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Genetic and Rare Diseases Information Center (GARD)

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Von Hippel-Lindau disease

Other Names for this Disease
  • VHL
  • VHL syndrome
  • Von Hippel-Lindau disease
  • Von Hippel-Lindau syndrome
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Your Question

I have VHL and have cysts in both of my kidneys. I have been experiencing new symptoms, including hearing loss on the left side, constant ringing in the left ear, problems with balance, and trouble catching my breath. There are times that I get flushed and very hot, and then my blood pressure drops very low. I live in Maine and the doctors here don't know much about VHL. Are these symptoms serious?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Is hearing loss associated with Von Hippel-Lindau disease (VHL)?

Yes, hearing loss and ringing in the ears (tinnitus) have been described in people with VHL who have endolymphatic sac tumors (ELSTs).[1] Problems with balance (vertigo) can also be caused by this type of tumor. In some cases, these tumors can be surgically removed. Early detection and treatment of small ELSTs may reduce the severity of the symptoms associated with these types of tumors.[2] If you are experiencing any of these symptoms we recommend that you consult a healthcare professional immediately.
Last updated: 5/20/2013

Is blood pressure affected in Von Hippel-Lindau disease (VHL)?

Individuals with VHL are at increased risk of developing a type of noncancerous tumor called a pheochromocytoma.[1] Pheochromocytomas affect the adrenal glands, which are small hormone-producing glands located on top of each kidney. These tumors often cause no symptoms, but in some cases can lead to dangerously high blood pressure.[1] We were not able to locate information on individuals with VHL having low blood pressure. We recommend discussing any abnormalities in blood pressure with your health care provider.
Last updated: 5/20/2013

I live in Maine and the doctors here don't know much about Von Hippel-Lindau disease (VHL). Where can I find a doctor who has experience with this condition?

VHL is a genetic condition, therefore we recommend that you consult with a medical geneticist. A genetics professional, particularly one who specializes in cancer, will be able to coordinate your medical care among other specialists, and inform them about VHL.

You can locate a genetics clinic in your area using the clinic directory available at Visit the following link for a listing of genetics clinics located in Maine.
Genetics Clinics in Maine
Last updated: 5/20/2013

  • Von Hippel-Lindau Syndrome. Genetics Home Reference (GHR). July 2008; Accessed 7/5/2011.
  • Kim HJ, Butman JA, Brewer C, Zalewski C, Vortmeyer AO, Glenn G, et. al.. Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: Implications for their natural history, diagnosis, and treatment. Journal of Neurosurgery. March 2005; 102:503-512.