Deafness: “Not a disease to cure”
We’ve all seen the heartwarming videos on YouTube of people with cochlear implants experiencing the joy of sound for the first time: the eight-month old baby hearing his mother’s voice, pacifier falling out of his mouth in wonder, and the 29 year-old woman hearing her own voice for the first time, immediately dissolving into happy tears. These are just two examples of how life-altering hearing can be.
Recently, a study in Nature reported that researchers were able to restore hearing in gerbils through use of stem cells, a breakthrough that puts us a step or two closer on the path to curing deafness. However, many people in the Deaf community don’t embrace these breakthroughs as enthusiastically. Actress Marlee Matlin, who is deaf, took to her Twitter account to decry this recent breakthrough, stating that deafness “is not a disease to cure.”
So is deafness a disease? Should people pursue every opportunity available to restore one of the basic animal senses? We’ve asked Jeffrey Harris, MD, PhD, chief of Otolaryngology at UC San Diego Health System and Distinguished Professor of Surgery in the UCSD School of Medicine, three questions about deafness.
Question: What are cochlear implants and how do they work? Can anyone with a hearing deficit use them?
Answer: Cochlear implants are devices that pick up sound and convert them into electrical impulses that travel down along a very thin electrode array that is surgically implanted into the inner ear (cochlea). If there are sufficient numbers of remaining nerve endings that can be stimulated, then hearing sound, words, speech and even music can be perceived by the deaf individual. Only people who no longer have benefit from hearing aids and meet the established hearing loss criteria are candidates for this surgery.
Q: Do you often meet resistance from patients with deafness when implants are suggested?
A: Interestingly enough the vast majority of patients that I now implant were originally hearing individuals. These individuals have lost their hearing for a number of different reasons and they greatly desire to have their hearing restored with a cochlear implant. Back in the 1980s, when these devices were first introduced, there were a great number of adults who were born deaf and learned sign language who had great hesitancy to undergoing this surgery for social and cultural reasons. And the results on late implantation were not good because the unstimulated cochlear nerves degenerated well before they could have undergone the operation. Today, most centers implant deaf children early on in order to prevent nerve degeneration from occurring and, as a result, they can be mainstreamed in school and can develop excellent speech and language skills.
Q: Marlee Matlin, who is an advocate for deaf people, stated that deafness is not a handicap and “there are millions of Americans who sign, who are deaf, and are not a ‘disease’ to cure.” Should deafness be approached as just another trait, like being born with blue eyes or having red hair?
A: I firmly believe the options for implantation in a child born deaf need to be presented in a fair and balanced, non-emotional way to the parents and then they can make that decision. Included in this presentation should be educational levels, average income, speech and language acquisition, and quality of life assessments (to name a few) achieved in hearing restored versus deaf children. One striking fact is that a child who is implanted early on, say at the age of two, can achieve the same or nearly close to the level of speech and education as that of a hearing child. If the implant is delayed, say to the age of six, the level of speech and education is significantly reduced. The dilemma, then, is that there is a window of opportunity that exists for the deaf child to be implanted, and, if it is delayed for too long, the child may never achieve the same hearing result because neural degeneration begins to occur in the unstimulated ear. Therefore, this is not a case where one can simply say “let’s leave it up to the child to decide when he or she is an adult and can decide for themselves.”
