Assistive Technology and Cochlear Implants for Children Who Are Deaf-Blind - Research Bibliography

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Updated 3/2015


Adapting Environments to Support the Inclusion of Students Who Are Deaf-Blind --Prickett, Jeanne Glidden; Welch, Therese Rafalowski. Baltimore: Paul H. Brookes Publishing Co. Welcoming Students Who Are Deaf-Blind into Typical Classrooms: Facilitating School Participation, Learning, and Friendships. Norris G. Haring and Lyle T. Romer (Eds.). (1995) Adaptations and modifications can be made to support full participation of students who are deaf-blind in typical classroom settings and activities. Chapter 9 outlines the rationale for making sensory adaptations and the ways in which adaptations can be made to meet the needs of a student who is deaf-blind. Nice organization of environmental and materials adaptations.


Assistive Technology for Students Who Are Blind or Visually Impaired : A Guide to Assessment --Presley, Ike; D'Andrea, Frances Mary. New York: AFB Press. (2008) This book is designed for teachers and other service providers who assess students for the purpose of documenting their educational needs and suggesting potential solutions through the use of assistive technology (AT). The suggestions and examples provided are generally aimed at K-12 students, but many may also be relevant to college students and adults. Part 1 is an overview of high-tech and low-tech AT tools and devices that can be used for learning and literacy, accessing print and electronic information, producing written information, and producing materials in alternate formats. Part 2 provides a detailed guide to the process of assistive technology assessment (preparation, conducting an assessment, writing recommendations, implementing recommendations). Legal issues regarding AT and special education are dealt with in an appendix. Available from AFB Press.  Phone: 800-232-3044. Publisher's web site:


Children with Cochlear Implants and Complex Needs : A Review of Outcome Research and Psychological Practice --Edwards, L. C. Journal of Deaf Studies And Deaf Education, 2007 Summer; Vol. 12 (3), pp. 258-68. (2007) In recent years, the number of children receiving cochlear implants who have significant disabilities in addition to their deafness has increased substantially. However, in comparison with the extensive literature on speech, language, and communication outcomes following pediatric implantation in children without complex needs, the available literature for this special group of children is relatively sparse. This article reviews the available research on outcomes, grouping studies according to the nature of the additional disabilities (including visual impairment) and specific etiologies of deafness. The methodological problems relating to outcome research in this field are outlined, followed by some tentative conclusions drawn from the literature base while bearing these problems in mind. The remainder of the article focuses on the challenges for clinical practice, from a psychological perspective, of implanting deaf children with complex needs. Two groups of children are considered, those whose additional disabilities have been identified prior to implantation and those whose difficulties become apparent at some point afterward, sometimes many years later. A case example describing the psychological assessment of a deaf-blind child being considered for implantation is presented.


Cochlear Implantation --Todd, N. Wendall, M.D, M.P.H. San Diego, CA: Plural Publishing. CHARGE Syndrome. Timothy S. Hartshorne, Margaret A. Hefner, Sandra L. H. Davenport, James W. Thelin (Eds.) (2011) Chapter 9 within the book CHARGE Syndrome covers cochlear implantation.  It includes sections on indications for cochlear implantations, complications and problems with cochlear implantation, outcomes, reports on two patients implanted by the author and concludes with a brief paragraph on cochlear implantation surgery in the future. Publisher's web site:


Cochlear Implantation in Children With CHARGE Association --Bauer, Paul W., MD; Wippold, Franz J. II, MD; Goldin, Jenifer, MS, CCC-A; Lusk, Rodney P., MD. ARCH OTOLARYNOGOL HEAD NECK SURGERY, vol. 128, September 2002, pp. 1013-1017. (2002) This study explores the anomalies of the temporal bone found on radiologic examination, technical challenges in cochlear implantation, and the audiologic benefit derived from cochlear implantation in children with CHARGE Association.  Conclusions found that individual outcomes after implantation may vary, parents should be counseled thoroughly and have appropriate expectations before proceeding with implantation.


Cochlear implantation in Children with CHARGE syndrome : Therapeutic decisions and outcomes --Lanson, Biana G.; Green, Janet E.; Roland, Thomas; Lalwani, Anil K.; Waltzman, Susan B. The Laryngoscope, 2007 Jul; Vol. 117, #7, pp. 1260-6. (2007) OBJECTIVES: Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome. STUDY DESIGN: Retrospective chart review. METHODS: Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome. RESULTS: All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS. CONCLUSIONS: Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population. Publisher's web site:


Cochlear Implantation in Children with CHARGE Syndrome --Southwell, Katherine E.; Bird, Philip A.; Murray, Daran P. COCHLEAR IMPLANTS INTERNATIONAL, vol. 11, #3, September 2010, pp. 170-183. (2010) This article presents details about three children (ages 3, 14, and 17) with CHARGE Syndrome who received cochlear implants. One of the children is described as having visual impairments (bilateral coloboma and glaucoma). All showed improvement in their audiological function--one child has high functioning verbal communication, one uses both sign and verbal communication with improved speech quality and ability to speech read, and the third responds reliably to sound, understands short phrases and attempts to vocalize (vocalizing limited by tracheostomy).


Cochlear Implantation in Individuals with Usher Type 1 Syndrome --Liu, Xue Z.; Angeli, Simon I.; Rajput, Kaukab; Yan, Denise; Hodges, Annelle V.; Eshraghi, Adrien; Telischi, Fred F.; Balkany, Thomas J. INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, vol. 72, pp. 841-847. (2008) This article reports the findings of a case series study of nine patients with Usher Syndrome Type 1. Pre- and post-implantation audiologic tests included pure tone audiometry, speech perception measures, and qualitative assessment of auditory performance.  Pre-implantation assessment indicated that all of the subjects were pre-linguistically profoundly deaf, had no consistent response to sound, had varying degrees of auditory-oral habilitation. Age at implantation ranged from 2 to 11 years. There was post-implantation improvement in sound detection and speech recognition measures in all patients. Children implanted at an age of 3 years or less showed good open-set speech perception with lip-reading. All patients are implant users. Those patients who do not show open-set perception still use the cochlear implant as an adjunct of lip-reading or total communication. Genetic testing for Usher Syndrome Type 1 allows early identification and intervention of children. Timely intervention is important to maximize the development of useful auditory-oral communication skills prior to the onset of the visual impairment.


Cochlear Implantation Outcomes in Children with Waardenburg Syndrome --Amirsalari, Susan; Ajallouyean, Mohammad; Saburi, Amin; Haddadi fard, Adel; Abed, Maryann; Ghazavi, Yasaman. Iran: Springer-Verlag. EUR ARCH OTORHINOLARYNGOL. (December 11, 2011) Waardenburg syndrome (WS) is an autosomal dominant disease, characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia iridis, white forelock, and congenital sensori-neural hearing loss (SNHL). The aim of this study was to evaluate the outcome of cochlear implantation in children with WS and compare it with children with pure SNHL. In a prospective study we evaluated 336 cochlear implanted children from 2008 to 2010. The WS was diagnosed by its established criteria and for control group children without any dysmorphic features, anatomical, behavioral, and developmental disorders were also enrolled. We evaluated children of both groups 1 year after cochlear implantation by categories of auditory performance (CAP) and speech intelligibility rating (SIR) tests. Eighty-one children out of the total 336 who had SNHL were included in study. Out of these 75 (22.3%) were healthy and six (1.78%) had WS. Of the 75 healthy children 40 (53.3%) were girls, while of the six children with WS, three (50%) were girls. There was a significant difference in SIR between WS and cases with pure SNHL (2.67 ± 1.03 vs. 3.79 ± 1.11, p = 021) however, the difference was not significant in CAP (4 ± 1.26 vs. 5.13 ± 1.13, p = 0.082). Prevalence of WS was 1.78% at Baqiyatallah Cochlear Implant Center. One year after implantation there was no significant difference in auditory outcome; however, the difference in speech outcome was significant between WS and cases with pure SNHL.


Cochlear Implants for Children with Blindness or Visual Impairments --Stremel, Kathleen. DVIQ, Spring 2009, pp.23-26. (2009) This article presents early findings and provides some strong general "indicators" of intervention startegies for children who are deaf-blind with cochlear implants. Available on the web:


Cochlear Implants in People Who Are Deaf-Blind --Chute, P. M.; Nevins, M. E. JOURNAL OF VISUAL IMPAIRMENT AND BLINDNESS, May-June 1995, pp. 297-301. (1995) The debate over providing cochlear implants for children continues. This article describes the evaluation process used by the Manhattan Eye, Ear and Throat Hospital to determine if a child is a good candidate for a cochlear implant.  The article then goes on to discuss the effects of cochlear implants on the lives of three deaf-blind children.


Congenitally Deafblind Children and Cochlear Implants : Effects on Communication --Dammeyer, Jesper. JOURNAL OF DEAF STUDIES AND DEAF EDUCATION, vol. 14, #2, Spring 2009, pp. 278-288. (2009) There has been much research conducted demonstrating the positive benefits of cochlear implantation (CI) in children who are deaf. Research on CI in children who are both deaf and blind, however, is lacking. The purpose of this article is to present a study of five congenitally deafblind children who received cochlear implants between 2.2 and 4.2 years of age. Ratings of video observations were used to measure the children's early communication development with and without the use of their cochlear implants. In addition, parental interviews were used to assess the benefits parents perceived regarding their children's cochlear implants. Two examples are included in this article to illustrate the parents' perspectives about CI in their deafblind children. Benefits of CI in this cohort of children included improved attention and emotional response as well as greater use of objects in interaction with adults. The best overall outcome of CI is not spoken language but better communication.


Family Survey Results : Children With Deaf-Blindness Who Have Cochlear Implants --Bashinski, Susan M.; Durando, Julie; Thomas, Kathleen Stremel. AER JOURNAL: RESEARCH AND PRACTICE IN VISUAL IMPAIRMENT AND BLINDNESS, Summer 2010, vol. 3, #3, pp. 81-90. (2010) This study examined families' feelings and perceptions about their child's cochlear implant and its impact on the child and the family. Survey research methodology involved both Web-based and paper and pencil formats to maximize the response rate. The survey questions asked about the process of implantation, child outcomes, and the availability and use of support services following implantation. Sixty families completed the survey. Eight-five percent indicated they would again decide to seek an implant for their child, knowing what they know now. Available on the web:


Follow Up of Cochlear Implanted Handicapped Children --Hamzavi, Jafar; Baumgartner, Wolf Dieter; Egelierler, Brigitte; Franz, Peter; Schenk, Barbara; Gstoettner, Wolfgang. INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, vol. 56, pp. 169-174. (2000) The purpose of this study was to document the progress and benefit of ten children with multiple disabilities using cochlear implants. The evaluation of auditory responses to speech (EARS) test battery was performed on the children in this study at regular intervals following implantation. All children went through individually tailored intensive audiological rehabilitation programs. The majority of children were successful implant users. One of the children was blind but the implant was not successful in this child.


Outcomes for Children With Deaf-Blindness With Cochlear Implants : A Multisite Observational Study --Wiley, Susan; Meinzen-Derr, Jareen; Stremel-Thomas, Kathleen; Schalock, Mark; Bashinski, Susan M.; Ruder, Charlotte. OTOLOGY AND NEUROTOLOGY, vol. 34, 2013, pp. 507-515. (2013) Introduction: Children with dual sensory impairments are receiving cochlear implants; however, little is known regarding their language outcomes. Materials and Methods: Children between the ages of 6 months and 8 years with dual sensory impairment and cochlear implant(s) were recruited from across the United States to participate in an evaluation of language skills using the Reynell-Zinkin Developmental Scales, a tool validated on children with vision impairment and adapted for children with hearing loss. Basic demographic information was also collected from care givers. Results: Ninety-one children completed assessments after implantation. For receptive language abilities, 32% of children obtained a level of sound detection, 15% obtained the ability to understand simple words, 21% could identify words, 5% could follow simple directions, and 22% could follow directions related to the functional use of objects. Four children had no response to sound after cochlear implantation. For expressive language abilities, 49% only had sound production skills, 9% could jargon, 18% could communicate with some words, 12% could communicate with simple sentences, and 12% could communicate with complex sentences. Children with lower developmental ages (or quotients) tended to obtain lower level expressive language skills such as sound production and jargoning. Developmental abilities, rather than age at implant, were the most robust predictor associated with outcomes. Discussion: This information can guide cochlear implant centers when discussing outcomes with families in the cochlear implant candidacy process. There is great heterogeneity in outcomes and caution should be used in discussing possible language outcomes for children with dual sensory impairments.


Pediatric Cochlear Implants : Additional Disabilities Prevalence, Risk Factors, and Effect on Language Outcomes --Birman, C.S.; Elliott, E.J.; Gibson, W.P.R. OTOLOGY & NEUROTOLOGY, vol. 33, 2012, pp. 1347-1352. (2012) Objective: To determine the prevalence of additional disabilities in a pediatric cochlear population, to identify medical and radiologic conditions associated with additional disabilities, and to identify the effect of additional disabilities on speech perception and language at 12 months postoperatively. Study Design: Retrospective case review. Setting: Tertiary referral center and cochlear implant program. Patients: Records were reviewed for children 0 to 16 years old inclusive, who had cochlear implantYrelated operations over a 12-month period. Interventions: Diagnostic and rehabilitative. Main Outcome Measures: Additional disabilities prevalence; medical history and radiologic abnormalities; and the effect on Categories of Auditory Performance (CAP) score at 12 months postoperatively.Results: Eighty-eight children having 96 operations were identified. The overall prevalence of additional disabilities (including developmental delay, cerebral palsy, visual impairment, autism and attention deficit disorder) was 33%. The main conditions associated with additional disabilities were syndromes and chromosomal abnormalities (87%), jaundice (86%), prematurity (62%), cytomegalovirus (60%), and inner ear abnormalities including cochlea nerve hypoplasia or aplasia (75%) and semi-circular canal anomalies (56%). At 12 months postoperatively, almost all (96%) of the children without additional disabilities had a CAP score of 5 or greater (speech), compared with 52% of children with additional disabilities. Children with developmental delay had a median CAP score of 4, at 12 months compared with 6 for those without developmental delay. Conclusion: Additional disabilities are prevalent in approximately a third of pediatric cochlear implant patients. Additional disabilities significantly affect the outcomes of cochlear implants.


Programmatic Approach to Use of Technology in Communication Instruction for Children with Dual Sensory Impairments --Mar, Harvey H.; Sall, Nancy. AUGMENTATIVE AND ALTERNATIVE COMMUNICATION,vol. 10, September 1994, pp. 138-150. Applications of Technology in the Communication Training of Children with Deaf-Blindness: A Programmatic Approach. Mar, Harvey H. and Nancy Sall. (1994) The purpose of this project was to develop and evaluate an intervention program involving applications of technology to enhance the communication skills and behaviors of children with dual sensory impairments.  Of interest were specific applications designed to increase attention, use of symbols, reception, expression of choice, awareness of contingencies, and social interaction.  Participants included 26 children, ages 3 to 15 years, with dual sensory impairments and other severe disabilities.  A team-collaboration model was utilized in which each child's communication goals were first generated, and intervention activities were then implemented and monitored.  One case example is presented to illustrate the multi-step process.  General results indicated that students with dual sensory impairments, including those with severe to profound cognitive disabilities, could achieve individualized communication goals through the use of microcomputers and other technological resources.  Analysis of intervention goals and activities revealed that, for students with nonsymbolic, nonintentional forms of communication, increasing social attention and contingency awareness were of primary concern. Increasing the use of symbols was most frequently targeted for students with higher levels of communication competence.


Quality of life and Cochlear Implantation in Usher Syndrome Type I --Damen, Godelieve W. J. A.; Pennings, Ronald J. E.; Snik, Ad F. M.; Mylanus, Emmanuel A. M. LARYNGOSCOPE, vol. 116, May 2006, pp. 723-728. (2006) This study from the Netherlands evaluated quality of life (QoL), hearing, and vision in patients with Usher syndrome type 1, with and without cochlear implants.  QoL of 14 patients (7 adults, 7 children) who had cochlear implants was compared with QoL of 14 patients (12 adults, 2 children) who did not have cochlear implants using three questionnaires and the Usher Lifestyle Survey.  A significant benefit of CI was seen in the hearing-specific questionnaire.  The Usher Lifestyle Survey indicated that patients with the CI tended to be able to live an independent life more easily than the profoundly deaf unimplanted patients.  Overall, the authors concluded that QoL can be enhanced by CI in patients with Usher 1, although effects are mostly seen in hearing-related QoL items.


Teaching Students to Request the Continuation of Routine Activities by Using Time Delay and Decreasing Physical Assistance in the Context of Chain Interruption --Gee, Kathleen; Graham, Nan; Goetz, Lori; Oshima, Gail; Yoshioka, Karen. JOURNAL OF THE ASSOCIATION FOR PERSONS WITH SEVERE HANDICAPS, vol. 16, #3, Fall 1991, pp.154-167. (1991) Three students (ages 5-10) with profound intellectual, sensory, and physical disabilities were taught to activate a communication device to request the continuation of activities in which they were involved. The intervention consisted of a consistent response interval and increasing time delay paired with decreasing physical assistance in operating the call device.


Technology-Aided Programs for Person with Severe/Profound and Multiple Disabilities : A Selective Review --Lancioni, Giulio E.; Singh, Nirbhay N.; O'Reilly, Mark F.; Sigafoos, Jeff; Oliva, Doretta. INNOVATIVE TEACHING, vol. 2, #1, 2013, pp 1-12. (2013) This paper represents a brief, selective review of technology-aided programs for persons with severe/profound and multiple disabilities.  Specifically, the paper provides detailed summaries of a number of illustrative studies conducted by these authors for the assessment of (a) microswitch-based programs aimed at promoting response engagement and control of environmental stimulation, (b) speech-generating devices and computer-aided programs directed at promoting communication and stimulus choice, (c) orientation technology solutions for prompting indoor travel, and (d) visual- and verbal-instruction technology for promoting performance of complex, multi-step tasks.  The studies included in the review provide a specific picture of the technological instruments adopted within the programs, of the participants involved, and of the outcomes obtained.  Questions of practical importance left unanswered by those studies and others in the field are also discussed as possible targets of new research.


Using a Color CCTV to Teach Children with Deaf-Blindness --Peck, F. R. JOURNAL OF VISUAL IMPAIRMENT AND BLINDNESS, May-June 1995, pp. 257-261. (1995) A research project was developed to evaluate the use of a color closed-circuit television (CCTV) for teaching students who are deaf-blind. This article presents an overview of the proposal for using the CCTV, the project design, and the outcome of the project.


Videophone Technology and Students with Deaf-Blindness : A Method for Increasing Access and Communication --Emerson, Judith; Bishop, John. JOURNAL OF VISUAL IMPAIRMENT & BLINDNESS, vol. 106, #10, October-November 2012, pp. 622-633. Seeing the Possibilities with Videophone Technology began as research project funded by the National Center for Technology Innovation. The project implemented a face-to-face social networking program for students with deaf-blindness to investigate the potential for increasing access and communication using videophone technology. Ten students with deaf-blindness aged 16 to 20 in four southeastern states were recruited through the network of Deaf-Blind Project offices throughout the United States. Criteria for selection to participate in the study were that the participants needed to have enough functional visual acuity to access a 22-inch videophone monitor and use manual sign language as a mode of communication. After a videophone was installed in each participant's home and school, data were collected over six months, using three primary methods of collection. The data were analyzed through a qualitative design method. The primary outcomes were increased accessibility for interpersonal communication among the students with deaf-blindness, seen notably in subscales of the Intrinsic Motivation Inventory (IMI) and through the development of themes involving the cultivation and maintenance of friendships with peers through interaction using videophone technology. With the role of interactive technologies in our ever-increasing digital landscapes, timing is ripe for research that aids the advancement of accessibility to information and social interaction, particularly among populations that have historically been marginalized in traditional educational systems. Dissemination of the results of the project through the National Consortium on Deaf-Blindness and the American Association of the Deaf-Blind will encourage practitioners in the field to replicate the project's activities with videophone technology to benefit youths who are deaf-blind.

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