Salt Lake Meeting

In Salt Lake we conducted the Annual Meeting, and heard from some very good speakers.

Vital Friends

Tom Rath shared with us his own experiences with VHL, and the research he has conducted at the Gallup Organization on how attitude contributes to organizational health as well as family and individual health. His new book, Vital Friends: The People You Can’t Afford to Life Without, is available August 1, 2006. Vital Friends helps readers identify the friendships that make their work and home life more positive and productive.

Insurance

Tom Rodenberg spoke with us about insurance and negotiating successfully when your insurance company denies a claim.

Endolymphatic Sac Tumors.

Dr. Russell Lonser of the Surgical Branch of the U.S. National Institute for Neurological Disorders and Stroke (NINDS), one of the National Institutes of Health (NIH) was our primary featured speaker. He updated us on their work on brain and spinal tumors of VHL, and especially shared with us his latest research on endolymphatic sac tumors (ELSTs). Since 1997, he and his group have been studying the natural history of ELSTs. They bring in a group of people with VHL for thorough checkups every 6-12 months, watching the progress of their symptoms and the growth of the tumor on imaging, and deciding when is the optimal moment to operate to get the best outcome.

They have learned a great deal about what causes symptoms to develop in this tiny structure. In 1997 we thought that the symptoms were related to the size of the tumor and the duration of symptoms. But that is proving not to be the case. Even very small tumors that cannot be seen on imaging can cause significant symptoms and hearing loss.

The sudden loss of hearing experienced by a number of people with VHL may be caused by a hemorrhage in the labyrinth, the coiled passages of the inner ear. Left untreated, this hemorrhage causes inflammation and eventual deterioration. Anyone with VHL who experiences a sudden drop in hearing should seek treatment immediately. Even after a hemorrhage, if the inflammation can be controlled there is some hope of regaining the hearing, but the time window of opportunity is short.

Lonser’s team have now operated on 16 patients. In all cases, hearing was preserved at the same level as before the operation, but with no improvement. They recommend surgery for people with an ELST visible on imaging, especially who can still hear, to preserve the hearing. Even if the hearing is gone, if there are increasing balance symptoms or other neurological issues, surgery may help to prevent a worsening of these symptoms.

Surgery may also be indicated if no tumor is seen on the images, but there is evidence of blood in the labyrinth and there are symptoms (hearing loss, tinnitus, and/or vertigo).

The technique they use is an incision behind the ear called a retrolabyrinthine posterior petrosectomy (RLPP). It is a smaller incision than most craniotomies, and does not require moving much muscle out of the way, so the recovery is easier. Some people had balance issues for a time after surgery, but these usually went away.

Kim, Butman, Glenn, Oldfield, Lonser, et al., Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment. J Neurosurg. 2005 Mar;102(3):503-12. PMID: 15796386

As printed in the VHL Family Forum 14:2, August/September 2006. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org