Gamma Knife Radiosurgery for Vestibular Schwannomas in NF2

NF2: What is it?

The rare genetic illness known as neurofibromatosis type 2 (NF2) mostly affects the neurological system. Numerous benign (non-cancerous) tumors growing throughout the nerves, particularly in the brain and spinal cord, are its defining feature. Bilateral vestibular schwannomas, or tumors that develop on both vestibular nerves involved in hearing and balance, are the defining feature of NF2. Adolescence or early adulthood is typically when this illness first appears, and it steadily worsens with time.

Vestibular Schwannomas: What Are They?

The Schwann cells that cover the vestibular nerve give birth to benign tumours called vestibular schwannomas, sometimes referred to as acoustic neuromas. This nerve is an essential component of the vestibulocochlear nerve, the eighth cranial nerve that sends information to the brain from the inner ear about balance and sound.

Vestibular schwannomas usually form on both sides of the brain in people with NF2, and because they are located close to important brain regions, they can cause a variety of symptoms and consequences.

Vestibular Schwannoma symptoms in NF2

Patients with NF2 frequently experience bilateral vestibular schwannomas, which can affect both sides. These symptoms can include:
Hearing Loss: The most prevalent symptom and sometimes one of the first indicators is progressive hearing loss.
Ear ringing or buzzing is known as tinnitus.
Balance problems include unsteadiness or trouble walking as a result of a malfunctioning balance.
Facial Weakness or Numbness: As tumours enlarge, they may compress nearby cranial nerves, impairing movement and sensation in the face.
In more severe situations, headaches are caused by elevated pressure inside the skull.

Vestibular Schwannoma Diagnosis in NF2

Vestibular schwannomas in NF2 are usually diagnosed by:

The gold standard for identifying vestibular schwannomas is magnetic resonance imaging, or MRI scans. It offers fine-grained pictures of the inner ear and brain.
Audiometry: To assess the degree of hearing loss, hearing tests are conducted.
Genetic Testing: To find mutations in the NF2 gene, genetic testing is frequently used to confirm NF2.

Vestibular Schwannoma Treatment Options in NF2

In order to treat vestibular schwannomas in NF2, a multidisciplinary approach is necessary. Controlling tumour growth, protecting hearing, and reducing neurological impairments are the objectives.

Observation

A “watch and wait” approach with routine MRI scans is frequently advised to track tumour progression in situations when tumours are small and symptoms are minimal.

Removal via Microsurgical

When large tumours press brain regions or cause severe symptoms, surgery may be necessary. However, there are dangers associated with surgery, including hearing loss, injury to the facial nerves, and balance issues, particularly in patients with NF2, where both sides are affected.

Radiosurgery using Gamma Knives

For NF2 patients with vestibular schwannomas, Gamma Knife radiosurgery is a very successful and less invasive therapy option.

Vestibular Schwannomas treated with Gamma Knife Radiosurgery in NF2

Gamma Knife radiosurgery eliminates the need for open surgery by accurately targeting vestibular schwannomas with concentrated, high-dose radiation beams. NF2 patients particularly benefit from this cutting-edge technology for a number of reasons:

Gamma Knife Radiosurgery Benefits:

Non-invasive: No general anaesthesia or incisions are needed.
In many situations, hearing preservation helps to halt the growth of tumours while maintaining hearing.
Minimal Recovery Time: Within a few days, patients can typically resume their regular activities and go back home the same day.
High Accuracy: The method lowers the chance of problems by focussing on the tumour while preserving the surrounding healthy brain tissue.
Slows Tumour Growth: Especially useful for managing small to medium-sized tumours, halting their growth and reducing the need for repeated operations.

Ideal Gamma Knife Candidates:

Individuals with vestibular schwannomas that range in size from tiny to medium.
People who want to maintain their hearing ability.
Those who have already had several procedures or for whom standard surgery involves significant hazards.

Overview of the Procedure:

Carried out under light sedation and local anaesthesia.
To ensure accuracy, a lightweight frame or mask is utilised.
The targeted radiation beams are guided straight to the tumour via MRI and imaging.
Patients usually return home the same day after the procedure, which takes a few hours.

In conclusion

The quality of life can be greatly impacted by vestibular schwannomas in NF2, although patients can obtain better symptom control and functional preservation with early identification and current treatment options like Gamma Knife radiosurgery. Particularly in NF2 instances when balancing tumour management and quality of life is the aim, Gamma Knife presents a possible substitute for surgery.

The management of this complicated condition can be significantly improved by early intervention and individualised therapy planning if you or a loved one has been diagnosed with NF2

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