Medical Oncologist
CION Cancer Clinics
VHL and hearing: endolymphatic sac tumours explained | CION Cancer Clinics
A small number of people with VHL develop a slow-growing tumour in the inner ear, called an endolymphatic sac tumour. It rarely spreads, but it can damage hearing and balance, sometimes suddenly and permanently. This page explains where it grows, the symptoms that should never wait, what a hearing check involves and why finding it early matters so much. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
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Medically reviewed by Dr. Naresh GunduConsultant Medical Oncologist · MBBS, DNB (Internal Medicine), DM (Medical Oncology, AIIMS) · last reviewed September 2026, next review due September 2027
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On this page
- Why does VHL affect hearing?
- Which ear symptoms should a VHL carrier never ignore?
- What happens at a VHL hearing check?
- The words you will meet, in plain language
- Why finding it before symptoms matters
- Four things people assume, and what is actually true
- What this page cannot tell you
- Common questions about VHL and hearing
The short answer
Why does VHL affect hearing?
A minority of people with VHL develop an endolymphatic sac tumour, a slow-growing growth in a tiny drainage pouch of the inner ear. It very rarely spreads, but it can damage hearing and balance, sometimes suddenly and for good. That is why hearing checks are part of VHL surveillance.
Where the tumour sits
Deep inside the bone behind each ear is the inner ear, which turns sound into signals for the brain and helps you balance. A small sac drains its fluid. In VHL, a growth rich in blood vessels can form in that sac. It can appear on one side or, less often, on both.
Why hearing can go suddenly
Even a small tumour can bleed into the inner ear or upset its fluid balance. When that happens, hearing can drop over hours or days, and the loss is often permanent. This is the reason teams look for these tumours before any symptom appears, and why a sudden change in hearing is treated as urgent.
Sudden hearing loss in one ear should be seen by an ENT doctor within a day. Say that you have VHL.What to notice
Which ear symptoms should a VHL carrier never ignore?
Any of these in someone with VHL deserves a hearing test and a call to the team, even if it seems minor.
Hearing loss
Sudden or gradual, usually in one ear. People often notice it first on the phone, or when they cannot tell which direction a sound is coming from. A drop that comes on over a day or two needs the most urgent attention.
Ringing or buzzing
A constant sound in one ear that nobody else can hear, called tinnitus. It is common in everyone, but new tinnitus on one side needs checking in VHL.
Spinning dizziness
Attacks where the room seems to spin, called vertigo, sometimes with vomiting. Unsteadiness when walking in the dark can also come from the inner ear.
Less common signs
- A feeling of fullness or pressure in the ear
- Weakness on one side of the face
- Pain or a lump behind the ear
Not sure whether this applies to you?
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What happens at a VHL hearing check?
A few questions first
The audiologist asks about any change in hearing, ringing, dizziness or ear infections since your last visit. Bring your previous hearing reports so the results can be compared.
The beep test
You sit in a quiet booth wearing headphones and press a button each time you hear a beep. It measures the softest sound each ear can pick up at different pitches. It does not hurt.
Words and ear pressure
You repeat words played at a set volume, and a soft probe checks how the eardrum moves. Together these tell an inner ear problem apart from a blocked or infected middle ear.
A scan if needed
Your routine brain MRI usually includes fine views of the inner ear. If the hearing test changes, a dedicated MRI or a CT scan of the ear bone may be arranged sooner.
On your report
The words you will meet, in plain language
- Endolymphatic sac
- A tiny pouch that drains fluid from the inner ear. It sits inside the bone behind the ear.
- Endolymphatic sac tumour
- A slow-growing, blood-vessel-rich growth in that pouch, often shortened to ELST. It rarely spreads but can damage the inner ear around it.
- Audiogram
- The chart from a hearing test. It shows the softest sounds each ear can hear, from low pitch to high.
- Tinnitus
- Ringing, buzzing or hissing in the ear that comes from inside, not from the room.
- Vertigo
- A spinning sensation caused by a problem with the balance part of the inner ear.
- Temporal bone
- The hard bone at the side of the skull that holds the inner ear. A CT scan of this bone shows whether a tumour has worn it away.
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Side by side
Why finding it before symptoms matters
Found on a scan, hearing normal
Found after hearing has changed
Hearing is usually still intact
Some hearing may already be lost for good
Surgery can aim to protect hearing
Surgery aims to stop further damage
The tumour is small and easier to remove whole
A larger tumour may lie close to the face nerve
Decisions can be planned calmly
Decisions often have to be made quickly
Commonly believed
Four things people assume, and what is actually true
"Hearing loss in a young person is just wax or an infection."
Often it is, in people without VHL. In someone with VHL, new hearing loss or ringing on one side needs a proper hearing test and a word with the team before anyone puts drops in the ear.
"If the tumour does not spread, it can be left alone."
It rarely travels elsewhere, but it can still destroy hearing and balance where it sits. Whether and when to operate depends on its size, your hearing and the other ear. Some small tumours are watched closely instead.
"An operation will bring lost hearing back."
Usually it will not. Surgery aims to stop further damage and protect what is left. That is why teams try to find these tumours while hearing is still normal.
"A normal hearing test means there is no tumour."
A small tumour can sit quietly with perfect hearing. The hearing test and the MRI look for different things, which is why both are part of the plan.
Being straight with you
What this page cannot tell you
It cannot tell you whether a change on your own scan or audiogram is an endolymphatic sac tumour, or what should be done about it. That needs an ENT surgeon who knows VHL, working with the doctor who plans the rest of your checks. What your specific variant means is a question for the counsellor who ordered the test.
The evidence is thin in places
These tumours are rare, even within VHL. Most of what is known comes from small groups of patients at a few specialist centres. Experts still differ on when to operate on a small tumour in someone whose hearing is normal. A good team will tell you that openly and explain the trade-off for your ear.
Who this does not apply to
If you have tested negative for your family's known VHL fault, none of this applies, and ordinary ear problems can be treated in the ordinary way. Most hearing loss in the general population has nothing to do with VHL. This page is for confirmed carriers and relatives who have not yet been tested.
Questions we are asked
Common questions about VHL and hearing
How common are ear tumours in VHL?
They affect a minority of people with VHL, far fewer than eye, brain or kidney growths. Estimates vary between studies, partly because small tumours were missed before MRI became routine. Because the consequence for hearing is serious, every carrier is checked, not only those with symptoms.
Is an endolymphatic sac tumour a cancer?
It is usually described as low grade. It grows slowly and very rarely spreads to other parts of the body. It can, however, wear away the surrounding bone and damage the inner ear, so it is taken seriously even though it does not behave like most cancers.
What should I do if my hearing drops suddenly?
Treat it as urgent. See an ENT doctor within a day, tell them you have VHL, and ask for a hearing test. Then call your VHL team, because an MRI may be needed sooner than planned. Do not wait to see whether it settles on its own.
How often should my hearing be tested?
Hearing tests usually start in late childhood and are repeated every other year or so, alongside the brain MRI. Any new symptom brings the next test forward. Your team may test more often if a small tumour is being watched. Keep every audiogram, because comparing them over the years is what shows a slow change.
Can both ears be affected?
Yes, although it is less common than one ear. This is one reason teams try hard to protect hearing in the first ear affected. If a tumour is found on one side, the other ear is watched with particular care from then on, and any change in it should be reported straight away.
Is the operation risky for the face?
The nerve that moves the face runs close to the inner ear, so every ear operation carries some risk to it. The risk is generally lower when the tumour is small. Ask your surgeon how often they operate on this area, and what they expect for your hearing and your face.
Is radiotherapy an option instead of surgery?
Sometimes, for a tumour that cannot be removed safely or for someone who cannot have an operation. Experience with it is limited and studies so far are small, so surgery is usually preferred where it is possible. Your team will explain which option suits your tumour, and why.
Will a hearing aid help if hearing is lost?
Often, yes, depending on how much hearing is left in that ear. Where one ear is badly affected, other devices can pass sound from that side to the better ear. An audiologist can test what suits you. Hearing aids are fitted to young people as well as older ones.
Your Specialists
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Medical Oncologist
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Medical Oncologist
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Medical Oncologist
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI) — Von Hippel-Lindau Syndrome
- MedlinePlus Genetics — Von Hippel-Lindau syndrome
- Cancer.Net (ASCO) — Von Hippel-Lindau Syndrome
- National Institute on Deafness and Other Communication Disorders — Sudden Deafness
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
Keep reading
Related pages
Von Hippel-Lindau: every organ involvedWhere the ear fits among the other organs VHL affectsBrain and spine surveillance in VHLThe MRI that usually also checks the inner earVHL surveillance scheduleEvery check a carrier needs, and roughly whenGrowing up with VHLHow hearing tests fit into a child's check-ups
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