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

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) PopulationTime Entire Group Median time (range) Mean time (SD) 2.8 mos (19 – 319 days) 3.6 mos (2.8 mos) CI group * Mean time (SD) 4 mos (3.2 mos) HI group Mean time (SD) 3.5 mos (2.8 mos) * 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) PopulationTime to amplification Entire Group Median (range) Mean (SD ) 2 mos (at diagnosis – 13 years) 1 year ( 2 years) CI group Median (range) Mean (SD) 1 month (at diagnosis - 4 mos) 1.2 month (1 month) HI group Median (range) Mean (SD) 2 mos (at diagnosis – 13 years) 1.2 years (2 years)

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

16. Etiology of Hearing Loss Genetic (31% of total group) Number% of genetic Presumed genetic (positive family history, GJB2 negative) 1238.7% GJB2 positive619.4% GJB2 indeterminate39.7% Pendrin positive13.2% Mitochondrial positive13.2% Genetic Syndrome (not Usher)619.4% Usher Syndrome26.5% 31100%

17. Etiology of Hearing Loss Etiology Number of total group (n=100) Unknown36 Structural Anomalies Large vestibular aqueduct10 Cochlear dysplasia9 Infectious Congenital CMV4 Meningitis1 Acquired Prematurity4 Ototoxicity (gentamycin, cisplatin)3 Trauma2

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 Type of ProblemNumber% Mild Motor Coordination (enrolled in OT or PT at some time ) 1424.1% Behavior1322.4% Cognitive712.1% Learning712.1% Cerebral Palsy58.6% Vision35.2% Sleep35.2% Oral motor apraxia35.2% Complex medical issues35.2% 58100%

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