Integrated Medical Care for Children who are Deaf/HOH

1. Integrated Medical Care for Children who are Deaf/HOH Quality Control Assessment Ellis Arjmand, MD, PhD Susan Wiley, MD Cincinnati Children’s Hospital Medical Center University of Cincinnati

2. Objectives • Present a rationale for interdisciplinary medical evaluations for children who are deaf/hard of hearing • Describe an integrated model of medical care for children who are deaf/hard of hearing • Present preliminary clinical outcomes seen in this setting

3. Premise Behind Clinic Model • Little consensus behind standardized medical evaluation (no evidence based guidelines) • Variable approach to work-up • Evaluation protocol often more standardized for children who are considering a cochlear implant • Etiology of hearing loss often unknown • Attempt to standardize care protocols • Provide quality of care to all children who are deaf/hoh

4. Pre-clinic Data • CCHMC otology and audiology serve approximately 150 newly identified children with hearing loss annually • This includes approximately 50 cochlear implant evaluations per year • Prior to the clinic model, many children had long waits for appointments with subspecialists such as ophthalmology, neurology, developmental pediatrics, genetics • Estimate of clinician time possible based on baseline data

5. Clinic Needs • Service coordination (scheduling) • Buy-in from specialists (ophthalmology, genetics, etc) • Standardized evaluation protocol • Comprehensive evaluations for all children irrespective of degree and type of hearing loss • Improved access for families/patients • Outcome data/QA • Support from institution (start-up costs)

6. Clinic Participants • Clinic coordination • One 0.5 FTE scheduler • Schedules appointments, collects reports from evaluations • Medical Sub-specialists • Pediatric Otologists • Genetics • Ophthalmology • Radiology/Laboratory Medicine • Developmental Pediatrics (as needed) • Neurology (as needed)

7. Clinic Participants • Allied Health Specialists • Pediatric Audiologists • Aural Rehabilitation Specialists • Speech/Language Pathologists • Social Work • Community Collaboration • Part C (Regional Infant Hearing Programs) • Part B (Local school programs)

8. Entry Criteria • Any child with a newly identified hearing loss irrespective of degree, side, or type of hearing loss • Any child with an identified hearing loss who needed collaborative approach to care (often at the discretion of ENT and Audiology)

9. Referral System • Referral by community physicians, audiologists, or otolaryngologists • Scheduler contacts families to confirm demographic information, contact information • Nurse intake by telephone • Pediatric otologist reviews intake information, develops treatment plan • Scheduler contact families to arrange appointments

10. Day of Appointments • Goal: Appointments range from occurring from a 1-2 day period to within a 2 week period • Results shared with families by specialists • Brief summary of findings from specialist faxed to managing otologist

11. Clinic Collaboration • Follow-up appointment with otologist after evaluations are complete • Summary letter sent to family, other providers, and referring physician • Format for discussion among providers at weekly team meetings

12. Clinic Data • Random selection of 100 children referred to the HDRC clinic between July 2005 and December 2006 • 18 were evaluated for a cochlear implant (CI) • 82 were referred for hearing loss in general • Of the 100, 10 did not complete the evaluation process (none were CI candidates)

13. Time to Completion of Appointments Among patients who completed the full evaluation process (n=90) *CI group calculated based on date of surgery, thus increasing the length of time through the process.

14. Time from Identification to Amplification Among those receiving amplification (n=60)

15. 100 children Mild to Moderate Hearing Loss N=38 Moderately Severe or Worse Hearing Loss N=41 Unilateral Hearing Loss N=21 Cochlear Implant Evaluation N=18 Left N=15 Right N=6 Clinic Data Median Age 5 ½ yrs at evaluation Range 1 mo – 16 yrs 94 SNHL 4 mixed 1 conductive 1 auditory neuropathy

16. Etiology of Hearing Loss

17. Etiology of Hearing Loss

18. Medical Evaluations Suggested in Literature • All children with hearing loss should see the following 3 medical specialists: • Pediatric Otolaryngology • Ophthalmology • Genetics

19. Pediatric Otolaryngology • 100% (by definition) saw a pediatric otolaryngologist • 76%with GJB2 testing (51/67 with indications) • Not indicated in 33 (unilateral, known syndrome, CMV, meningitis, CMV) • 51 subjects completed GJB2 testing • 6 positive (8% of those tested) • 3 indeterminate • CT results • 76 % completed • 42% of those tested had findings (32/76 patients)

20. Ophthalmology • 46% of entire group completed an eye exam • 54% of those with an eye exam (25/46 patients) had a significant finding • 18 with glasses • 7 with more significant vision problems • RP, coloboma, etc. • 47% of those with mild or unilateral HI completed an eye exam (28/59) • 53% (15/28) with findings

21. Genetics • 21 completed a genetics evaluation other than for genetic testing for hearing loss • 9 had a genetic syndrome, 1 was not the cause of the hearing loss. • Beckwith Wiedemann and connexin • CHARGE • CHARGE and XXY • Partial trisomy 3q and congenital CMV • Miller Syndrome • Campomelic dysplasia (2 sibs) • Usher Syndrome (2 patients)

22. Developmental Pediatrics • 58 patients completed a neurodevelopmental evaluation • 4 additional charts indicated issues, 2 did not keep appointment, 2 were not referred • 40 patients had findings which could impact education/development • 40% among entire population (n=100) • 69% among those referred and evaluated (n=58)

23. Developmental Pediatrics

24. Therapy Appointments • 52 patients completed a speech/language evaluation • 63% of those evaluated (33/52 patients) had an identified delay/issue requiring therapy • 42 patients completed an aural rehabilitation evaluation • 55% of those evaluated (23/42 patients) had an identified delay/issue requiring therapy

25. Unilateral/Mild HL 59 subjects had unilateral/mild hearing loss • 25 subjects (42%) saw DBP • 68% had findings (17/25) • 23 subjects (39%) saw SLP, 3 missing reports • 60% were suggested intervention (14/23) • 14 subjects (24%) saw AR, 1 missing report • 35% were suggested intervention (5/14)

26. Implications • Quality Improvement data to identify appropriate benchmarks or goals of care • Information to support the development of evidence-based clinical guidelines for the management of SNHL