Transcript Risk Factors For Permanent Hearing Loss

Risk Factors For Permanent
Hearing Loss
Betty Vohr, M.D.
Medical Director
Rhode Island Hearing Assessment Program
Professor of Pediatrics
Brown Medical School
Causes of Permanent Hearing
Loss in 100 Infants
50%
Environmental
50%
Genetic
50
30% syndromes (>300)
30
20% >75 genes ident
20
½ are GJB2 – Connexin 26
Genetic Causes
Single gene
Connexin 26
Gene + environment
Mitochondrial +
ototoxic
Gene + gene
Gene + other gene
Dilemma #1: Two Sets of Risk Factors
• Neonatal Risk Factors
• Late onset Risk Factors
JCIH Neonatal Risk Indicators for
neonates <28 d where there is
no universal screening
• Illness with admission to NICU > 48 hours
• Stigmata assoc with SNHL or Conductive HL
• Family hx of permanent childhood SNHL
• Craniofacial anomalies
• In-utero infections ie CMV, herpes,
toxoplasmosis or rubella
Risk Indicators for late onset HL
• Caregiver concern re: hearing, speech, language
• Family hx of permanent childhood HL
• Stigmata associated with SNHL or Conduct. HL
• Postnatal infections: ie meningitis
• In-utero infections ie CMV, herpes,
•
•
•
•
toxoplasmosis or rubella
Hyperbilirubinemia req Exchange, PPHN, ECMO
Syndromes assoc with SNHL
Neurodegenerative disorders
Head Trauma & persistent OM with effusion
Checklist of speech –language milestones
•
•
•
Birth to 3 months
Startles to loud noise
Awakens
Blinks or widens eyes in response to sound
3-4 months
Quiets to mother’s voice
Stops playing, listens to new sounds
Looks for source of sounds not in sight
6-9 months
Enjoys musical toys
Coos and gurgles with inflection
Says “mama”
Checklist of speech –language milestones
•
•
•
12-15 months
responds to his or her name and “no”
Follows simple requests
Uses expressive vocabulary: 3-5 words
Imitates sounds
18-24 months
knows body parts
20-50 words, 2 word phrases
50% of speech intelligible to strangers
36 months
approximately 500 words, 4-5 word sentences
80% intelligible to strangers: understands some verbs
JCIH Recommendation
• All children who pass the newborn screen
but have risk indicators for auditory
disorders or speech and language delay
should receive ongoing audiologic and
medical monitoring for HL and language
development for the first 3 years of life.
Dilemma #2: 2 sets of babies
• The NICU graduate- Considered high risk
for many ND sequelae. Many have routine
longitudinal surveillance for 1-3 years and
are less likely to fall through the cracks.
• The well baby nursery baby with a risk
factor. Monitoring considered less important
by family and primary provider and
therefore more likely to be lost to FU.
Questions ?
• How is tracking of risk factors accomplished ?
• Is it cost effective to track all children with a
risk factor ?
• Can it be accomplished on large populations ?
Longitudinal Assessment of Infants
who pass but have a risk factor
• Whose responsibility is it ?
The State EHDI system ?
The Medical Home ?
The Family ?
State EHDI Systems
• Tracking must be centralized
• There needs to be a centralized data
management system.
• We need to have quality indicators for
tracking infants with risk factors.
Data Management System Requirements
• Electronically store core demographic and
•
•
•
•
hearing screen & assessment data elements
Modifiable data model- protocols change
Multi-user ability to access & retrieve data
Protect and ensure security of confidential
health care information
Tracking and follow up capability
Benchmarking a Hearing Screen Program
Infants screened < 1 month
>99%
1st stage (TEOAE/AABR) fail rate
<4%
Rescreen return rate
>90%
Diagnostic procedures on refers
>90%
Referral age for intervention services
<6m
Number referred to EI
100%
Follow-up of Infants with Risk Factors
est 40%
Tracking for Risk Factors in
Rhode Island
• NICU – thorough chart review completed to
identify risk factors
• Well Baby Nursery – Risk factors identified
through notations in the child’s Kardex
entry and through nurse’s report
VRA Referral Process
Primarily Risk Factors
• After newborn screen
• Letter to parent and PCP
• Audiology Network list
• At age 5 months
• Reminder letter to parents
• At age 6 months
• Second letter to parents
Diagnostic Protocol
-six months corrected age
• Visual Reinforcement Audiometry
– insert earphones
– hidden, eye-level reinforcers
– 2-tester paradigm
• TEOAE or DPOAE
• Tympanometry/Acoustic Reflexes
Reporting of Dx Results
• Results requested for all RIHAP referrals
• To ensure all children receive follow-up
• Reporting is NOT mandatory
• Percentage Received
• VRA – 40%
Reporting of Dx Results
• Reports reviewed and coded by audiologist
when received
• Referral to Early Intervention if appropriate
• RIHAP as “safety net” for EI referrals
• Results entered into RITRACK by Data
Staff
RIHAP Referrals 2002
• 13,568 initial screens
• 232 (1.7%) did not pass the screen prior to
discharge
• 379 (2.8%) passed and were referred on for
monitoring due to risk factors
Determinants of successful Follow-up
• Medical Home awareness of risk factors,
speech and language milestones, and the
importance of follow-up.
• Family awareness of risk factors, speech and
language milestones and importance of
follow-up.
• A reliable tracking system
Unresolved issues
• What is the risk of late onset hearing loss
for the current individual risk factors ?
• Are there adequate resources for tracking all
children with risk factors ?