JULY 12, 2019 Hearing and Speech Outcomes in Children Following Cochlear Implantation … · 2019. 8. 12. · otologic and radiographic assessment • Cochlear implantation provides

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

Presenter

Presentation Notes

-In the United States and other developed nations, about 1 to 3 out of every 1000 children is born with sensorineural hearing loss. -According to Robert Jackler’s 1987 paper on this topic, congenital inner ear malformations are present in 20% of children born with sensorineural hearing loss (SNHL); other studies have found a prevalence as high as 35%. For these children, cochlear implantation is performed on the basis of preoperative audiologic, otologic, radiologic findings. -Cochlear implant candidacy has expanded in recent years due to successes in hearing and speech outcomes in these children; however, outcomes are often difficult to predict. -Many children with inner ear malformations who undergo cochlear implantation receive only marginal benefit. At the University of Michigan, we have extensive experience in this area, with an existing database of over 1500 implant recipients which includes data on preoperative audiometric testing and postoperative hearing and speech outcomes.

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.

Presenter

Presentation Notes

-The overall goal of this study was to develop statistical tools which will help with prognostic counseling. -We sought to create multivariate regression models to predict speech and hearing outcomes in children with congenital inner ear malformations who receive cochlear implantation.

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

Presenter

Presentation Notes

-Using a prospectively collected database at the University of Michigan, a retrospective chart and imaging review was conducted -921 patients were identified with implantation performed before age 18 -This was narrowed to 150 with some documentation of IEM. Our study cohort was then narrowed to 93 patients born with radiographic verification of IEM who underwent cochlear implantation before the age of 18. -The control group included 138 patients implanted before age 18, without IEM and pursuing oral communication. -Malformation type was assessed by preoperative CT scan. -Control group was accrued from an existing CI outcomes database of normal pediatric control patients, without inner ear malformations or cognitive delay -Narrow IAC – 3 mm at porous acousticus or 1.7 mm at cochlear canal/modiolus -EVA – 2mm at the operculum or 1 mm at the midpoint of the VA, in children (Cincinnati criteria)

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%

Presenter

Presentation Notes

-In order to measure outcomes across the groups, over 20 years of audiometric recording, we needed to define “good outcomes” to create binary outcome variables for logistic regression modeling. -Speech detection was recorded depending on presence of speech detection threshold at 35 dB. -Speech and language outcomes representing expressive and receptive language were measured by the Peabody Picture Vocabulary Test (PPVT), Preschool Language Scale (PLS) and Expressive Vocabulary Test (EVT), using the best score obtained during the first 2 years postoperatively. Outcomes were dichotomized, with a score of 5 representing an age-normal “successful” result. -Speech perception was measured by the Glendonald Auditory Screening Procedure (GASP), Lexical Neighborhood/Multisyllabic Lexical Neighborhood Test (LNT-MLNT), Hearing in Noise Test (HINT) and the pediatric AzBio test. Outcomes were dichotomized, with a score of 5 representing a “successful” result.

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

Presenter

Presentation Notes

-There were 93 patients in the malformation cohort and 138 in the control group -The two groups were notably and significantly different in terms of age of implantation. Those with malformation were implanted later, with an average age of 5 years compared with 2.7. Keep in mind this group includes patients as far back as 1994. -For bilateral implant recipients, outcomes were assessed following the patient’s first cochlear implant. -Comorbidities - 18 / 93 9 CHARGE - sig prob of no SFD 1 Trisomy 21 - no SFD 4 Autism - sig prob of no SFD 2 severe multisystem disorder - neither achieved SFD 2 severe developmental delay - not sig w/ SFD

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

Presenter

Presentation Notes

-PTA of no SFD: 124.5 CI (114.6084-134.3916) Comorbidities - 18 / 93 9 CHARGE - sig prob of no SFD 1 Trisomy 21 - no SFD 4 Autism - sig prob of no SFD 2 severe multisystem disorder - neither achieved SFD 2 severe developmental delay - not sig w/ 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

Presenter

Presentation Notes

-A broad multivariate regression model, which included all inner ear malformations showed a significant correlation to the groups cognitive comorbidity status. -An additional multivariate model was performed on each of our outcomes, using only variables that met a criteria of p<0.2 on univariate analysis. This did show a significant correlation of several inner ear malformations to the outcome of sound detection. Patients with Common cavity deformity, IP-1 and Narrow IAC were less likely than the controls to achieve adequate sound detection at 35 dB. Univariate logistic PTAprebest p<0.0001 (t-test) Comorbid bin p<0.0001 Malfctimp1 p=.001 (common cavity) Malfctimp3 p=0.07 (IP1) Malfctimp5 p<0.0001 (Narrow IAC/CC) All controls had +SFD

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

Presenter

Presentation Notes

-Speech and language outcomes reflected the best performance on the expressive and receptive language battery, of our cohort and controls within 2 years of implant. -Age at implantation showed significant correlation to speech and language outcomes, patients implanted earlier did better. -Interestingly, our groups of patients with IP-II (most of them with concomitant EVA), as well as isolated EVA, actually performed better than our control group as a whole. I think that speaks to the excellent care they receive here through our department, and the intensive rehabilitation achieved through audiology and speech therapy. SLcat binary model >5 Univariate logistic Age p=0.14 Comorbid binary p=0.16 Malfctimp2 p=0.11 (cochlear hypoplasia) Malfctimp3 p=0.15 (IP1) Malfctimp6 p=0.112 (OR 3.48) (isolated EVA)

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

Presenter

Presentation Notes

Additional analysis will need to be done to remove subjects with severe cognitive impairment from the group, as well as the isolated EVA patients. And with those extremes of the lowest and highest performers removed, we’ll what else we can find.

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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JULY 12, 2019 Hearing and Speech Outcomes in Children Following Cochlear Implantation … · 2019. 8. 12. · otologic and radiographic assessment • Cochlear implantation provides