Guzy nerwu słuchowego (schwannoma przedsionkowa)
Etiologia i przyczyny

Nerwiak osłonkowy nerwu przedsionkowego (vestibular schwannoma) to łagodny guz wywodzący się z komórek Schwanna nerwu VIII, głównie jego części przedsionkowej. Około 95% przypadków jest sporadycznych, związanych z mutacją genu NF2 na chromosomie 22q12.2, kodującego białko merlinę, które reguluje proliferację komórek Schwanna. W około 5% przypadków guz występuje w przebiegu neurofibromatozy typu 2 (NF2), dziedziczonej autosomalnie dominująco, charakteryzującej się obustronnymi nerwiakami i innymi nowotworami układu nerwowego. Jedynym potwierdzonym czynnikiem środowiskowym zwiększającym ryzyko jest ekspozycja na wysokie dawki promieniowania jonizującego w obrębie głowy i szyi, natomiast inne czynniki, takie jak hałas, używanie telefonów komórkowych czy nadczynność przytarczyc, nie mają jednoznacznego potwierdzenia. Częstość występowania wynosi około 1/100 000 rocznie, z największym ryzykiem w wieku 40-60 lat.

Etiologia i przyczyny występowania nerwiaka osłonkowego nerwu przedsionkowego (vestibular schwannoma)

Nerwiak osłonkowy nerwu przedsionkowego, znany również jako nerwiak nerwu słuchowego (acoustic neuroma), jest łagodnym (nienowotworowym) guzem, który rozwija się z komórek Schwanna otaczających nerw przedsionkowo-ślimakowy (VIII nerw czaszkowy), głównie z jego części przedsionkowej (odpowiedzialnej za równowagę), rzadziej z części ślimakowej (odpowiedzialnej za słuch)12. Guzy te powstają na skutek nadmiernej proliferacji komórek Schwanna, które w normalnych warunkach tworzą osłonkę mielinową wokół włókien nerwowych, zapewniając im izolację i wsparcie34.

Sporadyczne występowanie nerwiaków osłonkowych

Około 95% wszystkich przypadków nerwiaków osłonkowych nerwu przedsionkowego to przypadki sporadyczne (niedziedziczne), występujące jednostronnie, bez wyraźnej przyczyny56. W tych przypadkach naukowcy uważają, że dochodzi do utraty funkcji lub mutacji genu znajdującego się na chromosomie 22q12.2, który koduje białko zwane merliną (lub schwannominą)78. Białko to normalnie kontroluje wzrost komórek Schwanna, a jego dysfunkcja prowadzi do niekontrolowanej proliferacji tych komórek i powstania guza910.

Dokładny mechanizm, który prowadzi do mutacji genu NF2 w przypadkach sporadycznych, nie jest w pełni poznany11. Hipotezy naukowe sugerują, że gen ten w jakiś sposób traci zdolność do prawidłowego funkcjonowania, ale szczegółowe przyczyny tego zjawiska wciąż są przedmiotem badań12.

Neurofibromatoza typu 2 (NF2)

W około 5% przypadków nerwiak osłonkowy nerwu przedsionkowego jest związany z genetycznym zaburzeniem zwanym neurofibromatozą typu 2 (NF2)1314. NF2 jest chorobą dziedziczoną w sposób autosomalny dominujący, spowodowaną wadliwym genem supresorowym guza zlokalizowanym na chromosomie 22q12.215. Osoby z NF2 mają zwiększone ryzyko rozwoju obustronnych nerwiaków osłonkowych nerwu przedsionkowego i innych guzów układu nerwowego16.

U pacjentów z NF2 wadliwy gen na chromosomie 22 jest dziedziczony17. NF2 charakteryzuje się wystąpieniem obustronnych nerwiaków osłonkowych nerwu przedsionkowego, często towarzyszą im inne objawy, w tym obwodowe nerwiaki, oponiaki, glejaki i młodzieńcze tylne podtorebkowe zmętnienia soczewki18.

Około 50% osób z NF2 ma mutację de novo, a około 50% tych nowych mutacji będzie łagodnymi przypadkami mozaicyzmu, które są mniej prawdopodobne do przekazania potomstwu19.

Czynniki środowiskowe

Jedynym potwierdzonym czynnikiem środowiskowym związanym ze zwiększonym ryzykiem rozwoju nerwiaka osłonkowego nerwu przedsionkowego jest ekspozycja na wysokie dawki promieniowania jonizującego, szczególnie w obszarze głowy i szyi2021. Badania wykazały, że pacjenci, którzy w dzieciństwie byli poddawani radioterapii w obrębie głowy i szyi (np. w celu zmniejszenia rozmiaru migdałków i gruczołów), są narażeni na zwiększone ryzyko rozwoju nerwiaka osłonkowego nerwu przedsionkowego w późniejszym życiu2223.

Niskie dawki promieniowania jonizującego, takie jak stosowane w diagnostyce obrazowej (np. zdjęcia rentgenowskie zębów), nie zostały jednoznacznie powiązane ze zwiększonym ryzykiem2425.

Inne potencjalne czynniki ryzyka

Naukowcy badali również inne potencjalne czynniki ryzyka, jednak wyniki nie są jednoznaczne:

  • Ekspozycja na hałas: Niektóre badania sugerowały możliwy związek między długotrwałą ekspozycją na głośne dźwięki (np. muzyka, hałas w miejscu pracy) a rozwojem nerwiaka osłonkowego nerwu przedsionkowego, jednak związek ten nie został jednoznacznie potwierdzony2627.
  • Używanie telefonów komórkowych: Mimo obaw dotyczących promieniowania z telefonów komórkowych, liczne badania nie wykazały bezpośredniego związku przyczynowego między używaniem telefonów komórkowych a rozwojem nerwiaków osłonkowych nerwu przedsionkowego2829.
  • Nadczynność przytarczyc: Niektóre badania sugerowały możliwy związek między nadczynnością przytarczyc a zwiększonym ryzykiem rozwoju nerwiaka osłonkowego nerwu przedsionkowego, ale potrzebne są dalsze badania, aby potwierdzić ten związek30.
  • Palenie papierosów: Kilka badań sugerowało, że palenie papierosów może mieć działanie ochronne przed rozwojem nerwiaka osłonkowego nerwu przedsionkowego31.
  • Aspiryna: Sugerowano, że aspiryna może mieć potencjalne działanie ochronne przed rozwojem nerwiaka osłonkowego nerwu przedsionkowego3233.

Czynniki biologiczne i molekularne

Najnowsze badania koncentrują się na skomplikowanych szlakach molekularnych związanych z rozwojem nerwiaków osłonkowych nerwu przedsionkowego34. Kluczowym odkryciem w 1993 roku była identyfikacja genu NF2 i jego produktu białkowego merliny, który moduluje złożone szlaki sygnalizacji molekularnej kontrolujące proliferację komórek35.

Badania wskazują, że utrata funkcji białka merliny prowadzi do ekspansji klonalnej komórek Schwanna36. Ponadto, zaproponowano dodatkowy mechanizm immunologiczny jako główny czynnik przyczyniający się do różnic w tempie wzrostu obserwowanym w różnych guzach37.

Mikrośrodowisko guza podtrzymywane przez infiltrujące komórki immunologiczne prowadzi do wewnątrzguzowego stanu zapalnego, który odgrywa kluczową rolę w wzroście nerwiaka osłonkowego nerwu przedsionkowego38. Czynniki wzrostu takie jak TGF, FGF, NGF, BDNF są przykładem niektórych czynników uwalnianych w środowisku guza39.

Czynnik martwicy nowotworu alfa (TNF) został wskazany jako czynnik ototoksyczny, wydzielany przez nerwiaki osłonkowe nerwu przedsionkowego, którego poziomy były znacząco skorelowane z utratą słuchu40. Również ekspresja czynnika wzrostu śródbłonka naczyniowego (VEGF) jest znacznie wyższa w nerwiakach osłonkowych nerwu przedsionkowego niż w normalnym nerwie słuchowym i była związana z utratą słuchu41.

Epidemiologia i wiek występowania

Nerwiak osłonkowy nerwu przedsionkowego występuje z częstością około 1 przypadku na 100 000 osób rocznie42. Ryzyko rozwoju tego guza zwiększa się wraz z wiekiem, a największe jest u osób w wieku od 40 do 60 lat4344. U dzieci występuje rzadko45.

Częstość występowania nerwiaków osłonkowych nerwu przedsionkowego wydaje się wzrastać, co jest przynajmniej częściowo związane z przypadkowym rozpoznawaniem bezobjawowych zmian dzięki szerokiemu zastosowaniu obrazowania metodą rezonansu magnetycznego (MRI) i tomografii komputerowej (CT)46.

Co ciekawe, osoby z NF2 są często diagnozowane w znacznie młodszym wieku (późna adolescencja lub wczesna dorosłość), chociaż czasami mogą wystąpić później, w piątej do siódmej dekadzie życia, z wolno rosnącymi guzami47.

Podsumowanie etiologii nerwiaka osłonkowego nerwu przedsionkowego

Etiologia nerwiaka osłonkowego nerwu przedsionkowego (vestibular schwannoma) jest złożona i nie w pełni poznana. Większość przypadków (około 95%) to sporadyczne, jednostronne guzy bez wyraźnej przyczyny, związane najprawdopodobniej z mutacją genu na chromosomie 22. Około 5% przypadków jest związanych z dziedzicznym zaburzeniem – neurofibromatozą typu 2 (NF2), która powoduje obustronne nerwiaki osłonkowe nerwu przedsionkowego4849.

Jedynym potwierdzonym czynnikiem środowiskowym zwiększającym ryzyko rozwoju nerwiaka osłonkowego nerwu przedsionkowego jest ekspozycja na wysokie dawki promieniowania jonizującego w obszarze głowy i szyi. Inne potencjalne czynniki ryzyka, takie jak ekspozycja na hałas czy używanie telefonów komórkowych, nie zostały jednoznacznie potwierdzone50.

Badania molekularne wskazują na kluczową rolę białka merliny, którego dysfunkcja prowadzi do niekontrolowanej proliferacji komórek Schwanna. Aktualnie prowadzone są intensywne badania nad dokładnymi mechanizmami molekularnymi związanymi z rozwojem tych guzów, co może w przyszłości przyczynić się do opracowania nowych metod terapeutycznych51.

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  1. 11.04.2026
  2. www.leksykon.com.pl

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. […] 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. […] 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. […] 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.
  • #2 Vestibular Schwannoma (Acoustic Neuroma) & Neurofibromatosis | NIDCD
    https://www.nidcd.nih.gov/health/vestibular-schwannoma-acoustic-neuroma-and-neurofibromatosis
    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. […] Scientists believe that both unilateral and bilateral vestibular schwannomas form following the loss of the function of a gene on chromosome 22. […] Scientists believe that this particular gene on chromosome 22 produces a protein that controls the growth of Schwann cells. […] When this gene malfunctions, Schwann cell growth is uncontrolled, resulting in a tumor. […] In NF2 patients, the faulty gene on chromosome 22 is inherited. […] For individuals with unilateral vestibular schwannoma, however, some scientists hypothesize that this gene somehow loses its ability to function properly.
  • #3 Acoustic Neuroma (Vestibular Schwannoma): Symptoms
    https://my.clevelandclinic.org/health/diseases/16400-acoustic-neuroma
    Acoustic neuromas (vestibular schwannomas) are benign (noncancerous) tumors that can affect the nerves that help you hear and maintain your balance. […] Acoustic neuromas grow very slowly. As they grow, they may cause the following symptoms: […] What causes acoustic neuromas? These tumors develop when Schwann cells multiply. These cells support and protect the balance and hearing nerves in your peripheral nervous system. Researchers dont know why this happens. They do know people with neurofibromatosis type 2 may develop bilateral acoustic neuromas.
  • #4 Acoustic Neuroma | Vestibular Schwannoma | Dr LM Hofmeyr
    https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/acoustic-neuroma/
    There is no evidence that cell phones cause vestibular schwannomas. […] 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.
  • #5 Acoustic Neuroma Symptoms & Causes (Vestibular Schwannoma)
    https://health.ucsd.edu/care/ent/acoustic-neuroma/causes-and-symptoms/
    Radiation exposure to the head is the only environmental influence that has been definitively linked with an increased incidence of acoustic neuroma. […] Acoustic neuroma is not generally considered an inherited disease; however, 5% of cases are associated with a genetic disorder called neurofibromatosis type 2 (NF2). These individuals show two-sided vestibular tumors often associated with other tumors around the brain and/or in the spine. […] Most acoustic neuromas are sporadic (nonhereditary).
  • #6 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. […] 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.
  • #7 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. […] 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. […] 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. […] 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.
  • #8 Vestibular Schwannoma (Acoustic Neuroma) & Neurofibromatosis | NIDCD
    https://www.nidcd.nih.gov/health/vestibular-schwannoma-acoustic-neuroma-and-neurofibromatosis
    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. […] Scientists believe that both unilateral and bilateral vestibular schwannomas form following the loss of the function of a gene on chromosome 22. […] Scientists believe that this particular gene on chromosome 22 produces a protein that controls the growth of Schwann cells. […] When this gene malfunctions, Schwann cell growth is uncontrolled, resulting in a tumor. […] In NF2 patients, the faulty gene on chromosome 22 is inherited. […] For individuals with unilateral vestibular schwannoma, however, some scientists hypothesize that this gene somehow loses its ability to function properly.
  • #9 Acoustic Neuroma Causes | Expert Surgeon | Aaron Cohen-Gadol, MD
    https://www.aaroncohen-gadol.com/en/patients/acoustic-neuromas/types/causes
    Acoustic neuromas are abnormal growth of cells (Schwann cells) that form the myelin sheath that insulates certain nerves. An acoustic neuroma can be due to a sporadic (random) mutation or a problem with a gene on chromosome 22. This gene produces a protein that suppresses tumor development and controls the growth of Schwann cells. […] Many mutations of chromosome 22 are associated with a genetic disorder called neurofibromatosis type 2 (NF2) that is inherited from previous generations. […] Acoustic neuromas are caused by unregulated cell growth of Schwann cells, which surround nerves. […] Chromosome 22 gene mutations are associated with an increased risk of developing acoustic neuromas. […] Neurofibromatosis type 2 (NF2), an inherited disorder, is associated with bilateral acoustic neuromas.
  • #10 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. While studying the association between cell phone use and the development of these tumors is difficult, the scientific community does not generally accept that any relationship has been proven.
  • #11 Acoustic Neuroma – Vestibular Disorders Association
    https://vestibular.org/article/diagnosis-treatment/types-of-vestibular-disorders/acoustic-neuroma/
    Acoustic neuromas, also referred to as vestibular schwannomas, are non-cancerous (benign) tumors that develop from the eighth cranial nerve, between the ear and the brainstem. […] Virtually all acoustic neuromas have at least one mutation involving an important gene located on chromosome 22. When this gene is healthy (not mutated), it prevents the Schwann cells from growing into a tumor. However, when this gene is mutated, a tumor may form. […] Approximately 95% of all acoustic neuromas occur sporadically because of random mutations in the affected gene, meaning they are not part of an inherited genetic syndrome. […] There is no convincing evidence that certain foods, smoking, or other environmental factors increase the risk of developing an acoustic neuroma. Some publications suggest that a history of loud noise or cell-phone use may cause acoustic neuromas to develop; however, this is very controversial, and based on current information, these factors likely do not cause acoustic neuromas. […] Aside from some rare factors, such as radiation exposure at a young age, there is no identifiable cause to explain why most acoustic neuromas develop.
  • #12 Vestibular Schwannoma (Acoustic Neuroma) & Neurofibromatosis | NIDCD
    https://www.nidcd.nih.gov/health/vestibular-schwannoma-acoustic-neuroma-and-neurofibromatosis
    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. […] Scientists believe that both unilateral and bilateral vestibular schwannomas form following the loss of the function of a gene on chromosome 22. […] Scientists believe that this particular gene on chromosome 22 produces a protein that controls the growth of Schwann cells. […] When this gene malfunctions, Schwann cell growth is uncontrolled, resulting in a tumor. […] In NF2 patients, the faulty gene on chromosome 22 is inherited. […] For individuals with unilateral vestibular schwannoma, however, some scientists hypothesize that this gene somehow loses its ability to function properly.
  • #13 Acoustic Neuroma (Vestibular Schwannoma): Diagnosis, TreatmentSecond Opinion IconGroup 5
    https://www.barrowneuro.org/condition/acoustic-neuroma/
    An acoustic neuroma, also known as a vestibular schwannoma, is a benign (noncancerous) brain tumor that develops on the vestibulocochlear nerve, which carries sound and balance information from the inner ear to the brain. […] 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. […] 95 percent of these tumors occur spontaneously without any evidence of family history.
  • #14 Acoustic Neuroma (Vestibular Schwannoma) – UC Health
    https://www.uchealth.com/en/conditions/acoustic-neuroma
    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).
  • #15 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/882876-overview
    Most patients diagnosed with an acoustic neuroma have no apparent risk factors. Exposure to high-dose ionizing radiation is the only definite environmental factor associated with an increased risk of developing an acoustic neuroma. […] Neurofibromatosis type II (NF2) related schwannomatosis occurs in individuals who have a defective tumor suppressor gene located on chromosome 22q12.2; it is inherited in an autosomal dominant pattern. The defective protein produced by the gene is called merlin or schwannomin. Bilateral acoustic tumors are a principle clinical feature of NF2, although other manifestations, including peripheral neurofibromata, meningioma, glioma, and juvenile posterior subcapsular lenticular opacities, are often present as well. Many patients with NF2 present in late adolescence or early adulthood but occasionally may present later, in the fifth to seventh decade, with slowly growing tumors.
  • #16 Acoustic Neuroma | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/acoustic-neuroma.html
    Acoustic neuromas (also called vestibular schwannomas) are noncancerous brain tumors that can cause problems with hearing, balance, and facial movement and sensation. […] In most cases, we don’t know what causes acoustic neuromas. However, some factors may increase your risk, including: […] An inherited genetic condition called neurofibromatosis type 2, which can cause cells to grow out of control and can lead to cancer.
  • #17 Vestibular schwannoma | Handouts | MedLink Neurology
    https://www.medlink.com/handouts/acoustic-neurinoma1
    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. […] Scientists believe that both unilateral and bilateral vestibular schwannomas form following the loss of the function of a gene on chromosome 22. […] Scientists believe that this particular gene on chromosome 22 produces a protein that controls the growth of Schwann cells. […] In NF2 patients, the faulty gene on chromosome 22 is inherited. […] For individuals with unilateral vestibular schwannoma, however, some scientists hypothesize that this gene somehow loses its ability to function properly.
  • #18 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/882876-overview
    Most patients diagnosed with an acoustic neuroma have no apparent risk factors. Exposure to high-dose ionizing radiation is the only definite environmental factor associated with an increased risk of developing an acoustic neuroma. […] Neurofibromatosis type II (NF2) related schwannomatosis occurs in individuals who have a defective tumor suppressor gene located on chromosome 22q12.2; it is inherited in an autosomal dominant pattern. The defective protein produced by the gene is called merlin or schwannomin. Bilateral acoustic tumors are a principle clinical feature of NF2, although other manifestations, including peripheral neurofibromata, meningioma, glioma, and juvenile posterior subcapsular lenticular opacities, are often present as well. Many patients with NF2 present in late adolescence or early adulthood but occasionally may present later, in the fifth to seventh decade, with slowly growing tumors.
  • #19 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. […] The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head. […] Variations in the mutation determine the nature of the tumor’s development. […] About 50% of people with NF2 have a de novo mutation, and about 50% of these new mutations will be mild mosaic cases which are less likely to be passed on. […] The complexity of the molecular biology research involved is truly challenging. […] 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. […] The development of a new generation of drugs may become a secondary therapy in view of advances in genome editing during the 1990s that led to the invention of CRISPR in 2009.
  • #20 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/882876-overview
    Most patients diagnosed with an acoustic neuroma have no apparent risk factors. Exposure to high-dose ionizing radiation is the only definite environmental factor associated with an increased risk of developing an acoustic neuroma. […] Neurofibromatosis type II (NF2) related schwannomatosis occurs in individuals who have a defective tumor suppressor gene located on chromosome 22q12.2; it is inherited in an autosomal dominant pattern. The defective protein produced by the gene is called merlin or schwannomin. Bilateral acoustic tumors are a principle clinical feature of NF2, although other manifestations, including peripheral neurofibromata, meningioma, glioma, and juvenile posterior subcapsular lenticular opacities, are often present as well. Many patients with NF2 present in late adolescence or early adulthood but occasionally may present later, in the fifth to seventh decade, with slowly growing tumors.
  • #21 Acoustic Neuroma Symptoms & Causes (Vestibular Schwannoma)
    https://health.ucsd.edu/care/ent/acoustic-neuroma/causes-and-symptoms/
    Radiation exposure to the head is the only environmental influence that has been definitively linked with an increased incidence of acoustic neuroma. […] Acoustic neuroma is not generally considered an inherited disease; however, 5% of cases are associated with a genetic disorder called neurofibromatosis type 2 (NF2). These individuals show two-sided vestibular tumors often associated with other tumors around the brain and/or in the spine. […] Most acoustic neuromas are sporadic (nonhereditary).
  • #22 Content – Health Encyclopedia – University of Rochester Medical Center
    https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=85&contentid=P00438
    Acoustic neuroma is a rare tumor. It’s not cancer. It grows slowly from an overproduction of Schwann cells and is also called a vestibular schwannoma. Acoustic neuroma may be the result of nerve damage caused by environmental factors. The only certain environmental risk factor is past radiation to the head. […] Acoustic neuroma can be caused by: Neck or head radiation. This can lead to acoustic neuroma many years later. […] Neurofibromatosis type 2 (NF2). People with this disease are at higher risk. NF2 can run in families. […] It can be caused by radiation exposure to the head and neck. Some types are inherited.
  • #23 Acoustic Neuroma: Causes, Symptoms, and Treatment | Doctor
    https://patient.info/doctor/acoustic-neuromas
    Acoustic neuromas are tumours of the vestibulocochlear nerve (eighth cranial nerve), arising from the Schwann cells of the nerve sheath. […] Confirmed risk factors include: Neurofibromatosis. High-dose ionising radiation (children who received radiation for benign conditions of their head and neck – for example, to decrease the size of their tonsils and adenoids – were at increased risk of developing an acoustic neuroma much later in life.) However, the medical use of low-dose ionising radiation, such as used in imaging, has not been established as a risk. […] Many epidemiological studies have failed to find a link between acoustic neuroma and mobile phone use. A 2009 systematic review and meta-analysis evaluating long-term mobile phone use found a 60% increase in risk for acoustic neuroma following ten years of ipsilateral mobile phone usage, but the methodology used by this study has been questioned.
  • #24 Acoustic Neuroma Treatment & Symptoms | Brigham and Women’s Hospital
    https://www.brighamandwomens.org/neurosurgery/brain-tumors/acoustic-neuroma
    What Causes Acoustic Neuroma? Acoustic neuroma, also known as vestibular schwannoma, can happen to anyone, and some of the causes appear sporadic. Often experts can’t pinpoint what causes acoustic neuroma. Still, there are two known main acoustic neuroma risk factors: […] A rare genetic disorder that disrupts normal cell growth in the nervous system, neurofibromatosis can cause tumors to grow. In these cases, acoustic neuroma tends to develop bilaterally. […] If a patient has received high dose cranial radiation therapy for the treatment of cancer, they may be at risk of developing an acoustic neuroma. Procedures such as dental X-rays do not predispose a person to this disease. […] Not all causes of acoustic neuroma are known, although a rare genetic disorder, neurofibromatosis, is one of the known causes of a small number of cases.
  • #25 Acoustic Neuroma: Causes, Symptoms, and Treatment | Doctor
    https://patient.info/doctor/acoustic-neuromas
    Acoustic neuromas are tumours of the vestibulocochlear nerve (eighth cranial nerve), arising from the Schwann cells of the nerve sheath. […] Confirmed risk factors include: Neurofibromatosis. High-dose ionising radiation (children who received radiation for benign conditions of their head and neck – for example, to decrease the size of their tonsils and adenoids – were at increased risk of developing an acoustic neuroma much later in life.) However, the medical use of low-dose ionising radiation, such as used in imaging, has not been established as a risk. […] Many epidemiological studies have failed to find a link between acoustic neuroma and mobile phone use. A 2009 systematic review and meta-analysis evaluating long-term mobile phone use found a 60% increase in risk for acoustic neuroma following ten years of ipsilateral mobile phone usage, but the methodology used by this study has been questioned.
  • #26 Acoustic neuroma (Schwannoma): Treatment, symptoms, and causes
    https://www.medicalnewstoday.com/articles/186184
    Significant exposure to radiation to the head and neck during childhood may increase the risk later in life. […] Studies have suggested that some cases may be related to long-term exposure to loud noise. […] People have also speculated that cell phone use may contribute to the development of an acoustic neuroma, but research does not support this. […] Sometimes a person will develop neurofibromatosis type 2 (NF2), which causes acoustic neuromas to develop on both sides of the head. This is usually a hereditary or genetic condition.
  • #27 Azthena logo with the word Azthena
    https://www.news-medical.net/health/What-is-a-Vestibular-Schwannoma.aspx
    A vestibular schwannoma is a slow-growing, benign tumor developing in the nerves that connect the inner ear to the brain. […] The tumor may develop because of environment-induced damage to the nerve, but the exact risk factors are unclear. […] Some scientists think that both types are caused by defects in the same gene which causes Schwann cells to function. […] 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.
  • #28 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. […] 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. […] 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. […] 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.
  • #29 What is Acoustic Neuroma (Vestibular Schwannoma)? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/acoustic-neuroma.html
    Acoustic neuroma, also known as a vestibular schwannoma, is a benign (non-cancerous) tumor. Despite the name, these tumors do not develop from the acoustic nerve. They start in the vestibular nerve, which is associated with balance. […] The cause of most cases of acoustic neuroma is not known. Most occur randomly, thus they are called sporadic vestibular schwannomas. These sporadic tumors are considered to be different from genetic tumors. […] Although there are theories that exposure to loud noise or cellular phone use may increase the likelihood of acoustic neuromas, none of these have been scientifically proven.
  • #30 Causes of vestibular schwannoma – Louis Hofmeyr
    https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/acoustic-neuroma/elementor-21665/
    Numerous studies have investigated the potential connection between cell phones and VS, but the results have been inconclusive. […] While exposure to loud noises can cause damage to the inner ear and potentially lead to hearing loss, there is no scientific evidence to support the claim that it can cause vestibular schwannoma. […] Studies have shown that the majority of VS occur sporadically, with only a tiny percentage being associated with a genetic predisposition or exposure to high-dose radiation, such as that used in specific medical treatments. […] Some studies have suggested a possible association between hyperparathyroidism and an increased risk of developing VS, but further research is needed to confirm this link. […] Several studies have suggested that cigarette smoking may have a protective effect against the development of VS.
  • #31 Causes of vestibular schwannoma – Louis Hofmeyr
    https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/acoustic-neuroma/elementor-21665/
    Numerous studies have investigated the potential connection between cell phones and VS, but the results have been inconclusive. […] While exposure to loud noises can cause damage to the inner ear and potentially lead to hearing loss, there is no scientific evidence to support the claim that it can cause vestibular schwannoma. […] Studies have shown that the majority of VS occur sporadically, with only a tiny percentage being associated with a genetic predisposition or exposure to high-dose radiation, such as that used in specific medical treatments. […] Some studies have suggested a possible association between hyperparathyroidism and an increased risk of developing VS, but further research is needed to confirm this link. […] Several studies have suggested that cigarette smoking may have a protective effect against the development of VS.
  • #32 Causes of vestibular schwannoma – Louis Hofmeyr
    https://lmhofmeyr.co.za/conditions/conditions-we-specialise-in/acoustic-neuroma/elementor-21665/
    Aspirin has been suggested to have a potential protective effect against the development of VS. […] While viruses have been shown to be a cause of approximately 15% of all cancers, no direct relationship has been established between viruses and VS. […] There is currently not enough evidence to support pregnancy and hormonal contraceptives as a cause of the formation of vestibular schwannoma.
  • #33 Acoustic Neuroma: Causes, Symptoms, and Treatment | Doctor
    https://patient.info/doctor/acoustic-neuromas
    Bilateral acoustic neuroma occurs in neurofibromatosis-type 2 (NF2). NF2 is an autosomal dominant disorder (ie has a 50% risk of transmission from a parent) but also shows high levels of mosaicism. 7% of patients with acoustic neuroma also have NF2. Acoustic neuroma due to NF2 tends to present earlier, typically around 30 years old. […] Genetic screening for NF2 in patients presenting with sporadic, unilateral acoustic neuroma is usually only productive in cases of very early onset (younger than 20 years). NF2 patients are predisposed not only to developing acoustic neuroma but also schwannomas of other cranial nerves. […] Targeted biological therapies are emerging as potential treatments – eg, bevacizumab, everolimus, and lapatinib. There may also be a role for aspirin in slowing tumour growth.
  • #34 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. […] The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head. […] Variations in the mutation determine the nature of the tumor’s development. […] About 50% of people with NF2 have a de novo mutation, and about 50% of these new mutations will be mild mosaic cases which are less likely to be passed on. […] The complexity of the molecular biology research involved is truly challenging. […] 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. […] The development of a new generation of drugs may become a secondary therapy in view of advances in genome editing during the 1990s that led to the invention of CRISPR in 2009.
  • #35 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. […] The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head. […] Variations in the mutation determine the nature of the tumor’s development. […] About 50% of people with NF2 have a de novo mutation, and about 50% of these new mutations will be mild mosaic cases which are less likely to be passed on. […] The complexity of the molecular biology research involved is truly challenging. […] 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. […] The development of a new generation of drugs may become a secondary therapy in view of advances in genome editing during the 1990s that led to the invention of CRISPR in 2009.
  • #36 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    Several mechanisms and factors could at the base of VS onset and progression. 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. Indeed, the cause for the transformation of SCs into schwannomas is mainly credited to the inactivation of the neurofibromin 2 gene and the resulting loss of its protein merlin, which then leads to a clonal expansion of SCs. However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] Typically, about 1030% of patients with VS lesions present unilateral high-frequency SNHL, although they can also exhibit tinnitus, headache, vertigo, and motor/balance problems. Therefore, it could be hypothesized that the tumoral expansion of SCs, found in VS, may be one of the causes of SNHL. 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. Hence, the VS might be the consequence of the loss of crosstalk between merlin-deficient SGNs and SCs.
  • #37 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    Several mechanisms and factors could at the base of VS onset and progression. 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. Indeed, the cause for the transformation of SCs into schwannomas is mainly credited to the inactivation of the neurofibromin 2 gene and the resulting loss of its protein merlin, which then leads to a clonal expansion of SCs. However, an additional immune-mediated mechanism has been proposed as the major contributor to the differences in growth rate observed in various tumors. […] Typically, about 1030% of patients with VS lesions present unilateral high-frequency SNHL, although they can also exhibit tinnitus, headache, vertigo, and motor/balance problems. Therefore, it could be hypothesized that the tumoral expansion of SCs, found in VS, may be one of the causes of SNHL. 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. Hence, the VS might be the consequence of the loss of crosstalk between merlin-deficient SGNs and SCs.
  • #38 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    HL has been observed even in non-growing tumors anyhow, so great that the correlation between tumor size and HL suggested that mechanical compression could not be the unique cause of SNHL. Indeed, VS may cause HL also for the concomitant secretion of paracrine toxic substances into the inner ear or into the cochlear nerve. Hence, the tumor microenvironment sustained by infiltrated immune cells leads to intratumoral inflammation, playing a key role in VS growth. TGF, FGF, NGF, BDNF are an example of some of growth factors released in the tumoral milieu. Tumor necrosis factor alpha (TNF) was indicated as an ototoxic factor, secreted by VSs, which levels were significantly correlated with HL. Also the vascular endothelial growth factor (VEGF) expression is significantly higher in VS than in normal auditory nerve, and was associated with HL. This suggested that, at least partially, VS growth is achieved by promoting intratumoral angiogenesis.
  • #39 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    HL has been observed even in non-growing tumors anyhow, so great that the correlation between tumor size and HL suggested that mechanical compression could not be the unique cause of SNHL. Indeed, VS may cause HL also for the concomitant secretion of paracrine toxic substances into the inner ear or into the cochlear nerve. Hence, the tumor microenvironment sustained by infiltrated immune cells leads to intratumoral inflammation, playing a key role in VS growth. TGF, FGF, NGF, BDNF are an example of some of growth factors released in the tumoral milieu. Tumor necrosis factor alpha (TNF) was indicated as an ototoxic factor, secreted by VSs, which levels were significantly correlated with HL. Also the vascular endothelial growth factor (VEGF) expression is significantly higher in VS than in normal auditory nerve, and was associated with HL. This suggested that, at least partially, VS growth is achieved by promoting intratumoral angiogenesis.
  • #40 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    HL has been observed even in non-growing tumors anyhow, so great that the correlation between tumor size and HL suggested that mechanical compression could not be the unique cause of SNHL. Indeed, VS may cause HL also for the concomitant secretion of paracrine toxic substances into the inner ear or into the cochlear nerve. Hence, the tumor microenvironment sustained by infiltrated immune cells leads to intratumoral inflammation, playing a key role in VS growth. TGF, FGF, NGF, BDNF are an example of some of growth factors released in the tumoral milieu. Tumor necrosis factor alpha (TNF) was indicated as an ototoxic factor, secreted by VSs, which levels were significantly correlated with HL. Also the vascular endothelial growth factor (VEGF) expression is significantly higher in VS than in normal auditory nerve, and was associated with HL. This suggested that, at least partially, VS growth is achieved by promoting intratumoral angiogenesis.
  • #41 Hearing loss and vestibular schwannoma: new insights into Schwann cells implication | Cell Death & Disease
    https://www.nature.com/articles/s41419-023-06141-z
    HL has been observed even in non-growing tumors anyhow, so great that the correlation between tumor size and HL suggested that mechanical compression could not be the unique cause of SNHL. Indeed, VS may cause HL also for the concomitant secretion of paracrine toxic substances into the inner ear or into the cochlear nerve. Hence, the tumor microenvironment sustained by infiltrated immune cells leads to intratumoral inflammation, playing a key role in VS growth. TGF, FGF, NGF, BDNF are an example of some of growth factors released in the tumoral milieu. Tumor necrosis factor alpha (TNF) was indicated as an ototoxic factor, secreted by VSs, which levels were significantly correlated with HL. Also the vascular endothelial growth factor (VEGF) expression is significantly higher in VS than in normal auditory nerve, and was associated with HL. This suggested that, at least partially, VS growth is achieved by promoting intratumoral angiogenesis.
  • #42 Acoustic Neuroma (Vestibular Schwannoma) – Treatment abroad
    https://www.gsdinternational.com/conditions/acoustic-neuroma-vestibular-schwannoma
    The incidence is approximately one case per 100,000 population per year, but increases in patients with neurofibromatosis. […] Therefore, the Gamma Knife is considered the treatment of first choice in all patients with auditory neuroma smaller than 3 cm, with no evidence of brain stem compression or risk of hydrocephalus. […] Current surgical techniques allow a choice between different surgical approaches (retrosigmoid, translabial, extradural subtemporal) depending on the size and exact location of the neurinoma (intracanal or extracanal) as well as the possible neurological deficit found in the patient before surgery.
  • #43 Vestibular schwannoma (acoustic neuroma) | Macmillan Cancer Support
    https://www.macmillan.org.uk/cancer-information-and-support/brain-tumour/acoustic-neuroma
    Vestibular schwannomas are most common in people aged between 40 and 60, but they can affect people of any age. […] The cause of vestibular schwannomas is unknown in most cases, but research is being done to find out more. […] In a very small number of people, vestibular schwannoma is linked to NF2-related schwannomatosis.
  • #44 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). It occurs in about 4-5 persons per 100,000 per year. At least one out of 500 people will develop a vestibular schwannoma in their lifetime. […] Vestibular schwannoma occurs most commonly in the age group of 40- 60 years. It rarely occurs in children. It occurs evenly in male and female patients. […] In 95% of cases, patients will present with a solitary tumour. These cases are not inherited (genetic). […] In 5% of cases, patients present with a genetic form of which Neurofibromatosis type 2 is the most common cause. […] 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).
  • #45 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). It occurs in about 4-5 persons per 100,000 per year. At least one out of 500 people will develop a vestibular schwannoma in their lifetime. […] Vestibular schwannoma occurs most commonly in the age group of 40- 60 years. It rarely occurs in children. It occurs evenly in male and female patients. […] In 95% of cases, patients will present with a solitary tumour. These cases are not inherited (genetic). […] In 5% of cases, patients present with a genetic form of which Neurofibromatosis type 2 is the most common cause. […] 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).
  • #46 Vestibular schwannoma (acoustic neuroma) – UpToDate
    https://www.uptodate.com/contents/vestibular-schwannoma-acoustic-neuroma
    Vestibular schwannomas (also known as acoustic neuromas) are Schwann cell-derived, histologically benign tumors of the eighth cranial nerve, most commonly arising from the vestibular portion of the nerve. […] The incidence appears to be increasing, due at least in part to the incidental diagnosis of asymptomatic lesions with the widespread use of magnetic resonance imaging (MRI) and computed tomography (CT).
  • #47 Acoustic Neuroma: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/882876-overview
    Most patients diagnosed with an acoustic neuroma have no apparent risk factors. Exposure to high-dose ionizing radiation is the only definite environmental factor associated with an increased risk of developing an acoustic neuroma. […] Neurofibromatosis type II (NF2) related schwannomatosis occurs in individuals who have a defective tumor suppressor gene located on chromosome 22q12.2; it is inherited in an autosomal dominant pattern. The defective protein produced by the gene is called merlin or schwannomin. Bilateral acoustic tumors are a principle clinical feature of NF2, although other manifestations, including peripheral neurofibromata, meningioma, glioma, and juvenile posterior subcapsular lenticular opacities, are often present as well. Many patients with NF2 present in late adolescence or early adulthood but occasionally may present later, in the fifth to seventh decade, with slowly growing tumors.
  • #48
    https://med.uth.edu/orl/2021/01/26/vestibular-schwannoma-acoustic-neuroma/
    Vestibular schwannoma is a benign (non-cancerous) tumor that grows on the eighth cranial nerve, which is responsible for hearing and balance. […] 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. […] 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.
  • #49 Acoustic Neuroma (Vestibular Schwannoma) – UC Health
    https://www.uchealth.com/en/conditions/acoustic-neuroma
    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).
  • #50
    https://med.uth.edu/orl/2021/01/26/vestibular-schwannoma-acoustic-neuroma/
    Vestibular schwannoma is a benign (non-cancerous) tumor that grows on the eighth cranial nerve, which is responsible for hearing and balance. […] 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. […] 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.
  • #51 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. […] The only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head. […] Variations in the mutation determine the nature of the tumor’s development. […] About 50% of people with NF2 have a de novo mutation, and about 50% of these new mutations will be mild mosaic cases which are less likely to be passed on. […] The complexity of the molecular biology research involved is truly challenging. […] 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. […] The development of a new generation of drugs may become a secondary therapy in view of advances in genome editing during the 1990s that led to the invention of CRISPR in 2009.