JULY 12, 2019
Hearing and Speech Outcomes in Children Following Cochlear Implantation of Congenital Inner Ear MalformationsOwen Darr, MDFellowPediatric OtolaryngologyAssistant Professor
Disclosures
• None
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
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.
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
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%
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
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
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
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
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
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
Acknowledgments
• Marc Thorne, MD MPH• Teresa Zwolan, PhD• Ellen Thomas, M.A., CCC-SLP• Paul Kileny, PhD• UM Hearing Rehabilitation Center
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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