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………………..…………………………………………………………………………………………………………………………………….. The Impact of Cochlear Implantation on Speech and Language Outcomes in Children with Asymmetric Sensorineural Hearing Loss Prashant S. Malhotra, MD, Oliver Adunka, MD, Jaron Densky, MD, Manasa Melachuri, MS2 Samyuktha Melachuri, MS3, Amanda Onwuka, PhD, Krista Winner, AuD, Shana Lucius, CCC-SLP/AVT, Ursula Findlen, PhD

The Impact of Cochlear Implantation on Speech and ...The Impact of Cochlear Implantation on Speech and Language Outcomes in Children with Asymmetric Sensorineural Hearing Loss Prashant

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Page 1: The Impact of Cochlear Implantation on Speech and ...The Impact of Cochlear Implantation on Speech and Language Outcomes in Children with Asymmetric Sensorineural Hearing Loss Prashant

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The Impact of Cochlear Implantation on Speech and Language Outcomes in Children with Asymmetric Sensorineural Hearing Loss

Prashant S. Malhotra, MD, Oliver Adunka, MD, Jaron Densky, MD, Manasa Melachuri, MS2 Samyuktha Melachuri, MS3, Amanda Onwuka, PhD, Krista Winner, AuD, Shana Lucius, CCC-SLP/AVT, Ursula Findlen, PhD

Presenter
Presentation Notes
Thank you for staying until the very last talk of ACIA! It was worth it, this will blow your minds.
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Disclosures• Ursula Findlen, PhD- Research support from Advanced Bionics, Inc.• Prashant Malhotra, MD- Has served on Pediatric Advisory Board, Med-El• Oliver Adunka, MD:

– Consultant for:• Advanced Bionics Corporation• MED-EL Corporation• Advanced Genetics Technologies Corporation• Spiral Therapeutics

– Research Support:• MED-EL Corporation• Cochlear Corporation• Advanced Bionics Corporation

– Ownership:• Advanced Cochlear Diagnostics, LLC

Presenter
Presentation Notes
These are our disclosures
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NCH Hearing and Implant ProgramSurgeons:

Oliver Adunka, MD (Medical Director)Ed Dodson, MDPrashant Malhotra, MD

Pediatric Nursing/Otolaryngology:Kelly Brothers, CPNPEmily Seitz, RN

Speech Manager: Lindsey Pauline, MA, CCC-SLPPediatric Speech and Language Therapy:

Janelle Huefner, M.A. CCC-SLPShana Lucius, MA, CCC-SLP, LSLS Cert. AVTErin Stefanik. CCC-SLPLauren Wills, CCC-SLPLauren Yoshihiro, M.S., CF-SLP

Neuropsychology:Jennifer Cass, PhD

Audiology Manager: Gina Hounam, Ph.D.-CCC-A Pediatric Audiologists:

Sandra Alston, AuDRebecca Belt, Au.DVirginia Bolster, Au.DNikia L. Bridges, Au.DNicole Schuller, Au.DUrsula Findlen, PhDJamie Hadley Godsey, Au.DAlecia Jayne, Au.DDevon McIlvaine Springer, Au.DRebecca Matsche, Au.DLauren Durinka, Au.DChristine Schafer, AuDHolly Gerth, , AuDLindsey Cameron, Au.DMichelle Shannon, AuDCindi Warner, Au.DKrista Winner, AuD

Presenter
Presentation Notes
We have a big team at NCH, and I want to thank them.
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Expanding Indications…Significant benefit is observed for children not meeting typical candidacy:

o Asymmetric hearing losso Less severe hearing losses (e.g. moderate to profound)o Partial deafness (e.g. ototoxic/high frequency)o Auditory Neuropathy Spectrum Disordero Younger implantation, < 12 monthso Unilateral Profound Sensorineural Hearing Losso Complex medical and developmental comorbidities

Presenter
Presentation Notes
Indications for pediatric cochlear implantation (CI) are evolving Auditory progress more important than only assessing audiometric thresholds, resulting in children with greater amounts of residual hearing are being considered Youngest kids have most benefit, but strictest criteria for implantation… ----------------------------------- Missing HF sounds with the HA… Think beyond the audiogram. Rene Gifford (Vanderbilt from Mayo Clinic) ANSD: Breneman et al. (submitted). JAAA Teagle et al. (2010). Ear Hear
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Expanding IndicationsBilateral

Severe to Profound

Asymmetric SNHL

SSD

Presenter
Presentation Notes
Data exists on bilateral SPSNHL and is emerging also in SSD, which the is the most extreme version of asymmetric SNHL. Kids with unilateral HL have many issues related to hearing loss academically/behaviorally, but not always with speech and language. Are kids with ASNHL more like bilateral SPSNHL or more like SSD, or in between? It’s a bilateral hearing loss, so presumably do worse than SSD. Clinically, that IS what we see.
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Asymmetric Sensorineural Hearing Loss

Definition:Worse ear: severe to profound (≥70 dB)Better ear: >20-30dB and < 60-70dB

PTA-4Interaural difference 30dB

Some variability in definitions

Presenter
Presentation Notes
The definition of ASNHL can be variable, but generally indicates a bad ear in the normal candidacy range, and a better ear has a hearing loss in the mild to moderate hearing loss. AHL Poorer ear Severe to profound hearing loss Better ear >30 dB HL to 4,000 Hz inclusively ≤60 dB HL to 4,000 Hz inclusively Interaural asymmetry ≥30 dB (poorer ear PTA4 – better ear PTA4)
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CI in ASNHL• CI in children with asymmetric sensorineural hearing loss

(SNHL) has been described– Primarily audiologic outcomes (Tzifa 2013, Cadieux 2013)

• Speech Perception in the implanted ear improves (not surprising…)• Improved speech recognition in noise (adults… Arndt 2017)• Improved sound localization• Acceptance of CI is excellent (Sadadcharam 2016)• Acceptance of Acoustic + Electric signal generally high (>90%)

o Benefit may be task dependent (Crew et al 2015)

• Little is reported about impact on speech and language outcomes

Presenter
Presentation Notes
Outcomes of cochlear implantation in children with asymmetric hearing loss are emerging. Much of this is in terms of hearing outcomes – speech perception, sound localization, acceptance of CI. However, there is a paucity of information examining comprehensive speech and language outcomes in this population. Sadadcharam (2016): Pediatric study Only 1/47 rejected CI 86-90% of kids continued with CI +HA and few rejected the HA Arndt (2017) Adult study – SSD and ASNHL – improved speech in noise, Crew et al. (2015) - variable acceptance. Speech may be improved, but music perception may be better with HA alone. Noisy environments and music perception are hard with CI alone. Sound localization improved from 22 degrees (SSD) or 13 degrees (ASNHL). ------------------------------------------------------------------------- In children with single sided deafness, speech perception, sound localization and quality of life have been examined in case series1-3. 1Arndt, S., Prosse, S., Laszig, R., Wesarg, T., Aschendorff, A., & Hassepass, F. (2015). Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter? Audiology and Neurotology, 20(suppl 1), 21–30. 2Tzifa, K., & Hanvey, K. (2013). Cochlear implantation in asymmetrical hearing loss for children: our experience. Cochlear Implants International, 14 Suppl 4, S56–61. 3Cadieux, J. H., Firszt, J. B., & Reeder, R. M. (2013). Cochlear implantation in nontraditional candidates: preliminary results in adolescents with asymmetric hearing loss. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 34, 408–15.
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Our Study• IRB approved, Retrospective review • Free-standing, tertiary children’s hospitalAim:

To determine if children with bilateral asymmetric sensorineural hearing loss had improvements in speech and language outcomes after cochlear implantation– ASNHL in this study: audiometric thresholds < 70 dB HL at any

frequency in the better ear and thresholds ≥ 70 dB HL at most frequencies in the poorer ear

Presenter
Presentation Notes
This study retrospectively reviewed children who received cochlear implants for asymmetric hearing loss (severe to profound in implanted ear, and better than 70dB in the non-implanted ear) implanted at our institution (a large, freestanding tertiary care children’s hospital), with a focus on capturing information on the preoperative speech and language status of these children
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Methods • Inclusion criteria:

– <18 years old– Implanted with CI for ASNHL in poorer ear, 2014-2017– Maintained HA in better ear

• Excluded:– Single Sided Deafness– Auditory Neuropathy or cochlear nerve disorder in either ear– Multiply involved or cognitively impaired (other reasons for delays)– Poor CI use after surgery– Non-native English speakers

Presenter
Presentation Notes
Our study was in children, implanted in the poorer ear between 2014-2017. They maintained a HA in the better ear. Children that were multiply involved, non-native English speakers, cognitively impaired, or with cochlear nerve deficiency and auditory neuropathy were excluded.
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Outcomes• Preoperative and postoperative, ear-specific audiometry• (Speech Perception)• Speech and language outcomes (every 6 months)

– When available, measures of speech articulation, expressive and receptive language, and vocabulary

• Goldman-Fristoe Test of Articulation (GFTA-3)• Clinical Evaluation of Language Fundamentals (CELF-P or CELF-5)• Receptive-Expressive Emergent Language Test-Third Edition (REEL-3) battery• Receptive One-Word Picture Vocabulary Test (ROWPVT)• Expressive One Word Picture Vocabulary Test (EOWPVT)

Presenter
Presentation Notes
Demographics, pre= and post-operative audiometric thresholds, speech perception, and speech and language evaluations were collected. Postoperative (6 months, 12 months) speech perception and speech and language testing data were also collected when available. Preoperative and postoperative, ear-specific audiometry Developmentally appropriate speech recognition tasks (questionnaires, closed-set, and open-set measures) Measures of speech articulation, expressive and receptive language, and vocabulary Challenging to assess S&L because these change with age
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ResultsDemographic Characteristics N=26Length of follow up in months (Mean, SD) 30.1 11.8

Female 10 38%Etiology

Bilateral EVA 7 27%Bilateral EVA/Pendred's 4 15%Congenital CMV 2 8%Connexin 2 8%DFNB59 gene 1 4%Stroke/meningitis 1 4%Unknown 8 31%Usher/Von Willebrand 1 4%

Age at Coclear Implantation (Mean, SD) 7.9 4.6Implanted Ear: Right 13 50%Manufacturer

AB 8 31%Cochlear 14 54%Med El 4 15%

Progression of 2nd ear to CI 8 31%

Presenter
Presentation Notes
26 children had (preoperative audiometric thresholds and speech perception data), and 23 with both comprehensive preoperative speech and language evaluations pre and postoperatively Mean length of follow up was 30 months Average age of CI in this group (not routinely done in past, progressive loss) was 7.9 years. Etiology of hearing loss in this group was frequently isolated EVA or in Pendred’s for a combine 42% of the population, though other etiologies are listed here. 8 kids (31%) progressed to needing a 2nd CI at some point
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Unaided PTA-4 at time of CI: Better ear: 62dB HLWorse ear: 92dB HL

Presenter
Presentation Notes
This shows the asymmetry in our ears. Figure 1 shows the degree of preoperative unaided ear threshold asymmetry in the group – better ear in green, implanted ear black. At time of CI, unaided mean Pure Tone Average-4 (PTA4) for the better ear was 62dB HL, and worse ear was 92dB HL. Figure 2 represents the aided threshold benefit in the implanted ear – with preop HA in black, and postop CI in green
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Preoperative Speech and Language Deficits

Main Speech Indication for Surgery(Not mutually exclusive)

N=26 %

Audition 10 38%Articulation 14 54%Receptive Language 15 58%Expressive Language 17 65%High Frequency access (i.e plurals) 2 8%None 2 8%Auditory Closure 1 4%Global Issues 1 4%

Presenter
Presentation Notes
Based on pre-operative assessments, we did see that kids had deficits in speech and language I was surprised to see this - may indicate either that this group was biased towards poor performers (possibly true given that it is off-label indication and early), or that language is more impacted in ASNHL kids that we anticipated.
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Speech and Language EvaluationsSame Test Preop => Postop

Table 2: Speech and Language Evaluations (N=14)

Test N Standard/Scaled ScoreDifference in in Standard Score

Time of Eval after Surgery (months)

Preoperative PostoperativeGoldman Fristoe -2 5 74.8 ± 21.9 86.0 ± 28.9 11.2 ± 8.0 14.8 ± 5.4

Receptive One Word Picture Vocabulary

5 85.0 ± 16.3 87.8 ± 19.7 2.8 ± 7.9 15.6 ± 10.5

Expressive One Word Picture Vocabulary

4 87.8 ± 20.6 92.3 ± 21.5 4.5 ± 2.9 13.5 ± 5.7

CELF P-2 - Core Language 4 73.0 ± 16.3 83.3 ± 28.4 10.3 ± 17.9 13.3 ±5.9CELF 5 - Core Language 7 67.6 ± 15.1 74.6 ± 18.7 7.0 ± 11.3 8.3 ± 4.7

• (Speech perception improved, not surprisingly)• N = 13/14 (96%) who had same test, improved

Presenter
Presentation Notes
All preoperative speech and language evaluations were conducted within 7 months of cochlear implantation. Fourteen patients had preoperative and postoperative assessments on the same speech or language evaluations. Kids take harder tests as they get older, which makes things complicated. Among them, 93% showed improvement on at least one indicator (n=13). At the postoperative assessment (see last column), we observed an average improvement in the standard score in all test standard scores.. Numbers for each subtest are small, making statistical analysis limited – however differences seen were large in: Goldman Fristoe-2, CELF P-2 and CELF 5 core language tests. Goldman Fristoe increased by 11 points in the standard score in the 5 children, 14 months after CI. This reflects closing of a gap, as it is a scaled score, normed for age differences. Similarly, the standard score for the CELF 5 Core Language also improved by an average of 7 points in 8 months of follow up, and CELF-P2 increased by 10 points after 13 months of follow up. Receptive One Word Picture Vocab and Expressive One Word Picture Vocab improved in scaled score marginally. This may reflect a ceiling effect, as these were the tests that children did best on to start with.
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Speech and Language – Change Over TimeArticulation and Core language

Presenter
Presentation Notes
GFTA CELF core language (PS2 and 5)
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Speech and Language – Change Over TimeVocabulary

Presenter
Presentation Notes
Receptive and Expressive Vocabulary changed less… Flatter lines
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Limitations• Retrospective

– biased towards poor performers?• Inconsistent follow up and timing of

speech and language re-evaluations• Small number

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Conclusions• Age of CI is later (7.8 years) in ASNHL• 42% of kids implanted had bilateral EVA EVA/Pendred’s• Children had most difficulty preoperatively with audition,

articulation, receptive language, expressive language

• After CI, most improvement seen in Goldman-Fristoe 2 (articulation), CELF P2, and CELF 5 Core language tests

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Clinical Implications• Children with bilateral ASNHL do suffer from speech and

language deficits• Improvement in speech and language measures can be

demonstrated in children undergoing unilateral cochlear implantation for asymmetric sensorineural hearing losses.

• These children, who are not typical CI candidates, can benefit from a CI in the poorer ear.

Presenter
Presentation Notes
Read slide
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Thank You!