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Correspondence

Cochlear Implants

N Engl J Med 2008; 358:1522-1524April 3, 2008

Article

To the Editor:

The review article by Papsin and Gordon (Dec. 6, 2007, issue)1 provides useful information about cochlear implantation. However, additional practical points should be noted. The majority of patients with hearing impairment live in countries where the cost of a cochlear implant is the most influential factor. For example, in Iran, two thirds of the price of a $20,000 implant is covered by the government, and the perioperative costs would not exceed $2,000, but fewer than 2000 implants have been inserted in the past 10 years, whereas this figure reaches more than 40,000 in the United States.2 The difference in the gross domestic product per capita between Iran and the United States ($8,900 vs. $43,800) explains this difference.3 Genetics, first through genotype–phenotype studies and recently through gene-expression studies of the inner ear, is gaining a more important role in this field. Some mutations affecting the ganglion cells make implantation questionable, since this structure should be intact to have a functional implant.4 Finally, the trend toward preserving the residual hearing in the ear with the implant was reinforced after a successful multicenter Food and Drug Administration clinical trial of a new hybrid implant.5 These breakthroughs have substantially influenced the field of cochlear implantation since its clinical introduction in the 1980s.

Mahdi Malekpour, M.D.
Tehran University of Medical Sciences, Tehran 1145765111, Iran

5 References
  1. 1

    Papsin BC, Gordon KA. Cochlear implants for children with severe-to-profound hearing loss. N Engl J Med 2007;357:2380-2387
    Full Text | Web of Science | Medline

  2. 2

    Zarandy MM, Malekpour M. Two cochlear implants: halving the number of recipients. Lancet 2007;370:1686-1686
    CrossRef | Web of Science | Medline

  3. 3

    Malekpour M. A jumpstart to nowhere. Lancet 2007;370:317-317
    CrossRef | Web of Science | Medline

  4. 4

    Christiani TV, Alexandrino F, de Oliveira CA, et al. Molecular study in Brazilian cochlear implant recipients. Am J Med Genet A 2007;143:1580-1582

  5. 5

    Gantz BJ, Turner C, Gfeller KE. Acoustic plus electric speech processing: preliminary results of a multicenter clinical trial of the Iowa/Nucleus Hybrid implant. Audiol Neurootol 2006;11:Suppl 1:63-68
    CrossRef | Medline

To the Editor:

The recommendations of Papsin and Gordon to the family should include immediate, frequent exposure to fluent users of American Sign Language.1 As the authors note about one study of children with implants, “40% had speech that was intelligible to the average listener and 79% could use the telephone.” This means 60% had unintelligible speech and 21% could not use the telephone (a standard commonly used to classify a person as being “deaf”). The figure of approximately 20% or more for children with implants who remain deaf is recurrent and intractable.2 Children in this group who are not using sign language are linguistically deprived, and linguistic deprivation that continues through the first several years of life can result in serious cognitive deficits3 and psychological harm.4 The risk is too great. A child with an implant should initially also use American Sign Language while receiving oral therapy to ensure proper linguistic development. And if the child winds up in that 40% with speech that is intelligible to strangers, the child will then be fortunate to be bilingual.5

Donna Jo Napoli, Ph.D.
Swarthmore College, Swarthmore, PA 19081

5 References
  1. 1

    Allen S, DeLuca D, Napoli DJ. Societal responsibility and linguistic rights: the case of deaf children. J Res Educ 2007;17:41-53

  2. 2

    O'Reilly RA, Mangiardi AJ, Bunnell HT. Cochlear implants. In: Leigh IW, DeLuca D, Lindgren K, Napoli DJ, eds. Access: multiple avenues for deaf people. Washington, DC: Gallaudet University Press (in press).

  3. 3

    Sandler W, Lillo-Martin D. Sign language and linguistic universals. Cambridge, England: Cambridge University Press, 2006.

  4. 4

    Christiansen JB, Leigh IW. Cochlear implants in children: ethics and choices. Washington, DC: Gallaudet University Press, 2002.

  5. 5

    Wilbur R. Sign language and successful bilingual development of deaf children. J Inst Soc Res 2001;56:1039-1079

Author/Editor Response

Although cochlear implants should be universally available, we concur with Malekpour that fiscal inequality exists globally, affecting this and every other area of health care. We also agree that genetics now play an important role in the diagnosis of hearing loss, but we note that by far the most common form of genetic deafness (the GJB2 mutation) does not compromise the outcome of cochlear implantation.1 As we indicate in Figure 1 and Table 1 of our article, a viable auditory nerve is necessary for cochlear-implant function and should be examined as part of the candidacy assessment. The hybrid device was not discussed, since further testing in children is warranted, given the unknown long-term effects in a developing auditory system with unknown potential for progression of sensorineural hearing loss.

Like Napoli, we strive to provide children with hearing loss optimal opportunity to develop effective communicative skills, but we do not agree with her recommendation that all children be provided with “immediate, frequent exposure to fluent users of American Sign Language.” Parents or guardians of children with hearing loss are best positioned to choose the communication option that their child will use (i.e., oralism, manual forms of communication, or both). Parents seeking cochlear implants for their children aim to improve perception of oral speech. They should be made aware that many factors (shown in Table 1 of our article) influence a child's ability to understand and develop oral speech–language after implantation, including the mode of communication used.2 The studies we highlighted indicate that cochlear implants have provided children with hearing and oral language skills previously unknown in their peers who use hearing aids. This is particularly true when implants are provided at a young age (i.e., with a limited interval between the onset of deafness and implantation), thereby restricting the effects of deafness on the auditory system3 and allowing the best potential for acquisition of oral speech and language skills.4,5 Certainly, unilateral cochlear implants do not provide normal hearing. With ongoing research and improvements in technology, we aim to better understand the effects of deafness on developing auditory systems and to more effectively promote auditory development for the purpose of oral speech–language learning.

Blake C. Papsin, M.D.
Karen A. Gordon, Ph.D.
Hospital for Sick Children, Toronto, ON M5G 1X8, Canada

5 References
  1. 1

    Taitelbaum-Swead R, Brownstein Z, Muchnik C, et al. Connexin-associated deafness and speech perception outcome of cochlear implantation. Arch Otolaryngol Head Neck Surg 2006;132:495-500
    CrossRef | Web of Science | Medline

  2. 2

    Geers A, Brenner C, Davidson L. Factors associated with development of speech perception skills in children implanted by age five. Ear Hear 2003;24:Suppl:24S-35S
    CrossRef | Web of Science | Medline

  3. 3

    Lee DS, Lee JS, Oh SH, et al. Cross-modal plasticity and cochlear implants. Nature 2001;409:149-150
    CrossRef | Web of Science | Medline

  4. 4

    Harrison RV, Gordon KA, Mount RJ. Is there a critical period for cochlear implantation in congenitally deaf children? Analyses of hearing and speech perception performance after implantation. Dev Psychobiol 2005;46:252-261
    CrossRef | Web of Science | Medline

  5. 5

    Svirsky MA, Teoh SW, Neuburger H. Development of language and speech perception in congenitally, profoundly deaf children as a function of age at cochlear implantation. Audiol Neurootol 2004;9:224-233
    CrossRef | Web of Science | Medline