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February 7, 2003

No One Listening to Meningitis Concerns

From: Insight on the News, D.C. - 07 Feb 2003

Posted Feb. 7, 2003

By Timothy W. Maier

Radio host Rush Limbaugh had a cochlear implant installed two years ago, but was unaware of the alleged meningitis risk at the time.

When surgeons placed a cochlear implant inside Rush Limbaugh's left ear two years ago they failed to provide their famous patient with an important warning. They did not tell the conservative radio celebrity that the computerized device that helped restore his hearing was believed to have contributed to several deaths and dozens of cases of meningitis, a potentially fatal, contagious infection of the tissue layers surrounding the brain and its cerebrospinal fluid. The reason for the oversight? Apparently no one knew.

But concern soon prompted angry protests from deaf communities worldwide, particularly in England, where protesters have been calling for a ban on the cochlear implant, declaring: "Better Deaf Than Dead!" Protesters went so far as to accuse hospitals and manufacturers of manipulating research data for profit at the expense of human lives. Unlike the "Deaf President Now!" protest at Washington's Gallaudet University in 1988, demanding that the leader of the university be chosen from the deaf community, the protests about the cochlear implant have had little impact or publicity. But then neither has an apparently related worldwide meningitis outbreak that seemed to come out of nowhere after the cochlear products had been on the market for nearly two decades.

The protests failed to stop Heather Whitestone, the former Miss America, from going ahead with the $35,000 to $50,000 effort to improve her hearing even after the outbreak emerged. Whitestone wanted to hear the voices of her children. Appearing on Good Morning America afterward, she reported that while she can't yet understand what her children are saying, she hears and loves "the sound of running water."

The cochlear implant has three main parts: the internal chip, external processor and a magnet. Inside the internal chip are electrodes that are positioned in the cochlea of the inner ear to activate auditory-nerve fibers needed to transmit sound signals to the brain. An external processor that runs on batteries and amplifies the sound is worn on the body or ear in the same manner as a hearing aid. It is connected by a wire to a magnet that sits snugly on the side of the skull, where it helps transmit messages from the internal chip.

While not a cure for deafness, the implant allows patients to hear sounds they otherwise would not hear, though it can take several years before the patient can interpret what is being heard. At first, speech may seem more robotic than natural, and environmental sounds may seem like a series of beeps. This changes over time. Limbaugh, however, had nearly immediate success in understanding speech, as many newly deafened people do. But he also relies on alternative listening devices, such as a telephone that displays captions, and says he is "completely deaf" when he removes the external processor.

But now the early excitement about such successful implantations is being tempered with concern. The U.S. Food and Drug Administration (FDA) warns that there is a higher risk among cochlear-implant users of contracting bacterial meningitis than among the general population. Along with other regulatory agencies such as the Centers for Disease Control and Prevention (CDC), surgeons and manufacturers are conducting a study to try to understand the outbreak of bacterial meningitis among those with cochlear implants.

Most vulnerable to the disease are children under the age of 5 and the elderly. Deaf patients who have had congenital abnormalities of the inner ear also may be more prone to the disease. Common symptoms of meningitis include fever, irritability, lethargy and loss of appetite, and it is the primary cause of children being born deaf. Adults might experience headaches, stiff necks, nausea, vomiting and confusion. Meningitis can be contagious and the bacteria can spread through the exchange of respiratory and throat secretions, such as from coughing or kissing.

As for Limbaugh, he appears not to be among those at high risk of contracting meningitis. But the famed talk-show host will not know if he is totally clear of the disease for some time because the onset of meningitis symptoms among implant patients ranges from less than 24 hours to more than five years after implant. The ages of U.S. meningitis patients who have undergone implants range from 18 months to 84 years, but most were under 7 years of age at the time they developed meningitis.

The sudden outbreak has raised a series of hard questions and undoubtedly will lead to more calls for reforming both the FDA and the CDC, which apparently had not conducted a timely analysis of adverse-effect data supplied to them by hospitals and implant manufacturers. Only now is such data being collected after an international conference of European otolaryngologists publicly proclaimed concern in July.

Otolaryngologists Noel Cohen of New York University and Thomas Balkany of the University of Miami are surveying about 400 implant centers in North America. So far they have received information from about 400 surgeons who performed between 18,000 and 24,000 implants. The survey results are being shared with the FDA and the CDC and will be made public. According to published reports in trade journals, the meningitis outbreaks appear to have started in 2000 -- nearly two years before the FDA warning about the potential hazard. Many of those who contracted meningitis were not vaccinated, a procedure that likely would have saved lives. For those under the age of 2, no FDA-approved vaccine was available in 2000, although such a vaccine has been approved since then.

Of the 60,000 implant recipients worldwide, the FDA reports 17 deaths and 90 cases of meningitis, but those numbers have doubled in two months and are expected to increase as data continue to be collected. As of October, the FDA broke down the outbreak of meningitis cases related to the three licensed implant manufacturers as follows: California-based Advanced Bionics Corp. had 56 cases, Australia-based Cochlear Ltd. had 33 cases and Austrian-based MED-EL Corp. had one case. Cochlear Ltd, Advanced Bionics and MED-EL devices were approved in 1985, 1996 and 2001, respectively. The numbers of related meningitis cases nearly doubled after regulatory agencies began tracking the data in July.

In the United States, the FDA discovered 52 cases of postimplant meningitis: Advanced Bionics had 29 cases, Cochlear Ltd. had 22 cases and MED-EL had one. Five of the 17 worldwide deaths occurred in the United States. At least two of the five involved children aged 2 and 3. A majority of the deaths involved an Advanced Bionics device known as Clarion II Bionic Ear, which is the device implanted in Limbaugh.

The meningitis scare set off an escalating market war among the three major implant manufacturers. All downplayed the outbreak, claiming the chances of contracting the disease are small and can be reduced through vaccination. Both MED-EL and Cochlear Ltd. provided information to the FDA suggesting that victims who had their respective devices implanted had factors that predisposed them to meningitis and were unrelated to the implant. Regardless, the number of fatalities and incidents of meningitis raises a series of questions that have yet to be answered satisfactorily. What did the manufacturers, hospitals and federal regulatory agencies know about the deaths and onslaught of meningitis cases, and when did they know it?

These questions are attracting trial lawyers hoping to line up clients for wrongful-death suits. One of the largest law firms in the United States, Lieff Cabraser Heimann & Bernstein, has been trolling on the Internet for cochlear-implant clients. Attorney Stephen Cassidy says the firm has been contacted by two people, but so far there is "no smoking gun" that implicates anyone or suggests the companies covered up the deaths so they could continue to sell their products for thousands of dollars. The firm has yet to file a lawsuit. "What we want to know is when did they know there was a problem?" Cassidy says.

Insight has learned that the outbreak was in fact discussed and kept quiet by senior officials at the National Institutes of Health (NIH) and the FDA about a month before it hit the public's radar screen in July 2002, when otolaryngologists in Amsterdam exchanged information with American colleagues and made a series of recommendations about patient management. At a conference, otolaryngologists found a greater incidence of meningitis among cochlear-implant recipients who used Advanced Bionics, a two-component electrode array that includes a Silastic positioner that places the electrode close to the auditory nerve and, in theory, increases battery life. They recommended that the positioner be discontinued. Regulatory agencies in France, Germany and Spain immediately accepted the recommendations. The United States was the last to accept them.

While the FDA claimed there was no evidence to suggest that the devices were contaminated, sources inside the NIH indicate the devices with the positioners were not thoroughly evaluated in animals yet managed to be slipped through the FDA approval process. The NIH and FDA more than likely were tipped off by Advanced Bionics, which claimed it informed the FDA of the incidents of meningitis about two months before the July conference.

Meanwhile, the news from the international conference resulted in Cochlear Ltd.'s stock value dropping 20 percent as investors feared the worst. When the stock dropped Graeme Clark, the inventor of Cochlear Ltd.'s implant, went on the offensive. He claimed the outbreak should be blamed on the private American company Advanced Bionics for creating a new design with a positioner that created "dead space" within the ear, providing a home for infection and bacteria. "It is a very great problem if engineers pursue designs without due recourse to biologists and medical people," he charged. He subsequently boasted that none of the fatalities were from the Cochlear device, although later it appears that at least one fatality was from the company's nucleus implant, even though it is believed the victim was predisposed to meningitis.

The accusations by Clark, who publicly had promised to support his allegations with papers, hit a nerve with Advanced Bionics, which counterattacked aggressively. "I haven't seen those published papers," says Advanced Bionics spokesman Douglas Lynch. Nearly seven months later, apparently, neither has anyone else.

To its credit, Advanced Bionics was the first implant manufacturer to post information on its Website about rising health concerns. Cochlear Ltd. followed and then MED-EL. But being the first to acknowledge a possible problem may have more to do with the fact that a majority of the deaths were linked to the Advanced Bionics implant. At the same time, the company announced a recall of its Clarion II Bionic Ear implant with positioner. Asked if the FDA threatened Advanced Bionics to get the product removed, FDA scientist Eric Mann said, "Let's say there were discussions." He declined to elaborate. Asked if the positioner was behind the outbreak and deaths, Mann replied, "We are currently investigating that." He said he could not foresee whether the positioner would return to the market.

While the FDA does not have the authority to pull products off the market it can fine and seek criminal prosecutions to pressure companies to remove products from the shelves. In this case, Advanced Bionics claimed it voluntarily pulled its implant off the market in July. Since 1991 nearly 34,000 products, devices and drugs have been recalled in this way for safety reasons, according to the FDA. In September, Advanced Bionics received permission to bring back the Clarion II Bionic Ear, but without the positioner.

Despite the recall, Advanced Bionics appears to be no worse off, as patients postponed surgery at hospitals such as Johns Hopkins in Baltimore to wait for the Clarion II Bionic Ear to return to the market. The strongest selling point appears to be the high-resolution strategy that comes with the implant. The high resolution allows Advanced Bionics to stimulate auditory nerves faster than any commercially available implant and, according to company studies, provides a greater benefit for patients who have been deaf most of their life.

Lynch says the device without the positioner will have "no difference in performance" but may have a 5 percent decrease in battery life to about 6 to 8 hours, which is likely to be improved with a more battery-efficient processor due out in June. Aaron Parkinson, a research scientist with Cochlear argues, "Faster is not necessarily better," and notes its implant provides battery life that can last up to three days. Regardless, Cochlear will be putting out a much faster device than its current Nucleus Contour 24 in the near future. While MED-EL may become the first to roll out an all-implantable device with no external mechanism, Blake Wilson of the Research Triangle Institute in North Carolina, who once studied faster stimulation rates for MED-EL, says, "All three devices have quite a high level of performance. There is no clear evidence to discriminate between them, although high resolution may prove to be the way to go in the future."

Wilson says patients should not be alarmed by the recent scare. He and the FDA recommend a series of vaccination shots to help prevent meningitis. However, because of a vaccine shortage, the shots still are not available for children under the age of 2 unless they fall in the high-risk category. Limbaugh, who says he doesn't want to be an advocate for the hearing impaired or the deaf community, has yet to reveal publicly whether he has been vaccinated for meningitis, or to suggest that others should do the same.

Timothy W. Maier is a writer for Insight. His wife, Susan Boswell, recently underwent a cochlear implant. Read her firsthand account of that experience at "A Cochlear Implant Success Story."

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