See discussions, stats, and author profiles for this publication at: https://www.researchgate.

net/publication/335335513

Acoustic Neuroma (Vestibular Schwannoma)

Article · November 2018

CITATIONS READS
7 3,344

2 authors, including:

Mohammed Al-Dhahir
University of Florida
44 PUBLICATIONS 75 CITATIONS

SEE PROFILE

Some of the authors of this publication are also working on these related projects:

URGENT BRAIN TUMOR RESECTION SURGERY, WHEN TO DO? – A PROPOSED NEW INCLUSION CRITERIA View project

TRANSFUSION PRACTICES IN ACUTE TRAUMATIC BRAIN INJURY View project

All content following this page was uploaded by Mohammed Al-Dhahir on 22 August 2019.

The user has requested enhancement of the downloaded file.

8/22/2019 Acoustic Neuroma (Vestibular Schwannoma) - StatPearls - NCBI Bookshelf

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

Acoustic Neuroma (Vestibular Schwannoma)

Authors

Joshua Greene1; Mohammed A. Al-Dhahir.

Affiliations
1 Henry Ford Health System

Last Update: November 25, 2018.

Introduction
Acoustic neuromas are also called vestibular schwannoma, acoustic neurinoma, vestibular neuroma, and acoustic
neurofibroma. These are tumors that evolve from Schwann cell sheath and can be either intracranial or extra-axial.
They usually occur adjacent to the cochlear or vestibular nerve. Anatomically, acoustic neuromas tend to occupy the
cerebellopontine angle. About 20% of internal carotid artery (ICA) tumors are meningiomas and may occur elsewhere
in the brain. Bilateral acoustic neuromas also tend to be exclusively in individuals with type 2 neurofibromatosis.[1]
[2][3]

Etiology
Acoustic neuroma is allied to neurofibromatosis type 2 (defect on Chromosome 22) bilateral disease. Studies have
shown that acoustic neuroma had a causative predisposition mutation. Radiation exposure may predispose a patient to
the development of that condition as well.[4]

Epidemiology
Approximately 8% of all intracranial tumors which manifest clinically are attributed to schwannomas. The majority of
acoustic neuromas are unilateral and sporadic. Bilateral acoustic neuromas are in fact genetic and constitute a bit less
than 5% of all schwannomas. Acoustic neuromas in general, tend to present between the fourth to sixth decades of
life. Acoustic neuromas developing in individuals with neurofibromatosis type 2 (NF II) are likely to present earlier,
with a peak incidence around the third decade of life. Although rare, acoustic schwannomas can occur in children.
There is a small female preponderance with an aggravation of problems during pregnancy. Hereditary acoustic
neuroma occurs in NF II much more often compared to neurofibromatosis type 1 (NF I), although the latter is much
more common. Unilateral acoustic neuroma has been reported exclusively in 24% of cases with NF I, while bilateral
acoustic schwannoma is a hallmark of NF II. Both these conditions are autosomal dominant. The defective genetic
locus has been localized to chromosome 17 in NF I and chromosome 22 in NF II.[5][6]

Histopathology
Grossly, the tumor is rubbery-firm with a pale, gray color. It shows different degrees of vascularity and has a well-
defined capsule, which could be discoursed by the displaced and stretched nerve fibers. The cut section is usually pale
gray and firm with a finely trabeculated appearance. Often, evidence of cystic degeneration, hemorrhage,
xanthomatous changes and points of calcification may be present and are encountered in large tumors. These changes
give a variegated appearance in consistency and color to the giant tumors. The blood supply of the tumor comes
mainly from the internal auditory artery, which forks the surface of the tumor with several tiny branches. For larger
tumors, there might be blood supply through small branches of the neighboring cerebellar and pontine arteries.

On light microscopy, the tumor is made up of spindle cells that have elongated nuclei and fibrillary cytoplasm. Those
cells are arranged in two specific ways: Antoni A and Antoni B. Antoni A tissue is small, with a prominent,
organized, interwoven course of elongated bipolar cells. Occasionally, the arrangement of the nuclei and fibers results
https://www.ncbi.nlm.nih.gov/books/NBK470177/?report=printable 1/5
8/22/2019 Acoustic Neuroma (Vestibular Schwannoma) - StatPearls - NCBI Bookshelf

in a spiral framework simulating that seen in meningiomas. Antoni B is represented by a random gathering of
cells grouped around foci of cystic change, necrosis, old hemorrhage, and blood vessels. There is a variable amount of
lymphocytic infiltration of this tissue. The Antoni B tissue type, generally seen in large tumors, is thought to be the
outcome of ischemia. The consistency of the tumor is determined by the distant relative quantities of these two types.
Although these are benign changes, as malignant transformation almost never happens, nuclear pleomorphism is
typical in schwannomas. Mitotic figures are relatively rare. Necrosis, if found, is more due to a poor blood supply
rather than fast development. Edema, development of micro or possibly macrocysts, xanthomatous alteration, and
areas of calcification are attributed to the degenerative changes in the tumor tissue. Electron microscopy (EM) reveals
the characteristic basement membrane of the Schwann cells as well as the presence of wide-spaced collagen.[7]

History and Physical

The signs and symptoms of acoustic neuroma are attributed to the involvement of the cranial nerve VIII,
compressing surrounding cranial nerves, cerebellum, brainstem, as well as raised intracranial pressure (ICP). The
majority of acoustic neuromas present with unilateral hearing loss due to cochlear nerve interruption or impairment of
blood supply to the nerve. Other clinical features include tinnitus, decreased word understanding, vertigo, headaches,
and facial numbness. With enough growth, the mass of CP angle will eventually compress the brainstem and cause
gait abnormalities. The following is a summary of the detailed clinical features.

Due to the involvement of Cranial Nerve VIII

Auditory

1. Hearing impairment: the most common symptom, slowly progressive, high frequency retrocochlear
sensorineural type. May pass unnoticed due to its insidious onset. May be examined in the physical through
speech discrimination, using tuning forks of wide-range frequencies, Weber’s test as well as Rinne’s test.

2. Tinnitus: also a common symptom, can be intermittent

Vestibular: Instability while moving the head and nystagmus

Due to compression of other Cranial Nerves

Facial nerve: usually minimal with late presentation except for very large tumors. Depending on the degree
of engagement of the nerve, the symptoms may include twitching, increased lacrimation and facial weakness

Trigeminal Nerve: paraesthesia in the trigeminal distribution, tingling of the tongue, impairment of the corneal
reflex, and less commonly pain which may mimic typical trigeminal neuralgia

The glossopharyngeal and vagus nerves: palatal paresis, hoarseness of voice and dysphagia

Due to Cerebellar Compression

Occurs in large tumors

Symptoms include gait ataxia and incoordination of the upper limb, and rarely dysarthria

Due to Brainstem Compression/Torsion

Symptoms include pyramidal weakness, contralateral cranial nerves involvement, and nystagmus

Due to Raised ICP

These include, but are not limited to, headache, nausea, vomiting, increased blood pressure, decreased mental
abilities, confusion, disturbance of level of consciousness, and papilledema.

https://www.ncbi.nlm.nih.gov/books/NBK470177/?report=printable 2/5
8/22/2019 Acoustic Neuroma (Vestibular Schwannoma) - StatPearls - NCBI Bookshelf

Evaluation
The diagnosis of an acoustic neuroma is made with a contrast MRI or a CT scan. Contrast is essential; otherwise, the
non-enhanced scan can miss small tumors. If hearing impairment is present, audiometric tests are needed. Auditory
brainstem evoked response is not used frequently to screen for acoustic tumors as it can miss small malignancies.[8]
[9][10]

Treatment / Management
Acoustic neuroma may be treated by:

Observation if small and in elderly patients with numerous comorbidities

Stereotactic radiotherapy or

Open craniotomy. The type of treatment depends on surgeon experience and patient preferences. After surgery,
MRI is required within 6-12 months to document the extent of tumor removal and serve as a baseline. The most
common complications of surgery include injury to the anterior inferior cerebral artery, hemorrhage, cerebellar
trauma, facial paralysis, hearing loss (the most common)and hydrocephalus.

Differential Diagnosis
Acoustic neuroma accounts for around 80% to 90% of cerebellopontine angle (CPA) lesions. Differential diagnosis of
an acoustic neuroma include:

Meningioma (5% to 10% of CPA lesions)

Ectodermal inclusion tumors

1. Epidermoid (5% to 7% of CPA lesions)

2. Dermoid

Metastases

Neuroma from cranial nerves other than cranial nerve VIII:

1. Trigeminal neuroma.

2. Facial nerve neuroma

3. Neurinoma of the lowest four cranial nerves (IX, X, XI, XII)

Arachnoid Cyst

Neurenteric Cyst

Cholesterol granuloma (distinct from epidermoid)

Aneurysm

Dolichobasilar ectasia

Extensions of nearby lesions in the CP angle:

1. Brainstem or cerebellar glioma

2. Pituitary adenoma

3. Craniopharyngioma

https://www.ncbi.nlm.nih.gov/books/NBK470177/?report=printable 3/5
8/22/2019 Acoustic Neuroma (Vestibular Schwannoma) - StatPearls - NCBI Bookshelf

4. Chordoma and tumors of skull base

5. Fourth ventricle tumors (ependymoma, medulloblastoma)

6. Choroids plexus papilloma from the fourth ventricle through Foramen of Luschka

7. Glomus jugulare tumor

8. Tumors of temporal bone

Complications
Most complications are related to the surgery and include the following:

Injury to the anterior or posterior intracerebral arteries

Neurological injury

Brain herniation

Brain hemorrhage

Injury to the cerebellum

Facial paralysis

Hearing loss

Pearls and Other Issues

Recurrence is not common after removal, but tinnitus may worsen in some patients. Hearing loss and facial paralysis
may improve with time.

The pre-operative distinction between a meningioma and an acoustic schwannoma is important for technical and
prognostic reasons.

Enhancing Healthcare Team Outcomes

Acoustic neuromas are not common tumors in clinical practice. However, they often present with unilateral hearing
loss and thus healthcare workers should consider the lesion in the differential diagnosis. The tumor once diagnosed is
best managed by a multidisciplinary team that includes a neurosurgeon, neurologist, ENT surgeon, and a hearing
specialist. After surgery, tinnitus is a common problem in at least 10-20% of patients. The recurrence rate after
excision is less than 5%. Facial nerve paralysis appears to occur in about 15-30% of patients but most make a
complete recovery over time. Hearing loss occurs in more than 50% of patients and may not improve over time.
Residual hearing loss has a significant impact on the quality of life. [11][12][13] (Level V)

Questions
To access free multiple choice questions on this topic, click here.

References
1. West N, Sass H, Møller MN, Cayé-Thomasen P. Facial nerve schwannomas presenting with vestibular
dysfunction: a case series. Acta Neurochir (Wien). 2018 Dec;160(12):2315-2319. [PubMed: 30370441]
2. Meola A, Chang SD. Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2. N. Engl. J. Med. 2018 Oct
11;379(15):1463. [PubMed: 30304657]
3. Braunstein S, Ma L. Stereotactic radiosurgery for vestibular schwannomas. Cancer Manag Res. 2018;10:3733-
3740. [PMC free article: PMC6159807] [PubMed: 30288104]

https://www.ncbi.nlm.nih.gov/books/NBK470177/?report=printable 4/5
View publication stats

8/22/2019 Acoustic Neuroma (Vestibular Schwannoma) - StatPearls - NCBI Bookshelf

4. Zhao F, Yang Z, Chen Y, Zhou Q, Zhang J, Liu J, Wang B, He Q, Zhang L, Yu Y, Liu P. Deregulation of the Hippo
Pathway Promotes Tumor Cell Proliferation Through YAP Activity in Human Sporadic Vestibular Schwannoma.
World Neurosurg. 2018 Sep;117:e269-e279. [PubMed: 29902598]
5. Miller AB, Morgan LL, Udasin I, Davis DL. Cancer epidemiology update, following the 2011 IARC evaluation of
radiofrequency electromagnetic fields (Monograph 102). Environ. Res. 2018 Nov;167:673-683. [PubMed:
30196934]
6. Koo M, Lai JT, Yang EY, Liu TC, Hwang JH. Incidence of Vestibular Schwannoma in Taiwan from 2001 to 2012:
A Population-Based National Health Insurance Study. Ann. Otol. Rhinol. Laryngol. 2018 Oct;127(10):694-697.
[PubMed: 30032646]
7. Landegger LD, Sagers JE, Dilwali S, Fujita T, Sahin MI, Stankovic KM. A Unified Methodological Framework
for Vestibular Schwannoma Research. J Vis Exp. 2017 Jun 20;(124) [PMC free article: PMC5608471] [PubMed:
28654042]
8. Sass H, Cayé-Thomasen P. Contemporary Molecular Biology of Sporadic Vestibular Schwannomas: A Systematic
Review and Clinical Implications. J Int Adv Otol. 2018 Aug;14(2):322-329. [PMC free article: PMC6354447]
[PubMed: 30100540]
9. Lees KA, Tombers NM, Link MJ, Driscoll CL, Neff BA, Van Gompel JJ, Lane JI, Lohse CM, Carlson ML.
Natural History of Sporadic Vestibular Schwannoma: A Volumetric Study of Tumor Growth. Otolaryngol Head
Neck Surg. 2018 Sep;159(3):535-542. [PubMed: 29685084]
10. Sweeney AD, Carlson ML, Shepard NT, McCracken DJ, Vivas EX, Neff BA, Olson JJ. Congress of
Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Otologic and Audiologic
Screening for Patients With Vestibular Schwannomas. Neurosurgery. 2018 Feb 01;82(2):E29-E31. [PubMed:
29309699]
11. Lin KF, Stewart CR, Steig PE, Brennan CW, Gutin PH, Selesnick SH. Incidence of Prolonged Systemic Steroid
Treatment after Surgery for Acoustic Neuroma and Its Implications. J Neurol Surg B Skull Base. 2018
Dec;79(6):559-568. [PMC free article: PMC6239872] [PubMed: 30456025]
12. Peng KA, Chen BS, Lorenz MB, Lekovic GP, Schwartz MS, Slattery WH, Wilkinson EP. Revision Surgery for
Vestibular Schwannomas. J Neurol Surg B Skull Base. 2018 Dec;79(6):528-532. [PMC free article:
PMC6239869] [PubMed: 30456020]
13. Chiluwal AK, Rothman A, Svrakic M, Dehdashti AR. Surgical outcome in smaller symptomatic vestibular
schwannomas. Is there a role for surgery? Acta Neurochir (Wien). 2018 Nov;160(11):2263-2275. [PubMed:
30229403]

Copyright © 2019, StatPearls Publishing LLC.

This book is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/),
which permits use, duplication, adaptation, distribution, and reproduction in any medium or format, as long as you give appropriate credit to the original
author(s) and the source, a link is provided to the Creative Commons license, and any changes made are indicated.

Bookshelf ID: NBK470177 PMID: 29262098

https://www.ncbi.nlm.nih.gov/books/NBK470177/?report=printable 5/5