1. the ear group, Antwerp, Belgium The landmarks towards hearing Paul J Govaerts, MD, MS, PhD K Daemers, G De Ceulaer, M Yperman, C De Beukelaer the eargroup Antwerp-Deurne (Belgium)

2. the ear group, Antwerp, Belgium BELGIUM Flanders 60% of population approx 60.000 newborns Antwerp 1993 pilot study European concerted actions (Grandori) 1995 Well Baby Organization

3. the ear group, Antwerp, Belgium Flanders UNHS 2003 Flanders approx 60.000 newborns since 1999: universal screening (ALGO) coverage: 94-96% 5.7 % 0 retested >> 2 % 0 referred >> 0.9 % 0 of population with bilateral loss

4. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line Congenital SNHL the landmarks towards hearing …

5. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line  3 months: diagnostic work-up Congenital SNHL the landmarks towards hearing …

6. the ear group, Antwerp, Belgium Congenital SNHL Diagnostic work-up  Confirmation of hearing loss  Excluding other pathologies (syndromes)  Looking for the etiology

7. the ear group, Antwerp, Belgium Congenital SNHL Diagnostic work-up  Confirmation of hearing loss ABR under anaesthesia After paracenthesis With air and bone conduction  Excluding other pathologies (syndromes)  Looking for the etiology

8. the ear group, Antwerp, Belgium Congenital SNHL Diagnostic work-up  Confirmation of hearing loss ABR under anaesthesia After paracenthesis With air and bone conduction  Excluding other pathologies (syndromes) Clinical examination Ophtalmologic examination Ultrasound kidneys, urine ECG Chromosomal analysis  Looking for the etiology

9. the ear group, Antwerp, Belgium Congenital SNHL Diagnostic work-up  Confirmation of hearing loss ABR under anaesthesia After paracenthesis With air and bone conduction  Excluding other pathologies (syndromes) Clinical examination Ophtalmologic examination Ultrasound kidneys, urine ECG Chromosomal analysis  Looking for the etiology

10. the ear group, Antwerp, Belgium Congenital SNHL diagnostic work-up n 10 years ago: only diagnosis is 15% n Now: diagnosis in 75% Unknown25 % Genetic connexin-2625 % other10 % Labyrinthine malformation20 % Acquired20 % Blood exam ! MRI / CT scan !

11. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line  3 months: diagnostic work-up Congenital SNHL the landmarks towards hearing …

12. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line  3 months: diagnostic work-up  3-6 months: hearing aid fitting + rehab Congenital SNHL the landmarks towards hearing …

13. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line  3 months: diagnostic work-up  3-6 months: hearing aid fitting + rehab  9-12 months: evaluate hearing aids Congenital SNHL the landmarks towards hearing … Cochlear function - amplification (detection) - discrimination Audiometry ?? HA evaluation CI candidacy selection CI evaluation + programming

14. the ear group, Antwerp, Belgium *.wav files

15. the ear group, Antwerp, Belgium A§E Boy 11 months Connexin-26 deafness Hearing aids from age 3 months

16. the ear group, Antwerp, Belgium A§E Boy 11 months Connexin-26 deafness Hearing aids from age 3 months

17. the ear group, Antwerp, Belgium A§E CI @ 16 mos

18. the ear group, Antwerp, Belgium  1 month: screening + referral + expert counseling + setting out the time line  3 months: diagnostic work-up  3-6 months: hearing aid fitting + rehab  9-12 months: evaluate hearing aids Congenital SNHL the landmarks towards hearing … http:/www.eargroup.net Cochlear implant before 18 months Continu Hearing aids

19. the ear group, Antwerp, Belgium Age at implantation CAP: follow-up 2 years response to spoken language language discrimination telephone 22 1017 9 9 6

20. the ear group, Antwerp, Belgium Age at implantation babbling

21. the ear group, Antwerp, Belgium Receptive language development (Reynell) Courtesy of Leo De Raeve, KIDS, Hasselt, Belgium)

22. the ear group, Antwerp, Belgium Expressive language development (Reynell) Courtesy of Leo De Raeve, KIDS, Hasselt, Belgium)

23. the ear group, Antwerp, Belgium Age at implantation mainstream integration

24. the ear group, Antwerp, Belgium Conclusion the road towards hearing… http://eargroup.net Info@eargroup.net  1 month: screening  3 months: diagnostic work-up  < 6 months: hearing aid fitting + rehab  < 12 months: evaluate hearing aids  < 18 months: hearing restoration