeated intratumoral microhemorrhage; hyaline, fatty and mucinous degeneration; and microcystic changes.

• #36 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Historically known as acoustic neuromas, these tumors have been demonstrated not to involve the acoustic (cochlear) nerve in most cases, nor to be of neuroglial origin. Instead, they originate from Schwann cells, and they can occur anywhere lateral to the glial-schwannian junction. […] Two different tissue types may be present in vestibular schwannomas: Antoni type A tissue, which is compact and ordered with a palisading architecture, and Antoni type B tissue, which is myxoid and loose. Histological studies have shown no correlation between dominance of Antoni A or B tissue in either cystic or solid vestibular schwannomas. […] The rapid growth of cystic VS is attributed to expansion of the cystic component. On histological studies, the Ki-67 index of cystic VS is either lower or not significantly different to that of solid VS. Several mechanisms by which the cystic component grows have been proposed: extravasation of serum proteins from impaired blood-tumor barrier; protein secretion from tumor cells; isolated or repeated intratumoral microhemorrhage; hyaline, fatty and mucinous degeneration; and microcystic changes.

• #37 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Distinct areas designated as Antoni A and B are seen. Antoni A areas are densely cellular and interwoven, whereas Antoni B areas are less cellular, less structured, and often contain areas of microcyst formation and hemorrhage. […] The distal location of the glia to Schwann cell transition in the vestibular nerves distinguishes them from other cranial nerves and has been thought to predispose them to neoplastic transformation. […] This theory has been questioned, and the incidence of vestibular tumors remains unexplained. […] Specific loss of genetic material on chromosome 22 was a feature of 44% of informative cases of sporadic vestibular tumors when analyzed by the RFLP approach. […] This loss has been attributed to partial deletion or mitotic recombination. […] Further work with schwannomas from multiple locations narrowed the region of interest to an area between the CRYB2A and the MB loci, but cloning of the candidate gene came from analysis of neurofibromatosis type 2 cases.

• #38 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Distinct areas designated as Antoni A and B are seen. Antoni A areas are densely cellular and interwoven, whereas Antoni B areas are less cellular, less structured, and often contain areas of microcyst formation and hemorrhage. […] The distal location of the glia to Schwann cell transition in the vestibular nerves distinguishes them from other cranial nerves and has been thought to predispose them to neoplastic transformation. […] This theory has been questioned, and the incidence of vestibular tumors remains unexplained. […] Specific loss of genetic material on chromosome 22 was a feature of 44% of informative cases of sporadic vestibular tumors when analyzed by the RFLP approach. […] This loss has been attributed to partial deletion or mitotic recombination. […] Further work with schwannomas from multiple locations narrowed the region of interest to an area between the CRYB2A and the MB loci, but cloning of the candidate gene came from analysis of neurofibromatosis type 2 cases.

• #39 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Distinct areas designated as Antoni A and B are seen. Antoni A areas are densely cellular and interwoven, whereas Antoni B areas are less cellular, less structured, and often contain areas of microcyst formation and hemorrhage. […] The distal location of the glia to Schwann cell transition in the vestibular nerves distinguishes them from other cranial nerves and has been thought to predispose them to neoplastic transformation. […] This theory has been questioned, and the incidence of vestibular tumors remains unexplained. […] Specific loss of genetic material on chromosome 22 was a feature of 44% of informative cases of sporadic vestibular tumors when analyzed by the RFLP approach. […] This loss has been attributed to partial deletion or mitotic recombination. […] Further work with schwannomas from multiple locations narrowed the region of interest to an area between the CRYB2A and the MB loci, but cloning of the candidate gene came from analysis of neurofibromatosis type 2 cases.

• #40 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Childhood radiation to the head and neck has also been linked to increased risk of vestibular schwannoma. […] Inactivating mutations in the NF2 gene were found more commonly in the tumor specimens of smokers than in never smokers. […] At this time there is no definitive evidence for a genetic/molecular signature suggestive of radiation-related vestibular schwannoma. […] An inherited but nonsyndromic contribution to sporadic vestibular schwannoma has been suggested by genealogic studies in veterans and residents of Utah. […] The tumors are variegated in color, with areas of gray, red, and yellow corresponding to areas of dense cellularity, increased vascularity, and xanthomatous degeneration. […] Histologically, the tumors are formed of interweaving bundles of spindle-shaped cells with cigar-shaped nuclei.

• #41 Pathology of vestibular schwannoma – Louis Hofmeyr

  https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/pathology-of-vestibular-schwannoma/

  A vestibular schwannoma, also known as an acoustic neuroma, is a benign tumour that typically arises from the Schwann cells of the vestibular portion of the eighth cranial nerve. […] The presence of Antoni B areas in vestibular schwannomas is also associated with decreased cellularity, degeneration, and cyst formation within the tumour. […] SOX10 is involved in developing and maintaining Schwann cells, which are the cells responsible for forming the myelin sheath around nerves. […] Over time, microscopic ancient change of vestibular schwannoma can occur where the tumour becomes more fibrous and less cellular.

• #42 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?lang=us

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumors that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumors (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumors arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] A number of other possibilities have been proposed including perilymphatic CSF flow dysfunction, neurovascular compromise, or altered blood-labyrinth barrier permeability. […] Immunohistochemical staining is usually positive for S-100 protein.

• #43 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.icoradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=gb

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumours that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumours (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumours arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] They can display two types of growth patterns: Antoni A and Antoni B. […] Immunohistochemical staining is usually positive for S-100 protein.

• #44 Pathology of vestibular schwannoma – Louis Hofmeyr

  https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/pathology-of-vestibular-schwannoma/

  A vestibular schwannoma, also known as an acoustic neuroma, is a benign tumour that typically arises from the Schwann cells of the vestibular portion of the eighth cranial nerve. […] The presence of Antoni B areas in vestibular schwannomas is also associated with decreased cellularity, degeneration, and cyst formation within the tumour. […] SOX10 is involved in developing and maintaining Schwann cells, which are the cells responsible for forming the myelin sheath around nerves. […] Over time, microscopic ancient change of vestibular schwannoma can occur where the tumour becomes more fibrous and less cellular.

• #45 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Historically known as acoustic neuromas, these tumors have been demonstrated not to involve the acoustic (cochlear) nerve in most cases, nor to be of neuroglial origin. Instead, they originate from Schwann cells, and they can occur anywhere lateral to the glial-schwannian junction. […] Two different tissue types may be present in vestibular schwannomas: Antoni type A tissue, which is compact and ordered with a palisading architecture, and Antoni type B tissue, which is myxoid and loose. Histological studies have shown no correlation between dominance of Antoni A or B tissue in either cystic or solid vestibular schwannomas. […] The rapid growth of cystic VS is attributed to expansion of the cystic component. On histological studies, the Ki-67 index of cystic VS is either lower or not significantly different to that of solid VS. Several mechanisms by which the cystic component grows have been proposed: extravasation of serum proteins from impaired blood-tumor barrier; protein secretion from tumor cells; isolated or repeated intratumoral microhemorrhage; hyaline, fatty and mucinous degeneration; and microcystic changes.

• #46 Pathology of vestibular schwannoma – Louis Hofmeyr

  https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/pathology-of-vestibular-schwannoma/

  A vestibular schwannoma, also known as an acoustic neuroma, is a benign tumour that typically arises from the Schwann cells of the vestibular portion of the eighth cranial nerve. […] The presence of Antoni B areas in vestibular schwannomas is also associated with decreased cellularity, degeneration, and cyst formation within the tumour. […] SOX10 is involved in developing and maintaining Schwann cells, which are the cells responsible for forming the myelin sheath around nerves. […] Over time, microscopic ancient change of vestibular schwannoma can occur where the tumour becomes more fibrous and less cellular.

• #47 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Although some tumors adhere to one or another of these growth patterns, others appear to alternate between periods of no or slow growth and rapid growth. Tumors that have undergone cystic degeneration (presumably because they have outgrown their blood supply) are sometimes capable of relatively rapid expansion because of enlargement of their cystic component. […] Because acoustic tumors arise from the investing Schwann cell, tumor growth generally compresses vestibular fibers on the surface. Destruction of vestibular fibers is slow; consequently, many patients experience little or no disequilibrium or vertigo. Once the tumor has grown sufficiently large to fill the internal auditory canal, it may continue growth either by expanding bone or by extending into the cerebellopontine angle. Growth within the cerebellopontine angle is generally spherical, which is different than the sessile growth pattern seen in a meningioma of the cerebellopontine angle.

• #48 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic neuromas (also known as vestibular schwannomas) are non-cancerous intracranial, extra-axial tumors that arise from Schwann cells. Schwann cells myelinate and insulate peripheral nerves, aiding in both nerve conduction and axonal regeneration. […] In the case of acoustic neuromas/vestibular schwannomas, a benign tumor arises from the Schwann cells surrounding one of the vestibular nerves or, less commonly, the cochlear nerve. […] The vast majority of acoustic neuromas develop from the Schwann cells investing the vestibular portion of the vestibulocochlear nerve. Less than 5% of tumors arise from the cochlear nerve. The superior and inferior vestibular nerves appear to be the nerves of origin with about equal frequency. Overall, three separate growth patterns can be distinguished within acoustic tumors, as follows: (1) no growth or very slow growth, (2) slow growth (ie, 2 mm/y), and (3) fast growth (ie, 1.0 cm/y on imaging studies). Although most acoustic neuromas grow slowly, rarely, a tumor may grow quickly and may double in volume within 6 months to a year.

• #49 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Although some tumors adhere to one or another of these growth patterns, others appear to alternate between periods of no or slow growth and rapid growth. Tumors that have undergone cystic degeneration (presumably because they have outgrown their blood supply) are sometimes capable of relatively rapid expansion because of enlargement of their cystic component. […] Because acoustic tumors arise from the investing Schwann cell, tumor growth generally compresses vestibular fibers on the surface. Destruction of vestibular fibers is slow; consequently, many patients experience little or no disequilibrium or vertigo. Once the tumor has grown sufficiently large to fill the internal auditory canal, it may continue growth either by expanding bone or by extending into the cerebellopontine angle. Growth within the cerebellopontine angle is generally spherical, which is different than the sessile growth pattern seen in a meningioma of the cerebellopontine angle.

• #50 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Although some tumors adhere to one or another of these growth patterns, others appear to alternate between periods of no or slow growth and rapid growth. Tumors that have undergone cystic degeneration (presumably because they have outgrown their blood supply) are sometimes capable of relatively rapid expansion because of enlargement of their cystic component. […] Because acoustic tumors arise from the investing Schwann cell, tumor growth generally compresses vestibular fibers on the surface. Destruction of vestibular fibers is slow; consequently, many patients experience little or no disequilibrium or vertigo. Once the tumor has grown sufficiently large to fill the internal auditory canal, it may continue growth either by expanding bone or by extending into the cerebellopontine angle. Growth within the cerebellopontine angle is generally spherical, which is different than the sessile growth pattern seen in a meningioma of the cerebellopontine angle.

• #51 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Historically known as acoustic neuromas, these tumors have been demonstrated not to involve the acoustic (cochlear) nerve in most cases, nor to be of neuroglial origin. Instead, they originate from Schwann cells, and they can occur anywhere lateral to the glial-schwannian junction. […] Two different tissue types may be present in vestibular schwannomas: Antoni type A tissue, which is compact and ordered with a palisading architecture, and Antoni type B tissue, which is myxoid and loose. Histological studies have shown no correlation between dominance of Antoni A or B tissue in either cystic or solid vestibular schwannomas. […] The rapid growth of cystic VS is attributed to expansion of the cystic component. On histological studies, the Ki-67 index of cystic VS is either lower or not significantly different to that of solid VS. Several mechanisms by which the cystic component grows have been proposed: extravasation of serum proteins from impaired blood-tumor barrier; protein secretion from tumor cells; isolated or repeated intratumoral microhemorrhage; hyaline, fatty and mucinous degeneration; and microcystic changes.

• #52 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic tumors, like other space-occupying lesions, produce symptoms by any of four recognizable mechanisms: (1) compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3) compression of vessels producing ischemia or infarction, or (4) compression and/or attenuation of nerves. […] Because the cerebellopontine angle is relatively empty, tumors can continue to grow, reaching 2 cm before they contact important structures such as other cranial nerves or the brain stem. Growth is often sufficiently slow that the facial nerve can accommodate to the stretching imposed by tumor growth without clinically apparent deterioration of function. Tumors that arise within the internal auditory canal may produce early symptoms in the form of hearing loss or vestibular disturbance by compressing the cochlear nerve, vestibular nerve, or labyrinthine artery against the bony walls of the internal auditory canal.

• #53 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic tumors, like other space-occupying lesions, produce symptoms by any of four recognizable mechanisms: (1) compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3) compression of vessels producing ischemia or infarction, or (4) compression and/or attenuation of nerves. […] Because the cerebellopontine angle is relatively empty, tumors can continue to grow, reaching 2 cm before they contact important structures such as other cranial nerves or the brain stem. Growth is often sufficiently slow that the facial nerve can accommodate to the stretching imposed by tumor growth without clinically apparent deterioration of function. Tumors that arise within the internal auditory canal may produce early symptoms in the form of hearing loss or vestibular disturbance by compressing the cochlear nerve, vestibular nerve, or labyrinthine artery against the bony walls of the internal auditory canal.

• #54 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic tumors, like other space-occupying lesions, produce symptoms by any of four recognizable mechanisms: (1) compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3) compression of vessels producing ischemia or infarction, or (4) compression and/or attenuation of nerves. […] Because the cerebellopontine angle is relatively empty, tumors can continue to grow, reaching 2 cm before they contact important structures such as other cranial nerves or the brain stem. Growth is often sufficiently slow that the facial nerve can accommodate to the stretching imposed by tumor growth without clinically apparent deterioration of function. Tumors that arise within the internal auditory canal may produce early symptoms in the form of hearing loss or vestibular disturbance by compressing the cochlear nerve, vestibular nerve, or labyrinthine artery against the bony walls of the internal auditory canal.

• #55 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic tumors, like other space-occupying lesions, produce symptoms by any of four recognizable mechanisms: (1) compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3) compression of vessels producing ischemia or infarction, or (4) compression and/or attenuation of nerves. […] Because the cerebellopontine angle is relatively empty, tumors can continue to grow, reaching 2 cm before they contact important structures such as other cranial nerves or the brain stem. Growth is often sufficiently slow that the facial nerve can accommodate to the stretching imposed by tumor growth without clinically apparent deterioration of function. Tumors that arise within the internal auditory canal may produce early symptoms in the form of hearing loss or vestibular disturbance by compressing the cochlear nerve, vestibular nerve, or labyrinthine artery against the bony walls of the internal auditory canal.

• #56 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Acoustic tumors, like other space-occupying lesions, produce symptoms by any of four recognizable mechanisms: (1) compression or distortion of the spinal fluid spaces, (2) displacement of the brain stem, (3) compression of vessels producing ischemia or infarction, or (4) compression and/or attenuation of nerves. […] Because the cerebellopontine angle is relatively empty, tumors can continue to grow, reaching 2 cm before they contact important structures such as other cranial nerves or the brain stem. Growth is often sufficiently slow that the facial nerve can accommodate to the stretching imposed by tumor growth without clinically apparent deterioration of function. Tumors that arise within the internal auditory canal may produce early symptoms in the form of hearing loss or vestibular disturbance by compressing the cochlear nerve, vestibular nerve, or labyrinthine artery against the bony walls of the internal auditory canal.

• #57 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  Hearing loss (HL) is the most common and heterogeneous disorder of the sensory system, with a large morbidity in the worldwide population. […] Notably, a benign SC-derived tumor of the acoustic nerve, named vestibular schwannoma (VS), has been indicated as cause of HL. […] 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. […] The first step of the pathologic process leading to SNHL, with SGN degeneration, entails a rapid and extensive loss of the unmyelinated axonal processes within the organ of Corti, followed by a gradual degeneration of the myelinated portion of the peripheral axons (within the osseous spiral lamina), and of the SGN soma inside the Rosenthals canal.

• #58 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  From a biomolecular point of view, the etiology of sporadic VS as well as that of NF2 is ascribable to the loss of function of the protein named merlin. […] However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] The failure of SCs capacity to dedifferentiate into the stage of repair/myelinating cells is likely due to a heterozygous NF2 mutation either in the neuronal or in the SCs compartment, which in turn leads to the development of VS. […] The VS compresses mechanically the auditory nerve or the labyrinthine artery, producing a mechanical damage and leading to the unilateral SNHL. […] In accordance to the failure-of-nerve regeneration theory, the VS preferentially develops in a location prone to the nerve injury, given by compression or physical trauma.

• #59 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  The resulting SCs demyelination determines an increase of the neural membrane capacitance, reducing the capability of a neuron in initiating and propagating action potentials in response to electrical stimuli, and leading to a delayed nerve excitation. […] The SCs surrounding the SGNs, indeed, mostly myelinate these neurons and are a source for the neurotrophic support (i.e., producing growth factors like brain derived neurotrophic factor, BDNF, or neurotrophin-3, NT-3) of SGNs. […] Overall, SCs modify their physiologic state responding to other growth factors, including the fibroblast growth factor (FGF). […] In this light, it was assumed that SCs in the injured acoustic nerve can re-differentiate in regenerative cells, although their clear involvement in the pathogenesis of HL has not been so far investigated and is matter of discussion in this review.

• #60 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  The resulting SCs demyelination determines an increase of the neural membrane capacitance, reducing the capability of a neuron in initiating and propagating action potentials in response to electrical stimuli, and leading to a delayed nerve excitation. […] The SCs surrounding the SGNs, indeed, mostly myelinate these neurons and are a source for the neurotrophic support (i.e., producing growth factors like brain derived neurotrophic factor, BDNF, or neurotrophin-3, NT-3) of SGNs. […] Overall, SCs modify their physiologic state responding to other growth factors, including the fibroblast growth factor (FGF). […] In this light, it was assumed that SCs in the injured acoustic nerve can re-differentiate in regenerative cells, although their clear involvement in the pathogenesis of HL has not been so far investigated and is matter of discussion in this review.

• #61 Vestibular schwannoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vestibular-schwannoma?lang=us

  Vestibular schwannomas, also known as acoustic neuromas, are relatively common tumors that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle (CPA) masses. […] Vestibular schwannomas are benign tumors (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII). […] They were classically described as originating near the transition zone between glial and Schwann cells but contemporary data suggests they can originate at any point along the nerve. […] In over 90% of cases, these tumors arise from the inferior division of the vestibular nerve. […] Less than 5% cases arise from the cochlear component of the vestibulocochlear nerve (CN VIII). […] A number of other possibilities have been proposed including perilymphatic CSF flow dysfunction, neurovascular compromise, or altered blood-labyrinth barrier permeability. […] Immunohistochemical staining is usually positive for S-100 protein.

• #62 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  Hearing loss (HL) is the most common and heterogeneous disorder of the sensory system, with a large morbidity in the worldwide population. […] Notably, a benign SC-derived tumor of the acoustic nerve, named vestibular schwannoma (VS), has been indicated as cause of HL. […] 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. […] The first step of the pathologic process leading to SNHL, with SGN degeneration, entails a rapid and extensive loss of the unmyelinated axonal processes within the organ of Corti, followed by a gradual degeneration of the myelinated portion of the peripheral axons (within the osseous spiral lamina), and of the SGN soma inside the Rosenthals canal.

• #63

  https://www.karmanos.org/karmanos/acoustic-neuroma-treated-at-karmanos

  A vestibular schwannoma (also known as acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) is a benign, usually slow-growing tumor that develops from the balance and hearing nerves supplying the inner ear. The tumor comes from an overproduction of Schwann cells the cells that normally wrap around nerve fibers like onion skin to help support and insulate nerves. […] As the vestibular schwannoma grows, it affects the hearing and balance nerves, usually causing unilateral (one-sided) or asymmetric hearing loss, tinnitus (ringing in the ear), and dizziness/loss of balance. […] If the tumor becomes large, it will eventually press against nearby brain structures (such as the brainstem and the cerebellum), becoming life-threatening.

• #64 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  Although some tumors adhere to one or another of these growth patterns, others appear to alternate between periods of no or slow growth and rapid growth. Tumors that have undergone cystic degeneration (presumably because they have outgrown their blood supply) are sometimes capable of relatively rapid expansion because of enlargement of their cystic component. […] Because acoustic tumors arise from the investing Schwann cell, tumor growth generally compresses vestibular fibers on the surface. Destruction of vestibular fibers is slow; consequently, many patients experience little or no disequilibrium or vertigo. Once the tumor has grown sufficiently large to fill the internal auditory canal, it may continue growth either by expanding bone or by extending into the cerebellopontine angle. Growth within the cerebellopontine angle is generally spherical, which is different than the sessile growth pattern seen in a meningioma of the cerebellopontine angle.

• #65 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  As the tumor grows beyond 2 cm, it can begin to compress the lateral surface of the brain stem. Further growth can occur by compressing or displacing the brain stem, which can result in hydrocephalus by compression of the fourth ventricle. The growing tumor can also extend sufficiently far anteriorly to compress the trigeminal nerve and produce facial hypoesthesia.

• #66 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  As the tumor grows beyond 2 cm, it can begin to compress the lateral surface of the brain stem. Further growth can occur by compressing or displacing the brain stem, which can result in hydrocephalus by compression of the fourth ventricle. The growing tumor can also extend sufficiently far anteriorly to compress the trigeminal nerve and produce facial hypoesthesia.

• #67 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology

  https://emedicine.medscape.com/article/882876-overview

  As the tumor grows beyond 2 cm, it can begin to compress the lateral surface of the brain stem. Further growth can occur by compressing or displacing the brain stem, which can result in hydrocephalus by compression of the fourth ventricle. The growing tumor can also extend sufficiently far anteriorly to compress the trigeminal nerve and produce facial hypoesthesia.

• #68 Vestibular schwannomas: A Review | Applied Radiology

  https://appliedradiology.com/articles/vestibular-schwannomas-a-review

  Historically known as acoustic neuromas, these tumors have been demonstrated not to involve the acoustic (cochlear) nerve in most cases, nor to be of neuroglial origin. Instead, they originate from Schwann cells, and they can occur anywhere lateral to the glial-schwannian junction. […] Two different tissue types may be present in vestibular schwannomas: Antoni type A tissue, which is compact and ordered with a palisading architecture, and Antoni type B tissue, which is myxoid and loose. Histological studies have shown no correlation between dominance of Antoni A or B tissue in either cystic or solid vestibular schwannomas. […] The rapid growth of cystic VS is attributed to expansion of the cystic component. On histological studies, the Ki-67 index of cystic VS is either lower or not significantly different to that of solid VS. Several mechanisms by which the cystic component grows have been proposed: extravasation of serum proteins from impaired blood-tumor barrier; protein secretion from tumor cells; isolated or repeated intratumoral microhemorrhage; hyaline, fatty and mucinous degeneration; and microcystic changes.

• #69 Acoustic Neuroma (Vestibular Schwannoma): Surgery, Treatment & Symptoms | Skull Base Institute

  http://www.skullbaseinstitute.com/acoustic-neuroma/

  Symptoms and signs associated with acoustic neuromas have been well known for over 150 years and are due to direct tumor compression. […] These tumors can reach a remarkable size causing severe compression of the brain stem, the cerebellum and the surrounding cranial nerves such as the seventh (facial) or fifth (trigeminal) nerves, they can induce bony changes with invasion of the surrounding pneumatic cells and marrow spaces and may lead to a life threatening condition. […] The prognosis for someone with a unilateral acoustic neuroma is usually quite good provided the tumor is diagnosed early and appropriate treatment is instituted. In spite of the fact that an acoustic neuroma is a benign tumor, it can still be fatal if left untreated.

• #70 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  Somatic alterations in the NF2 gene are critical factors in the pathogenesis of sporadic vestibular schwannomas. […] It has been revealed that somatic mutations in both alleles of this gene trigger tumorigenesis in vestibular schwannoma. […] The mutation or inactivation of the NF2 gene is important for the tumorigenesis of sporadic vestibular schwannoma. […] Understanding the molecular mechanisms underlying the development of sporadic vestibular schwannoma may assist in developing new drug therapies. […] The mechanism of neurological damage, however, has not yet been clarified, making it difficult to predict the progression of hearing loss or sudden hearing loss caused by vestibular schwannoma.

• #71 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  From a biomolecular point of view, the etiology of sporadic VS as well as that of NF2 is ascribable to the loss of function of the protein named merlin. […] However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] The failure of SCs capacity to dedifferentiate into the stage of repair/myelinating cells is likely due to a heterozygous NF2 mutation either in the neuronal or in the SCs compartment, which in turn leads to the development of VS. […] The VS compresses mechanically the auditory nerve or the labyrinthine artery, producing a mechanical damage and leading to the unilateral SNHL. […] In accordance to the failure-of-nerve regeneration theory, the VS preferentially develops in a location prone to the nerve injury, given by compression or physical trauma.

• #72 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  The occurrence of vestibular schwannomas and meningiomas in association with the loss of function of a gene suggests that the neurofibromatosis type 2 gene is a tumor suppressor gene involved with the growth control of cells of neural crest origin. […] The protein is thought to exist in both an unfolded and a folded form, with the latter mediating the tumor suppressor activity. […] The protein PAK phosphorylates merlin at S518, disrupting the maintenance of the folded state and tumor suppressor function. […] The NF2 gene product inhibits directly the kinase PAK1, which is essential for ras transformation, and may be a target for therapy in NF2. […] Merlin has been shown to be a negative regulator of the mammalian target of rapamycin complex 1 (mTORC1) with elevated mTORC1 signaling in NF2 tumors. […] The role of micro RNAs (miRNAs) in schwannoma development is also being investigated.

• #73 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  The occurrence of vestibular schwannomas and meningiomas in association with the loss of function of a gene suggests that the neurofibromatosis type 2 gene is a tumor suppressor gene involved with the growth control of cells of neural crest origin. […] The protein is thought to exist in both an unfolded and a folded form, with the latter mediating the tumor suppressor activity. […] The protein PAK phosphorylates merlin at S518, disrupting the maintenance of the folded state and tumor suppressor function. […] The NF2 gene product inhibits directly the kinase PAK1, which is essential for ras transformation, and may be a target for therapy in NF2. […] Merlin has been shown to be a negative regulator of the mammalian target of rapamycin complex 1 (mTORC1) with elevated mTORC1 signaling in NF2 tumors. […] The role of micro RNAs (miRNAs) in schwannoma development is also being investigated.

• #74 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  The occurrence of vestibular schwannomas and meningiomas in association with the loss of function of a gene suggests that the neurofibromatosis type 2 gene is a tumor suppressor gene involved with the growth control of cells of neural crest origin. […] The protein is thought to exist in both an unfolded and a folded form, with the latter mediating the tumor suppressor activity. […] The protein PAK phosphorylates merlin at S518, disrupting the maintenance of the folded state and tumor suppressor function. […] The NF2 gene product inhibits directly the kinase PAK1, which is essential for ras transformation, and may be a target for therapy in NF2. […] Merlin has been shown to be a negative regulator of the mammalian target of rapamycin complex 1 (mTORC1) with elevated mTORC1 signaling in NF2 tumors. […] The role of micro RNAs (miRNAs) in schwannoma development is also being investigated.

• #75 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  The occurrence of vestibular schwannomas and meningiomas in association with the loss of function of a gene suggests that the neurofibromatosis type 2 gene is a tumor suppressor gene involved with the growth control of cells of neural crest origin. […] The protein is thought to exist in both an unfolded and a folded form, with the latter mediating the tumor suppressor activity. […] The protein PAK phosphorylates merlin at S518, disrupting the maintenance of the folded state and tumor suppressor function. […] The NF2 gene product inhibits directly the kinase PAK1, which is essential for ras transformation, and may be a target for therapy in NF2. […] Merlin has been shown to be a negative regulator of the mammalian target of rapamycin complex 1 (mTORC1) with elevated mTORC1 signaling in NF2 tumors. […] The role of micro RNAs (miRNAs) in schwannoma development is also being investigated.

• #76 Vestibular Schwannoma: What We Know and Where We are Heading

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7669921/

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. […] Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. […] Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. […] This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #77

  https://link.springer.com/article/10.1007/s12105-020-01155-x

  Mutations to NF2, a tumour suppressor gene on chromosome 22, play a vital role in the development of both sporadic and NF2-related disease. Inactivation of the NF2 protein product, Merlin (schwannomin), leads to deregulation of various intracellular signalling pathways such as Rac1, Ras, PAK1, and mTORC1. Inactivation of other tumour suppressor genes including LZTR1, SMARCB1, and COQ6 are also linked to schwannoma development. […] Although the role of NF2 mutations was reinforced by recent large-scale sequencing studies, there are data to suggest that NF2-associated VS has a different, polyclonal mutation pattern. This has been postulated to account for variance in treatment outcomes as compared to sporadic VS.

• #78 The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies | Acoustic Neuroma Association of Canada

  https://www.anac.ca/the-current-landscape-of-vestibular-schwannoma-therapy-development-of-novel-targeted-therapies

  An acoustic neuroma, also known as a vestibular schwannoma, is a benign tumour that arises from Schwann cells of the vestibulocochlear nerve. […] The loss of the NF2 gene is linked to pathogenesis of acoustic neuromas, both in sporadic and genetic conditions. About 60% of sporadic unilateral acoustic neuromas have mutations in the NF2 gene. Mutations of the NF2 gene lead to deregulation of important pathways that regulate tumour growth such as RAS and mTORC1 pathway. […] Further work is needed to understand the biological alterations that are driving the tumour development, in order to better develop targeted therapies that will effectively treat this disease process.

• #79 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #80 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #81 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #82 Acoustic Neuroma (aka acoustic schwannoma, vestibular schwannoma) » Lillian S. Wells Department of Neurosurgery at the University of Florida » College of Medicine » University of Florida

  https://neurosurgery.ufl.edu/patient-care/diseases-conditions/acoustic-neuroma/

  Acoustic Neuromas are benign slow growing tumors arising on the nerves of hearing and balance. […] The common name is a misnomer as this tumor most commonly originates from vestibular nerve, a component of the vestibulo-cochlear nerve bundle, also known as the eighth cranial nerve. […] Additionally, the tumor type is schwannoma, rather than a neuroma. […] Thus, the correct name is vestibular schwannoma. […] Over the past decade, another treatment approach, called radiosurgery, has become increasingly popular. […] Long term tumor control rates are quite high and the risk is very low. […] Over the past several years, a third treatment approach, a medical therapy using an intravenous medication called Avastin (Bevacizumab), is being used in certain patients. […] In several small studies, this medication has been shown to improve hearing, reduce tumor size, and delay the time until need for open surgery in certain patients.

• #83 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  The occurrence of vestibular schwannomas and meningiomas in association with the loss of function of a gene suggests that the neurofibromatosis type 2 gene is a tumor suppressor gene involved with the growth control of cells of neural crest origin. […] The protein is thought to exist in both an unfolded and a folded form, with the latter mediating the tumor suppressor activity. […] The protein PAK phosphorylates merlin at S518, disrupting the maintenance of the folded state and tumor suppressor function. […] The NF2 gene product inhibits directly the kinase PAK1, which is essential for ras transformation, and may be a target for therapy in NF2. […] Merlin has been shown to be a negative regulator of the mammalian target of rapamycin complex 1 (mTORC1) with elevated mTORC1 signaling in NF2 tumors. […] The role of micro RNAs (miRNAs) in schwannoma development is also being investigated.

• #84 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  The resulting SCs demyelination determines an increase of the neural membrane capacitance, reducing the capability of a neuron in initiating and propagating action potentials in response to electrical stimuli, and leading to a delayed nerve excitation. […] The SCs surrounding the SGNs, indeed, mostly myelinate these neurons and are a source for the neurotrophic support (i.e., producing growth factors like brain derived neurotrophic factor, BDNF, or neurotrophin-3, NT-3) of SGNs. […] Overall, SCs modify their physiologic state responding to other growth factors, including the fibroblast growth factor (FGF). […] In this light, it was assumed that SCs in the injured acoustic nerve can re-differentiate in regenerative cells, although their clear involvement in the pathogenesis of HL has not been so far investigated and is matter of discussion in this review.

• #85 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  As reported above, SCs alterations in the auditory system have been hypothesized as one of the possible causes of HL or HHL. […] The SCs have been implicated also in the origin of some kinds of tumors, likely through a partial EMT process. […] The VS, also named acoustic neurinoma, is a benign SC-derived tumor of the vestibulocochlear nerve (VIII cranial nerve), coming along the internal auditory canal or the cerebellopontine angle. […] Histologically, the VS is mostly composed by SCs, showing some typical microscopic features: biphasic architecture (Antoni A and Antoni B patterns), nuclear palisading (Verocay bodies), fibrous capsule with displaced parent nerve and degenerative signs, such as hyalinized vessels, nuclear pleomorphism, hemosiderin deposition. […] Several mechanisms and factors could at the base of VS onset and progression.

• #86 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  From a biomolecular point of view, the etiology of sporadic VS as well as that of NF2 is ascribable to the loss of function of the protein named merlin. […] However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] The failure of SCs capacity to dedifferentiate into the stage of repair/myelinating cells is likely due to a heterozygous NF2 mutation either in the neuronal or in the SCs compartment, which in turn leads to the development of VS. […] The VS compresses mechanically the auditory nerve or the labyrinthine artery, producing a mechanical damage and leading to the unilateral SNHL. […] In accordance to the failure-of-nerve regeneration theory, the VS preferentially develops in a location prone to the nerve injury, given by compression or physical trauma.

• #87 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  From a biomolecular point of view, the etiology of sporadic VS as well as that of NF2 is ascribable to the loss of function of the protein named merlin. […] However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] The failure of SCs capacity to dedifferentiate into the stage of repair/myelinating cells is likely due to a heterozygous NF2 mutation either in the neuronal or in the SCs compartment, which in turn leads to the development of VS. […] The VS compresses mechanically the auditory nerve or the labyrinthine artery, producing a mechanical damage and leading to the unilateral SNHL. […] In accordance to the failure-of-nerve regeneration theory, the VS preferentially develops in a location prone to the nerve injury, given by compression or physical trauma.

• #88 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  Vestibular schwannoma is a clinically benign schwannoma that arises from the vestibulocochlear nerve that causes sensorineural hearing loss. […] Despite being a benign tumor, its management is difficult and controversial due to the potential serious complications, such as irreversible sensorineural hearing loss, of current interventions. […] Improvements have been reported recently in the treatment of vestibular schwannomas. […] Precise genomic analysis of the tumor would be helpful in determining the characteristics of the tumor for each patient, leading to a better hearing prognosis. […] This review summarizes recent advances in vestibular schwannoma management and treatment, especially in hearing preservation. […] In addition, recent advances in the understanding of the molecular mechanisms underlying vestibular schwannomas and how these advances can be applied in clinical practice are outlined and discussed, respectively.

• #89 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #90 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #91 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #92 Vestibular Schwannomas or Acoustic Neuromas by Another Name | Canadian Audiologist

  https://canadianaudiologist.ca/vestibular-schwannomas-or-acoustic-neuromas-by-another-name/

  While medical therapy does not play an active role yet in the management of VSs there has been interest in molecules such as bevacizumab (a neutralizing anti-vascular endothelial growth factor antibody) and imatinib (that affects platelet derived growth factor receptors) in the hopes that they might slow tumour growth.

• #93 Vestibular Schwannomas or Acoustic Neuromas by Another Name | Canadian Audiologist

  https://canadianaudiologist.ca/vestibular-schwannomas-or-acoustic-neuromas-by-another-name/

  While medical therapy does not play an active role yet in the management of VSs there has been interest in molecules such as bevacizumab (a neutralizing anti-vascular endothelial growth factor antibody) and imatinib (that affects platelet derived growth factor receptors) in the hopes that they might slow tumour growth.

• #94 Vestibular schwannoma – Wikipedia

  https://en.wikipedia.org/wiki/Vestibular_schwannoma

  The complexity of the molecular biology research involved is truly challenging. Clinical trials are in progress for other drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk therapeutic option, but emerging evidence suggests that aspirin and other NSAID use may not prevent VS tumor growth. […] A key step forward in 1993 was the identification of the NF2 gene and its protein product Merlin, which modulates the complex molecular signaling pathways that control cell proliferation. These pathways that drive VS formation (tumorigenesis) and growth are currently under investigation. […] A second important field of study in molecular biology investigates ways to stop the formation (angiogenesis) of the new blood vessels that are needed to support tumor growth by supplying nutrients and oxygen. In 1998, the glycoprotein named VEGF (vascular endothelial growth factor) that initiates proliferation was discovered. An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged treatment resulting in hypertension and impaired wound healing.

• #95 The Current Landscape of Vestibular Schwannoma Therapy: Development of Novel Targeted Therapies | Acoustic Neuroma Association of Canada

  https://www.anac.ca/the-current-landscape-of-vestibular-schwannoma-therapy-development-of-novel-targeted-therapies

  An acoustic neuroma, also known as a vestibular schwannoma, is a benign tumour that arises from Schwann cells of the vestibulocochlear nerve. […] The loss of the NF2 gene is linked to pathogenesis of acoustic neuromas, both in sporadic and genetic conditions. About 60% of sporadic unilateral acoustic neuromas have mutations in the NF2 gene. Mutations of the NF2 gene lead to deregulation of important pathways that regulate tumour growth such as RAS and mTORC1 pathway. […] Further work is needed to understand the biological alterations that are driving the tumour development, in order to better develop targeted therapies that will effectively treat this disease process.

• #96 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  Vestibular schwannoma is a clinically benign schwannoma that arises from the vestibulocochlear nerve that causes sensorineural hearing loss. […] Despite being a benign tumor, its management is difficult and controversial due to the potential serious complications, such as irreversible sensorineural hearing loss, of current interventions. […] Improvements have been reported recently in the treatment of vestibular schwannomas. […] Precise genomic analysis of the tumor would be helpful in determining the characteristics of the tumor for each patient, leading to a better hearing prognosis. […] This review summarizes recent advances in vestibular schwannoma management and treatment, especially in hearing preservation. […] In addition, recent advances in the understanding of the molecular mechanisms underlying vestibular schwannomas and how these advances can be applied in clinical practice are outlined and discussed, respectively.

• #97 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  Somatic alterations in the NF2 gene are critical factors in the pathogenesis of sporadic vestibular schwannomas. […] It has been revealed that somatic mutations in both alleles of this gene trigger tumorigenesis in vestibular schwannoma. […] The mutation or inactivation of the NF2 gene is important for the tumorigenesis of sporadic vestibular schwannoma. […] Understanding the molecular mechanisms underlying the development of sporadic vestibular schwannoma may assist in developing new drug therapies. […] The mechanism of neurological damage, however, has not yet been clarified, making it difficult to predict the progression of hearing loss or sudden hearing loss caused by vestibular schwannoma.

• #98 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease

  https://www.nature.com/articles/s41419-023-06141-z

  The onset of HL is frequently unpredictable, since not all VS cause profound HL. […] The surgical treatment, even representing the most common approach among the therapies for VS, should be considered as a high-risk morbidity for HL. […] Emerging treatments for VS are mostly addressed to target SCs, restraining the molecular pathways downstream merlin and/or reducing tumor growth.

• #99 Vestibular schwannoma: evolution of diagnosis and treatment | Egyptian Journal of Neurosurgery | Full Text

  https://ejns.springeropen.com/articles/10.1186/s41984-025-00349-1

  Vestibular Schwannomas are benign tumors of the vestibular branch of the eight cranial nerves usually seen in adults who present with sensorineural hearing loss and tinnitus. […] The tumors may originate anywhere along the path of the eight cranial nerves from the internal acoustic meatus to proximity of the brainstem growing into the Cerebellopontine Angle (CPA) in variable presentation and unpredictable ways, often compressing the adjacent vital structures. […] The molecular hallmark for sporadic and NF2-associated VS tumor is believed to be due to biallelic inactivation of the merlin gene also known as NF2 gene cytogenetically located on the long (q) arm of chromosome 22, at band 12.2 (22q12.2). Merlin is the protein coded by the NF2 gene. This gene acts as a tumor suppressor thus its deletion prevents formation of merlin protein which in turn leads to schwannoma proliferation. […] The knowledge about the molecular biology of schwannomas led to emergence of targeted medications as another option for treatment. The targeted therapies are aimed at the mechanisms by which merlin dysregulation as well as the signaling pathways lead to tumor growth.

• #100 Vestibular schwannoma: evolution of diagnosis and treatment | Egyptian Journal of Neurosurgery | Full Text

  https://ejns.springeropen.com/articles/10.1186/s41984-025-00349-1

  Vestibular Schwannomas are benign tumors of the vestibular branch of the eight cranial nerves usually seen in adults who present with sensorineural hearing loss and tinnitus. […] The tumors may originate anywhere along the path of the eight cranial nerves from the internal acoustic meatus to proximity of the brainstem growing into the Cerebellopontine Angle (CPA) in variable presentation and unpredictable ways, often compressing the adjacent vital structures. […] The molecular hallmark for sporadic and NF2-associated VS tumor is believed to be due to biallelic inactivation of the merlin gene also known as NF2 gene cytogenetically located on the long (q) arm of chromosome 22, at band 12.2 (22q12.2). Merlin is the protein coded by the NF2 gene. This gene acts as a tumor suppressor thus its deletion prevents formation of merlin protein which in turn leads to schwannoma proliferation. […] The knowledge about the molecular biology of schwannomas led to emergence of targeted medications as another option for treatment. The targeted therapies are aimed at the mechanisms by which merlin dysregulation as well as the signaling pathways lead to tumor growth.

• #101 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Studies in rats and mice suggest a carcinogenic effect of whole body exposure to radiofrequency radiation. […] Cell cultures of human Schwann cells exposed to electromagnetic fields showed alterations in proliferation, intracellular signaling, metabolic pathways, and in the expression of genes related to hearing loss. […] In 2013 and 2019, Hardell and Carlberg concluded that emissions from wireless phones should be regarded as group 1 carcinogens. […] Some are skeptical of this interpretation, citing the fact that cell phone emissions are not energetic enough to break molecular bonds within cells, a necessary step toward oncogenesis. […] At least two studies have implicated loud noise exposure in the genesis of vestibular schwannoma, but others have shown no increased risk or an indeterminate risk.

• #102 Vestibular schwannoma | MedLink Neurology

  https://www.medlink.com/articles/vestibular-schwannoma

  Studies in rats and mice suggest a carcinogenic effect of whole body exposure to radiofrequency radiation. […] Cell cultures of human Schwann cells exposed to electromagnetic fields showed alterations in proliferation, intracellular signaling, metabolic pathways, and in the expression of genes related to hearing loss. […] In 2013 and 2019, Hardell and Carlberg concluded that emissions from wireless phones should be regarded as group 1 carcinogens. […] Some are skeptical of this interpretation, citing the fact that cell phone emissions are not energetic enough to break molecular bonds within cells, a necessary step toward oncogenesis. […] At least two studies have implicated loud noise exposure in the genesis of vestibular schwannoma, but others have shown no increased risk or an indeterminate risk.

• #103 Vestibular Health — Vestibular schwannoma or acoustic neuroma

  https://www.vestibularhealth.ca/blog/vestibular-schwannoma-acoustic-neuroma

  Vestibular schwannomas make up about 8% of all intracranial tumours. […] If large enough, the tumor can press against nearby cranial nerves. […] If the tumor is very large, it can press against the brainstem and cerebellum and can prevent the normal flow of cerebrospinal fluid (causing hydrocephalus) – this leads to symptoms such as headaches, nausea/vomiting, and impaired coordination of movement. […] The choice of treatment is determined by the size of the tumour, your age, your level of hearing and balance function, and your individual symptoms and preferences. […] Radiation decreases the size of the tumor and prevents growth – the tumor will not completely disappear. […] Vestibular rehabilitation physiotherapy can help decrease symptoms of dizziness, improve your balance, and maximize your physical functioning. […] Your vestibular physiotherapist can provide an individualized treatment program to improve symptoms of dizziness – particularly dizziness provoked by head movement, improve your gaze stability – the ability for you to keep your vision focused while you are moving around, and improve your balance.

• #104 Vestibular Health — Vestibular schwannoma or acoustic neuroma

  https://www.vestibularhealth.ca/blog/vestibular-schwannoma-acoustic-neuroma

  Vestibular schwannomas make up about 8% of all intracranial tumours. […] If large enough, the tumor can press against nearby cranial nerves. […] If the tumor is very large, it can press against the brainstem and cerebellum and can prevent the normal flow of cerebrospinal fluid (causing hydrocephalus) – this leads to symptoms such as headaches, nausea/vomiting, and impaired coordination of movement. […] The choice of treatment is determined by the size of the tumour, your age, your level of hearing and balance function, and your individual symptoms and preferences. […] Radiation decreases the size of the tumor and prevents growth – the tumor will not completely disappear. […] Vestibular rehabilitation physiotherapy can help decrease symptoms of dizziness, improve your balance, and maximize your physical functioning. […] Your vestibular physiotherapist can provide an individualized treatment program to improve symptoms of dizziness – particularly dizziness provoked by head movement, improve your gaze stability – the ability for you to keep your vision focused while you are moving around, and improve your balance.

• #105 Vestibular Health — Vestibular schwannoma or acoustic neuroma

  https://www.vestibularhealth.ca/blog/vestibular-schwannoma-acoustic-neuroma

  Vestibular schwannomas make up about 8% of all intracranial tumours. […] If large enough, the tumor can press against nearby cranial nerves. […] If the tumor is very large, it can press against the brainstem and cerebellum and can prevent the normal flow of cerebrospinal fluid (causing hydrocephalus) – this leads to symptoms such as headaches, nausea/vomiting, and impaired coordination of movement. […] The choice of treatment is determined by the size of the tumour, your age, your level of hearing and balance function, and your individual symptoms and preferences. […] Radiation decreases the size of the tumor and prevents growth – the tumor will not completely disappear. […] Vestibular rehabilitation physiotherapy can help decrease symptoms of dizziness, improve your balance, and maximize your physical functioning. […] Your vestibular physiotherapist can provide an individualized treatment program to improve symptoms of dizziness – particularly dizziness provoked by head movement, improve your gaze stability – the ability for you to keep your vision focused while you are moving around, and improve your balance.

• #106 Vestibular Health — Vestibular schwannoma or acoustic neuroma

  https://www.vestibularhealth.ca/blog/vestibular-schwannoma-acoustic-neuroma

  Vestibular schwannomas make up about 8% of all intracranial tumours. […] If large enough, the tumor can press against nearby cranial nerves. […] If the tumor is very large, it can press against the brainstem and cerebellum and can prevent the normal flow of cerebrospinal fluid (causing hydrocephalus) – this leads to symptoms such as headaches, nausea/vomiting, and impaired coordination of movement. […] The choice of treatment is determined by the size of the tumour, your age, your level of hearing and balance function, and your individual symptoms and preferences. […] Radiation decreases the size of the tumor and prevents growth – the tumor will not completely disappear. […] Vestibular rehabilitation physiotherapy can help decrease symptoms of dizziness, improve your balance, and maximize your physical functioning. […] Your vestibular physiotherapist can provide an individualized treatment program to improve symptoms of dizziness – particularly dizziness provoked by head movement, improve your gaze stability – the ability for you to keep your vision focused while you are moving around, and improve your balance.

• #107 Acoustic Neuroma

  https://www.physio.co.uk/what-we-treat/vestibular/acoustic-neuroma.php

  Acoustic neuroma is a non-malignant tumour that develops on the hearing and balance nerves which supply the inner ear. […] It is an overproduction of the cells which line the nerves called Schwann cells thus can also be known as vestibular schwannoma. […] Acoustic Neuromas have no hereditary link and there are no known direct causes of the tumour. […] Vestibular physiotherapy – passive resetting exercises. […] There are 3 main options for the treatment of Acoustic Neuroma which depend on the patients overall health and the size and severity of the symptoms. […] Radiotherapy or radiosurgery is another alternative which can reduce the size of the Acoustic Neuroma and limit its progression. […] Physiotherapy rehabilitation of Acoustic Neuroma is normally post-surgical. […] Due to the position of the tumour surgery can affect the normal function of these nerves and rehabilitation can help in restoring function or teaching the brain to compensate for the permanent damage. […] Acoustic Neuroma is a non-malignant tumour that affects the vestibular nerves.

• #108 Understanding the Molecular Mechanism of Vestibular Schwannoma for Hearing Preservation Surgery: Otologists’ Perspective from Bedside to Bench

  https://www.mdpi.com/2075-4418/12/5/1044

  The vestibular schwannoma can arise anywhere along the course of the vestibular nerve, running from the cistern to inside the vestibular organ. […] Vestibular schwannomas are clinically and oncologically regarded as benign tumors. […] Despite this, the management of vestibular schwannoma is difficult and controversial because vestibular schwannoma interventions can cause complications. […] Improving vestibular schwannoma management to prevent complications such as hearing loss and facial nerve palsy is desirable. […] With recent advances in surgical equipment systems and the molecular understanding of the disease, vestibular schwannoma treatment has changed and improved. […] This review summarizes the recent advances in vestibular schwannoma management and treatment. […] The molecular mechanisms underlying tumorigenesis need to be investigated in these cases.

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