Cochlear Implants in Children with Congenital Deaf-Blindness or Other Multiple Disabilities Bibliography

by DB-LINK on May 1, 2009
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This is a partial list of materials on this topic available from the DB-LINK Catalog Database.  If you have additional questions, please contact us via email:

Updated 7/2009


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.


Abstracts of the 5th Latin American Congress of Clinical Neurophysiology 7-11 October 2008, Puebla, Mexico : Neurophysiologic and Neuro-Image Methods in the Candidacy for Cochlear Implantation in Deaf and Deaf Blind children --Pérez-Abalo, M. C.; Charro, L; Hernández, MC; Hernández, N; Galan, L. CLINICAL NEUROPHYSIOLOGY, vol. 119, pp. S143-S177. (2008) Brief abstract describing a study that investigated the role of event related potentials (ERPs) and electroencephalography (EEG) to aid in decision-making for cochlear implant candidacy in difficult-to-test children with multiple sensory or neurological impairments (18 were deaf-blind).


Assessing Joint Attention and Symbolic Play in Children with Cochlear Implants and Multiple Disabilities : Two Case Studies --Johnson, Karen C.; DesJardin, Jean L.; Barker, David H.; Quittner, Alexandra L.; Winter, Margaret E. OTOLOGY & NEUROLOGY, vol. 29, #2, pp. 246-250. (2008) Using a prospective case study design, this study investigated how measures of joint attention and symbolic play contributed to an understanding of progress in language acquisition following implantation for two young children with developmental disabilities in addition to hearing loss. One child, identified with global developmental delays, was implanted at the age of 2 years 8 months. The other was diagnosed with autism spectrum disorder and implanted at age of 4 years 4 months. Communication assessments were conducted using the Reynell Developmental Language Scales and the MacArthur-Bates Communicative Development Inventories before implantation (baseline) and at 12 months postimplant. Children were also videotaped during a 10-minute free play with their mothers (joint attention task) and 5-minute solitary play (symbolic play task) at baseline, 6 months, and 12 months postimplant. Videotapes were coded for child attention and play states. The MacArthur-Bates Communicative Development Inventories provided important information regarding both children's emerging joint attention and symbolic play skills that are typically not assessed by direct measures of early child language. Videotaped parent-child interaction revealed qualitative differences in the nature of these children's attention and play, which has important implications for intervention. For these two children, obtaining developmental information from various sources, including precursor skills to the development of oral language, provided a more complete picture of each child than conventional clinician-elicited language assessments alone.


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 Implant Candidacy in Children with Autism --Hayman, Carmen D.; Franck, Kevin H. SEMINARS IN HEARING, vol., #4, pp. 217-225. (2005) Cochlear implant candidacy and follow-up for children with autism have important differences to consider when compared with children without cognitive disabilities. This article describes how general cochlear implant candidacy is modified for children with autism. It specifically addresses the influence of age at implantation, language modality and therapy, and parental expectations. It also describes post implant management issues, including the need for team collaboration with outside schools and therapies, and the use of objective measures for cochlear implant programming and processor settings. The article describes three case examples: two of children who received implants (one with possible cortical blindness), and one for whom implantation was not recommended.


Cochlear Implantation for Children with Hearing Loss and Multiple Disabilities : An Evaluation from an Educator's Perspective --Bertram, Bodo, Ph.D. VOLTA REVIEW, Volume 104 (4), Winter 2004, pp. 349-359. (2004) In recent years, parents of children with hearing loss and various additional disabilities have shown increasing interest in cochlear implants. This article describes how the program in Hanover, Germany is attempting to respond to this interest. It discusses the selection process for children who receive cochlear implants who have multiple disabilities, the necessity of in-depth information and counseling for parents, and the role that additional disabilities play in determining the success of (re)habilitation. A multi-dimensional, team approach is proposed to maximize success for both the child and the family.


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.


Cochlear Implantation in Children with Multiple Disabilities --Streubel, Sven-Olrik; Tatlipinar, Arzu; Limb, Charles, J. OTOLARYNGOLOGY - HEAD AND NECK SURGERY, vol. 133, #2, Supplement, August 2005, pp. P190. (2005) This abstract from the American Academy of Otolaryngology - Head Neck Surgery Foundation's 2005 Annual Meeting describes a study of speech perception outcomes in 40 children with profound sensorineural hearing loss in association with at least one cognitive or physical disability, who underwent cochlear implantation at the Johns Hopkins Hospital between 1980 and 2004. Approximately 80% had cognitive disabilities, of which half also had additional physical disabilities. The remaining 20% had physical disabilities alone. Following implantation, in cases of cognitive deficit, there was an improvement in speech perception test scores in 75%. The authors conclude that despite the challenges of combined auditory, cognitive, and physical deficits, cochlear implantation leads to improved speech perception and enhances communication skills even in this high-risk group.


Cochlear Implantation in Children with Severe Developmental Delays --Simon, Lawrence M.; Oghalai, John S.; Haymond, Jody; Tonini, Ross; Caudle, Susan E. OTOLARYNGOLOGY - HEAD AND NECK SURGERY, vol. 133, #2, Supplement, August 2005, pp. P237-P238. (2005) This abstract from the American Academy of Otolaryngology - Head Neck Surgery Foundation's 2005 Annual Meeting describes a study of four children with severe developmental delays who underwent cochlear implantation. All four have improved self-confidence, communication, and social interactions after implantation, manifested as longer attention spans and decreased timidity and anxiety. All four are now vocalizing, with three correctly imitating sounds and producing specific phonemes. One uses the world "no." Three of the children are calmed when their implant is turned on, one enjoys listening to music, and one has increased his sign language vocabulary from three to sixteen signs by three months post-implantation. The authors conclude that cochlear implantation can provide considerable quality of life improvements for deaf children with severe developmental delays, even though normal speech may not be expected.


Cochlear Implantation in Children with Waardenburg Syndrome --Daneshi, A; Hassanzadeh, S; Farhadi, M. JOURNAL OF LARYNGOLOGY & OTOLOGY vol. 119, #9, pp. 719-723. (2005) Waardenburg syndrome is a genetic disorder characterized by varying degrees of congenital hearing loss; dystopia canthorum; synophrys; broad nasal root; depigmentation of hair (white forelock), skin or both; and heterochromic or hypochromic irides. This retrospective study assessed speech perception, speech production, general intelligence, and educational setting in six profoundly hearing-impaired children with Waardenburg syndrome, aged 2 to 14 years. All of the children's speech perception and speech intelligibility capabilities improved considerably after receiving the implants and they were able to be placed in regular educational settings.


Cochlear Implantation in Deaf Children with Associated Disabilities : Challenges and Outcomes --Berrettini, Stefano; Forli, Francesca; Genovese, Elisabetta; Santarelli, Rosamaria; Arslan, Edoardo; Chilosi, Anna Maria; Cipriani, Paola. INTERNATIONAL JOURNAL OF AUDIOLOGY, vol. 47, # 4, pP. 199-208, (2008) The issue of cochlear implantation in deaf children with associated disabilities is an emerging subject. Currently, there is no consensus on whether to implant children with multiple impairments; moreover, it may be difficult to evaluate these children with standard tests pre- or post-implantation. In addition, these children often have poor speech perception and language skills, making assessment more difficult. Despite these factors, these children often receive important benefits in daily life, with an overall improvement in quality of life. In the present study, post-implant outcomes of 23 profoundly deaf children with neuropsychiatric disorders (e.g., cerebral palsy, mental retardation, autism) were analysed, using objective measures of speech perception, and a questionnaire administered to the parents, aimed at evaluating the benefits in daily life after implantation. The results were quite variable, but overall positive, in terms of speech perception, communication abilities, and improvement in quality of life. The findings add an additional piece of evidence to support the effectiveness of cochlear implantation in these special cases. The vision status of the children is not included in the article.


Cochlear Implantation in Prelingually Deaf Persons with Additional Disability --Daneshi, A; Hassanzadeh, S. JOURNAL OF LARYNGOLOGY & OTOLOGY vol. 121, #9, pp. 635-638. (2007) OBJECTIVES: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-implanted, prelingually deaf persons: mild and moderate mental retardation; learning disability; attention deficit/hyperactivity disorder; cerebral palsy; congenital blindness; and autism. We also aimed to document the development of auditory perception in patients having one of these additional disabilities. STUDY DESIGN: A retrospective study was designed to pursue the above aims.Methods: We examined the records of 398 cochlear-implanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Patients were selected who showed a delay in motor, cognitive or emotional development. The selected cases were referred for psychological evaluation in order to identify patients with additional disabilities. We then compared these patients' auditory perception prior to and one year following cochlear implantation. RESULTS: A total of 60 (15 per cent) cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. These were classified as: mild mental retardation in eight cases (13.33 per cent); moderate mental retardation in five (8.33 per cent); learning disability in 20 (33.33 per cent); attention deficit/hyperactivity disorder in 15 (25 per cent); cerebral palsy in five (8.33); congenital blindness in three (5 per cent); and autism in four (6.66 per cent). All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. CONCLUSION: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation. These patients require unique rehabilitation in order to achieve more auditory development.


Cochlear Implants For Young Children Who Are Deaf-Blind --Stremel, Kathleen; Malloy, Peggy. OR: DEAF-BLIND PERSPECTIVES, vol. 13 #2, Winter 2006, pp. 1-5. (2006) Many state deaf-blind projects are tracking the number of deaf-blind children with cochlear implants, which has seen a dramatic increase in recent years. This article describes the history of cochlear implants, how they work, criteria for implantation, as well as outcomes of implantation. It addresses special issues to consider when implanting a deaf-blind child, including habilitation, parent involvement, and the need for continued and additional research. Available on the web:


Cochlear Implants in Children with Craniofacial Syndromes : Assessment and Outcomes --MacArdle, B. M.; Bailey, C. M.; Phelps, P. D.; Bradley, J.; Brown, T.; Wheeler, A. INTERNATIONAL JOURNAL OF AUDIOLOGY, vol. 41, #6, PP. 347-356. (2002) This study reviewed the outcomes for four children with craniofacial syndromes who had received a cochlear implant. Two had CHARGE syndrome, one had Goldenhar Syndrome, and one had brachio-oculo-facial syndrome. One of the CHARGE children had bilateral coloboma with partial sight and the other had a right coloboma. The child with brachio-oculo-facial syndrome had a left choroidal coloboma. At follow-up, three of the children demonstrated benefit in detection, recognition, and identification of environmental sounds, and they continued to gain receptive spoken language skills, although none had intelligible speech. The group required careful mapping and higher levels of electrical stimulation of the implant compared to typical implantees. Stimulation of the facial nerve was a problem with one child. The pre-implantation assessment of these children requires extensive interdisciplinary discussion and careful radiological investigation. Cases should be carefully selected and parents should receive realistic counseling about outcomes and the time commitment necessary, as habilitation of these children can take twice as long as that of children without additional special needs. Post-implantation, these children continue to require well-coordinated medical and interdisciplinary management.


Cochlear Implants in Congenitally Deafblind Children --Ostli, Elin. NUD BULLETIN, January 2001, pp. 26-27. (2001) This is an article discussing the impact of cochlear implants in congenitally deafblind children in the areas of education and habilitation. Cochlear implantation is different in congenitally deafblind children than in congenitally deaf children and eligibility for it varies as well. Discusses key concepts in early development of these children including habilitative needs. Encourages early implantation for best results.


Cochlear implants in special cases : Deafness in the presence of disabilities and/or associated problems --Filipo, R.; Bosco, P. Mancini; Ballantyne, D. Acta Oto-Laryngologica, Volume 124, Supplement 552, April 2004, pp. 74-80. (2004) The aim of the present study is to construct a reference model with the indication for the attitude, the requirements and the resources needed in order to be able to deal with deafness in the presence of disabilities or associated problems. The study group consisted of 13 adults and 18 children affected by profound deafness, with associated problems and disabilities, who were implanted with Clarion® and Med-El® devices. Selection criteria for candidacy to cochlear implantation and counseling, hospitalization, fitting and speech therapy/rehabilitation are described. Findings were assessed evaluating: (i) use of acoustic feedback, on the ground of Erber's model; (ii) self-sufficiency: assessed by a questionnaire; and (iii) social and family relationships: qualitative judgment based on direct observation, analysis of drawings and structured interviews with family, teachers and therapists. The whole group showed benefit from cochlear implantation, with particular satisfaction for post-lingual deaf-blind adults, as well as for subjects with associated psychopathologies and mental retardation. In conclusion, cochlear implants can improve life quality in profoundly deaf subjects with associated disabilities, increasing both listening and communication skills as well as self-sufficiency while family and social relationships tend to remain stable.


Cochlear Implants in Waardenburg Syndrome --Cullen, Robert D.; Zdanski, Carlton; Roush, Patricia; Brown, Carolyn; Teagle, Holly; Pillsbury, Harold C.; Buchman, Craig. LARYNGOSCOPE, vol. 116, #7, pp. 1273-1275. (2006) This retrospective review describes the outcomes for seven children with Waardenburg syndrome who have received cochlear implants. The average age of implantation was 37 months. All of the children were active users of their cochlear implants and performed well after implantation. None of the children are described as having vision impairment.


Congenital Deafblindness and the Current Debate on Habilitation of Children with Cochlear Implants NUD BULLETIN, January, 2002, p. 7. (2002) This article proposes the benefits of cochlear implantation for congenitally deafblind children. Outlines the benefits as well as the results of the few cases in the Nordic that have underwent implantation.


Cross-Modal Association and Cerebral Plasticity : Implications for Assessment of the Deaf-Blind --Borchgrevink, Hans M. Oslo, Norway: International Conference on Sensation, Perception and Construction of Meaning in Congenital Deaf-Blindness, Gothenburg, August 28-September 1, 2002. (2002) This paper discusses the recent neurobiological research on cross-modal integration and cerebral plasticity and their implications for assessment of deaf-blind people. It reviews the concept of perception and cross-modal association and the sensory organs that transform external stimuli into memory. Discusses perception and the construction of meaning. Reviews cochlear implant technology and the importance of early implantation for people who are deafblind.


CT Diagnosis of Temporal Bone Anomalies and Cochlear Implant Surgery in CHARGE Association --Stjernholm, Christina; Muren, Catharina; Bredberg, Goran. COCHLEAR IMPLANTS INTERNATIONAL, vol. 2, #1, pp. 57-71. (2001) Children with CHARGE Syndrome have multiple disabilities. Impairments of vision and hearing, balance problems, and facial palsy are common. This report describes the temporal bone findings (as seen with computed tomography) and surgical findings of one child with CHARGE and one with a CHARGE-like condition. Both received cochlear implants. The findings included absent semicircular canals, narrow orifice for the cochlear nerve, and abnormalities of the oval and round window, the facial canal, and the ossicles. Outcomes following implantation are also described. Both children had severe visual impairments. Programming of the implant was more difficult than usual because of the their decreased ability to receive visual stimuli and interpret auditory signals. Additionally, they were unable to express their reactions because their facial expression was poor and phonation not yet developed. One year after surgery it was found that the more severely affected child had definitely gained a new way of communication through the cochlear implant. He now reacts to auditory stimuli and enjoys music. He produces more sounds and can interact with sounds in a play situation. The second child also reacts to auditory stimuli when using the implant but prefers sign language and removes the device.


Deaf-Blind Shown to Benefit From Cochlear Implant (2001) This article describes a study of eight deafblind people who received coclear implants, six as adults and two as children. It shows favorable results, especially in speech recognition. The study was conducted by the University of Michigan Health System. Available on the web:


ENT Management of a Child with CHARGE Association --Au, Dennis K. K.; Hui, Yau; Tsang, Amy, W. C.; Wei, William I. ASIAN JOURNAL OF SURGERY, vol. 27, #2, April 2004, pp. 141-3. (2004) A report of a case of a child with CHARGE association who underwent cochlear implantation using an unconventional surgical approach. Includes a review of the child's postoperative speech perception results and the benefits of cochlear implantation in children with multiple congenital defects. The child had strabismus, but did not require corrective lenses. 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.


GJB2 Mutations and Additional Disabilities in a Pediatric Cochlear Implant Population --Wiley, S.; Choo, D.; Meinzen-Derr, J.; Hilbert, L.; Greinwald, J. INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, vol. 70, pp. 493-500. (2006) This study investigated the presence of additional disabilities among children with and without GJP2 mutations who received cochlear implants. Children with severe to profound sensory hearing loss due to GJP2 mutations have often been deemed good cochlear implant candidates. Among the study subjects, 41% with GJB2-related deafness and 33% with GJP2 unrelated deafness had an additional disability other than hearing loss. Additional disabilities were also present in 41% who did not receive GJP2 testing. Other disabilities included learning disabilities, apraxia, epileptiform aphasia, attention deficit disorder, developmental delay, gross motor delay, language delay, and autism. The proportion of children with at least six months CI use who relied on oral communication was 62% in the GJB2 positive group, 66% in the GJB2 negative group, and 38% in the untested group.


Language Development of a Multiply Handicapped Child After Cochlear Implantation --Fukada, Shoichiro; Fukushima, Kunihiro; Maeda, Yukihide; Tsukamura, Keiko; Nagayasu, Rie; Toida, Naomi; Kibayashi, Namiki; Kasai, Norio; Sugata, Akemi; Nishizaki, Kazunori. INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, vol. 67, pp. 627-633. (2003) The presence of additional handicaps in hearing impaired children makes the prediction of language ability after cochlear implantation unreliable. Only limited follow-up data on developmental improvement after implantation among multiply handicapped children is available. The present study reports the course of development (audiological and linguistic) after cochlear implantation in one subject with moderate mental retardation. Preoperatively, his language development showed 34 months delay when compared to chronological age. The difference had shortened to 23 months by 2 years post-surgery. The subject's cognitive delay had not changed upon 2-year follow-up. The cochlear implant can be credited to his improvement in language development.


Measuring Progress in Children With Autism Spectrum Disorder Who Have Cochlear Implants --Donaldson, Amy Isaacs; Heavner, Krista S.; Zwolan, Teresa A. ARCHIVES OF OTOLARYNGOLOGY HEAD NECK SURGERY, vol. 130, May 2004, pp.666-671. (2004) A study of 6 children with autism spectrum disorder who received cochlear implants. Although no deaf-blind children were included, this is one of very few published studies about the impact of cochlear implants in children with multiple disabilities. It found improved standardized expressive and receptive vocabulary scores in children in whom testing was possible. Results also suggested changes in responsiveness to sound, interest in music, vocalization, and eye contact. Gains were small compared with the general implant population but improvements in behaviors and interaction point to a quality of life benefit following implantation that is difficult to quantify.


Multi-handicapped Children - Cochlear Implant Users and Their Education --Potmesil, Milon. DBI REVIEW, #38, July-December 2006, pp.56-59. (2006) The author of this article writes about the quality of life concerning multi-handicapped children. He suggests some changes that may be enabled by the use and accessibility of cochlear implantation.


Multichannel Cochlear Implantation in Visually Impaired Patients --El-Kashlan, Hussam K.; Boerst, Angelique; Telian, Steven A. University of Michigan, Ann Arbor. Otology & Neurotology, vol. 22, 2001, pp. 53-56. (2001) This article describes eight case histories of visually impaired patients who had multichannel cochlear implants. This paper lends support to the increasing feeling that patients who are deaf and blind may be very good candidates for cochlear implantation. It includes descriptions of each case along with discussion, conclusion, references, and comments on each of the seven case studies.


Our Experiences (to Date) with Sam's Cochlear Implant --Butalla, Shannon. OR: DEAF-BLIND PERSPECTIVES, vol. 13 #2, Winter 2006, pp. 5-7. (2006) Parents of a deaf-blind son describe their personal experiences with their son's cochlear implant. Available on the web:


Performance of Multiply Handicapped Children Using Cochlear Implants --Waltzman, Susan B.; Scalchunes, Valerie; Cohen, Noel L. THE AMERICAN JOURNAL OF OTOLOGY, vol. 21, 2000, pp. 329-335. (2000) This research article looks at expanding cochlear implantation guidelines to include the multiply handicapped population and how to determine the pattern of development of perceptual skills in deaf children who have other impairments. Conclusions from the study demonstrate multiply handicapped children generally benefit from implantation, although the rate of growth of perceptual skills is slower than for deaf children with no complicating factors.


Speech Perception Results for Children Using Cochlear Implants Who Have Additional Special Needs --Dettman, Shani J.; Fiket, Hayley; Dowell, Richard C.; Charlton, Margaret; Williams, Sarah S.; Tomov, Alexandra M.; Barker, Elizabeth J. THE VOLTA REVIEW, vol. 104, #4, Winter 2004, pp. 361-392. (2004) Speech perception outcomes in young children with cochlear implants are affected by a number of variables including the age of implantation, duration of implantation, mode of communication, and the presence of a developmental delay or additional disability. The aim of this study was to examine the association between degree of developmental delay and speech perception outcomes for a group of young children using cochlear implants. Forty-nine children who received cochlear implants at the Royal Victorian Eye and Ear Hospital between 1993 and 2001 were assessed by an educational psychologist. The choice of test used for the psychological, cognitive, and motor evaluations depended on the age and development of each child participant. Results suggested that there was a significant association between cognitive development and speech perception based on the categorical scale of outcomes. There was insufficient data available from the formal speech perception test scores for children with a range of cognitive delay to enable valid statistical analysis. Some children with significant cognitive delay demonstrated speech perception benefit from the cochlear implant. This result suggests that presence of a cognitive delay should not preclude children from being considered for receiving cochlear implants.


Successful Cochlear Implantation in a Child with Keratosis, Icthiosis, and Deafness (KID) Syndrome and Dandy-Walker Malformation --Cushing, Sharon. L.; MacDonald, Laurie; Propst, Evan J.; Sharma, Alok; Stockley, Tracy; Blaser, Susan L.; James, Adrian, L.; Papsin, Blake C. INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, vol. 72, pp. 693-698. (2008) Keratosis, Icthiosis, and Deafness (KID) Syndrome is a rare congenital disorder affecting the skin, corneal epithelium, and inner ear. Given eventual visual impairment, cochlear implantation is an important consideration despite an increased risk of wound complications. This article presents a child with KID Syndrome and bilateral profound sensorineural hearing loss. Imaging revealed mild cochlear malformation and Dandy-Walker malformation. The child received a cochlear implant at the age of 12 months, using a small incision approach. Following an immediate minor wound infection, implantation has been successful without further complication over 4 years.

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