charge syndromecharge syndrome: characteristics and outcomes in pediatric cochlear implantation...
TRANSCRIPT
![Page 1: CHARGE SyndromeCHARGE Syndrome: Characteristics and Outcomes in Pediatric Cochlear Implantation Nancy Young, MD, Beth Tournis, AuD, Jenelle Sandy, AuD Ann & Robert H. Lurie Children’sCHARGE](https://reader035.vdocument.in/reader035/viewer/2022070719/5eded251ad6a402d666a2c8d/html5/thumbnails/1.jpg)
CHARGE Syndrome: Characteristics and Outcomes in Pediatric Cochlear Implantation
Nancy Young, MD, Beth Tournis, AuD, Jenelle Sandy, AuD Ann & Robert H. Lurie Children’s Hospital of Chicago
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Disclosures Cochlear Americas Audiology Advisory Board Member
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Lurie Children’s CI Team
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Helping Children To Achieve Their Full Potential
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CHARGE • Complex disorder usually affecting hearing, balance and vision • Life threatening heart and airway problems often present at birth • Malformed cochlea present in 90% • Prediction of cognitive potential is not possible early in life:
– Intelligence in normal hearing cannot be accurately predicted – Underestimation of intelligence likely in deaf children due to low language level – Present of impaired vision and motor function further increases difficulty of evaluation and
likelihood of underestimation of cognitive potential
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Study Design • Retrospective chart review • Subject inclusion criteria
– Diagnosis of CHARGE Syndrome – 12 month minimum of device use
• Outcomes Post-CI – Auditory level & device use – Communication mode(s) for receptive & expressive language
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Auditory Level • Level 1
– No benefit, minimal detection
• Level 2 – Improved detection (SAT < 30dB)
• Level 3 – Closed-set speech perception (ESP 2,3,4)
• Level 4 – Open-set speech perception
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Subject Characteristics (N=12) • Average age at CI: 3.5 years (range 1.7 - 8.2) • Implanted unilaterally with full electrode array • Gender: 7 males and 5 females • Average length of follow up: 4.8 years (range 2.0 – 10.1) • All children received long-term aural habilitation
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Auditory Level At Last Follow Up: No Detection/Detection only
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Patient Pre CI Auditory Level & aided SAT (dB)
Post CI Auditory Level & aided SAT (dB)
Follow Up Interval (yrs)
CND Expressive Communication Mode Post CI
2 1 55 1 50 2.2 - Sign
4 1 55 2 20 4.7 - Sign/oral
5 1 30 2 20 2.8 - Sign/oral
7 1 85 1 85 10.1* + Sign/AC^
8 1 55 2 25 3.9 + Sign/AC^
11 1 40 2 25 3.7 - Sign/oral
Level 1= no benefit; Level 2= SAT< 30dB; Level 3= closed-set; Level 4= open-set
^AC=augmentative communication
*Inconsistent use
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Auditory Level At Last Follow Up: Closed-set
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Patient Pre CI Auditory Level & Perception Category
Post CI Auditory Level
Follow Up Interval (yrs)
CND Expressive Communication Mode Post CI
1 1 - 3 ESP 2 6.2 + Sign/oral/AC^
9 1 - 3 ESP 2 5.2 + Sign/oral
10 3 ESP 3 3 ESP 2* 2.5 + Oral*
*inconsistent CI use, open-set in non-CI ear
Level 1= no benefit; Level 2= SAT< 30dB; Level 3= closed-set; Level 4= open-set
^AC=augmentative communication
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Auditory Level At Last Follow Up: Open-set
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Patient Pre CI Auditory Level & Perception Category
Post CI Auditory Level
Follow Up Interval (yrs)
CND Expressive Communication Mode Post CI
3 1 - 4 Open-set 5.0 - Sign/oral
6 1 - 4 Open-set 9.3 - Sign/oral/AC^
12 2 ESP 2
4 Open-set 2.0 + Sign/oral
Level 1= no benefit; Level 2= SAT< 30dB; Level 3= closed-set; Level 4= open-set ^AC=augmentative communication
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Time Course For Development Of Speech Perception
Patient ESP 2 (years)
ESP 3 (years)
ESP 4 (years)
Open-set (years)
1 2
3 1 1.5 2.5
6 1.5 1.5 3.0
9 6
10* 1.5
12 0.5 0.8
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*ESP 3 pre-implant; Limited use at school only, has open-set in non-CI ear
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Ethan: Age 8, 5 Years Post CI (implanted age 36 months)
• Tracheotomy • Pre CI
– Bilateral severe to profound – Aided SAT 55dB – Receptive: none measurable – Expressive: natural gesture – Implanted in ear with normal 8th nerve
• Post CI – Open-set achieved: 36 months – Receptive: oral with sign
– Expressive language: • Sign • Augmentative communication
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Johan: Age 6 Years (Implanted 4 years, 4 months) • Pre-implant: MAIS=70%
– Right: mild sloping to moderately-severe unaided thresholds • Aided SAT 20dB
– Left: moderately-severe sloping to profound unaided thresholds • Aided SAT 25dB
– Sign and Spoken language (poor intelligibility) – Imaging: left eighth nerve hypoplasia in addition to bilateral vestibular malformation
• Post CI Left Ear – Open-set achieved by 9 months – MLNT 42% in aided and CI ear at 2 years – Improved speech intelligibility
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Summary • Majority (10 of 12)
– Achieved at least significant improvement detection – Consistent device use
• Measurable improvements in auditory skill usually occurred over a long time course • Improved detection and speech perception may occur despite cochlear nerve
deficiency as defined by MRI • Multiple communication strategies were used
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Conclusion • Children with CHARGE syndrome are viable cochlear implant candidates although
outcomes are difficult to predict • Benefits range from improved detection to open-set speech perception and spoken
language • Family counseling should include:
– Expected longer time course for auditory skills to emerge – Need for aural habilitation despite initial lack of progress – Sign language & augmentative communication are likely to be integral to communication
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Everybody is a genius. But if you judge a fish by its ability to climb a tree, it will spend its whole life thinking its stupid.
- Albert Einstein