1. Impact of Degree of Hearing Loss and Very Early Enrollment in EI on Language Betty Vohr, MD Julie Jodoin-Krauzyk MED, MA Richard Tucker BA, Mary Jane Johnson MED, Deborah Topol, BA, Marianne Ahlgren PhD, Women & Infants Hospital, RIHAP, Family Guidance, Rhode Island School for the Deaf Brown Medical School

2. Funding Cooperative Agreement from the Centers for Disease Control and Prevention #URs/CCU12033-01 AUCD from the Centers for Disease Control and Prevention

3. Background 33 babies are born each day in the US with HL 12,000 to 16,000 babies are born each year with HL The rate at birth is 2-3 per 1000 NICU rate is 10 per 1000 Well baby rate is 1 per 1000

4. Approximately 95% of newborns have their hearing screened in the United States We are continuing to learn about the outcomes of these babies and factors predicting outcome

5. Moderate, Severe to Profound HL There is strong evidence that congenital bilateral moderate, severe and profound hearing loss compromises communication skills, language and behavior, at school age.

6. Mild Hearing Loss There is increasing evidence that unilateral or mild/minimal hearing loss < 40 dB also impacts academic and language outcomes at school age. This impact is important since in some states infants with minimal or unilateral HL are not eligible for early intervention.

7. Hearing Loss and Early Intervention There is evidence that infants with congenital HL without other disabilities who receive early intervention services before 6m will have language development similar to hearing peers. Yoshinaga-Itano 1998; Moeller 2000.

8. Age of Identification In Rhode Island over time has decreased significantly Prior to hospital screening30 to 60m 198624-30m 2007 3m Rhode Island Data

9. Objectives To determine the language outcomes of infants with: Moderate to profound bilateral HL (Mod/P HL) Mild (< 40dB) or unilateral hearing loss (Min HL) Normal hearing controls To identify factors contributing to language scores

10. Hypotheses Infants with Min HL will have language scores similar to hearing infants at 12-16 m. Infants with Mod/P HL will have less optimal language outcomes at 12-16 m. Infants enrolled in EI 3 m will have better language scores at 12-16 m compared to infants enrolled > 3 m.

11. Study Design Resources/Support Early Intervention Degree of HL NICU Language at 12-16 months Mod/Prof HL Mild HL Typical Hearing Study Groups Mediators Outcome Outcome

12. Subject Recruitment Eligible families were identified in the RI Hearing Assessment database (RITRACK). Families were recruited through the Family Guidance Program (statewide specialty EI program for children with HL). Families not in the program were recruited by mail. Infants born between 10-02 and 1-05 were eligible

13. Methods Infants with HL were identified and enrolled. Matching for Controls was based on a hierarchical matching procedure for gender, DOB ±90days, birthing hospital, NICU status, maternal education, race, and health insurance. 33 infants with all degrees of HL were enrolled. Informed consent was obtained for all subjects.

14. Study Subjects 30 children with Hearing Loss were seen at 12+4 m. Children Mothers Moderate/Profound HL 18*15 Mild HL 1212 Hearing controls 9685 * 6/18 have profound HL Home visits were conducted

15. Assessments at 12+4 months 1. Family Resource Scale (Dunst & Leet) 2. Family Support Scale (Dunst et al) 3. Parenting Stress Index (Abidin) 4. Impact on the Family (Stein & Reissman) 5. Family Demographics 6. MacArthur-Bates Communicative Inventory: Words & Gestures (CDI)

16. MacArthur Communicative Inventory (CDI) Words and Gestures were completed by the mothers Part I: Early Words includes 33 questions about child comprehension of initial phrases and a 396 vocabulary checklist. Part II: Actions and Gestures has a 63 gesture checklist. Percentile scores are available for: phrases understood, words understood, words produced, and early, late, and total gestures.

17. Administration English speaking families: standard CDI Native Spanish speakers: the normed Spanish version ASL families: The ASL version of the CDI If signed vocabulary was understood or used by the child, it was counted accordingly on the English version. Scores are reported separately for 2 non-native English speakers: 1 Control – Mandarin language 1 Mod HL – American Sign Language

18. Data Analysis 1.Three-Way Analysis –Mod/P HL vs. Mild HL vs. Controls –ANOVA to analyze differences among group means – Ҳ 2 to analyze differences among proportions 2.Regression Models were run to show relationships of predictors with outcomes for the total cohort and for the group with HL controlling for confounders.

19. Maternal Characteristics* Mod/P HL (n=15) Mild HL (n=12) Control (n=85) P Ag e 32 ± 732 ± 6 0.999 Married 80%83%87%0.748 high school 27%16%11%0.390 Primary English 87%92% 98%0.124 Medicaid / No Ins. 20%16%17%0.909 Socio-economic status 41 ± 1641 ± 1345 ± 110.309 *Mothers with multiples are counted once and assigned to most severe HL group

20. Child Characteristics Mod/P HL (n=18) Mild HL (n=12) Control (n=96) P Female39%42%36%0.929 White94%92%95%0.689 NICU83%33%55%0.019 <37 weeks83%42%46%0.011 EI100%83%28%0.001

21. Types of Hearing Loss * ** *1 mild bilateral **1 mild bilateral

22. Mean Chronological* Age at Visit GroupMod/P HLMildHLControlP Age (m)16.4±215.1±2 15.6±20.237 Range (11-17)(11-17) (11-17) * Scores were derived from corrected ages for premature infants

23. Percent Early Intervention 3 months p=0.176 6/18(33%) 7/12 (58%)

24. Maternal Report at 12+4 months Mod/P HLMild HLControlP Total Support36.4±937.3±1135.2±110.75 Total Resources121±11126±7125±130.54 Total Impact26.8±525.2±424.9±40.19 Total Stress64.6±1167.8±1567.9±170.70

25. MacArthur CDI % Scores at 12+4 months Score N Mod/P HL 17 Mild HL 9 Control 77 P Phrases Understood % 20±25 +* 52±2241±300.002 Words Understood % 17±24 * 49±25 35±280.012 Words Produced % 20±21 * 49±34 36±260.015 Early Gestures % 29±30 * 59±30 44±310.060 Late Gestures % 12±8 +* 42±30 33±280.006 Total Gestures % 12±10 +* 45±30 34±280.003 + versus Control * versus Mild HL

26. Words Understood: Percent < 10 th 65% P<0.0086 25%27% 16.4 m 15.1m 15.6m

27. Words Produced: Percent < 10 th 53% P<0.0650 25% 16.4 m 15.1m 15.6m

28. Mean Number of Words Produced at Visit 5 P<0.0114 # 20 17 16.4 m 15.1m 15.6m # Analyses of log transformed data

29. Imitation Skills Present (yes/no) 41% NS 67% 65 % 16.4 m 15.1m 15.6m

30. Labeling Skills Present (yes/no) 6% 42%45 % P=.009 16.4 m 15.1m 15.6m

31. Early and Later Gestures Produced: Percent< 10 th P=0.1178 P=0.0953 532528 % 593332

32. Two Additional Children with CDI Data Language ASL Mandarin Study Group Mod/P HL Control Words Understood 4341 Produced26 2 Total Gestures 2421

33. # Words Produced at Visit for Infants with HL Enrolled in EI 3 m and Hearing Controls NS 20 17

34. Mean # Words Produced Reported at Visit by Age of Entry to Early Intervention for Children with HL P=0.02 20 Words 4 words

35. < 10 th Percentile for Words and Phrases Understood by Age of Entry to EI NS

36. Gestures Percentile for Children with HL by Age of Entry to EI.0001.02.01

37. Total Cohort: Regression to Predict CDI Words Understood % Predictor b p NICU -14.7.01 Mod/P HL -16.0.03 Mild HL 9.3.32 SES - 0.3.22 Support - 0.2.56 Resources - 0.3.19 Model R 2 =.19.002 Total Gestures % Predictor b p NICU -13.4.01 Mod/P HL -20.7.004 Mild HL 6.4.48 SES -0.3.22 Support 0.1.61 Resources -0.3.09 Model R 2 =.21.0009

38. Group with HL: Regression to Predict CDI Words Produced % Predictor b p NICU 11.8.32 Mod/P HL -25.1.03 EI 3 m 37.4.0008 Model R 2 =.56.0003 Early Gestures % Predictor b p NICU -3.6.77 Mod/P HL -14.4.21 EI 3 m 40.7.0006 Model R 2 =.59.0002

39. Conclusions Infants with Moderate to Profound HL who have hearing parents have significantly more language delays at 12+4m than infants with Minimal HL and controls. Infants with HL enrolled in Early Intervention 3m have better language skills at 12m than infants enrolled > 3m. These skills are similar to hearing controls.

40. Strengths of Study Strengths of this study are : Infants with all degrees of HL are included Very early enrollment in EI is evaluated The study includes control children with typical hearing. Weaknesses of the study are: The language information at 12+4 m was obtained by parent report and not direct assessment. The majority of children with Moderate-Profound HL were NICU infants.

41. Conclusions All infants diagnosed with permanent HL benefit from specialized intervention programs which meet their developmental and communicative needs and optimize outcomes.

43. CDI: Imitation and Labeling Examples Imitation: child parrots or imitates things s/he has heard including phrases and words s/he is just learning Labeling: child goes around proudly naming or labeling things in her/his environment

44. CDI: Early Gestures Examples Initial Communicative Gestures like pointing, waving, putting arms up to be picked up, shaking or nodding head, reaching arm to show or give toy, shrugging shoulders as if "all gone" or "where'd it go", blowing kisses, etc Initial Games/Routines like peek-a-boo, chase, singing, dancing, etc.

45. CDI: Late Gestures Examples Actions with Objects such as brushing teeth, putting phone to ear, throwing a ball, pouring liquid, making a plane fly, using a spoon, etc; Pretending to be a Parent such as putting a stuffed animal or doll to bed, pretending to feed it, rock it, talk to it, etc; Imitating Adult Actions such as housework, reading, driving, shoveling, putting on glasses, etc.