QuestionSBA
QuestionChoice
This is age-related sensorineural hearing loss. We can be fairly certain that the hearing loss are from his bilateral vestibular schwannomas which are shown on the MRI. This would not explain why he has bilateral vestibular schwannomas
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Presbycusis
QuestionChoice
Neurofibromatosis type 2 is a genetic condition characterised by tumour formation within nervous tissue. It is associated with bilateral vestibular schwannomas. They normally present by this age, unlike unilateral vestibular schwannomas which tend to present in older people
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Neurofibromatosis type 2 (NF2)
QuestionChoice
Neurofibromatosis type 1 is also a genetic condition characterised by tumour formation within nervous tissue, and is more common than NF2, however is not associated with bilateral vestibular schwannomas. NF1 is associated with optic gliomas
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Neurofibromatosis type 1 (NF1)
QuestionChoice
There is no known association between HIV/acquired immune deficiency syndrome (AIDS) and vestibular schwannomas
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HIV
QuestionChoice
This is not a cause of bilateral vestibular schwannomas
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Benign paroxysmal positional vertigo (BPPV)
QuestionComment
Vestibular Schwannomas on BOTH sides = NF is 2
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Yellow Nightshift
QuestionComment
Vestibular schwannoma on 2 sides = NF 2 
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QuestionComment
so good u gotta say it twice
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Flutter Hypertension
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Yellow Nightshift
Concept
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# Summary

A vestibular schwannoma is a benign subarachnoid tumour that predominantly affects the VIII cranial nerve. Key signs and symptoms include unilateral hearing loss, progressive ipsilateral tinnitus, and in severe cases, signs of raised intracranial pressure due to mass effect. Key investigations primarily involve an MRI scan, especially in patients presenting with unilateral tinnitus and sensorineural deafness. The definitive management strategy is surgical intervention for tumours over 40mm, while those under 40mm are monitored with 6-monthly annual surveillance scans via MRI.

# Definition

A vestibular schwannoma is defined as a benign subarachnoid tumour that exerts local pressure effects on the VIII cranial nerve.

# Epidemiology

In the UK, vestibular schwannomas, also known as acoustic neuromas, are relatively rare and are estimated to occur in about 1 in 100,000 people annually, most commonly presenting in adults between the ages of 40 and 60.

# Aetiology

Vestibular schwannomas, are benign tumors that develop from the Schwann cells of the vestibulocochlear nerve, with the majority being sporadic cases, though a small percentage are associated with a genetic disorder called neurofibromatosis type II.

# Signs and Symptoms

- The most common symptoms reported are asymmetric or unilateral hearing loss and progressive ipsilateral tinnitus.
- Larger tumours may cause a mass effect leading to signs of raised intracranial pressure and can result in focal neurology including compression of the fifth, seventh, and eighth cranial nerves.
- Additional symptoms include: dizziness, headaches, and disequilibrium.

# Differential Diagnosis


* **Meniere's Disease:** Characterized by recurrent episodes of vertigo, hearing loss, and tinnitus. While both conditions may cause vertigo, Meniere's disease has a different clinical presentation and is not a tumor.
* **Labyrinthitis:** An inflammation of the inner ear, often caused by viral or bacterial infections, resulting in symptoms similar to vestibular neuromas.
* **Benign Paroxysmal Positional Vertigo (BPPV):** A common cause of recurrent vertigo, often triggered by head movements. BPPV is not associated with tumors but with dislodged otoliths in the inner ear.
* **Migraine-Associated Vertigo:** Individuals with migraines may experience vestibular symptoms, which can sometimes be mistaken for vestibular neuromas.
* **Other Intracranial Lesions:** While less common, intracranial lesions, such as brainstem or cerebellar tumors, can present with similar vestibular symptoms.
* **Posterior Circulation Stroke:** Vertigo can be a symptom of a stroke, especially when associated with other neurological deficits.
* **Autoimmune Inner Ear Disease:** An immune-mediated condition affecting the inner ear, causing hearing loss and vestibular symptoms.
* **Otosclerosis:** An abnormal bone growth in the middle ear that can lead to hearing loss and sometimes imbalance.
* **Superior Semicircular Canal Dehiscence (SSCD):** A rare condition where there is a hole in the bone covering the superior semicircular canal, leading to vertigo triggered by loud sounds or pressure changes.



# Investigations

In patients presenting with unilateral tinnitus and sensorineural deafness, an MRI scan should be performed to exclude the evidence of malignancy.

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# Management

The definitive management of vestibular schwannoma is surgery, specifically for tumours over 40mm in size. For tumours under 40mm in size, 6-monthly annual surveillance scans via MRI are recommended.

# References

[Click here for NICE CKS on hearing loss in adults](https://cks.nice.org.uk/topics/hearing-loss-in-adults/)

[Click here for NICE CKS on tinnitus](https://cks.nice.org.uk/topics/tinnitus/)
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A vestibular schwannoma seen on an MRI head.
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Vestibular Schwannoma
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Ear, Nose & Throat
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Neurofibromatosis type 2 is characterised by bilateral vestibular schwannomas, typically presenting in young adults with hearing loss and tinnitus.
A 30 year old man is referred to the outpatient ENT clinic with gradual onset bilateral sensorineural hearing loss and tinnitus.

On examination, he has absent corneal reflexes bilaterally.

An MRI cerebellopontine angle confirms bilateral vestibular schwannomas.

What is the most likely underlying diagnosis?
a