Single sided deafness in children: risks and intervention

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1 Single sided deafness in children: risks and intervention Astrid van Wieringen Anouk Sangen Christian Desloovere Jan Wouters Experimental ORL, Dept Neurosciences Leuven, Belgium

2 Introduction Unilateral Hearing Loss/ Single sided deafness (UHL/SSD): o prevalence in Flanders: 39% of kids diagnosed with SSD (± 60 kids a year) o prevalence increases in older children/adults due to acquired and progressive hearing lossess o No benefit from a HA or Bone conduction device for binaural hearing o Is UHL/SSD a problem/risk factor? More than spatial hearing? picture

3 Content today s talk Limitations of SSD: to what extent does it impact communication? (no hearing in deaf ear) Clinical study (SSD & age matched NH) Language & memory tests Speech in noise localization Intervention with CI? picture

4 Children with SSD versus NH peers/siblings 3/16 delays in auditory behavior and preverbal vocalizations much more common in infants with UHL, delayed acquisition of 2-word phrases - Kishon-Rabin et al., 2015; Kiese-Himmel 2002; Lieu 2013 significantly lower scores on different language tests and (verbal) cognitive/iq tests, a complex WM task and several aspects of phonological processing - Borg, 2002, 4-6 year olds; Lieu et al., 2010, 6-12 year olds; Lieu et al., 2012, 6-8 year olds; Lieu 2013, 6-12 year olds; Ead et al., 2013, 9-14 year olds; Fisher & Lieu, 2014, year olds; Martínez-Cruz, 2009, 6-8 year olds, Schmithorst, 2014, 7-12 year olds increased rate of repetition of a grade in school, more likely to have individualized education plans and speech therapy, lower scores on a questionnaire of performance and behavior in school - Bess & Tharpe, 1984; Oyler et al, 1988; Lieu 2010, Lieu et al., 2012; Dancer, 1995 reports of behavioral problems (attention problems, internalizing or externalizing problems), stress, fatigue, decreased self-esteem, difficulties in social settings and increased listening effort - Lieu 2012; Hassepass 2012, Borton et al., 2010, Rachakonda; Noh & Park, 2012 Problems persist throughout the years: still worse overall and expressive language scores and lower IQ scores in UHL adolescents Fisher & Lieu, longitudinal study: language and verbal IQ scores increased but problems with behavior, school performance and academic weaknesses persisted (no NH control group) Lieu 2012

5 Auditory, language and cognitive outcomes of school-aged children with severe-to-profound UHL SSD Normal Hearing 22 children clinical sample UZ Leuven 5-15 years of age Congenital & sensorineural severe-to- profound UHL/SSD no intervention, no comorbidities normal education 11 boys, 11 girls Language morphology syntax expressive vocabulary Hearing ability including error analysis: where & which? STM WM SPIN at the good ear Poster 10 - Anouk Sangen, SSQ PhD student ExpORL School behavior SIFTER. 44 children age- and gender matched picture left: picture right:

6 Zooming into specific skills Morphology (word structure), CELF-4-NL A branch of grammar devoted to the study of the structure or forms of words children had to complete a sentence after seeing a color illustration, using a targeted word structure only 5-9 yrs of age He meet s the un happi est boy s

7 Formulating Sentences (FS) expressive syntactic skills formulate a sentence about a color illustration using a targeted word or phrase

8 Expressive One Word Picture Vocabulary Test Martin & Brownell, 2011 children are asked to name in one word, objects, actions and concepts presented with color illustrations

9 Group data

10 Conclusions clinical study 10/16 Children with SSD lag behind in complex language tests expressive vocabulary and syntactic skills No difference between SSD and NH for short term / working memory abilities and speech perception in noise at the good ear Children with SSD report significantly more problems with regard to spatial hearing, speech in noise and some aspects of quality than their NH peers Intervention? If so, at what age?

11 Single Sided Deafness (SSD) Infants: Kishon-Rabin et al., 2015: delays in auditory behavior and preverbal vocalizations What happens early in life is important! Cochlear implant? Távora-Vieira & Rajan (2015) and Arndt & Prosse et al. (2015): 2 very young children with congenital SSD (17 and 20 months) no formal testing yet but the children appear to perform better but not for children with congenital hearing loss implanted at a later age Ruscetta et al., 2005; Priwin et al., 2007; Lieu et al., 2013, Reeder, Cadieux & Firszt, 2015 picture left:

12 A CI offers the potential to restore binaural hearing 13/16 Novel univ hospital Leuven (with partner univ Hospital Antwerp & St Augustinus Antwerp) 10 children with profound SSD, CI between 18 months and 24 yrs o o (Cochlear provides CIs, Research group ExpORL: researcher) 3 children implanted o age- and gender-matched control groups: control group 1: children with profound SSD not receiving a CI control group 1: normal hearing children Testing 1. pre-surgery CI surgery 1st fitting CI Testing 2. 3 months post 1st fitting Testing 3. 9 months post 1st fitting...

13 Protocol Tests language comprension language production words and sentences content of spontaneous speech cognition / IQ Questionnaires language development social-emotional behavior & adaptive behavior development of auditory behavior aspects of hearing disability in daily life 14/16 Environment language environment analysis Expectations: CI will promote (near-)normal auditory, linguistic and cognitive skills in the children with SSD and as a group, these children will outperform a matched group of children with profound SSD without a CI. picture:

14 Expressive Vocabulary First Results (S1) Language Comprehension Syntactic Skills Scores with 90% confidence interval (µ=100, sd=15) Thick line = child with SSD and CI Thin lines = NH control children = first fitting of the CI data points: before CI 3 months after first fitting 9 months after first fitting

15 First Results (S1, S2, S3) Auditory Development Cognition Scores with 90% confidence interval (µ=100, sd=15) Thick lines = children with SSD and CI Thin lines = NH control children = first fitting of the CI data points: before CI 3 months after first fitting 9 months after first fitting

16 General conclusions Language problems (and spatial problems) persist Audiological rehabilitation should target improving specific skills - morphology important for fluent reading (identifying meaninful chunks ), development of vocabulary: changing tense of a word, adding affixes, improve comprehension CI at young age should provide the necessary binaural hearing & improve speech in noise Reorganization of auditory brain areas and pathways Gordon et al., 2013 and 2015; Kral & Sharma, 2012 sensitive period for binaural auditory development: ~1.5 years To be continued!

17 Thank you for you attention! Acknowledgements FP7 ITN ICARE Concerns optimizing inclusion of children with a hearing impairment in an oral society through evidence-based research

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