july 12, 2019 hearing and speech outcomes in children following cochlear implantation … · 2019....
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JULY 12, 2019
Hearing and Speech Outcomes in Children Following Cochlear Implantation of Congenital Inner Ear MalformationsOwen Darr, MDFellowPediatric OtolaryngologyAssistant Professor
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Disclosures
• None
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Background• 20% of children with congenital SNHL have inner ear
malformations (IEM)
• Pre-operative CI candidacy is based on audiologic, otologic and radiographic assessment
• Cochlear implantation provides the best potential for hearing, speech and language development– Results can be difficult to predict for some
malformations
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Goals and Hypothesis
• Improve prognostic counseling for children with IEM undergoing cochlear implantation
• Using multiple patient variables, create logistic regression models to correlate preoperative factors (age, hearing status, anatomy) to postoperative hearing and speech outcomes.
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UM CI outcomes database query921 candidates with CI at age <18 (1992-2016)
150 candidates with IEM documented
93 patients with radiology demonstrating IEMExcluded explant/reimplant cases
93 with IEM & 138 controls without IEM>1 year follow up
Best score within 2 years of follow-up
EMR word search
CT review / exclusions
CI outcomes database
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Methods - Outcome Measures
Analysis performed on STATA• T-test and Chi-squared for comparative statistics• Univariate logistic regression for each variable• Multivariate logistic regression
– Optimal outcomes in red used for dichotomous outcomes
SPEECH AND LANGUAGE OUTCOME CATEGORIES
BEST SCORE FROM PLS, PPVT AND EVT
1 <552 55-643 65-744 75-845 85*-946 95-1047 >105
*SCORES >85 CORRESPOND TO AGE-APPROPRIATE NORMAL RANGE
SOUNDFIELD DETECTION1 No Detection at 35 dB2 Detection of 3/5 thresholds at 35 dB or better
SPEECH PERCEPTION OUTCOME CATEGORIES1 Speech detection only (no measureable speech recognition)2 Greater than chance on closed set tests, GASP word or sentence score ≤25%3 GASP word or sentence score 26-80%, or closed set speech score >80%4 MLNT/LNT score 5-49%, GASP word/sentence score >80%, or HINT/AzBio 5-49%5 MLNT/LNT score 50-79%, HINT/AzBio 50-79%6 MLNT/LNT or HINT/AzBio sentences score ≥80%
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IEM and Control Groups
IEM OF IMPLANTED EAR N (%) +EVA Common Cavity 5 (5.3) 1Cochlear Hypoplasia 7 (7.5) 0
Incomplete Partition, Type I 11 (11.8) 8Incomplete Partition, Type II 47 (50.5) 35Narrow IAC or Cochlear Canal 11 (11.8) 0Isolated EVA 12 (12.9) 12
IEM COHORT CONTROL COHORTTotal No. 93 138Sex (M:F) 45 M : 48 F 64 M : 74 FAge
Range 1-17 years 7 months – 4.9 yearsMean (SD) 5 (4) * 2.7 (1.2) *
Unilateral CI 63 87Bilateral CI
Sequential 25 49Simultaneous 5 1
Cognitive Delay 18 (19.4%) * 0 **P<.0001
18/93 with cognitive delay or comorbid condition• 9 CHARGE • 1 Trisomy 21 • 4 Autism• 2 severe multisystem congenital disorder• 2 severe developmental delay
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Sound Detection Outcomes• Post-operative soundfield detection @ 35 dB
– No SFD in 14/93 patients with IEM– SFD present in all controls
• Correlated to pre-op PTA in better ear (p<0.0001)– 101 dB (95% CI 99-104) with +SFD– 124 dB (95% CI 115-134) without SFD
• Comorbid cognitive delay (p<0.0001)– 8/14 patients without SFD
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Soundfield Detection at 35 dBSOUNDFIELD DETECTION - MULTIVARIATE
OR Std. Err. z P>z 95% CIPre-op PTA 0.982 0.022 -0.82 0.410 0.94-1.03Cognitive Delay 0.070 0.071 -2.62 0.009 0.001-0.51Common Cavity 0.042 0.072 -1.84 0.065 0.001-1.21Cochlear Hypoplasia 0.126 0.225 -1.16 0.246 .004-4.15Incomplete Partition, Type I 0.149 0.243 -1.17 0.243 .006-3.65Incomplete Partition, Type II 1.310 1.955 0.18 0.856 0.07-24.4Narrow IAC or Cochlear Canal 0.134 0.196 -1.37 0.170 0.007-2.37
SOUNDFIELD DETECTION - SELECTED MULTIVARIATEOR Std. Err. z P>z 95% CI
Pre-op PTA 0.982 0.022 -0.81 0.418 0.94-1.03Cognitive Delay 0.047 0.043 -3.35 0.001 0.007-0.28Common Cavity 0.021 0.028 -2.92 0.004 0.002-0.28Incomplete Partition, Type I 0.076 0.089 -2.20 0.028 0.008-0.76Narrow IAC or Cochlear Canal 0.079 0.083 -2.41 0.016 0.01-0.62
Inclusion of variables with p<0.2 on univariate regression
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Speech Perception Outcomes• Univariate logistic regression showed a wide
variation across malformations– IP-II (p<0.001) and isolated EVA (p=0.016) better
speech perception than controls– CC, IP-I, NIAC/CC no SP assessments, omitted
from model
• Lower pre-op PTA correlated to better SP outcome– 93.6 (95% CI 88.9-98.4), p=0.0002
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Speech and Language OutcomesSPEECH AND LANGUAGE - MULTIVARIATE
OR Std. Err. z P>z 95% CIAge (months) 0.973 0.008 -3.42 0.001 0.958-0.988Common Cavity 0.495 0.630 -0.55 0.581 0.041-5.99Cochlear Hypoplasia 0.471 0.576 -0.06 0.538 0.043-5.17Incomplete Partition, Type I 0.963 0.998 -0.04 0.972 0.127-7.34Incomplete Partition, Type II 5.270 2.850 3.07 0.002 1.82-15.2Isolated EVA 75.23 104.600 3.11 0.002 4.93-1148Narrow IAC or Cochlear Canal (Omitted – predicted failure)Cognitive Delay (Omitted – predicted failure)
SPEECH AND LANGUAGE - SELECTED MULTIVARIATEOR Std. Err. z P>z 95% CI
Age (months) 0.984 0.007 -2.43 0.015 0.971-0.997
Cochlear Hypoplasia 0.348 0.406 -0.90 0.366 0.04-3.4Incomplete Partition, Type I 0.864 0.884 -0.14 0.886 0.12-6.4Isolated EVA 23.2 28.2 2.59 0.010 2.2-250
Inclusion of variables with p<0.2 on univariate regression
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Summary• Children with comorbidities associated with
cognitive delay were limited in their speech and language function after cochlear implantation
• Lower PTA preoperatively is associated with better sound detection and speech perception outcomes
• Patients with IP-II or isolated EVA performed better than other malformations
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Acknowledgments
• Marc Thorne, MD MPH• Teresa Zwolan, PhD• Ellen Thomas, M.A., CCC-SLP• Paul Kileny, PhD• UM Hearing Rehabilitation Center
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ReferencesJackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope. 1987 Mar;97:2-14.
Sennaroglu, L. and I. Saatci, A new classification for cochleovestibular malformations. Laryngoscope, 2002. 112(12): p. 2230-41.
Kim A et al. Role of electrically evoked auditory brainstem response in cochlear implantation of children with inner ear malformations. Otol Neurotol. 2008 Aug;29(5):626-34.
Chadha NK et al. Bilateral cochlear implantation in children with anomalous cochleovestibular anatomy. Arch Otolaryngol Head Neck Surg. 2009 Sep;135(9):903-9.
Papsin BC. Cochlear implantation in children with anomalous cochleovestibular anatomy. Laryngoscope. 2005 Jan;115:1-26.
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Questions?
Thank you!