Transcript Identification of Late

Identification of
Late-Onset Hearing
Loss
As a supplement to Universal
Newborn Hearing Screening
Programs.
PRESENTERS
Yusnita Weirather – Kapiolani Medical
Center, Hawaii
Karl White – Utah State University.
Child A: DOB: 05-22-1998
Age of identification: 5 years and 4 months
Failed behavioral hearing screening at pediatrician’s
office during routine physical
Passed newborn hearing screening with OAE
Hospitalized for 3 days within the 1st year of life due
to fever with unknown diagnosis.
No reported history of otitis media.
Right ear: normal hearing thresholds
Left ear: moderate sloping to mild sensorineural
hearing loss
Audiological findings
Late onset vs Progressive
Hearing Loss
LATE ONSET HL
Onset time: after birth
PROGRESSIVE HL
Onset time: at birth or
after birth
Indicators of progressive hearing loss :
Delayed or abnormality in motoric development
unilateral and asymmetric hearing loss
Children with dizziness or vestibular problem
Changes in auditory behaviors
Late onset hearing loss
Defining feature: Normal hearing at birth
Onset time: several days or months after
birth or later in life
Screening for late onset loss particularly
important before age 5
Lack of clarity of infant’s
hearing at birth
Approximately 5-20 % of infants screened do not
complete the screening process every year.
Some NICU children screen weeks after birth.
Some parents or physicians do not have access
to the results if the infant passes screening.
An electronic statewide newborn hearing
screening database is not available in all states.
Etiology of Hearing Loss
Genetic
Environmental
Uncertain
Child B
Age of identification: 1 year chronological
age
Failed hearing screening at one year
chronological age
Delayed screening was because of her
medical condition and life support
equipment
Mother reported that her child gradually
became unresponsive to her voice.
24 weeks premature, 923 grams
Audiological and otological
findings
Normal CT scan findings of temporal bone
structure
Normal middle ear functions
Absent OAEs and ABRs to click and tone
stimuli.
Vibrotactile bone conduction responses to
250 and 500 Hz at 60 dBHL
Pre-lingual vs post-lingual
hearing loss
Easier detection by parents or caregivers if
post-lingual
Less effect in post-lingual children
Easier to fit hearing aids for post-lingual
children.
Late Onset Hearing Loss
 What do we know about late-onset hearing
loss (LOHL) ?
 How are states implementing surveillance?
 What are obstacles / challenges in
implementing surveillance?
 Considerations for implementing
surveillance
“JCIH 2000” indicators for late
onset or progressive HL
Family history
Parental concern
In utero and postnatal infections
Physical findings: syndromal stigmata,
craniofacial anomalies
Neurodegenerative disorders
Temporal bone trauma
Neonatal indicators (continued)
“JCIH 2000” indicators for late
onset or progressive HL
continued ………….
Neonatal indicators—hyperbilirubinemia at a serum
level requiring exchange transfusion, persistent pulmonary
hypertension associated with mechanical ventilation, and
conditions requiring the use of extracorporeal membrane
oxygenation (ECMO).
% of children with hearing loss
who have the following risk factors
12
11.7
Syndromes
10
8
Family history
6.6
5.5
6
Meningitis
4.7
4
Craniofacial
1.5
2
Antibiotics
0
% of cases
Cone-Wesson et al. (2000) taken from Fortnum & Davis, 1997 and
the NIH multicenter study (Norton et al., 2000)
Reported Etiology of HL by onset, for the
estimated population of Deaf and HH students
in The US, 1992-1993 (N= 48,300)
CMV
At
birth
After
birth
Rubella
Hereditary
Birth
complication
Other
causes
Unknown
1.3
2.1
13
8.7
4.5
OM
Trauma
Infection
Meningitis
Other Uncauses known
3.7
0.6
4
8.1
1.5
Onset not known: 29.4%
1992-1993 Annual survey of hearing impaired children and youth, Center for Assessment and
Demographic Studies, Gallaudet University.
17.8
5.3
Gallaudet Research Institute Reported
Etiology of HL, Jan 2003 (N= 42,361)



Genetic / Hereditary / Familial
Cause can not be determined / DNA
Pregnancy related






Maternal rubella
CMV
Prematurity
Trauma at birth / complications
Other causes
20.8 %
52.4 %
0.8 %
1.8 %
4.2 %
5.3 %
1.3 %
Post Birth Disease / injury






OM
Meningitis
Other infections
Medications
Trauma
Other
5.6 %
4.7 %
1.7 %
1.3 %
0.9 %
3.3 %
Regional and National Summary Report of Data from 2001-2002 Annual survey of Deaf and Hard of
Hearing Children and Youth. Washington, DC: GRI, Gallaudet University
Examples how states are
implementing surveillance for LOHL
Provide parents with a list of risk indicators
Send NHS results to the child’s physician
“Tickler file” to call back children with risk
indicators
Hearing screening for children in Head Start,
Part C, and Preschool programs.
Hearing screenings by physicians as part of
children’s annual physicals.
Obstacles / challenges in
implementing surveillance for LOHL
NHS is still voluntary in some states / hospitals.
Some risk indicators are difficult to assess (hypoxia).
Some etiologies are unknown or difficult to access.
Lack of familiarity with auditory development and
auditory behaviors
Comprehensive electronic population data
management is unavailable.
Inconsistency exists in the schedule and protocol for
audiological follow up across the country.
Child C: DOB: 03-09-1998
Age of identification: 7 months at first follow up
requested by hospital hearing screening
program due to PPHN
Passed newborn hearing screening with AABR
Medical history: 37 ½ weeks gestational age,
meconium aspiration, maternal history of
substance abuse, amp/gent/lasix, PPHN,
hydronephrosis, mild chronic lung disease.
Audiological findings: Bilateral moderate
sensorineural hearing loss
Child D: DOB: 08-19-2000
 Age of identification: 3 years of age.
 Passed newborn hearing screening with
AABR.
 Medical history: Meconium aspiration
and PPHN requiring treatment with
ECMO
Audiological findings
 Normal middle ear functions
 Absent DPOAE in both ears
 No response to click and tone air conduction
ABR at 90 dBnHL and no response to bone
conduction ABR at 65 dBnHL.
Possible referral sources
for detecting LOHL
 NHS program
 Parents
 Physician
 School
 Other health professional
Considerations for implementing
surveillance
Parental education through NHS
Educate medical home providers about risk
indicators
Education in auditory development and auditory
behaviors to health care providers, day care
providers, and preschool teachers
Complete recording of medical history
Appropriate protocol for audiological follow up
based on the disorder
Create a LOHL Registry
Parental education
during NHS
More than just leaving a
brochure in the infant’s crib
Include newborn hearing
screening results in the
immunization record
Developmental examples on
the importance of good
hearing
The effect of hearing loss
can be minimized or
managed
Education for health care
providers, day care providers, and
preschool teachers
Include in the overall developmental
milestones
Include in the activities to stimulate
developmental skill
Perform hearing screening as part of
physical check up
Complete recording of
medical history
 Pre and postnatal history
 Primary and secondary diagnosis
 Medical management
 Screening results
 Family history
Obstacles to follow up
after NHS
Medical chart review - Finding risk
indicators
Contacting the family
Performing audiological evaluations
for children under 3 years of age
Financial consequences
Family compliance
JCIH Recommendation for
Detecting LOHL
Any infant with [the following] risk indicators for progressive or
delayed-onset hearing loss who has passed the birth screen should,
receive audiologic monitoring every 6 months until 3 years.
(a) Parental or caregiver concern.
(b) Family history of permanent childhood hearing loss
(c) Stigmata associated with a syndrome known to include a SNHL or conductive hearing loss
(d) Postnatal infections (e.g., bacterial meningitis)
(e) In-utero infections (e.g., CMV, herpes, rubella, syphilis, and toxoplasmosis.
(f)
Neonatal indicators -- especially hyperbilirubinemia, PPHN, ECMO
(g) Syndromes associated with progressive hearing loss
(h) Neurodegenerative disorders sensory motor neuropathies (e.g., Hunter syndrome,
Friedreich’s ataxia, Charcot-Marie-Tooth syndrome)
(i)
Head trauma
(j)
Recurrent or persistent otitis media with effusion for at least 3 months
(See Section E of the JCIH Year 2000 Statement
Logistical Considerations for
Implementing JCIH Recommendation
 Assume 8% of all children passing newborn hearing screening have a risk
indicator
 4 million births per year x .08 = 320,000 babies with risk indicators born each year
 Assessing each baby with a risk indicator every 6 months until 3 years of age
would require:

320,000 babies x 6 assessments;

or 1,920,000 assessments each year
 Currently, about 1% of all births (40,000) are referred from NBHS programs for
audiological assessment each year

The most serious concern of State EDHI coordinators is the lack of experienced pediatric
audiologists

Audiologic monitoring may not require a full diagnostic assessment
Expected “Yield” of Regular Monitoring of
0-3 Year- old Children for LOHL
 Not enough data for a definitive estimate
 University of Washington Study

4,911 children (mostly NICU) were screened at birth and asked to return
for VRA at 8-12 months of age regardless of newborn hearing screening
result (~65% had useable VRA results)

56 children with permanent hearing loss were identified

Investigators concluded only 1 of these children had late onset loss
 Anecdotal evidence

States with long-established EHDI programs (CO and RI) report about 5%
of children with hearing loss are late onset
Late onset or Progressive
Hearing Loss?
 Although the terms are often used interchangeably, they
should not be
Congenital
Late-onset
Progressive
Stable
 From University of Washington study

1 of 56 children (1.79%) was late onset

7 of 56 children (12.50%) were progressive
Hawaii Data
1998
# of birth
1999
2000
2001
11,997 15,009 14,989 14,979
# need f/u
154
326
159
124
# received f/u
59
71
51
44
7
5
2
4
62
59
65
64
# confirmed
# confirmed loss
by NHS
Risk indicators among LOHL
children in Hawaii
 2 with Family History of hearing loss
 2 with Parental concern
 2 with in utero and postnatal infections
 1 with syndromal stigmata
 9 with Neonatal indicators ( ECMO=1, PPHN=3,
hyperbilirubinemia=3, mechanical ventilation=2)
 1 with Ototoxic medication: 1
Hawaii Data (continued)
5
4.5
4
3.5
3
2.5
2
1.5
1
0.5
0
5
3
2
2
2
2
2
0
1998
1999
2000
2001
Unilateral
Bilateral
Take-Home Messages
 Late-onset and progressive hearing loss should
not be used interchangeably
 More data is needed about the incidence of both
late-onset and progressive hearing loss
 Following all babies with risk-indicators who
pass the newborn hearing screening test would
be very expensive and logistically difficult
 Evaluations of alternative approaches needed
(e.g., screening in the medical home and/or early childhood
programs, parent education, ????)