Integrated Medical Care for Children who

are Deaf/HOH

Ellis Arjmand, MD, PhDSusan Wiley, MD

Cincinnati Children’s Hospital Medical CenterUniversity of Cincinnati

Quality Control Assessment

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

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

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

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)

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)

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)

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)

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

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

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

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)

Time to Completion of Appointments

Among patients who completed the full evaluation process (n=90)

Population Time

Entire GroupMedian 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 groupMean 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.

Time from Identification to Amplification

Among those receiving amplification (n=60)

Population Time to amplification

Entire GroupMedian (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 groupMedian (range)

Mean (SD)

2 mos (at diagnosis – 13 years)1.2 years (2 years)

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 SNHL4 mixed 1 conductive1 auditory neuropathy

Median Age 5 ½ yrs

at evaluation

Range 1 mo – 16 yrs

Etiology of Hearing LossGenetic(31% of total group)

Number % of genetic

Presumed genetic(positive family history, GJB2 negative)

12 38.7%

GJB2 positive 6 19.4%

GJB2 indeterminate 3 9.7%

Pendrin positive 1 3.2%

Mitochondrial positive 1 3.2%

Genetic Syndrome (not Usher) 6 19.4%

Usher Syndrome 2 6.5%

31 100%

Etiology of Hearing LossEtiology Number of total

group (n=100)Unknown 36

Structural Anomalies

Large vestibular aqueduct 10

Cochlear dysplasia 9

Infectious

Congenital CMV 4

Meningitis 1

Acquired

Prematurity 4

Ototoxicity (gentamycin, cisplatin)

3

Trauma 2

Medical Evaluations Suggested in Literature

• All children with hearing loss should see the following 3 medical specialists:

– Pediatric Otolaryngology– Ophthalmology– Genetics

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)

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

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)

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)

Developmental PediatricsType of Problem Number %

Mild Motor Coordination (enrolled in OT or PT at some time)

14 24.1%

Behavior 13 22.4%

Cognitive 7 12.1%

Learning 7 12.1%

Cerebral Palsy 5 8.6%

Vision 3 5.2%

Sleep 3 5.2%

Oral motor apraxia 3 5.2%

Complex medical issues 3 5.2%

58 100%

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

Unilateral/Mild HL59 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)

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