BALTIMORE, MD. (Ivanhoe Newswire) — Neurofibromatoses are a group of disorders that cause tumors to grow in the nervous system. One of those conditions, NF 2, causes many patients to go deaf because the tumors grow on the nerves responsible for hearing. A drug already in use for some cancers is not only halting the hearing loss in some patients, but reversing it.
Heather Sheeley-Johns can walk along a busy city street without worry. For years, cars, horns and sirens were impossible to hear.
“I think things were a little more muffled. Having a very difficult time figuring out where the sound was coming from,” described Sheeley-Johns.
Sheeley-Johns was diagnosed with neurofibromatosis 2 in her late twenties. Tumors on one cranial nerve caused her hearing to get progressively worse.
Jaishri Blakeley, M.D., a neurosurgeon at Johns Hopkins University School of Medicine in Baltimore, Maryland, detailed, “Losing hearing at that age can be very traumatic and very isolating.”
Dr. Blakeley is an expert in tumors of the nervous system. NFs are not cancer but grow relentlessly and have no effective therapies.
“Surgery is for sure the standard of care. The trouble with surgery is it’s very difficult to remove the tumor, without damaging the nerve,” explained Dr. Blakeley.
Dr. Blakely is studying the impact of an already FDA-approved drug on NF 2 tumors. Bevacizumab is given intravenously every three weeks for 30 to 60 minutes. The drug shrinks the blood vessels near the tumor, making the tumor smaller.
“If there was something that could prolong my hearing there wasn’t a doubt, I had to try,” Sheeley-Johns told Ivanhoe.
Thirty-six percent of the trial participants gained back hearing on the drug. Sixty percent of those patients kept their hearing, even when they were off the drug for three months.
“It has not only stopped the hearing loss but my hearing has actually gotten better by about 30 percent,” said Sheeley-Johns.
Life-changing treatment for those in the prime of their lives.
Sheeley-Johns’ hearing gains have lasted several years, but since this is an experimental treatment, doctors aren’t sure if the results will be permanent. Dr. Blakeley said this drug is not for every patient, since side effects include an increase in blood pressure, change in ovarian function, and the possibility of kidney damage.
Contributors to this news report include: Cyndy McGrath, Supervising and Field Producer; Milvionne Chery, Assistant Producer; Tony D’Astoli, Editor; Kirk Manson, Videographer.
TOPIC: NF Tumors: Hearing Can Be Restored
REPORT: MB #4150
BACKGROUND: Neurofibromatosis Type 2, or NF2, is a disorder characterized by the growth of noncancerous tumors in the nervous system. Neurofibromatosis is a rare occurrence, with an estimated incidence of one in 33,000 people worldwide. It is a genetic condition with 50 percent of cases inherited from a parent. In the other half of cases, it occurs as a result of a spontaneous mutation. The most common tumors associated with the disease are called vestibular schwannomas or acoustic neuromas. These growths develop along the nerve that carries information from the inner ear to the brain, or the auditory nerve. Tumors that occur on other nerves may be also found in this condition. The signs and symptoms usually appear in young adulthood or adolescence. Common symptoms include hearing loss, ringing in the ears, and problems with balance. In most cases, tumors occur in both ears by age 30. Complications of tumor growth can include changes in vision, numbness or weakness in the arms or legs, and fluid buildup in the brain. Some people also experience vision clouding beginning in childhood. A diagnosis of NF2 is made based on hearing and vision evaluations. In some cases, however, genetic testing may be required to make a diagnosis.
TREATMENT: There is currently no cure for Neurofibromatosis type 2, so treatments focus on managing symptoms and preventing complications. Surgery may be used to remove tumors that are pushing on the spinal cord or are causing seizures. Surgery is typically only used on problematic tumors, and is seen as a last resort. Medication can sometimes be useful for managing symptoms like ringing in the ears, headaches, and pain caused by the benign tumors. Those who have problems with balance often benefit from physical therapy by strengthening core muscles and learning techniques to prevent falls. Supportive devices such as hearing aids are often recommended for those who suffer hearing loss as a result of NF2. When hearing loss progresses, surgical removal of tumor, hearing aids, cochlear implants, and eventually learning lip reading and sign language are available options to manage the negative effects.
NEW TREATMENT: In a small clinical study at Johns Hopkins Comprehensive Neurofibromatosis Center with an anticancer drug that stops blood vessel growth, people with neurofibromatosis type 2 experienced restoration of hearing. Bevacizumab, an FDA approved cancer drug, has had previously well-documented effects in other diseases. Doctors treated 14 patients with NF2 and hearing loss with the drug, in hopes that it would shrink their tumors. Nine patients reported significant hearing improvements, while all patients stopped experiencing further hearing loss. Five patients maintained the improvement for six months after treatment stopped, leading researchers to believe that the drug may be used intermittently to manage hearing problems for those diagnosed with NF2.
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