Acoustic Neuroma

Updated: August 24, 2023

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Background

Acoustic Neuroma, also referred to as vestibular schwannoma, is a tumor that is non-malignant (not cancerous) and arises on the eighth cranial nerve. This nerve is responsible for conveying balance and sound data from the inner ear to the brain and is also known as the vestibulocochlear or auditory-vestibular nerve.

It arises from a specific type of cell called Schwann cells, which form the protective covering (myelin sheath) around the nerves. These tumors grow slowly and usually originate from the Schwann cells lining the vestibular portion of the eighth cranial nerve.

Epidemiology

The epidemiology of Acoustic Neuroma:

  • Incidence and Prevalence: Acoustic Neuroma are uncommon growths, comprising approximately 8-10% of all tumors within the skull. It is approximated that there are 1 to 2 instances of this condition per 100,000 people annually.
  • Age distribution: Although Acoustic Neuroma may develop at any point in time, they are typically identified in individuals aged 30 to 60 years. The highest occurrence of cases is frequently observed during the fifth and sixth decades of life.
  • Sporadic vs. hereditary cases: Acoustic Neuroma are sporadic, occurring without any known genetic predisposition. Approximately 5-10% of cases are associated with a condition called neurofibromatosis type 2 (NF2), an inherited disorder characterized by the development of tumors in the nervous system.

Anatomy

Pathophysiology

The pathophysiology of Acoustic Neuroma involves the abnormal growth of Schwann cells on the eighth cranial nerve, which is responsible for transmitting sound and balance information from the inner ear to the brain.

  • Proliferation of Schwann cells: Acoustic Neuroma arises from Schwann cells, which usually offer backing and safeguarding to nerve fibers.
  • Loss of tumor suppressor genes: In certain instances, the formation of Acoustic Neuroma might be linked with the depletion or deactivation of definite tumor suppressor genes.
  • Compression and displacement: As the Acoustic Neuroma grow, it can exert pressure on surrounding structures, including the adjacent cranial nerves, brainstem, and blood vessels. This compression can cause various symptoms, such as hearing loss, balance problems, facial numbness or weakness, and headaches.
  • Impaired nerve function: The tumor can disrupt the normal functioning of the affected cranial nerve. In the case of Acoustic Neuroma, the vestibulocochlear nerve is affected, leading to hearing loss, tinnitus, and balance disturbances.

Etiology

Tumor growth and compression: Acoustic Neuroma usually proliferate at a gradual pace and as they expand in size, they can put pressure on neighboring formations, such as the contiguous cranial nerves, brainstem, and blood vessels. The exact indications are determined by the location and magnitude of the tumor.

  • Disruption of nerve function: The growth disrupts the typical operation of the vestibulocochlear nerve. The pressure applied by the tumor on the nerve fibers hinders the transfer of auditory and equilibrium signals to the brain.
  • Angiogenesis: As the tumor expands, it stimulates the emergence of fresh blood vessels to furnish oxygen and nutrients for its heightened metabolic necessities. This angiogenesis can contribute to the advancement and expansion of the tumor.

Genetics

Prognostic Factors

Several prognostic factors can help predict the outcome and guide the management of Acoustic Neuroma. These factors provide insights into the tumor’s behavior, growth pattern, and the potential impact on a patient’s quality of life.

  • Size of tumor: The magnitude of the Acoustic Neuroma during detection is a crucial indicator of future outcomes. The smaller growth usually has a positive prognosis and is linked to a reduced risk of complications.
  • Auditory condition: The hearing condition before surgery is a vital indicator of future outcomes, particularly for patients with functional hearing. The conservation of hearing is contingent on the magnitude, positioning of the growth, and the surgical method used.
  • Facial nerve function: The status of the facial nerve, which controls the muscles of facial expression, is another important prognostic factor. Preservation of facial nerve function and avoidance of facial nerve injury during tumor removal surgery.
  • Age: The age of diagnosis may impact the prognosis of Acoustic Neuroma. Typically, younger patients experience more favorable outcomes due to fewer comorbidities and better overall health.

Clinical History

CLINICAL HISTORY

Age Group:

  • Acoustic Neuroma may emerge at any stage of life, though they are generally identified in grown-ups ranging from 30 to 60 years old. The highest frequency of acoustic tumors typically arises during the fifth and sixth decades of existence.
  • Acoustic Neuroma are rare in younger individuals, particularly those below 18 years of age. When children experience acoustic tumors, they are usually connected to genetic disorders like neurofibromatosis type 2 (NF2).

Physical Examination

PHYSICAL EXAMINATION

  • Otoscopy: The medical practitioner will employ an otoscope to inspect the outer ear and ear canal. This evaluation assists in eliminating other ear ailments and evaluating the general well-being of the ear.
  • Audiometry: A hearing examination, such as pure-tone audiometry, is carried out to assess the patient’s hearing capacity. This procedure measures the patient’s hearing levels at various pitches and helps to establish the degree of hearing loss, particularly in the impacted ear.
  • Neurological examination: A comprehensive neurological examination is conducted to evaluate other neurological functions. This may include assessing muscle strength, reflexes, sensation, and coordination.

Age group

Associated comorbidity

Associated Comorbidity or Activity:

Some of the commonly observed comorbidities in Acoustic Neuroma include:

  • Auditory impairment and tinnitus: Acoustic Neuroma frequently exhibit indications associated with auditory impairment and ringing in the ears. These indications can have a considerable effect on the patient’s ability to communicate and their quality of life.
  • Cognitive and psychological impacts: Living with an Acoustic Neuroma and managing its associated symptoms can have psychological and cognitive effects. Patients may experience anxiety, depression, or cognitive difficulties due to the stress of the diagnosis.
  • Equilibrium and Coordination difficulties: As a result of the vestibular segment of the eighth cranial nerve being affected, Acoustic Neuroma can lead to equilibrium disruptions, vertigo, and difficulties with synchronization.
  • Facial nerve dysfunction: The facial nerve runs near the vestibulocochlear nerve, which is affected by Acoustic Neuroma.

Associated activity

Acuity of presentation

Acuity of Presentation:

  • Acute or immediate onset: Although less frequent, certain individuals may encounter an abrupt or immediate manifestation of symptoms. This can transpire if the tumor undergoes rapid expansion or if there is an abrupt alteration in its size or location.
  • Slow onset: In many cases, the indications of Acoustic Neuroma appear gradually over a period. Individuals may undergo a gradual hearing impairment in one ear, frequently accompanied by ringing in the ears. At first, these indications may be inconspicuous and remain unnoticed.
  • Asymptomatic: In certain cases, Acoustic Neuroma may be detected incidentally during imaging studies performed for unrelated reasons, such as a head CT or MRI.

Differential Diagnoses

DIFFERENTIAL DIAGNOSIS

  • Meniere’s syndrome: Meniere’s syndrome is a condition of the internal ear marked by repeated bouts of vertigo, hearing impairment, buzzing in the ear, and a sensation of pressure in the ear.
  • Benign paroxysmal positional vertigo (BPPV): BPPV is a frequent condition of the inner ear that causes short bouts of vertigo activated by specific head motions. It may give rise to symptoms such as lightheadedness, instability, and nausea.
  • Vertigo induced by Migraine: A few people who suffer from migraines can undergo episodes of dizziness and vertigo, frequently together with headache, nausea, and hypersensitivity to light and sound.
  • Acute vestibular neuritis: The sudden inflammation of the vestibular nerve, generally due to a viral infection, is known as acute vestibular neuritis. It manifests as severe vertigo, instability, nausea, and vomiting.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

TREATMENT PARADIGM

Modification of Environment:

Modifying the environment can help individuals with Acoustic Neuroma manage their symptoms and improve their overall well-being.

  • Ensure good lighting and visibility: Acoustic Neuroma can affect balance and coordination, increasing the risk of falls. Ensuring adequate lighting and removing tripping hazards in the environment can help improve visibility and reduce the risk of accidents.
  • Reduce noise exposure: Excessive noise can worsen symptoms and increase discomfort for individuals with acoustic neuroma. It is advisable to limit exposure to loud noises, such as concerts, noisy workplaces, or loud machinery.

Administration of Pharmaceutical Agents with Drugs:

  • Medications for dizziness and vertigo: Drugs that combat vertigo, such as dimenhydrinate, meclizine, or betahistine, may be recommended to mitigate symptoms of dizziness and vertigo that are linked to acoustic neuroma.
  • Antiemetics: Antiemetic medications, for instance, promethazine or ondansetron, can be utilized to control nausea and vomiting that could be present alongside vertigo or dizziness.
  • Corticosteroids: Short-term use of corticosteroids, such as prednisone, may be prescribed to reduce inflammation and swelling around the Acoustic Neuroma. Steroids can help alleviate symptoms like facial nerve compression and improve overall comfort.

Intervention with a Procedure:

  • Microscopic resection: The tumor can be eliminated surgically with the aid of microscopic techniques, such as retrosigmoid or translabyrinthine approaches. The aim of the operation is to completely eradicate the tumor while safeguarding neurological function, particularly the facial nerve and hearing if possible.
  • Decompression surgery: In some cases, when complete tumor removal is not possible or when the tumor is causing severe symptoms, decompression surgery may be performed.

Phase of Management:

The phases of management typically include:

  • Assessment and Analysis: The first step comprises the assessment of Acoustic Neuroma via a blend of clinical analysis, scrutiny of medical history, bodily inspection, and diagnostic assessments. These assessments might encompass audiometry, MRI scans, and infrequently supplementary imaging or genetic testing.
  • Decision-Making and Treatment Planning: The treatment options may include observation (watchful waiting), surgery, or radiation therapy (such as stereotactic radiosurgery). The benefits, risks, and potential outcomes of each option are discussed to help the patient make an informed decision.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

Acoustic Neuroma – StatPearls – NCBI Bookshelf (nih.gov)

Acoustic Neuroma

Updated : August 24, 2023

Mail Whatsapp PDF Image



Acoustic Neuroma, also referred to as vestibular schwannoma, is a tumor that is non-malignant (not cancerous) and arises on the eighth cranial nerve. This nerve is responsible for conveying balance and sound data from the inner ear to the brain and is also known as the vestibulocochlear or auditory-vestibular nerve.

It arises from a specific type of cell called Schwann cells, which form the protective covering (myelin sheath) around the nerves. These tumors grow slowly and usually originate from the Schwann cells lining the vestibular portion of the eighth cranial nerve.

The epidemiology of Acoustic Neuroma:

  • Incidence and Prevalence: Acoustic Neuroma are uncommon growths, comprising approximately 8-10% of all tumors within the skull. It is approximated that there are 1 to 2 instances of this condition per 100,000 people annually.
  • Age distribution: Although Acoustic Neuroma may develop at any point in time, they are typically identified in individuals aged 30 to 60 years. The highest occurrence of cases is frequently observed during the fifth and sixth decades of life.
  • Sporadic vs. hereditary cases: Acoustic Neuroma are sporadic, occurring without any known genetic predisposition. Approximately 5-10% of cases are associated with a condition called neurofibromatosis type 2 (NF2), an inherited disorder characterized by the development of tumors in the nervous system.

The pathophysiology of Acoustic Neuroma involves the abnormal growth of Schwann cells on the eighth cranial nerve, which is responsible for transmitting sound and balance information from the inner ear to the brain.

  • Proliferation of Schwann cells: Acoustic Neuroma arises from Schwann cells, which usually offer backing and safeguarding to nerve fibers.
  • Loss of tumor suppressor genes: In certain instances, the formation of Acoustic Neuroma might be linked with the depletion or deactivation of definite tumor suppressor genes.
  • Compression and displacement: As the Acoustic Neuroma grow, it can exert pressure on surrounding structures, including the adjacent cranial nerves, brainstem, and blood vessels. This compression can cause various symptoms, such as hearing loss, balance problems, facial numbness or weakness, and headaches.
  • Impaired nerve function: The tumor can disrupt the normal functioning of the affected cranial nerve. In the case of Acoustic Neuroma, the vestibulocochlear nerve is affected, leading to hearing loss, tinnitus, and balance disturbances.

Tumor growth and compression: Acoustic Neuroma usually proliferate at a gradual pace and as they expand in size, they can put pressure on neighboring formations, such as the contiguous cranial nerves, brainstem, and blood vessels. The exact indications are determined by the location and magnitude of the tumor.

  • Disruption of nerve function: The growth disrupts the typical operation of the vestibulocochlear nerve. The pressure applied by the tumor on the nerve fibers hinders the transfer of auditory and equilibrium signals to the brain.
  • Angiogenesis: As the tumor expands, it stimulates the emergence of fresh blood vessels to furnish oxygen and nutrients for its heightened metabolic necessities. This angiogenesis can contribute to the advancement and expansion of the tumor.

Several prognostic factors can help predict the outcome and guide the management of Acoustic Neuroma. These factors provide insights into the tumor’s behavior, growth pattern, and the potential impact on a patient’s quality of life.

  • Size of tumor: The magnitude of the Acoustic Neuroma during detection is a crucial indicator of future outcomes. The smaller growth usually has a positive prognosis and is linked to a reduced risk of complications.
  • Auditory condition: The hearing condition before surgery is a vital indicator of future outcomes, particularly for patients with functional hearing. The conservation of hearing is contingent on the magnitude, positioning of the growth, and the surgical method used.
  • Facial nerve function: The status of the facial nerve, which controls the muscles of facial expression, is another important prognostic factor. Preservation of facial nerve function and avoidance of facial nerve injury during tumor removal surgery.
  • Age: The age of diagnosis may impact the prognosis of Acoustic Neuroma. Typically, younger patients experience more favorable outcomes due to fewer comorbidities and better overall health.

CLINICAL HISTORY

Age Group:

  • Acoustic Neuroma may emerge at any stage of life, though they are generally identified in grown-ups ranging from 30 to 60 years old. The highest frequency of acoustic tumors typically arises during the fifth and sixth decades of existence.
  • Acoustic Neuroma are rare in younger individuals, particularly those below 18 years of age. When children experience acoustic tumors, they are usually connected to genetic disorders like neurofibromatosis type 2 (NF2).

PHYSICAL EXAMINATION

  • Otoscopy: The medical practitioner will employ an otoscope to inspect the outer ear and ear canal. This evaluation assists in eliminating other ear ailments and evaluating the general well-being of the ear.
  • Audiometry: A hearing examination, such as pure-tone audiometry, is carried out to assess the patient’s hearing capacity. This procedure measures the patient’s hearing levels at various pitches and helps to establish the degree of hearing loss, particularly in the impacted ear.
  • Neurological examination: A comprehensive neurological examination is conducted to evaluate other neurological functions. This may include assessing muscle strength, reflexes, sensation, and coordination.

Associated Comorbidity or Activity:

Some of the commonly observed comorbidities in Acoustic Neuroma include:

  • Auditory impairment and tinnitus: Acoustic Neuroma frequently exhibit indications associated with auditory impairment and ringing in the ears. These indications can have a considerable effect on the patient’s ability to communicate and their quality of life.
  • Cognitive and psychological impacts: Living with an Acoustic Neuroma and managing its associated symptoms can have psychological and cognitive effects. Patients may experience anxiety, depression, or cognitive difficulties due to the stress of the diagnosis.
  • Equilibrium and Coordination difficulties: As a result of the vestibular segment of the eighth cranial nerve being affected, Acoustic Neuroma can lead to equilibrium disruptions, vertigo, and difficulties with synchronization.
  • Facial nerve dysfunction: The facial nerve runs near the vestibulocochlear nerve, which is affected by Acoustic Neuroma.

Acuity of Presentation:

  • Acute or immediate onset: Although less frequent, certain individuals may encounter an abrupt or immediate manifestation of symptoms. This can transpire if the tumor undergoes rapid expansion or if there is an abrupt alteration in its size or location.
  • Slow onset: In many cases, the indications of Acoustic Neuroma appear gradually over a period. Individuals may undergo a gradual hearing impairment in one ear, frequently accompanied by ringing in the ears. At first, these indications may be inconspicuous and remain unnoticed.
  • Asymptomatic: In certain cases, Acoustic Neuroma may be detected incidentally during imaging studies performed for unrelated reasons, such as a head CT or MRI.

DIFFERENTIAL DIAGNOSIS

  • Meniere’s syndrome: Meniere’s syndrome is a condition of the internal ear marked by repeated bouts of vertigo, hearing impairment, buzzing in the ear, and a sensation of pressure in the ear.
  • Benign paroxysmal positional vertigo (BPPV): BPPV is a frequent condition of the inner ear that causes short bouts of vertigo activated by specific head motions. It may give rise to symptoms such as lightheadedness, instability, and nausea.
  • Vertigo induced by Migraine: A few people who suffer from migraines can undergo episodes of dizziness and vertigo, frequently together with headache, nausea, and hypersensitivity to light and sound.
  • Acute vestibular neuritis: The sudden inflammation of the vestibular nerve, generally due to a viral infection, is known as acute vestibular neuritis. It manifests as severe vertigo, instability, nausea, and vomiting.

TREATMENT PARADIGM

Modification of Environment:

Modifying the environment can help individuals with Acoustic Neuroma manage their symptoms and improve their overall well-being.

  • Ensure good lighting and visibility: Acoustic Neuroma can affect balance and coordination, increasing the risk of falls. Ensuring adequate lighting and removing tripping hazards in the environment can help improve visibility and reduce the risk of accidents.
  • Reduce noise exposure: Excessive noise can worsen symptoms and increase discomfort for individuals with acoustic neuroma. It is advisable to limit exposure to loud noises, such as concerts, noisy workplaces, or loud machinery.

Administration of Pharmaceutical Agents with Drugs:

  • Medications for dizziness and vertigo: Drugs that combat vertigo, such as dimenhydrinate, meclizine, or betahistine, may be recommended to mitigate symptoms of dizziness and vertigo that are linked to acoustic neuroma.
  • Antiemetics: Antiemetic medications, for instance, promethazine or ondansetron, can be utilized to control nausea and vomiting that could be present alongside vertigo or dizziness.
  • Corticosteroids: Short-term use of corticosteroids, such as prednisone, may be prescribed to reduce inflammation and swelling around the Acoustic Neuroma. Steroids can help alleviate symptoms like facial nerve compression and improve overall comfort.

Intervention with a Procedure:

  • Microscopic resection: The tumor can be eliminated surgically with the aid of microscopic techniques, such as retrosigmoid or translabyrinthine approaches. The aim of the operation is to completely eradicate the tumor while safeguarding neurological function, particularly the facial nerve and hearing if possible.
  • Decompression surgery: In some cases, when complete tumor removal is not possible or when the tumor is causing severe symptoms, decompression surgery may be performed.

Phase of Management:

The phases of management typically include:

  • Assessment and Analysis: The first step comprises the assessment of Acoustic Neuroma via a blend of clinical analysis, scrutiny of medical history, bodily inspection, and diagnostic assessments. These assessments might encompass audiometry, MRI scans, and infrequently supplementary imaging or genetic testing.
  • Decision-Making and Treatment Planning: The treatment options may include observation (watchful waiting), surgery, or radiation therapy (such as stereotactic radiosurgery). The benefits, risks, and potential outcomes of each option are discussed to help the patient make an informed decision.

Acoustic Neuroma – StatPearls – NCBI Bookshelf (nih.gov)

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