Acoustic neuroma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Acoustic neuromas, also known as vestibular schwannomas, are relatively common tumors that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle masses.[1] It is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (8th cranial nerve). This tumor arises from the Schwann cells responsible for the myelin sheath that helps keep peripheral nerves insulated.[2] On microscopic histopathological analysis, acoustic neuroma is characterized into two types, Antoni A and Antoni B, based on growth patterns.[3] Majority of the cases of acoustic neuroma are idiopathic. Less common causes include defect in tumor suppressor genes and high-dose ionizing radiation.[4] Common risk factors in the development of acoustic neuroma are neurofibromatosis type 2 and low dose radiation. Symptoms of acoustic neuroma include hearing loss, tinnitus, vertigo, unsteadiness, pressure in the ears, headaches and facial pain.[5] Gadolinium-enhanced MRI scan is diagnostic of acoutic neuroma. On brain MRI, acoustic neuroma is characterized by hypointense mass on T1-weighted MRI and hyperintense mass on T2-weighted MRI.[6] Head CT scan may be diagnostic of acoustic neuroma. Findings on CT scan diagnostic of acoustic neuroma include erosion and widening of the internal acoustic canal.[7] Other diagnostic studies for acoustic neuroma include audiometry, auditory brainstem response test, and electronystagmography.[8] Optimal therapy for acoustic neuroma includes observation, surgery, and radiation therapy.[9] Surgery is the mainstay of treatment for acoustic neuroma. [10]

Classification

Acoustic neuroma may be classified into three subtypes based on MRI, and into four subtypes based on microscopic histopathology.[11]

Pathophysiology

On microscopic histopathological analysis, acoustic neuroma is characterized into two types, Antoni A and Antoni B, based on growth patterns.[12]

Causes

Majority of the cases of acoustic neuroma are idiopathic. Less common causes include defect in tumor suppressor genes, and high-dose ionizing radiation.[13]

Acoustic neuroma differential diagnosis

Acoustic neuroma must be differentiated from meningioma, epidermoid, facial nerve schwannoma, trigeminal schwannoma, ependymoma, leiomymoma, intranodal palisaded myofibroblastoma, malignant peripheral nerve sheath tumour(MPNST), gastrointestinal stromal tumor, neurofibroma, meniere's disease, and bell's palsy. [14]

Epidemiology And Demographics

The incidence of acoustic neuroma is approximately 1 per 100,000 individuals worldwide. The prevalence of acoustic neuroma is approximately 2,500 new cases per 100,000 individuals. Women are more commonly affected with acoustic neuroma than men. Most cases of acoustic neuroma develop in individuals between the ages of 30 and 60.[15]

Risk Factors

Common risk factors in the development of acoustic neuroma are neurofibromatosis type 2, and low dose radiation.

Natural History

Common complications of acoustic neuroma include hearing loss, facial numbness and weakness, difficulty with balance, ringing in the ear, hydrocephalus and recurrence of tumor. Due to improved surgical techniques and early diagnosis, the morbidity associated with surgical removal of these lesions is reduced. Acoustic neuroma patients have a very good prognosis with minimal complications if treated.[16] [17] [18]

History and Symptoms

Symptoms of acoustic neuroma include hearing loss, tinnitus, vertigo, unsteadiness, pressure in the ears, headaches, and facial pain.[19]

Physical Examination

Common physical examination findings of acoustic neuroma include abnormal Weber test, decreased or absent Ipsilateral corneal reflex, facial twitching or hypesthesia, and abnormal caloric stimulation test.[20]

MRI

Gadolinium-enhanced MRI scan is diagnostic of acoutic neuroma. On brain MRI, acoustic neuroma is characterized by hypointense mass on T1-weighted MRI, and hyperintense mass on T2-weighted MRI. [21]

CT

Findings on CT scan diagnostic of acoustic neuroma include erosion, and widening of the internal acoustic canal.[22]

Laboratory Tests

There are no diagnostic lab findings associated with acoustic neuroma.

Other Diagnostic Studies

Other diagnostic studies for acoustic neuroma include audiometry, auditory brainstem response test, and electronystagmography.[23]

Medical Therapy

Optimal therapy for acoustic neuroma includes observation, surgery, and radiation therapy.[24]

Surgery

Surgery is the mainstay of treatment for acoustic neuroma. [25]

References

  1. "Wikipedia acoustic neuroma".
  2. "Radiopedia acoustic neuroma overview".
  3. "Radiopedia acoustic neuroma pathology".
  4. "Wikipedia acoustic neuroma causes".
  5. "NIH Acoustic neuroma symptoms".
  6. "Wikipedia acoustic neuroma diagnosis".
  7. "Radiopedia CT findings".
  8. "Wikipedia acoustic neuroma diagnosis".
  9. "Wikipedia Acoustic neuroma treatment".
  10. "Wikipedia Acoustic neuroma treatment".
  11. "Libre Pathology schwannoma acoustic neuroma subtypes".
  12. "Radiopedia acoustic neuroma pathology".
  13. "Wikipedia acoustic neuroma causes".
  14. "Radiopedia Differential diagnosis of Acoustic Neuroma".
  15. "NIH Acoustic neuroma Incidence".
  16. "NIH acoustic neuroma Prognosis".
  17. Strasnick B, Glasscock ME, Haynes D, McMenomey SO, Minor LB (1994). "The natural history of untreated acoustic neuromas". Laryngoscope. 104 (9): 1115–9. doi:10.1288/00005537-199409000-00011. PMID 8072358.
  18. "NHS choices acoustic neuroma complications".
  19. "NIH Acoustic neuroma symptoms".
  20. "NIH acoustic neuroma Exam and Tests".
  21. "Wikipedia acoustic neuroma diagnosis".
  22. "Radiopedia CT findings".
  23. "Wikipedia acoustic neuroma diagnosis".
  24. "Wikipedia Acoustic neuroma treatment".
  25. "Wikipedia Acoustic neuroma treatment".


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