Acoustic Neuroma (Acoustic Neurinoma)


Acoustic neuroma is a rare tumor that originates from the Schwann cells of the eighth cranial nerve, also known as vestibulocochlear nerve. This condition is characterized by unilateral hearing loss but has other sequelae as well.

Acoustic Neuroma is the consequence of the following process: neoplastic.


Acoustic neuromas are unilateral in greater than 90% of patients [1]. The bilateral cases are confined to neurofibromatosis type 2 [7].

The most common presenting feature in acoustic neuroma is the unilateral sensorineural hearing loss, which is slow and gradual in the majority of patients. This occurs secondary to either vascular insufficiency or direct injury to the cochlear nerve. The hearing deficit can be sudden or it can fluctuate depending on cochlear perfusion and its interruption. Some patients may recover their hearing spontaneously. Note that hearing impairment is not related to the size of the acoustic neuroma.

Unilateral tinnitus very often coexists with hearing loss. Although if present alone, the presence of tinnitus should prompt suspicion for acoustic neuroma.

The majority of patients will have low speech discrimination scores as displayed on audiological tests. According to one study, almost 65% of individuals demonstrated a link between hearing loss severity and reduced speech discrimination [8].

Approximately half of acoustic neuroma patients will experience balance disturbance although vertigo and disequilibrium are not present initially.

At least half of the patients exhibit headaches especially in cases with growing tumors and those with obstructive hydrocephalus.

Another manifestation is facial numbness, which is exhibited in approximately 25% of patients. Up to 70% of those with large tumors exhibit hypoesthesia on exam but are not aware of it.

Facial weakness is rare and should prompt investigation of differential diagnoses such as arteriovenous malformation (AVM), meningioma, hemangioma, and facial neuroma.

  • In some patients the tinnitus is a pure tone, and in others the tinnitus is a noise.[]
  • Tinnitus is very frequent in AN as it is many inner ear diseases.[]
  • Not all patients with tinnitus have acoustic neuroma and not all AN patients have tinnitus.[]
  • Tinnitus The increasing hearing difficulties are often accompanied tinnitus, often referred to as "ringing in the ears", Tinnitus is the sensation of hearing ringing, buzzing, hissing, chirping, whistling, or other sounds.[]
  • These problems may need additional treatment – read more about treating hearing loss and treating tinnitus .[]
Hearing Impairment
Sudden Hearing Loss
  • Conversely, sudden hearing loss is attributed to an acoustic neuroma patient only about 1 percent to 5 percent of the time, as there are many causes of sudden hearing loss.[]
  • Acoustic neuroma in patients with completely resolved sudden hearing loss.[]
  • Presumably, sudden hearing loss results from tumor compression.[]
  • A sudden hearing loss occurs in about 25 percent of patients with acoustic neuroma.[]
  • A sudden hearing loss occurs in about 25% of patients with acoustic neuroma.[]
Hearing Problem
  • Our Mission The Acoustic Neuroma and Hearing Preservation Program at UCSF comprises a multidisciplinary team of doctors, nurses, and scientists devoted to the care of patients with complex hearing problems.[]
  • 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[]
  • 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 a magnetic resonance[]
  • Those affected often notice the hearing problem very late or by chance, for example when telephoning or during a routine examination.[]
  • Published: October 8, 2013 Updated: October 9, 2013 at 03:55 PM ZEPHYRHILLS — Occupational therapist Evelyn Lopez was treating a patient at Florida Hospital Zephyrhills when she casually mentioned her hearing problem to the student observer working with[]
  • The clinical signs may be characterized as: a) progressive unilateral or asymmetrical sensorineural dysacusis, b) intermittent hearing loss, c) tinnitus and sensation of fullness in the ear, d) lasting positional vertigo, e) difficulties in walking, f[]
Fluctuating Hearing Loss
  • Sudden and fluctuating hearing losses are more easily explained on the basis of disruption of cochlear blood supply.[]
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  • neurologic
    • Headache [ edit ] Recurring headaches are an uncommon symptom, also tending to occur only in cases of larger tumors.[]
    • Headache after acoustic neuroma excision.[]
    • Compression and attentuation of vital structures in this stage can lead to increased headaches and numbness of the face.[]
    • Some less common symptoms include: headache problems with vision difficulty understanding speech pain in the face or ear numbness in the face or ear fatigue Diagnosis of Acoustic Neuroma If you experience hearing loss or other neurologic symptoms, it[]
    • The headache that results from the acoustic neuroma can be dull or aching in quality and is usually unilateral.[]
    • When to Contact a Medical Professional Call your provider if you have: Hearing loss that is sudden or getting worse Ringing in one ear Dizziness (vertigo) References Arriaga MA, Brackmann DE.[]
    • When to Contact a Medical Professional Call your health care provider if you have: Hearing loss that is new or getting worse Ringing in one ear Dizziness (vertigo) visHeader References Baloh RW, Jen J.[]
    • Lifestyle impact - dizziness and loss of balance can make daily activities difficult to do.[]
    • Unilateral/asymmetric hearing loss and/or tinnitus and loss of balance/dizziness are early signs of a vestibular schwannoma.[]
    • Car or motorcycle licence You must tell DVLA if you suffer from sudden and disabling dizziness.[]
    • True vertigo is not commonly associated with AN.[]
    • When to Contact a Medical Professional Call your provider if you have: Hearing loss that is sudden or getting worse Ringing in one ear Dizziness (vertigo) References Arriaga MA, Brackmann DE.[]
    • When to Contact a Medical Professional Call your health care provider if you have: Hearing loss that is new or getting worse Ringing in one ear Dizziness (vertigo) visHeader References Baloh RW, Jen J.[]
    • The first signs or symptoms usually are related to ear function and include ear noise, hearing loss, and imbalance or vertigo.[]
    • Vertigo — A feeling of spinning or whirling.[]
    • Although little information is available on its exact incidence, truncal (trunk, abdomen, chest) ataxia appears to be more common than limb ataxia.[]
    • […] body) vertigo (the sensation that you're moving or spinning) A large acoustic neuroma can also sometimes cause: persistent headaches temporary blurred or double vision numbness, pain or weakness on one side of the face problems with limb co-ordination (ataxia[]
    • ) vertigo (the sensation that you're moving or spinning) A large acoustic neuroma can also sometimes cause: persistent headaches temporary blurred or double vision numbness, pain or weakness on one side of the face problems with limb co-ordination ( ataxia[]
    • Once the tumor has extended into the cerebello-pontine angle, it may encroach on other cranial nerves such as the 5th cranial nerve, causing facial numbness, or compress the brain stem causing ataxia.[]
    • Alternatively the patient may have signs of ataxia [1] .[]
    • One would expect that this would create timing differences between the ears without spontaneous nystagmus, and result in head-shaking nystagmus , without spontaneous or hyperventilation induced nystagmus.[]
    • Ipsilateral beating nystagmus after acoustic schwannoma resection.[]
    Facial Muscle Weakness
    • Patients and their physicians need to pay close attention for hearing loss, balance and facial muscle weakness.[]
    • Shands at the University of Florida also provides comprehensive rehabilitative services for commonly associated symptoms, such as vertigo or dizziness, tinnitus (ringing in the ears), and facial muscle weakness.[]
    Cranial Nerve Involvement
    • Clinical Presentation [ edit ] The symptoms involved with acoustic neuroma are due to cranial nerve involvement and tumor progression.[]
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  • Face, Head & Neck
    Facial Numbness
    • Complications An acoustic neuroma may cause a variety of permanent complications, including: Hearing loss Facial numbness and weakness Difficulties with balance Ringing in the ear Large tumors may press on your brainstem, preventing the normal flow of[]
    • As it grows, an acoustic neuroma can produce a number of characteristic clinical features, such as: Hearing loss Tinnitus (ringing in the ear) Dizziness Facial numbness (when the tumour is 2.5cm ) Severe imbalance, headaches, nausea, facial weakness ([]
    • Advanced symptoms of acoustic neuroma Symptoms of advanced acoustic neuroma can include: headache pain in the face facial numbness facial twitches visual disturbances, such as double vision difficulties swallowing eventual death as the functioning of[]
    • Unilateral sensorineural hearing loss is the most common symptom, followed by intermittent dizziness and facial numbness.[]
    • Facial numbness is not as common as the facial weakness.[]
    Facial Pain
    • Large tumours may produce additional symptoms including headache, facial pain, numbness or twitching, double vision, speech difficulties, and swallowing problems.[]
    • Intracranial tumors in patiens with facial pain.[]
    • If the 5th cranial nerve (nervus trigeminus) is impaired this leads to sensation problems or facial pain.[]
    • Rarely, facial pain occurs, usually as a sharp stabbing pain.[]
    • If the 5th cranial nerve (Trigeminal Nerve) is impaired this leads to sensation problems, numbness or facial pain, i.e. trigeminal neuralgia.[]
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  • Entire body system
    Impaired Balance
    • Although acoustic neuromas mostly originate from the upper part of the balance nerve, vertigo and impaired balance rank only in third place as a symptom of an acoustic neuroma.[]
    • Balance/Vertigo Although acoustic neuromas mostly originate from the upper part of the balance nerve, vertigo and impaired balance rank only in third place as a symptom of an acoustic neuroma.[]
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  • Workup

    Individuals presenting with unilateral sensorineural hearing deficits should be presumed to have an acoustic neuroma. The diagnosis should be confirmed through evaluation of the clinical picture, history, a physical exam with focus on hearing and neurologic assessment, and imaging studies.


    The confirmatory imaging modality is the gadolinium-enhanced MRI, which detects small lesions measuring 1 to 2mm in diameter. This is contraindicated in patients with ferromagnetic implants. Adding a fast-spin echo feature to MRI imaging is rapid and inexpensive but is ineffective in diagnosing differentials such as demyelinating disease. Fine-cut CT with contrast can identify medium or large tumors in the internal auditory canal but fail to discover lesions less than 1 to 1.5cm.


    Histologic analysis of tissue samples of portrays characteristic histologic findings. There are two types of tissues: Antoni A and Antoni B. The former exhibits a pattern of spindle cells with palisading nuclei referred to as Verocay bodies while the latter displays loose stroma with fewer cells.


    While previously common, audiometric studies are not used for diagnostic purposes anymore.


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  • Laboratory

    Cerebrospinal Fluid
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  • Imaging

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  • CT
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  • Test Results

    Other Test Results
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  • Lumbar Puncture
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  • Treatment

    The are three main therapeutic approaches in the treatment of acoustic neuroma which include observation, stereotactic radiation therapy, and surgical removal of the lesion.

    When deciding the best course of management, the medical team should assess numerous factors [9] such as the parent's hearing status, the size and growth rate of the tumor, the patient's life expectancy, the probability of developing facial nerve paralysis, and the risks of surgery. The latter includes outcomes such as hearing loss and other postsurgical complications. Another variable for consideration is whether the patient has NF2.


    These tumors are typically benign and slow growing. Therefore, careful observation is appropriate for those with small tumors, particularly in the elderly. Groups who are good candidates for observation are the elderly, those with comorbid medical conditions that render them risky for surgery, and patients with the tumor presenting on the side of the unaffected ear. Furthermore, patient preference may favor observation.

    Studies have indicated that up to 40% of patients under observation will eventually warrant intervention and many will become ineligible for surgery during this time period.

    Stereotactic radiotherapy

    As an alternative to microsurgery, this treatment modality applies radiation to target tissues hence minimizing the potential exposure to nearby tissues. The goals of radiation are to inhibit the growth of the tumor cells and to obliterate the lesion's vascular perfusion. One study reported that Gamma Knife radiation is effective in tumor control [10]. although another investigation observed that these tumors are resistant to low dose radiation [11].


    Surgical excision is the preferred treatment for the elimination of acoustic neuroma. There are numerous techniques that may be utilized depending on the surgeon's technical skills, size and location of the tumor, and the desire for hearing preservation.

    When choosing between radiation and microsurgery, there are many variables to be considered such as postsrgical morbidity and mortality, hospital length of stay and cost, etc.


    The patient is monitored with periodic imaging studies and hearing tests.


    The prognosis can be evaluated by the outcome of each symptom. For example, cases with hearing preservation have been noted to increase during the last decade. It is likely that post-radiation and post-surgical patients have similar rates of conservation. However, hearing impairment has been observed in patients who underwent surgery.

    Although tinnitus may improve after surgery, it can actually worsen in a minority of patients. Furthermore, it may develop postoperatively as well. The post-surgical recurrence rate of acoustic neuroma is less than 5%. Note that patients may have residual tumor since intraoperative visualization is quite challenging in many cases.


    Hearing loss following surgical resection may require rehabilitation such as the bone-anchored hearing aid (BAHA) or contralateral routing of signals (CROS) hearing aid. Additionally, patients with post-surgical facial weakness warrant artificial tears and other eye care products until the nerve recovers. Generally, facial nerve recovery is associated with tumors that are smaller than 1.5cm. Moreover, the utilization of intraoperative facial nerve monitoring may lead to the improvement of function.


    • NIH: National Institute on Deafness and Communication Disorders NIDCD Glossary (National Institute on Deafness and Other Communication Disorders)[]
    • Some people are partly or completely deaf after this type of operation.[]
    • Consequently, the ear is made permanently deaf.[]
    • There are now several options to try to rehabilitate deafness in NF2 patients.[]
    • Hearing loss can vary from mild hearing loss to complete deafness.[]
    Sensorineural Hearing Loss
    • Unilateral sensorineural hearing loss is the most common symptom, followed by intermittent dizziness and facial numbness.[]
    • The most common presenting symptoms are unilateral sensorineural hearing loss, tinnitus and imbalance.[]
    • The most common abnormality is an asymmetrical high-frequency sensorineural hearing loss.[]
    • The typical presentation is with adult-onset sensorineural hearing loss or tinnitus .[]
    Acoustic Neuroma
    • Acoustic neuromas are rare.[]
    • Causes of acoustic neuroma The cause of most acoustic neuromas is not known.[]
    Bell's Palsy
    • In this particular case, the facial nerve was damaged causing hearing loss and Bell's palsy on the affected side.[]
    • Neck Surgery, University of Texas Southwestern Medical School Joe Walter Kutz, Jr, MD, FACS is a member of the following medical societies: Alpha Omega Alpha , American Academy of Otolaryngology-Head and Neck Surgery , American Neurotology Society , Otosclerosis[]
    Facial Nerve Disorder
    • Coauthor(s) Peter S Roland, MD Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director, Clinical Center for Auditory, Vestibular, and Facial Nerve Disorders, Chief of Pediatric[]


    The major known risk factor for developing an acoustic neuroma is high-dose ionizing radiation, although the majority of affected individuals do not have apparent risk factors. Some studies suggest that exposure to loud noise [1] [2] and nonmedullary thyroid cancer [3] may be risk factors for developing this tumor.

    Neurofibromatosis type 2

    Acoustic neurofibroma is the major clinical feature of the genetic disorder, neurofibromatosis type 2, which is the result of a mutation of a tumor suppressor gene that is found on chromosome 22.


    The incidence of acoustic neuroma is 1 per population of 100,000 annually. The incidence is rising which may be explained by the widespread utilization of various imaging modalities such as MRI and computed tomography (CT), leading to a higher number of incidental diagnoses [4] [5]. The prevalence in the population is likely higher according to autopsy studies [6] [7].

    With regards to patient demographics, the median age at diagnosis of the condition is about 50 years old [5]. This tumor comprises almost 8% of adult intracranial tumors and at least 80% of cerebellopontine tumors.

    Sex distribution
    Age distribution


    Acoustic neuromas are benign, slow growing, and noninvasive. They rarely undergo malignant transformation. Additionally, there are three distinct patterns of growth as some will remain dormant, while others may grow slowly or rapidly. While most are slow growers, tumors may erratically alternate between these patterns.

    Location of tumor and symptomatology

    In addition to its size, the tumor is also characterized by its location. It may develop in the auditory canal and expand into the cerebellopontine angle. The symptoms result from the compression of nerves, vessels, spinal fluid spaces, as well as the displacement of the brain stem.

    Most of acoustic neuromas arise from Schwann cells located on the vestibular segment of the vestibulocochlear nerve. When these tumors compress the vestibular fibers, the resultant damage ensues in a gradual and slow manner. Therefore, vertigo is not a prominent feature.

    Note that lesions emerging in the internal auditory canal may compress and impinge upon the vestibular nerve, cochlear nerve, or labyrinthine artery. These mechanisms result in hearing loss and vestibular disturbance.

    When these tumors grow, they may extend into the cerebellopontine angle, which is an empty space that contains spinal fluid. Larger lesions can compress other cranial nerves, impinge on neighboring structures, and shift the position of the brain stem. If greater than 4cm, they cause hydrocephalus.


    There are no preventative measures. However, it is important for patients with hearing loss to seek medical care in a timely manner.


    Acoustic neuroma, also referred to as vestibular schwannoma, is a benign tumor that derives from the Schwann cells of the vestibulocochlear nerve. Specifically, the vestibular branch of the nerve accounts for almost all cases of this condition. Most tumors are slow growing and some can expand from the internal auditory canal to the cerebellopontine angle. If the lesion enlarges, it may encompass the brainstem, cerebellum, and compress other cranial nerves. The etiology is unknown but there are potential risk factors.

    The chief symptom at initial presentation is the unilateral sensorineural hearing loss. Other symptoms that develop as the tumor progresses include tinnitus, disequilibrium, headache, and other abnormalities that reflect affected anatomic structures.

    Unilateral hearing impairment should warrant a high index of suspicion for acoustic neuroma. The workup consists of a detailed assessment of the patient's clinical picture and history, a physical examination with a hearing evaluation, and imaging tests. Due to the availability of gadolinium-enhanced magnetic resonance imaging (MRI), the detection of acoustic neuromas is on the rise.

    Patients with acoustic neuroma can be managed by observation, radiation therapy, or microsurgery. The appropriate treatment depends on variables such as the size, location, and growth rate of the tumor, as well as the patient's age, comorbid medical issues, and patient preference.

    Patient Information

    What is an acoustic neuroma?

    This is a rare tumor that grows on the cranial nerve called vestibulocochlear nerve which travels from the brainstem to the ear canal. Specifically, this nerve is responsible for hearing and balance maintenance. The exact cause of this condition is unknown.

    This benign tumor rarely becomes cancerous. Also, most are slow growing and non-invasive.

    What are the risk factors for developing this condition?

    Exposure to loud noise may increase the probability of developing acoustic neuroma. Also, having a personal or family history of neurofibromatosis type 2 (NF2) may also be a risk factor.

    What are the signs and symptoms?

    Although this is a benign tumor, it can cause serious problems for the patient. Early signs and symptoms are as follows:

    • Hearing loss in one ear (while the other ear is normal)
    • Tinnitus: ringing in the same ear as hearing loss
    • Difficulty with discriminating between sounds

    The growing neuroma can cause:

    How is it diagnosed?

    The clinician will assess the patient's symptoms, obtain the medical history, perform a physical exam and a detailed hearing evaluation. Also, imaging tests such as the following will be ordered:

    • MRI
    • CT scan

    How is it treated?

    The medical team will choose the most appropriate treatment by considering factors such as the size, location, and growth of the acoustic tumor. Also, the patient's age, overall health, and preference will also be important factors. There are 3 main options for treatment:

    1. Observation: small tumors can be monitored, especially in the elderly
    2. Radiation: this kills tumor cells and prevents further growth. It is an option for small tumors that are difficult to operate on.
    3. Surgical removal of the tumor: this is warranted when the tumor grows and hearing loss occurs. There are complications such as deafness and paralysis of the muscles on the face.

    The patients will follow-up with their doctor and undergo periodic imaging and hearing tests.

    What is the prognosis?

    The probability of the tumor recurring after surgery is less than 5%.

    Hearing may be preserved in patients who have undergone surgery as well as those who received radiation. However, hearing loss may occur after surgery as well.

    Other symptoms

    Neurofibromatosis Type 2
    • Risk factors Neurofibromatosis type 2 The only confirmed risk factor for acoustic neuroma is having a parent with the rare genetic disorder neurofibromatosis type 2.[]
    • When tumors are present on both right and left sides of the brain, a condition called neurofibromatosis type 2 exists.[]
    • Cause Until recently the cause was unknown except for a small group of patients who have Neurofibromatosis type 2 which is genetically determined.[]
    • Family history of neurofibromatosis type 2 - around 5 percent of people with acoustic neuroma have neurofibromatosis type 2 (NF-2), in which a person develops acoustic neuromas on both auditory nerves.[]
    • Bilateral acoustic neuromas are a common feature of neurofibromatosis type 2.[]
    Acoustic Trauma
    • Risk Factors [ edit ] Acoustic trauma: OR of 2.2 if 10 years exposure to extremely loud noise, OR of 13.1 if 20 or more years of exposure.[]
    Neurofibromatosis Type 1
    • Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease, is a separate genetic disease resembling NF-2, and in extremely rare occasions can manifest with an acoustic neuroma.[]


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    7. Eldridge R, Parry D. Vestibular schwannoma (acoustic neuroma). Consensus development conference. Neurosurgery 1992;30(6):962.
    8. Lee SH, Choi SK, Lim YJ, et al. Otologic manifestations of acoustic neuroma. Acta Oto-Laryngologica. 2015;135(2):140-6.
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    10. Boari N, Bailo M, Gagliardi F, et al. Gamma Knife radiosurgery for vestibular schwannoma: clinical results at long-term follow-up in a series of 379 patients. Journal of Neurosurgery. 2014; 121 Suppl:123-42.
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    • Resolution nonenhanced fast spin echo magnetic resonance imaging: cost-effective screening for acoustic neuroma in patients with sudden sensorineural hearing loss - RL Daniels, C Shelton - Otolaryngology--Head and , 1998 -
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    • 3719-Vol. 74/Ed 4/in 2008 Section: Relato de Caso Pages: 639 to 639 - AHB DellAringa, LFP Sena, R Teixeira, AR DellAringa -
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    • Auditory neuropathy - A Starr, TW Picton, Y Sininger, LJ Hood, CI Berlin - Brain, 1996 - Oxford Univ Press
    • Abnormal vestibular evoked myogenic potentials in the presence of normal caloric responses - S Iwasaki, Y Takai, K Ito, T Murofushi - Otology & Neurotology, 2005 -
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