Guzy nerwu słuchowego (schwannoma przedsionkowa)
Diagnostyka i diagnoza
Nerwiakowłókniak słuchowy (schwannoma przedsionkowy) to łagodny, wolno rosnący guz wywodzący się z osłonek nerwu przedsionkowo-ślimakowego (VIII nerw czaszkowy), najczęściej z jego części przedsionkowej. Klinicznie manifestuje się jednostronną, czuciowo-nerwową utratą słuchu, często z towarzyszącym tinnitus oraz zaburzeniami równowagi. Diagnostyka rozpoczyna się od audiometrii tonalnej, która wykazuje asymetryczną utratę słuchu oraz dysproporcję w rozumieniu mowy. Badania uzupełniające obejmują słuchowe potencjały wywołane pnia mózgu (ABR) z charakterystycznym brakiem fal III i V przy obecności fali I, oraz badania równowagi (ENG/VNG, VEMP, VHIT), które mogą wykazać jednostronne osłabienie funkcji przedsionkowej u około 60% pacjentów. W diagnostyce obrazowej złotym standardem jest MRI z kontrastem gadolinowym, umożliwiające wykrycie guzów o średnicy 1-2 mm, z typowym obrazem izointensywnym lub hipointensywnym w T1 i silnym wzmocnieniem kontrastowym. Alternatywnie stosuje się CT z kontrastem, choć jest mniej czułe na małe zmiany. Klasyfikacje Koosa i Hannover służą ocenie wielkości i lokalizacji guza, co jest kluczowe dla planowania terapii.
Diagnostyka nerwiakowłókniaka słuchowego (schwannoma przedsionkowego)
Nerwiakowłókniak słuchowy (zwany również schwannoma przedsionkowym) to łagodny, wolno rosnący guz rozwijający się z osłonek nerwu przedsionkowo-ślimakowego (VIII nerw czaszkowy), najczęściej z części przedsionkowej tego nerwu. Wczesna diagnostyka tego schorzenia może być trudna, ponieważ objawy rozwijają się powoli i mogą przypominać inne problemy ucha środkowego i wewnętrznego.123
Badanie fizykalne i wywiad
Pierwszym krokiem w diagnostyce nerwiakowłókniaka słuchowego jest dokładne badanie fizykalne, zwłaszcza badanie ucha, oraz szczegółowy wywiad z pacjentem dotyczący objawów i ich rozwoju w czasie. Najczęściej występującym objawem, który może sugerować obecność nerwiakowłókniaka słuchowego, jest jednostronna utrata słuchu, której nierzadko towarzyszy szum w uszach (tinnitus) oraz zawroty głowy lub problemy z równowagą.145
Ze względu na to, że objawy nerwiakowłókniaka słuchowego mogą przypominać inne schorzenia ucha, takie jak choroba Ménière’a, konieczne jest przeprowadzenie dokładnej diagnostyki różnicowej przez specjalistów w zakresie laryngologii, neurologii lub neurootologii.6
Badania słuchu
Audiometria tonalna jest najczęściej wykonywanym pierwszym badaniem diagnostycznym przy podejrzeniu nerwiakowłókniaka słuchowego. Badanie to zazwyczaj wykazuje asymetryczną utratę słuchu typu odbiorczego (czuciowo-nerwową) oraz większe upośledzenie rozumienia mowy niż można by oczekiwać przy danym stopniu utraty słuchu. Taki wynik badania wskazuje na potrzebę wykonania badań obrazowych.278
Dodatkowe badania słuchu, które mogą być wykonywane w diagnostyce nerwiakowłókniaka słuchowego, obejmują:
- Słuchowe potencjały wywołane pnia mózgu (ABR) – badanie to mierzy aktywność elektryczną w drodze słuchowej do pnia mózgu i może wykazać brak odpowiedzi falowych i/lub zwiększoną latencję fali V. Charakterystycznym objawem nerwiakowłókniaka słuchowego może być obecność fali I przy braku fal III lub V.2910
- Badanie odruchu strzemiączkowego – może wykazać rozpad odruchu strzemiączkowego w tympanometrii.211
Badania równowagi
Chociaż badania równowagi nie są zwykle wymagane w rutynowej ocenie pacjenta z asymetryczną utratą słuchu typu czuciowo-nerwowego, mogą one dostarczyć dodatkowych informacji diagnostycznych:
- Elektronystagmografia (ENG) lub wideonystagmografia (VNG) – badania te oceniają funkcję układu przedsionkowego i mogą wykazać znaczne osłabienie odpowiedzi przedsionkowej (porażenie kanału) po stronie dotkniętej guzem. U około 60% pacjentów z nerwiakowłókniakiem słuchowym obserwuje się jednostronną utratę reakcji na próbę kaloryczną.21110
- Przedsionkowe miogenne potencjały wywołane (VEMP) – badanie to dostarcza informacji o nerwiakowłókniaku słuchowym, który może istnieć na dolnym nerwie przedsionkowym. VEMPy są zazwyczaj nieobecne u około 80% pacjentów z nerwiakowłókniakiem słuchowym.1012
- Badanie wideo głowy (VHIT) – jest znacznie szybsze niż badanie ENG i może być dobrą procedurą przesiewową u osób z podejrzeniem nerwiakowłókniaka słuchowego.11
Badania obrazowe
Badania obrazowe odgrywają kluczową rolę w potwierdzeniu diagnozy nerwiakowłókniaka słuchowego oraz w określeniu jego wielkości i lokalizacji, co ma istotne znaczenie przy planowaniu leczenia.
Rezonans magnetyczny (MRI)
MRI z kontrastem gadolinowym jest uważany za „złoty standard” w diagnostyce nerwiakowłókniaka słuchowego. Badanie to może wykryć guzy o średnicy nawet 1-2 mm. Kontrast gadolinowy jest kluczowy, ponieważ MRI bez kontrastu może nie wykazać małych guzów.1134
W diagnostyce nerwiakowłókniaka słuchowego przy użyciu MRI wykorzystuje się następujące sekwencje:
- Obrazy T1-zależne wzmocnione kontrastem – uważane za złoty standard dla początkowej oceny i pooperacyjnej oceny nawrotu lub pozostałości guza13
- Obrazy T2-zależne o wysokiej rozdzielczości – pomagają w wizualizacji przebiegu nerwu twarzowego jako część oceny przedoperacyjnej14
- Sekwencje CISS lub FIESTA – specjalne sekwencje T2-zależne używane do przedoperacyjnego nadzoru wzrostu nerwiakowłókniaka słuchowego14
W przypadku nerwiakowłókniaków typowy obraz MRI pokazuje okrągły, owalny lub płatowy guz, który jest izointensywny lub hipointensywny w stosunku do tkanki mózgowej w obrazach T1-zależnych i wykazuje silne, jednorodne wzmocnienie po podaniu kontrastu. Niektóre guzy mogą wykazywać niejednorodne wzmocnienie kontrastowe, co może być spowodowane zmianami torbielowatymi. Torbielowate nerwiakowłókniaki będą wykazywać hiperintensywne torbiele w obrazach T2-zależnych, które są hipointensywne w obrazach T1-zależnych.15
Tomografia komputerowa (CT)
Jeśli MRI nie jest dostępny lub pacjent nie może poddać się badaniu MRI (np. z powodu wszczepionego rozrusznika serca), można zastosować tomografię komputerową (CT) z kontrastem. Należy jednak pamiętać, że badanie CT może nie wykryć małych guzów i jest mniej dokładne niż MRI.146
Systemy klasyfikacji guzów
Do oceny wielkości i lokalizacji nerwiakowłókniaków słuchowych stosuje się różne systemy klasyfikacji, które pomagają w planowaniu leczenia:
- System klasyfikacji Koosa – opisuje wielkość guza i jego lokalizację od małych guzów wyłącznie wewnątrzkanałowych do dużych guzów powodujących przemieszczenie pnia mózgu15
- System klasyfikacji rozszerzeń guza Hannover – dodaje kryteria, które obejmują rozszerzenie na kąt mostowo-móżdżkowy z i bez kontaktu z pniem mózgu oraz ucisk pnia mózgu z i bez deformacji czwartej komory15
Diagnoza różnicowa
Diagnoza nerwiakowłókniaka słuchowego może być trudna ze względu na podobieństwo objawów do innych schorzeń ucha środkowego i wewnętrznego. W rozpoznaniu różnicowym należy wziąć pod uwagę:
- Chorobę Ménière’a6
- Inne guzy kąta mostowo-móżdżkowego, takie jak oponiaki, nerwiakowłókniaki nerwu twarzowego, torbiele naskórkowe, torbiele pajęczynówki, tętniaki i przerzuty do mózgu1611
- Inne przyczyny asymetrycznej utraty słuchu, takie jak ekspozycja na hałas, wiek, urazy czy infekcje17
Obserwacja i monitorowanie
Po zdiagnozowaniu nerwiakowłókniaka słuchowego, w zależności od wielkości guza, objawów i stanu pacjenta, lekarz może zalecić jedną z trzech opcji postępowania:
- Obserwacja – w przypadku małych guzów bez objawów lub u pacjentów, którzy nie są kandydatami do operacji z powodu innych problemów zdrowotnych187
- Mikrochirurgia – usunięcie guza187
- Radioterapia stereotaktyczna – leczenie guzów za pomocą precyzyjnie skierowanych promieni radiacyjnych187
W przypadku wyboru opcji obserwacji, pacjent jest regularnie monitorowany za pomocą badań MRI i testów słuchu, aby ocenić wzrost guza i funkcję słuchową. Rekomendowane jest wykonywanie badań MRI co roku przez 5 lat, a następnie wydłużanie odstępów między badaniami, jeśli guz jest stabilny.1419
Pacjenci z neurofibromatozą typu 2
Szczególnym przypadkiem są pacjenci z neurofibromatozą typu 2 (NF2), gdzie nerwiakowłókniaki słuchowe mogą występować obustronnie. Diagnoza NF2 może być postawiona u osób, które mają dwa nerwiakowłókniaki słuchowe, jeden nerwiakowłókniak słuchowy z rodzinną historią NF2 lub jeden nerwiakowłókniak słuchowy w połączeniu z innymi guzami mózgu lub rdzenia kręgowego.5
W przypadku pacjentów z NF2 zaleca się częstsze obrazowanie ze względu na bardziej zmienną szybkość wzrostu nerwiakowłókniaków słuchowych, a badania obrazowe mogą być kontynuowane co roku po ustaleniu tempa wzrostu.14
Podsumowanie diagnostyki
Diagnostyka nerwiakowłókniaka słuchowego wymaga kompleksowego podejścia i zwykle obejmuje:
- Dokładny wywiad i badanie fizykalne, zwłaszcza badanie ucha1
- Badania słuchu (audiometria tonalna, ABR) wykazujące asymetryczną utratę słuchu typu czuciowo-nerwowego2
- Badanie MRI z kontrastem gadolinowym, które jest najbardziej czułym testem w diagnostyce nerwiakowłókniaków słuchowych13
- W niektórych przypadkach badania równowagi (ENG/VNG, VEMP) dla uzupełnienia obrazu klinicznego10
Wczesna diagnoza nerwiakowłókniaka słuchowego jest kluczowa dla zapobiegania jego poważnym konsekwencjom, takim jak całkowita utrata słuchu, i pozwala na wybór najbardziej odpowiedniej opcji leczenia.1820
Należy pamiętać, że o ile asymetryczna utrata słuchu jest czułym wskaźnikiem możliwości wystąpienia nerwiakowłókniaka słuchowego, to jej swoistość jest niska, gdyż występuje w wielu innych schorzeniach ucha. Dlatego istotna jest integracja wszystkich dostępnych danych klinicznych przez specjalistę z doświadczeniem w otologii, aby postawić prawidłową diagnozę.1717
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Materiały źródłowe
- #1 Acoustic neuroma – Diagnosis and treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/acoustic-neuroma/diagnosis-treatment/drc-20356132
A thorough physical exam, including an ear exam, is often the first step in acoustic neuroma diagnosis and treatment. […] An acoustic neuroma is often hard to diagnose in the early stages because symptoms may be easy to miss and develop slowly over time. Common symptoms such as hearing loss also are associated with many other middle and inner ear problems. […] After asking questions about your symptoms, a member of your health care team conducts an ear exam. You may need the following tests: […] Magnetic resonance imaging (MRI) with contrast dye is usually used to diagnose an acoustic neuroma. This imaging test can detect tumors as small as 1 to 2 millimeters in diameter. If MRI is not available or you can’t have an MRI scan, computerized tomography (CT) may be used. However, CT scans may miss small tumors.
- #2 Vestibular Schwannoma – Ear, Nose, and Throat Disorders – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/ear-nose-and-throat-disorders/inner-ear-disorders/vestibular-schwannoma
A vestibular schwannoma (acoustic neuroma) is a Schwann cell-derived tumor of the 8th cranial nerve. […] Diagnosis is based on audiology and confirmed by MRI. […] Most commonly, an audiogram is the first test done to diagnose vestibular schwannoma. It usually detects an asymmetric sensorineural hearing loss and a greater impairment of speech discrimination than would be expected for the degree of hearing loss. Such findings indicate the need for imaging tests, preferably gadolinium-enhanced MRI. […] Other findings include presence of acoustic reflex decay on tympanometry. Auditory brain stem response testing may show the absence of waveforms and/or increased latency of the 5th waveform. […] Although not usually required in the routine evaluation of a patient with asymmetric sensorineural hearing loss, caloric testing shows marked vestibular hypoactivity (canal paresis) on the affected side.
- #3 Acoustic Neuroma (Vestibular Schwannoma): Diagnosis, TreatmentSecond Opinion IconGroup 5https://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. […] Diagnostic tests by a medical professional are needed for confirmation. […] 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. […] The following may be used to diagnose an acoustic neuroma tumor: Hearing tests, Balance tests, CT or MRI scans.
- #4 Diagnosing Acoustic Neuroma | NYU Langone Healthhttps://nyulangone.org/conditions/acoustic-neuroma/diagnosis
Acoustic neuroma, also called vestibular schwannoma, is a slow-growing, benign tumor that develops on the eighth cranial nerve the nerve that carries signals from the inner ear to the brain. […] To diagnose an acoustic neuroma, NYU Langone doctors perform a physical exam, ask about your medical history and symptoms, and request hearing testing and brain imaging. […] These results can tell your doctor how a tumor might be affecting your hearing. Asymmetric unilateral hearing loss, meaning the loss occurs in or is greater in one ear, may be a sign of acoustic neuroma; it usually prompts your doctor to order other tests. […] NYU Langone physicians use an MRI scan to confirm if an acoustic neuroma is present. This scan uses a magnetic field to create computerized, three-dimensional pictures of the soft tissues in and around the brain. […] Instead, the doctor may use a CT scan. For this test, a series of X-ray images is sent to a computer to create three-dimensional, cross-sectional pictures of the head.
- #5 Acoustic Neuroma – Vestibular Disorders Associationhttps://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. […] The diagnosis of NF2 can be firmly made in people who have two acoustic neuromas, or one acoustic neuroma with a family history of NF2, or one acoustic neuroma and a combination of other brain or spinal tumors. […] In most cases, the first symptoms that develop with an acoustic neuroma are hearing loss and tinnitus (i.e., ringing in the ear) on the side with the tumor. […] If the hearing test confirms that one ear has more hearing loss than the other, then a magnetic resonance imaging (MRI) scan of the head is typically ordered. […] Currently in the United States, the gold standard test used to diagnose an acoustic neuroma is an MRI scan of the head with contrast. […] Acoustic neuromas have a classic appearance on MRI such that a tumor biopsy is almost never needed to establish a diagnosis. Rather, in most cases, a diagnosis can be made solely based on the results of the MRI.
- #6 Acoustic neuroma (vestibular schwannoma)https://www.nhs.uk/conditions/acoustic-neuroma/
An acoustic neuroma is a type of non-cancerous (benign) brain tumour. It’s also known as a vestibular schwannoma. […] Acoustic neuromas can be difficult to diagnose because the symptoms can be caused by other conditions, such as Mnire’s disease. […] If your GP thinks you could have an acoustic neuroma, you’ll be referred to a hospital or clinic for further tests, such as: hearing tests to check for hearing problems and determine whether they’re caused by a problem with your nerves, an MRI scan, which uses strong magnetic fields and radio waves to produce a detailed picture of the inside of your head, a CT scan, which uses a series of X-rays to create a detailed image of the inside of your head.
- #7 Acoustic Neuroma (Vestibular Schwannoma): Symptomshttps://my.clevelandclinic.org/health/diseases/16400-acoustic-neuroma
Hearing tests, including auditory brainstem response. […] Balance tests. […] Electronystagmography. […] Treatment options include: Observation: If tests show the tumor isnt growing and you have no symptoms, your provider may recommend observation. […] Stereotactic radiosurgery: This therapy targets tumors with beams of radiation focused on the tumor while limiting the impact on nearby healthy tissue. […] Microsurgery: Neurosurgeons use special instruments that let them do surgery in very small areas of your body. This is the only treatment that can eliminate an acoustic neuroma. […] In rare cases, providers may use the chemotherapy drug bevacizumab (Alymsys, Avastin, MVASI, Zirabeva). […] Vestibular rehabilitation therapy: Sometimes, surgery involves removing parts of nerves that control balance.
- #8 Acoustic Neuroma Symptoms, Surgery and Treatment | UPMChttps://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. […] Doctors use MRI or CT scans to diagnose and confirm the presence of an acoustic neuroma. […] Other diagnostic tests may include: Audiogram to evaluate hearing in both ears. […] Auditory brainstem response test (ABR, BAER, or BSER) to measure the rate of electric impulses traveling from the inner ear to the brainstem. A slowed or absent impulse may indicate the presence of an acoustic neuroma. […] Electronystagmography to test balance.
- #9 Patient Education Article | KIDZ Medical Serviceshttps://www.kidzmedical.com/patient-education-article/acoustic-neuroma-584/
Contact your doctor if you experience the symptoms of acoustic neuroma. Your doctor can diagnose acoustic neuroma by reviewing your medical history and conducting examinations. You may be referred to a neurologist, audiologist, or ear, nose, and throat specialist. […] Magnetic resonance imaging (MRI) scans and computed tomography (CT) scans are used to diagnose this tumor. Hearing tests (audiometry) are used to determine your degree of hearing loss. Brainstem auditory evoked response (BAER) is used to evaluate hearing and neurological functions. The test involves listening to sounds while a device records your brainâs activity. […] Electronystagmography (ENG) is used to identify nerve damage in the ear. ENG testing uses receptors to measure involuntary eye movements during certain procedures, such as caloric testing. Caloric testing checks involuntary eye movements when hot or cold water is placed in the ear.
- #10 Acoustic Neuroma: An Overview – Article 11407https://www.audiologyonline.com/articles/acoustic-neuroma-an-overview-11407
As we know, steroids are something that can treat sudden sensorineural hearing loss, and I personally have had a few patients whose sudden hearing loss improved with the use of steroids but still have an acoustic neuroma. Just because a patient’s hearing has improved with steroids it does not mean a tumor has been ruled out. There is still a need for an MRI on patients that have had a unilateral sensorineural hearing loss, even if hearing improved with steroids. […] ABR testing is less sensitive than the MRI as Dr. Battista said, but is less expensive than the MRI. It can be important in the follow-up care of the patient. A characteristic finding on the ABR is a present wave I but absent waves III or V. You may also see a delayed I-III absolute latency or a delayed wave V latency. It is also important to pay attention to the differences between ears and look for any significant interaural differences, because that can indicate possible acoustic neuroma. Keep in mind that there are high false-positive and false-negative rates associated with the ABR. Patients with small tumors can have normal ABRs. The ABR is more accurate the larger the tumor is, so it has 92 to 98% accuracy when the acoustic neuroma is greater than one-and-a-half centimeters, but as it gets smaller than that, the accuracy drops to 60 to 70%. Even if a patient has a normal ABR, an acoustic neuroma cannot be ruled out.
- #10 Acoustic Neuroma: An Overview – Article 11407https://www.audiologyonline.com/articles/acoustic-neuroma-an-overview-11407
Electronystagmography and videonystagmography (ENG/VNG) is another tool that can give us information about acoustic neuroma. Many patients will have a unilateral caloric weakness on ENG or VNG testing. Caloric weaknesses, however, can be caused by other conditions, so it is not a specific test. Vestibular-evoked myogenic potential (VEMP) testing is something we would want to complete which provides insight into an acoustic neuroma that may exist on the inferior vestibular nerve. VEMPs tend to be absent in approximately 80% of patients with acoustic neuroma.
- #11 Acoustic Neuroma Diagnostic testshttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/tests.html
Acoustic reflex decay is also insensitive to acoustic neuroma (about 36%) and this test is rarely used for this purpose. […] As mentioned above, VEMP testing would be expected to be sensitive to acoustic neuroma’s. […] VHIT testing is much quicker than ENG (caloric) testing, and for this reason, we think might be a good screening procedure in persons where an acoustic is suspect, such as someone with progressive asymmetrical hearing. […] Although it is more costly compared to audiometry or ABR, the optimal test for excluding an acoustic neuroma is a gadolinium enhanced T1 MRI. […] While MRI’s are the most sensitive test to acoustics, they also can make errors. False negative errors mainly occur in persons with very small tumors, or very bad scans (such as a scan done in a low-field unit, such as an open MRI, without contrast).
- #11 Acoustic Neuroma Diagnostic testshttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/tests.html
Symmetrical hearing impairment or even normal hearing does not exclude an acoustic, but it is very rare. […] ABR testing is less sensitive than MRI (false negative rate about 33%), but it is considerably less expensive. […] A characteristic finding on ABR in a person with an acoustic neuroma would be a wave I with nothing after it — no waves 3 or 5 (10-20% of cases). […] At this writing, we have ENG testing, Rotatory chair testing, VHIT testing, VEMP testing, and Posturography to choose from when we are attempting to diagnose dizzy patients. […] Electronystagmography, (ENG testing) is frequently abnormal in persons with acoustic neuromas and about 60 percent of all tumors are associated with unilateral loss of calorics. […] Nevertheless, ENG is not a sufficient test by itself for acoustic neuroma because it is not specific, and also because there are far more other causes for caloric loss than acoustic neuromas.
- #11 Acoustic Neuroma Diagnostic testshttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/tests.html
Acoustic neuromas range in size up to 4 cm. […] Tumors are staged by a combination of their location and size. […] Acoustic neuroma caused by type-II neurofibromatosis should be suspected in young patients and those with a family history of neural tumors. […] There are several other tumors that can occur in the same region of the brain, the cerebellopontine angle or the CPA, as acoustic neuromas. […] As CT and MRI scans become more commonly used, there are more acoustics being discovered accidentally — serendipitously. […] For this reason, extreme caution is suggested — in our opinion, except for very large tumors, it is best to have objective evidence — i.e. progression of hearing loss or a enlargement on MRI — that the tumor is growing before embarking on surgery or radiation treatment.
- #12 Acoustic Neuroma ENT Treatment in Utah – ENT Center of Utahhttps://entcenterutah.com/adult-care/otology-neurotology/acoustic-neuroma/
Acoustic neuromas (vestibular schwannomas or acoustic tumors) are benign tumors that arise from either the balance or auditory nerves. […] Most cases are diagnosed when a patient experiences hearing loss that is worse on one side. Often this is accompanied by tinnitus on the same side. Usually an audiogram (hearing test) is obtained that verifies this hearing loss. An MRI then is performed in the majority of cases that demonstrates the tumors. […] After the diagnosis is made often balance testing with videonystagmography (VNG) and vestibular-evoked myogenic potentials (VEMP) are used to obtain information on ones balance function. Additionally if there is concern of a patients facial nerve function then electroneurography (ENoG) may be performed to look at facial nerve function.
- #13 EANO guideline on the diagnosis and treatment of vestibular schwannomahttps://pmc.ncbi.nlm.nih.gov/articles/PMC6954440/
The radiological diagnosis of vestibular schwannoma is made by magnetic resonance imaging. Histological verification of the diagnosis is not always required. […] MRI is the method of choice for the identification of suspected VS, with contrast-enhanced T1-weighted scans considered to be the gold standard for the initial evaluation and postoperative assessment of recurrence or residual tumors. […] The diagnosis is made according to the World Health Organization (WHO) 2016 classification. […] The histological picture of conventional VS on hematoxylin/eosin-stained sections parallels that of schwannomas in other localizations and is specific enough for a morphological diagnosis in the vast majority of cases. […] The task of observational management is to monitor tumor growth and hearing function to obtain data for a potential decision for therapy.
- #14 Guidelines on the Management of Patients with Vestibular Schwannomahttps://www.cns.org/guidelines/management-patients-vestibular-schwannoma/5-role-of-imaging-in-diagnosis-management-of-patie
Level 3: Imaging used to detect vestibular schwannomas should use high-resolution T2-weighted and contrast-enhanced T1-weighted MRI. […] Level 3: Standard T1, T2, FLAIR, and DWI MR sequences obtained in axial, coronal, and sagittal plane may be used for detection of vestibular schwannomas. […] Level 3: Preoperative surveillance for growth of a vestibular schwannoma should be followed with either contrast-enhanced 3D T1 MPRAGE or high-resolution T2 (including CISS or FIESTA sequences) MR imaging. […] Level 2: Postoperative evaluation should be performed with post-contrast 3D T1 MPRAGE, with nodular enhancement considered suspicious for recurrence. […] Level 3: T2-weighted MRI may be used to augment visualization of the facial nerve course as part of preoperative evaluation. […] Level 3: MRIs should be obtained annually for 5 years, with interval lengthening thereafter with tumor stability.
- #14 Guidelines on the Management of Patients with Vestibular Schwannomahttps://www.cns.org/guidelines/management-patients-vestibular-schwannoma/5-role-of-imaging-in-diagnosis-management-of-patie
Level 3: Adults with cystic vestibular schwannomas should be counseled that their tumors may more often be associated with rapid growth, lower rates of complete resection, and facial nerve outcomes that may be inferior in the immediate postoperative period but similar to non-cystic schwannomas over time. […] Level 3: The degree of lateral IAC involvement by tumor adversely affects facial nerve and hearing outcomes and should be emphasized when interpreting imaging for preoperative planning. […] Level 3: In general, vestibular schwannomas associated with NF2 should be imaged (similar to sporadic schwannomas) with the following caveats: More frequent imaging may be adopted in NF2 patients because of a more variable growth rate for vestibular schwannomas, and annual imaging may ensue once the growth rate is established.
- #15 Vestibular schwannomas: A Review | Applied Radiologyhttps://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. This paper will review the clinical presentation, natural history, and pathology of vestibular schwannomas, as well as discuss the cystic subtypes and provide an overview of imaging diagnosis and measurement techniques. […] Magnetic resonance imaging (MRI) is the gold standard of imaging diagnosis, owing to excellent soft tissue contrast. Tumors are typically round, ovoid, or lobulated. Vestibular schwannomas on MRI are typically T1 isointense or hypointense to adjacent brain tissue and show avid, homogeneous contrast enhancement. Some tumors may show heterogeneous contrast enhancement, which can be due to cystic change. Cystic VS will show T2 hyperintense cysts, which are hypointense on T1W imaging.
- #15 Vestibular schwannomas: A Review | Applied Radiologyhttps://appliedradiology.com/articles/vestibular-schwannomas-a-review
Commonly used tumor grading systems are the Koos grading system and Hannover tumor extension grading system. Koos grade I IV describes tumor size and location from being small and purely intrameatal through to large tumors causing displacement of the brainstem. The Hannover tumor extension grading system also adds criteria that include CPA extension with and without brainstem contact and brainstem compression with and without fourth ventricular deformity. […] Measuring VSs can be challenging, as these tumors often have an irregular shape, and linear measurements can suffer considerable interobserver variability. The Consensus Meeting on Systems for Reporting Results in Vestibular Schwannoma described a standardized measurement technique which includes size based on measurements in millimeters instead of volumetric measurements.
- #16 Vestibular schwannoma – Wikipediahttps://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 only environmental exposure that has been definitely associated with the growth of a VS is therapeutic radiation exposure to the head. […] Preliminary diagnostic procedures include ear examination, hearing and vestibular testing. Typical symptoms include unilateral tinnitus, progressive hearing loss and vertigo. […] MRI scan is the imaging of choice because it can more accurately differentiate the mass from other tumors such as meningioma, facial nerve schwannoma, epidermoid cyst, arachnoid cyst, aneurysm, and brain metastasis. […] The overall mortality rate for VS surgery is around 0.2% – 0.5%. […] The 'Patient Survey’ in the U.S. in 2014 by the national Acoustic Neuroma Association showed that 29% of VS patients reported radiosurgery (17%) or radiotherapy (12%) as their treatment of choice.
- #17 Acoustic Neuroma: overviewhttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/index.html
The lack of specificity has been commented upon by Margolis and Saly (2008). […] This conclusion needs to be tempered by other clinical information – -someone with a progressive asymmetrical sensorineural hearing reduction would (in our opinion) be far more likely to have an acoustic than someone with a static or improving asymmetry. […] Nevertheless, if the chance of finding an acoustic in someone with asymmetrical hearing is between 1/1000 and 1/10,000 and the cost of an MRI is roughly $2000, then it costs between 2 million and 20 million $ in MRI studies to diagnose every acoustic neuroma.
- #17 Acoustic Neuroma: overviewhttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/index.html
The best tests to diagnose acoustic neuroma are audiometry (hearing testing) and MRI scanning of the head with gadolinium contrast. […] Diagnosis of an acoustic usually requires either a physician with otologic expertise who can integrate together the entire picture, or an MRI with gadolinium. […] Because acoustic neuromas are very rare, and MRI’s are very expensive, in our opinion — all patients with a substantial risk of having an acoustic should be evaluated by a physician with otologic expertise. […] The following text describes how this integration process can be done. […] While hearing loss is common in acoustic neuroma (i.e. it is sensitive), there are myriads of other causes of hearing loss (i.e. hearing loss is very nonspecific). […] Roughly 20% of adults have hearing impairment of 25 dB or more.
- #17 Acoustic Neuroma: overviewhttps://dizziness-and-balance.com/disorders/tumors/acoustic_neuroma/index.html
Because of the high sensitivity but low specificity, routine use of a very expensive diagnostic test such as a gad-MRI of the IAC’s in all persons with asymmetrical hearing is not always justified. […] Hearing loss is the most frequent symptom of acoustic neuroma, occurring in more than 95 percent of patients. […] Clinicians often attempt to estimate the risk of an acoustic neuroma by looking at the pattern of hearing loss. […] A sudden hearing loss occurs in about 25 percent of patients with acoustic neuroma. […] Asymmetrical hearing is sensitive but extremely nonspecific for acoustic neuroma. […] The lack of specificity and rarity of acoustic neuromas compared to the myriad of other causes of asymmetrical hearing makes the „cost” of scanning every person with asymmetrical hearing to find an acoustic in 1/1000 persons (or even less) extremely high.
- #18 Vestibular Schwannoma (Acoustic Neuroma) & Neurofibromatosis | NIDCDhttps://www.nidcd.nih.gov/health/vestibular-schwannoma-acoustic-neuroma-and-neurofibromatosis
Early diagnosis of a vestibular schwannoma is key to preventing its serious consequences. […] Determining the best management of the vestibular schwannomas as well as the additional nerve, brain, and spinal cord tumors is more complicated than deciding how to treat a unilateral vestibular schwannoma. […] There are three options for managing a vestibular schwannoma: (1) surgical removal, (2) radiation, and (3) observation.
- #19 Acoustic Neuroma, vestibular schwannoma | Mayfield Brain & Spinehttps://mayfieldclinic.com/pe-acoustic.htm
Magnetic Resonance Imaging (MRI) is a noninvasive test that uses a magnetic field and radiofrequency waves to give a detailed view of the soft tissues of the brain. […] Computed Tomography (CT) is a noninvasive test that uses X-rays and a computer to view anatomical structures within the brain. […] Auditory Brainstem Response (ABR) is a test that checks the hearing pathway to the brainstem. […] The treatment that is right for you will depend on your age, general health, hearing status, and the tumor size. […] Acoustic neuromas that are small and have few symptoms may be observed with MRI scans every year until tumor growth or symptoms change. […] Surgical removal is a common treatment for acoustic neuromas, especially large ones. […] The goal of radiosurgery is to stop or control tumor growth. It does not remove the tumor. […] Acoustic neuromas sometimes recur after radiation or surgery. Periodic MRI scans (every 1 to 2 years) and hearing tests are important for long-term monitoring.
- #20 Acoustic neuroma – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/acoustic-neuroma/symptoms-causes/syc-20356127
An acoustic neuroma, also known as a vestibular schwannoma, is a noncancerous tumor. […] Early diagnosis of an acoustic neuroma may help keep the tumor from growing large enough to cause complications such as total hearing loss.