Question: I have been having vertigo and tinnitus problems since early 1994. I had the ear area scanned with one of my regular MRIs about that time and no tumors were found. I was diagnosed with Meniere’s disease and have been treated for such since that time with some positive results.

I read with great interest your two articles on ELST in the September 1996 issue. I sent copies to both of my physicians. They reviewed my prior scans with a neuroradiologist and still saw nothing, but they recommended a CT scan of the temporal bone area. An ELST the size of a quarter was found. The tumor was successfully removed in February, without any hearing loss or balance problems. The remaining tinnitus faded as the swelling went down.

The one thing my doctors disagreed with in the article was the statement that "they are often best left in place." My doctors felt that these tumors must be removed because of the dangers they pose if they hemorrhage.

Thank you again for your great work in keeping VHL patients informed. I would never have known of these tumors and their association with VHL without your great work. I should have better hearing and balance in the future because of these articles.

-- Marty M., Illinois

Answer: Although the existing medical literature suggests that endolymphatic tumors frequently enlarge rapidly and often produce life-threatening circumstances, our experience with many patients with von Hippel-Lindau syndrome and ELST suggests that this is not accurate.

Our review of these patients indicates that the tendency of these tumors to ultimately produce complete hearing loss, since degree of hearing loss is related to the duration of symptoms. However, it is somewhat unusual for these tumors to reach a life threatening size and it seems even more unusual for them to suddenly hemorrhage, as I have known of no patients with this disorder to hemorrhage in the many that we have seen.

The natural history of these tumors is not completely established, but they certainly grow slower and tend to reach a much smaller size than the medical literature now suggests.

It is now possible to remove these tumors and preserve hearing. I have similar patients who have received surgery at the NIH. Several tumors have been removed with no degradation in hearing, and in a few cases hearing has improved after surgery. In fact, surgery of the endolymphatic sac is commonly performed for Menière’s disease and usually does not produce a hearing loss. However, the consistency with which these tumors can be removed with preservation of hearing has not been established.

Early results are hopeful, and we encourage patients and physicians to consult with Dr. Daniel Choo or myself on possible surgical approaches to ELST.

-- Dr. Edward H. Oldfield, Chief, Surgical Unit, National Institute of Neurological Disorders & Stroke, Bethesda, Maryland.

Dr. Choo Tel: 301-496-5368; Fax: 301-402-0409 or
Dr. Oldfield Tel: 301-496-2921; Fax: 301-402-0380.

As published in the VHL Family Forum 5:4, December 1997. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.