Ethics for the Pediatrician

A cochlear implant is a small, complex electronic device that can help to provide a sense of sound to a person who is profoundly deaf or severely hard-of-hearing. Implants all have four key components: (1) a microphone, which picks up sound from the environment; (2) a processor, which selects and arranges sounds picked up by the microphone; (3) a transmitter and receiver/stimulator, which converts signals from the processor into electric impulses; and (4) a group of electrodes that sends impulses from the stimulator to different regions of the auditory nerve. After implantation of the device, recipients must undergo an extensive program of speech therapy to learn how to make the implant useful. As of December 2010, ∼219,000 people had received implants worldwide, including 71,000 people in the United States, of whom 28,400 were children. (1)Over the past two decades, doctors, parents, policymakers, and members of the Deaf community have struggled to understand the benefits, risks, and implications of using cochlear implants for children. These struggles were the latest episode in a debate about medical and educational approaches to deafness that have lasted over a century. They also reflected the fact that there were no good long-term outcome data on cochlear implants. Thus, complex issues of clinical and research ethics are intertwined with complex cultural issues and a long history of discrimination and stigmatization of children who are deaf. In this article, I will review the origins of the controversy, discuss its most heated moments, and summarize the current state of the debate.Much of the ethical controversy about cochlear implants reflects a debate that has been going on for >100 years about the best way to educate children who are deaf in order to maximize their opportunities in life. Some people, notably Thomas Gallaudet, were of the opinion that children who are deaf all should be taught sign language and educated by teachers who know sign language. This approach became known as “manualism.” Others, notably Alexander Graham Bell and Horace Mann, thought it was preferable to educate children who are deaf to speak and to read lips. This philosophy was known as “oralism.”Bell, whose wife was deaf, was himself fluent in sign language. He argued that sign language should be discouraged because it would lead to isolation of individuals who are deaf from hearing people. He also feared that more marriages between people who are deaf would result in a perpetuation of deafness. Thus, his program was motivated by eugenic concerns as well as educational considerations. Bell went as far as advocating a ban on marriages between people who are deaf. (2)Although Bell’s extreme views about marriage never prevailed, his views on the superiority of oralism over signing did prevail in many schools for the deaf. Throughout the early 20th century, many such schools prohibited signing. (3) By the middle of the 20th century, however, oralism had been discredited thoroughly. A 1964 report to Congress on deaf education called oralism a “dismal failure.” The report recommended new approaches to the education of children who are deaf. (4) Oralism failed, in part, because lip reading (or “speechreading”) is difficult and never perfect. Only ∼30% to 35% of English sounds can be speechread. Words as different as “queen” and “white” look the same on a person’s lips. So do the three words in the sentence, “Buy my pie”. (5) Sign language, by contrast, allows students who are deaf to understand all that is being communicated.In recent years, this stark exclusivity has all but disappeared. Most educators today prefer methods that combine lip reading, speech, and sign language, and are known collectively as “total communication”. (6) Still, the years during which signing was prohibited in schools for the deaf left deep scars. Signing became a matter of deep pride and cultural identity within the Deaf community. (7) This history explains, in part, the response of many in the Deaf community to cochlear implants. They saw implants as a return to the philosophy of oralism and a rejection of sign language.In 1957, Djourno and Eyries were the first surgeons to implant an electrode in a patient’s cochlea. (8) Over the next decades, others developed the technology. Between 1965 and 1970, House developed single-electrode cochlear implants and teamed up with 3M to make the first commercial cochlear implants in the United States. These devices were first marketed in 1972, and in 1984 they were approved by the Food and Drug Administration (FDA) for use in adults.In the mid-1970s, Clarke developed a multielectrode cochlear implant, and first implanted an adult patient with his prototype device in 1978. Multichannel devices divide the incoming signal into various frequency bands that are then transmitted to various sites of stimulation spanning the inner ear. Low-pitch sounds are sent to one part of the cochlea, high-pitch sounds to another, more closely mimicking the human ear. Because multiple channels provide a more detailed representation of sound, they are thought to allow better speech understanding than do single-channel devices. Clarke’s multichannel device was approved in 1985. Throughout these years, implants were being offered to children, although they were not yet approved by the FDA for use in pediatric populations.In 1990, the FDA approved the use of cochlear implants for children over age 2 years. This decision led to a firestorm of controversy. The next year, the National Association of the Deaf (NAD) issued a statement deploring the FDA’s approval decision. (9) They argued that cochlear implants for children remained highly experimental. They accused the FDA of “failing to consult formally with organizations of deaf Americans and with deaf leaders and scholars knowledgeable about the acquisition and use of sign communication and English in deaf children, the psycho-social development and education of deaf children, and the social organization and culture of the American Deaf community.” Most controversially, the NAD claimed that deafness “comprises a linguistic and cultural minority,” and that “we should not seek the scientific tools nor use them, if available, to change a child biologically so he or she will belong to the majority rather than the minority – even if we believe that this biological engineering might reduce the burdens the child will bear as a member of a minority.” They recommended a national conference to address the ethical issues surrounding cochlear implants for children.In 1995, a conference was convened at the National Institutes of Health (NIH) to address some of the issues raised by cochlear implants. Of note, it was not the conference that the NAD had recommended. Instead, the scope was much more limited. According to the organizers, “The conference was convened to summarize current knowledge about the range of benefits and limitations of cochlear implantation that have accrued to date. Such knowledge is an important basis for informed choices for individuals and their families whose philosophy of communication is dedicated to spoken discourse. Issues related to the acquisition of sign language were not addressed directly by the panel, because the focus of the conference was on new information on cochlear implant technology and its use. The panel acknowledges the value and contributions of bilingual and bicultural approaches to deafness”. (10) Conference panelists were drawn from “the fields of otolaryngology, audiology, speech-language pathology, pediatrics, psychology, and education, and including a public representative.” There was no representative from Deaf culture. The goal of the conference was to make recommendations to the NIH and the FDA about cochlear implants. More than 600 people attended.The conclusions of the consensus conference were as follows: “Cochlear implantation improves communication ability in most adults with severe-to-profound deafness and frequently leads to positive psychological and social benefits as well. Currently, children at least 2 years old and adults with profound deafness are candidates for implantation. Cochlear implant candidacy should be extended to adults with severe hearing impairment and open-set sentence discrimination that is less than or equal to 30% in the best-aided condition. Access to optimal education and (re)habilitation services is important for adults and is critical for children to maximize the benefits available from cochlear implantation.”Critics of the NIH conclusions pointed out that there were no rigorous, controlled studies of outcomes for children. In particular, they worried that only surrogate end points and controversial markers of language acquisition were being used. In a review published in 1997, Lane and Grodin noted that “the decision to have the surgery and habilitation may promote delays in the child's acquisition of ASL [American Sign Language] and therefore may delay the time when that child has any full language at his or her command. Developmental milestones for signed languages are similar to those for spoken languages, and the later the acquisition of ASL, the poorer its mastery on the average. If implanted children become fluent neither in English nor in ASL, their intellectual, social, and psychological development may be compromised”. (11)In a comprehensive review published in 1998, Lane and Bahan noted that, until long-term outcome studies were done to quantify linguistic, psychosocial, and educational attainments of implanted children, the procedure should be considered innovative or experimental and should be conducted only in carefully designed research protocols. (12) These articles were asking questions that were partly methodological, partly ethical. By asking which outcome measures should be used, they were asking how to define success. Should it be word recognition? School performance? Quality of life? Or something else?At this point, both sides in the battle seemed to have firm and incompatible positions. On the one hand, the surgeons and audiologists were enamored of the new technology and even, perhaps, a little starry-eyed about the potential benefits. Reports in the mid-1990s often included individual case reports, but no careful studies of series of patients who had received implants. (13) Such individual case reports were usually optimistic, but not scientifically rigorous. Thus, members of the Deaf community remained skeptical and unsure of the efficacy or the implications of this new technology. These critics within the Deaf community seemed to be the voice of reason, calling for careful study and evaluation. But the real-world situation was never really so simple or dichotomous.Some advocates within the Deaf community, as well as some bioethicists, suggested that even a treatment that would be 100% effective (what is 100% effective?) was undesirable. (14) Some of these critics saw attempts to treat or cure deafness as a genocidal attack on the Deaf community, an extension of the oralist tradition of Bell and others. An article in The Washington Post in 1997 described this group as “a radical segment” who “…call cochlear implants ‘the devil’s work;’ who consider Miss America 1995, the deaf Heather Whitestone, a charlatan because she speaks; who picket oralist deaf schools and stop parents of children with implants in malls, demanding to know why they butcher deaf babies”. (15) In the eyes of this group, cochlear implants were seen as an intervention that was offered not to improve the outcome for individual children but, instead, as an assault on the Deaf community. Given these views, it seemed that there could be no reconciliation in views between the proponents of implants and their opponents.Then, somehow, and rather suddenly, the terms of the controversy seemed to change. The Deaf community softened its opposition. Cochlear implants came to be seen as one acceptable option among many. The reasons for the transition in attitudes are complex.One explanation for the shift was that it was a response to public pressure on the NAD. This is the view of I. King Jordan, President Emeritus of Gallaudet University: “The NAD position received adverse attention from the media and criticism from moderates in the deaf community and from physicians and allied health professionals who considered cochlear implants an appropriate option”. (16) In response to this adverse attention, the NAD withdrew its controversial position paper. In 2000, they released a new position paper that recognizes cochlear implants as one of multiple options. Note that this change in outlook does not mean that the NAD’s earlier concerns were wrong. The new statement endorsed informed parental choice, recognized that there is diversity within the Deaf community, and supported parents’ right to make the decision that they think best for their child.Another reason why the debate shifted is that follow-up studies have answered some of the questions that could not be answered in the early days. When cochlear implants first came into use for children, it was not clear whether the implants would cause more harm than good by promising more hearing, and thus more verbal linguistic functioning, than they could deliver. The earliest outcome studies confirmed some of these fears. Many children who received cochlear implants continued to have poor language acquisition and to do poorly in school.Later studies showed that outcomes were, in fact, variable. Some children who receive implants are able to function well in the hearing world. More often, however, they continue to require special assistance or to use sign language along with spoken language. Thus, many children who have had a cochlear implant still go to school in classrooms for the deaf, or in hearing classrooms but with special services. For many such children, the approach has changed from an either/or, oralism/manualism choice to an approach that stresses bilingualism (that is, the use of both ASL and oral communication) in an approach that is individualized and uses all available technology. (18)Some issues remain contentious. The question of whether parents who are deaf should be able to choose to have a child who is deaf, either through preimplantation genetic diagnosis or through prenatal testing and termination of pregnancy if the child is not deaf, remains as contentious as ever. (19) Some advocates for the deaf (20) and some bioethicists (21) insist that this option should be available to parents who want an infant who is deaf. Others insist that it is unethical, even though understandable, for parents to choose deafness for a child. (22)Of course, some forms of reproduction are easier to regulate than others. Couples may be denied access to preimplantation genetic diagnosis. But it is difficult to imagine regulations that would prevent two carriers of an autosomal dominant form of deafness from having children together.Questions have arisen about the cost of cochlear implants and of access to both the technology and the extensive postsurgical habilitation that is necessary. A recent study from Cleveland showed that poor children were just as likely to receive a cochlear implant as children of higher socioeconomic status, but that they had more complications and poorer follow-up care. (23) Such outcomes are not unique to cochlear implants but are true for many medical treatments that require long-term follow-up. Although disparities in access to health care are common throughout medicine and throughout the world, the issues surrounding deafness raise unique issues, because most newborns in the United States are now screened for hearing loss. (24) In general, screening programs should be implemented only if there is access to appropriate treatment for those who test positive. (25)