Cochlear Implants & Multiple Disabilities: What We’ve Learned So Far

Ella L. TaylorWestern Oregon University

Susan M. BashinskiUniversity of Kansas

2008 Council for Exception Children ConferenceBoston, MA

Session GoalsStatus of research on children

with multiple disabilities and cochlear implants

Challenges of working with multiple partners

Characteristics of children with deaf-blindness who have cochlear implants

Impact of CI on communication

Trends in Cochlear Implantation

FDA continues to lower the age of eligibility for receiving a cochlear implant

Cochlear implant centers are reporting a dramatic increase in the number of bilateral implants

CI technology continues to advance (i.e., the number of implant channels, amount of damage done to ear physiology by implant, etc.)

Research on CI & Multiple ImpairmentsEdwards (2007) reviews the small

amount of research focusing on CI and children with multiple disabilities

General findings from her examination:◦Cognitive functioning is one of the strongest

predictors of progress in developing speech perception and speech production

◦Parents report satisfaction with CI due to increased eye contact, awareness of the environment and response to requests.

Trends in Results of Cochlear Implantation (HI only)

Earlier ages for implantation are associated with more significant, and rapid, progress

Variability in outcomes continues to exist, especially for children who receive implants at older ages

Orally-focused post-implant habilitation is, generally, associated with more positive outcomes than is total communication

Importance of This Research ProjectStates across the country are

reporting:

A significant increase in the number of children with DB receiving implants

Increasing numbers of children with multiple disabilities receiving implants

Importance of This Research ProjectCurrent extant literature base

nearly entirely reports data re: cochlear implants with children who are deaf

Very limited data exist regarding post-implant outcomes for children with deaf-blindness

Outcomes with children who are deaf are NOT transferable to children with deaf-blindness

PurposeThe overall purpose of this

project is NEITHER to: encourage families of children who have DB to seek out an implant

NOR to discourage families from doing so.

The purpose of the study is to establish a database that

families, in the future, might consult to make more informed decisions.

Research Partners

Deaf-Blind Services Projects in 22 states (AZ, CA, CT, DE, FL, GA, IL, KS, KY, MA, MD, MN, MO, NC, NE, NJ, NY, OH, OK, OR, PA, TX)

Cochlear Implant Centers: Midwest Ear Institute (Kansas City, MO) Cincinnati Children’s Hospital (Ohio) Boys’ Town National Research Hospital

(Omaha, NE)

ChallengesNumerous collaborative partners

exacerbate the challenge of compliance with IRB requirements

Completion of subcontracts and / or collaborative agreements has had a negative impact on ID & consent with participants

Identification and assessment of pre-implant participants has been difficult because of health and scheduling variables

Assessment PackageCIDB Project Demographic Survey

MacArthur-Bates Communicative Development Inventories Words & Gestures Words & Sentences

Communication & Symbolic Behavior Scales Developmental Profile (CSBS-DP)

Reynell-Zinkin Developmental Scales for Young Children with Visual Impairments

Speech Intelligibility Rubric

C-SIM

Assessment Schedule

Participants are divided into three groups, for purposes of assessment:

Pre-implant Post-implant

Implant less than seven years Implant for seven years or longer

Assessment schedule varies according to the amount of time the child has had a cochlear implant

ParticipantsDemographics reported here are in regard to 63 participants:

Chronological age: 13 mos. to 12 yrs. 7 mos.

Gender: 64% male, 36% female

Ethnicity: 70% Caucasian 13% Latino 13% African-American 2% Native American 3% Other

Prematurity : 1 out of every 2 born prematurely

CI Status: Pre-implant (19%), Post-implant (81%)

47% were premature

Etiology Percentage

Complications of prematurity 26%

Unknown 24%

CHARGE 20%

Meningitis 6%

Refsum Syndrome 4%

CMV (Cytomeglovirus) 4%

Asphyxia 2%

Klippel-Feil sequence 2%

Pierre-Robin syndrome 2%

Usher syndrome 2%

Congenital Rubella 2%

Hydrocephaly 2%

Maternal drug use 2%

Leber congenital amaurosis 2%

Participants’ Vision Status

Vision Impairment Participants

Low vision (<20/200) 11%

Legally blind 29%

Light perception only 6%

Totally blind 17%

CVI 23%

Diagnosed progressive loss

4%

Further testing needed 11%

Participants’ Hearing StatusALL participants have severe or

profound hearing loss-MUST have to qualify for cochlear implant

76% born with hearing impairment

6% of those tested diagnosed with central auditory processing disorder (CAPD)

27% of those tested diagnosed with auditory neuropathy (AN)

Six children have bilateral CI

Participants’ Age at ImplantDemographics reported below

are in regard to 51post-implant participants:

Range: 6 months to 10 years, 7 months

≤12 months = 12% 13 – 24 months = 33%25 – 36 months = 18%37 – 48 months = 11%49 – 60 months = 12% > 5 years = 14%

Median implant age: 28 months

Time in Sound (months)

Duration with Implant“Time in Sound”

Children with CHARGE Syndrome

46

12

33

58

47

3140

54

12

113

43

60

96

133

41

52

147

54

0

20

40

60

80

100

120

140

160

Child1

Child2

Child3

Child4

Child5

Child6

Child7

Child8

Child9

Ag

e in

Mo

nth

s

Age at ImplantCurrent Age

Additional disabilities

Percentage with additional impairments

Communication (initial assessment)Large percentage of participants

demonstrate little vocabulary comprehension (> 75%)◦92% pre have less than 5 words◦75% post have less than 5 words

Large percentage have little vocabulary production (>80%)◦92% pre have less than 10 words◦88% post have less than 10 words

Data Trends: MacArthur-Bates(initial assessments)

Pre-implant Post-implant

Min

Max Mean Min

Max Mean

Vocab comprehension(407 items)

0 0 0 0 347 45.88

Vocab production(407 items)

0 32 6.17 0 85 9.27

First communicative gestures (12 items)

0 5 2.16 0 11 3.35

Pretending to be a parent (13 items)

0 1 0.17 0 13 1.12

Imitating adult actions(15 items)

0 9 1.50 0 15 2.73

First Communicative Gestures(initial assessment)

DATA TRENDS: MACARTHUR-BATES

Children who had implants for longer duration demonstrated increased output of “first communicative gestures” (n = 23, p < .10)

(e.g., Extends arm to give something, Extends arms to be picked-up)

Bilateral Cochlear ImplantsSix participants have bilateral

cochlear implantsTend to be youngerIntroducing Grace

Preliminary FindingsPopulation of children with DB is

extremely diverse; receiving a cochlear implant is not associated with any particular etiology

Majority of participants have two or more additional disabilities (beyond DB)

Younger participants in study, as a group, received their implants at earlier ages

Participants in study, who had more additional disabilities, as a group received their implants at older ages

Participants appear to be demonstrating tremendous variability in their outcomes

Preliminary FindingsImplantation at an earlier age appears

to be associated with more rapid, significant progress

Participants with longer “time in sound” appear to demonstrate increased output

Participants appear to be showing increased vocalizations during play, after CI

Participants appear to begin to show small increases in response to gestures and words

Concerns and LimitationsIncredible variety in participants’

cochlear implant wearing patterns

Reportedly: some children wear their implants during

all waking hours many children wear their implants only

at school a number of children wear their implants

inconsistently-home and school some participants’ families have

discontinued use of their child’s implant (as much as 10% reported in some states)

Concerns and Limitations

Intensity, frequency, and types of available intervention / habilitation vary widely across children and families

Tremendous need exists for appropriate intervention / habilitation techniques for implementation with children who have concurrent vision and hearing losses AND additional disabilities

Concerns and Limitations

Severe shortage of personnel trained in auditory-verbal (A-V) therapy techniques

Even therapists trained in AV techniques are unsure of how to adapt these for implementation with children with DB

Acknowledgements

USDE grant award H327A050079Co-Investigators:

Kat Stremel, NCDBLisa Cowdrey, Midwest Ear InstituteState Deaf-Blind Project directors

Teachers and families who allowed us to complete research in their classrooms and homes

Contact InfoSusan M. Bashinski (

sbashins@ku.edu)

Ella Taylor (taylore@wou.edu)

Project Websitehttp://www.wou.edu/cidb