Disclosures 1/28/19. Pediatric CI Team. Current CI criteria for children. Evolution of pediatric implant candidacy indications

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1 Evidence for the expansion of pediatric cochlear implant candidacy René H. Gifford, PhD Department of Hearing and Speech Sciences Vanderbilt University Medical Center KSHA Lexington, KY February 22, 2019 Disclosures Audiology Advisory Board Advanced Bionics Cochlear Americas Scientific Advisory Board Frequency Therapeutics Jourdan Holder, AuD Adrian Taylor, AuD Christine Brown, AuD Kelley Corcoran, AuD Ally Sisler-Dinwiddie, AuD Lindsey Kanes, MS Susan Marko, MS Ciara Alley, MS Geneine Snell, MS Delores Smith, MS Sarah Wilhite, MS Pediatric CI Team David Haynes, MD Alejandro Rivas, MD Marc Bennett, MD Rob Labadie, MD, PhD Matt O Malley, MD Christopher Wootten, MD Frank Virgin, MD Evolution of pediatric implant candidacy indications FDA approval 6/27/1990: Nucleus 22 (n = 142) F0/F1/F2-WSP III & Multipeak-MSP Profound SNHL bilaterally 2 to 17 years of age no open set speech recognition auditory oral education program Pediatric Cochlear Implant Criteria based primarily on the audiogram and auditory progress (or lack thereof ) Current CI criteria for children varies with age each manufacturer outlines slightly different criteria Many clinicians are uncomfortable recommending CI for children who do not meet all criteria. 1

2 Degree of hearing loss < 2 years AB, Cochlear, & MED-EL: profound bilateral SNHL Degree of hearing loss > 2 years Cochlear: severe-to-profound bilateral SNHL AB & MED-EL: profound bilateral SNHL Auditory progress with HAs: Younger children Cochlear, AB & MED-EL: little to no progress with appropriately fitted HAs e.g., IT-MAIS, MAIS, LittlEARS Auditory progress with HAs: Older children Cochlear: 30% word recognition (MLNT or LNT) AB: < 12% word recognition (PBK) or < 30% HINT-C sentence recognition MED-EL: < 20% word recognition (MLNT or LNT) Children must miss 70 to 88% of the signal to qualify. Otol Neurotol. 36(1): Evidence for the expansion of pediatric cochlear implant candidacy HYPOTHESIS: Children who are non-traditional CI candidates, but are not making progress with appropriately fitted HAs and intervention will demonstrate significant benefit from cochlear implantation as defined by improvement in: 1) Speech perception and/or 2) Auditory skill development 2

3 Study inclusion criteria CI recipients < 18 years of age with SNHL and one or both of the following: < 70 db HL PTA for children between 2 and 17 years of age or < 90 db HL PTA for those under 24 months Age appropriate word and/or sentence recognition scores > 30% in the best-aided condition Primary outcome measures Pre- & post-ci results for age appropriate materials: Speech recognition tests in the CI ear, contralateral ear and best-aided conditions NUCHIPs, MLNT, LNT, CNC HINT-C, BabyBio, AzBio Parental questionnaires gauging auditory skills development IT-MAIS/MAIS, LittlEARS, PEACH Participants Participants 51 patients (across 2 centers) 39 unilateral, 12 bilateral Mean age of implantation: 8.3 years Range: 7.0 months to 17.6 years Mean duration of CI experience at reported follow-up: 17.1 months Range: 2.5 to 46.5 months All were implanted with the most recent technology AB: 13, Cochlear: 44, MED-EL: 6 Participants Speech perception testing: PREOP 3

4 63-percentage point improvement 40-percentage point improvement 27-percentage point improvement No decline in performance Mean improvement in total language: 9.9 points (p = 0.024) Carlson et al. (2015) SUMMARY Children w/ less severe hearing losses than specified by FDA labeling, gain significant benefit from CI All but 2 demonstrated equivocal or significantly better outcomes 2 children showed a decrement in best-aided condition (words) These 2 children demonstrated benefit for sentences. We have not seen a definitive point of diminishing returns. A large-scale reassessment of peds CI candidacy is warranted to allow more children access to the benefits of CI. Leigh et al. (2016). Intl J Audiol, 55: S9 S18 STUDY OVERVIEW: retrospective study n = 140 children sensory hearing loss, no additional disabilities 78 CI recipients all implanted < 3 years 62 hearing aid (HA) users RESEARCH QUESTIONS: 1) Which children are truly CI candidates? 2) What is the optimal age for implantation? Leigh et al. (2016). Intl J Audiol, 55: S9 S18 STUDY OVERVIEW: retrospective study n = 140 children SNHL, no additional disabilities 78 CI recipients all implanted < 3 years 62 hearing aid (HA) users RESEARCH QUESTIONS: 1) Which children are truly CI candidates? 2) What is the optimal age for implantation? 4

5 Leigh et al. (2016). Intl J Audiol, 55: S9 S18 Leigh et al. (2016). Intl J Audiol, 55: S9 S18 EXPERIMENT 1: audiometric criteria for implantation CI group: CI < 2.5 years HA group: HA fitting < 2.5 years of age Monosyllabic word recognition at 5 years of age n = 17 n = 21 n = 38 n = 78 Leigh et al. (2016). Intl J Audiol, 55: S9 S18 phonemes Quadratic regression Lower limit of IQR Leigh et al. (2016). Intl J Audiol, 55: S9 S18 EXPERIMENT 2: optimal age at implantation 32 CI recipients All implanted < 2.5 years Language assessed postoperatively at 1, 2, 3, and 5 years post-activation Rossetti Infant-Toddler Language Scale (RITLS) Peabody Picture Vocabulary Test (PPVT) Leigh et al. (2016). Intl J Audiol, 55: S9 S18 Leigh et al. (2016). Intl J Audiol, 55: S9 S18 SUMMARY Children with PTA 65 db HL will have a higher likelihood of exhibiting improvement with CI vs. HA Equivalent to adult indications Moderate to profound SNHL Children tend to be exhibit language delay appx equivalent to the duration of auditory deprivation (prior to CI) Earlier is better à minimize or eliminate delay! 5

6 Other studies Dettman et al. (2004) Arch Otolaryngol Head Neck Surg, 130: Leigh et al. (2011). Ear Hear, 32, Implanting children under 12 months of age Cadieux et al. (2013). Otol Neurotol, 34: Hassepass et al. (2013). Otol Neurotol, 34: Vincenti et al. (2014). Ital J Pediatr, 40: 72. Fitzpatrick et al. (2015). Ear Hear, 36: Dettman et al. (2016). Otol Neurotol. 37:e Cosetti and Roland (2010). Trends Amplif, 14: A major concern is the issue of specificity: the risk of implanting a child without SNHL Anesthesia concerns and complications: incidence of morbidity, mortality, and life-threatening adverse events in children < 12 months was significantly higher than children older than 1 year of age Cosetti and Roland (2010). Trends Amplif, 14: Anesthesia concerns and complications: These concerns from prior papers were mostly due to: lack of fasting very young age (< 1 month) emergency surgery Cosetti and Roland (2010). Trends Amplif, 14: Multiple studies demonstrate no greater anesthetic risk for children < 12 months for CI surgery. Bertram and Lenarz, 2004; James and Papsin, 2004; Coletti et al., 2005; Miyamoto et al., 2005; Waltzman and Roland, 2005; Dettman et al., 2007; Valencia et al., 2008; Miyamoto et al., 2008 Recall that audiometric criteria for pediatric CI - most stringent for youngest children - our youngest language learners 6

7 Pediatric Cochlear Implant Criteria Age at implantation matters Infants can link sound patterns with meaning by 6 months (mommy, daddy, no, bye bye, etc.) Word segmentation abilities develop rapidly between 7.5 and 10.5 months (Jusczyk, 2002). 8 months of age: 60+ words/concepts Pediatric Cochlear Implant Criteria Age at implantation matters 8-month olds: long-term storage of words (up to 2 weeks) important prerequisite for learning language! Houston et al., 2009, 2012; Bergeson et al., 2010: children implanted < 12 months à significantly better word learning abilities Pediatric Cochlear Implant Criteria Age at implantation matters Tomblin et al. (2005): children implanted b/tw months had significantly better expressive language Hearing and/or language learning opportunities likely begin BEFORE birth (DeCasper et al., 1980, 1986; Kisilevsky et al., 2003; Moon et al., 2013; Partanen et al., 2013) Age matters! But current CI criteria are strictest for the youngest children. Dettman et al. (2016). Otol Neurotol, 37:e82 e95. 3 Australian CI centers, n = 125 prospective assessment: speech perception, language, & speech production Assessment time points: school entry & late primary/early secondary Dettman et al. (2016). Otol Neurotol, 37:e82 e95. Group 1: implanted < 12 months Age normative range 7

8 Dettman et al. (2016). Otol Neurotol, 37:e82 e95. n = 160 Age normative range Age normative range CI in 1 st year of life Tobey et al. (2013). Intl J Audiol, 52: Tobey et al. (2013). Intl J Audiol, 52: Age normative range Receptive Language Tobey et al. (2013). Intl J Audiol, 52: Age normative range Expressive Language Dashed lines: 95% confidence limits Outcomes for children implanted < 12 months Houston and Miyamoto (2010). Otol Neurotol, 31: Bergeson et al., (2010). Restorative Neurology and Neuroscience, 28: Houston et al. (2012). Developmental Science. 15(3): Houston et al. (2012). J Am Acad Audiol. 23(6): Leigh et al. (2013). Otol Neurotol, 34(3): Holman et al. (2013). Otol Neurotol, 34(2): Dettman et al. (2016). Otol Neurotol, 37:e82 e95. Guerzoni et al (2016). Laryngoscope, 126: Case studies 8

9 4.5-year old female Term birth, no complications No family history of hearing loss, passed NBHS Identified at 3 years of age with severe-to-profound SNHL in the high-frequency range Etiology: mutations in TMPRSS3 gene Autosomal recessive nonsyndromic hearing loss Fitted with HAs at 3 years of age, home FM systems Auditory verbal therapy 2x/week Home audiologist and ENT à too much hearing for CI KBIT-2: Nonverbal IQ standard score: 121 Verbal IQ standard score: 88 BabyBio words Quiet LEFT: 29% RIGHT: 25% BILATERAL: 30% Did not test QUick Interactive Language Screener (QUILS) Golinkoff, Villiers, Hirsh-Pasek, Iglesias, & Wilson, 2017 culturally-sensitive, computer-based receptive language assessment validated in children with normal hearing Kessler et al. (2018) significant correlation with normreferenced assessments of language in 22 preschoolers with hearing loss PP1122 AAA 2018: Evaluation of the Computerized Preschool Language Assessment (QUILS) for Children with Hearing Loss QUick Interactive Language Screener (QUILS) Prepositional Phrases: Find the kitten in a cup with a yellow ribbon. 9

10 QUick Interactive Language Screener (QUILS) QUick Interactive Language Screener (QUILS) Embedded Clauses: Cowboy Bob told Mia to go to the tool shed and bring him a hammer. But instead, Mia is outside riding her skate board. What did Cowboy Bob tell Mia to do? Known Nouns: Find the doorknob. QUick Interactive Language Screener (QUILS) CI in RIGHT ear 1 month after CI workup 4.5 years old Nucleus CI522 preop CI ear 10

11 1 month 6 month CI in RIGHT ear 1 month after CI workup 4.5 years old Nucleus CI522 3 months after RIGHT CI à CI532 in LEFT ear 6 month PRE-CI LEFT PRE-CI LEFT 6 month 6 month 3 month 11

12 RIGHT CI ear alone LEFT CI ear alone Bilateral CI + Bilateral HA (BiBi) Language and speech production 6 months post activation RIGHT, 3 months post activation LEFT Did not test Language and speech production Language Did not test Did not test Did not test 12

13 Language Case year old male Term birth, no complications No family history of hearing loss Failed NBHS Bilateral moderate-to-severe SNHL identified at 2 months Fitted with HAs at 3 months of age 2 younger siblings Case 2 Case 2 Case 2 Case 2 13

14 Case 2 Case 2 Case 2 CI in RIGHT ear 3 months after CI workup 1 month before 5 th birthday CI512 Case 2 Case 2 Case 2 14

15 Case 2 BabyBio Case 2 Case 2 BabyBio CONCLUSIONS current CI criteria for children are set too low re: audiogram, function, and age pediatric criteria are much more stringent than labeled adult criteria (even more so than Medicare!) requiring children with the best, appropriately fitted HAs to miss 70 to 88% of the signal! and that s in the quiet sound booth CONCLUSIONS CIs provide auditory access to HF information that HAs just cannot provide for those with sev-to-profound SNHL this is critical because children are learning language! Pay attention to auditory and language progress with appropriately fitted HAs more valuable than audiogram Crukley et al. (2011). J Educat Audiol, 17:

16 CONCLUSIONS Children with LESS SEVERE hearing losses than specified by current FDA labeling, gain significant benefit from CI Leigh et al. (2016) PTA = 65 db HL or poorer CI should be considered for children with SNHL who 1. make full-time use of appropriately fitted HAs 2. comply with recommended therapy & intervention 3. but who are NOT making month-for-month progress for auditory, speech, and language skills Large-scale reassessment of peds CI candidacy is needed to allow more children access benefits of CI Questions? Comments? rene.gifford@vanderbilt.edu 16