Download Presentation Title - Idaho Perinatal Project

Physician role in risk monitoring
for delayed onset and progressive
hearing loss
Dr. Jessica Stich-Hennen, Au.D., PASC
Doctor of Audiology
Specialty Certification in Pediatric Audiology
Elks Hearing & Balance Center - Boise
St. Luke’s Pediatric Otolaryngology - Boise
208-489-4999
[email protected]
Learner Goals
• Understand the importance of early detection and
intervention of childhood hearing loss
• Understand risk indicators for delayed onset hearing
loss in children
• Understand the pediatric audiologist role in newborn
hearing screening and risk indicator monitoring for
delayed onset hearing loss
• Understand the physician/medical home role in
newborn hearing screening and risk indicator
monitoring for delayed onset hearing loss
EARLY DETECTION & INTERVENTION OF
CHILDHOOD HEARING LOSS
Importance of Newborn Hearing Screening
• Hearing loss is invisible
• 3/1000 newborn infants are identified with permanent
hearing loss
– 1/1000 babies have profound hearing loss
– 2/1000 have lesser degrees of hearing loss
• If a child with hearing loss is identified early and given
appropriate services (educational, medical, and
audiological), over $400,000 can be saved in special
education costs.
Effects of Early Identification
 Intervention by 6 month old is key
 Children identified < 6 months develop significantly better language,
vocabulary and social skills (Yoshinaga-Itano)
 Lack of appropriate intervention results in the re-organization
of auditory tissue in the brain
 In the absence of sound, the brain begins to reorganize itself to
receive information from other senses (i.e. vision).
Idaho Sound Beginnings
• Hearing screening by 1 month old
• Audiological testing by 3 months old
• Early intervention services by 6 months old
Idaho Sound Beginnings
• Idaho is 1 of 7 states without legislation for newborn
hearing screening
• In 2012, 98.7% of newborns were screened in Idaho
– ~4% of infants referred to audiology
– 3/1000 infants diagnosed with hearing loss at birth
Idaho Sound Beginnings
• In 2007-2011, 3.5/10,000 infants were diagnosed
with a delayed onset hearing loss.
– 1/10,000 infants had no risk indicators
– 2.5/10,000 infants had risk indicators
– 75% of these infants had multiple risk indicators reported
RISK INDICATORS FOR DELAYED ONSET
HEARING LOSS IN CHILDREN
Joint Committee on Infant Hearing (JCIH)
Established in 1969
Comprised of:
• American Academy of Pediatrics
• American Academy of Ophthalmology and
Otolaryngology
• American Speech & Hearing Association
JCIH 1972 Position Statement
• High risk criteria
• Family history of childhood hearing loss
•
•
•
•
Intrauterine fetal infection (Rubella)
Defects of ear, nose or throat (atresia, cleft lip/palate)
Low birth weight (<1500 grams)
High bilirubin levels
JCIH 1982 Position Statement
–High risk criteria
»Bacterial meningitis, severe asphyxia (i.e. low APGAR)
were added
JCIH 1990 Position Statement
–High risk criteria additions:
»Ototoxic medications
»Prolonged mechanical ventilation
»Physical findings of syndromes
»Parent/caregiver concerns
»Head trauma
»Neurodegenerative disorders
»Infectious diseases associated with hearing loss
JCIH 1994 Position Statement
–Studies has shown that only 50% of all hearing loss
were being identified using the High Risk Register
JCIH 2007: Risk indicators for delayed onset hearing loss
 Caregiver concerns (re: hearing, speech, language, or developmental delay)
 Family history of permanent childhood hearing loss
 Neonatal Intensive Care (NICU) of more than 5 days or any of the following regardless of
length of stay: ECMO, assisted ventilation, exposure to ototoxic medications (gentimycin
and tobramycin) or loop diuretics (furosemide, Lasix), and hyperbilirubinemia that requires
exchange transfusion.
 In-utero infections
 Craniofacial anomalies
 Known physical findings associated with a syndrome
 Syndromes associated with hearing loss, progressive hearing loss or late-onset hearing loss
neurodegenerative disorders
 Culture-positive postnatal infections associated with hearing loss
 Head trauma, especially basal skull/temporal bone, requiring hospitalization
 Chemotherapy
Most frequently occurring risk factors
•
Ototoxic Medications (>70%)
•
Severe Asphyxia (>50%)
•
Mechanical Ventilation less than 5 days (>25%)
•
Low birth weight (>20%)
•
Parental/Physician concerns (>15%)
•
ECMO (>10%)
(Cone-Wesson, et al., 2000; Van Riper & Kileny, 2002, Hall, 2007)
Least frequently occurring risk factors (<10%)
•Hyperbilirubinemia
•Craniofacial anomalies
•Family history
•Congenital infections
•Bacterial meningitis
•Substance abuse (maternal)
•Neurodegenerative disorders
(Cone-Wesson, et al., 2000; Van Riper & Kileny, 2002, Hall, 2007)
Frequency of hearing loss among high risk indicators
•
Craniofacial anomalies (>50%)
•
ECMO treatments (>20%)
•
Severe Asphyxia/ Mechanical ventilation (>15%)
•
Congenital infections (>15%)
•
Family History (>15%)
•
Bacterial meningitis (>10%)
•
Other risk indicators (<10%)
(Cone-Wesson, et al., 2000; Fligor, 2008; Van Riper & Kileny, 2002, Hall,
2007)
Craniofacial anomalies
•
•
•
•
•
•
•
•
•
•
Head trauma
Recurrent OME
Cleft palate
Abnormal pinna
Abnormal ear canal
Ear tags and pits
Malformed eyes
Choanal atresia
Craniosynostosis
Hemifacial microsomia
Incidence of hearing loss in cleft palate patients
•
Children’s Hospital of Philadelphia (1972-1976)
• n = 70 (2 - 21 year olds) with cleft palate
• 50% conductive hearing loss (microtia, OME, tympanosclerosis, cerumen
impactions, external ear deformities )
•
Viswanathan et al (2008)
• n = 90 infants with cleft palate
• 82% (74) hearing loss (varying from mild to severe)
» 7 mixed hearing loss
» 1 unilateral SNHL
» 66 conductive hearing loss
• 18% (16) normal hearing
Idaho Cleft Palate & Craniofacial Deformities team
• Chart review (October 2007-February 2010)
• N = 210
– 104 (Normal hearing) = 50%
• At least 50% of these children have a history of OME and PE tubes
– 94 (Conductive hearing loss) = 45%
• 2 bilateral microtia
– 4 (Mixed hearing loss) = 1%
– 8 (Sensorineural hearing loss) = 4%
• 3 unilateral – profound left ear
• 5 bilateral
ECMO treatments
• Expracorporeal Membrane Oxygenation (ECMO)- is an
aggressive treatment that is used for the life support in infants
with respiratory or cardiopulmonary failure
(Fligor, 2008)
Mechanical ventilation
** Estimates 1/56 children with permanent hearing loss
at age 1, had the following risk factors:
Respiratory distress syndrome
Bronchiopulmonary dysplasia
Mechanical Ventlitation >36 days
Cone-Wesson et. al (2000)
Infections
Congenital Infections
• Cytomegalovirus (CMV)
• Herpes
• Rubella
• Syphilis
• Toxoplasmosis
Postnatal infections
• Bacterial or viral meningitis
Hearing Loss in Children with CMV
Congenital or Acquired
• 35-65% of symptomatic congenital CMV will
develop sensorineural hearing loss.
• 7-15% of asymptomatic congenital CMV will
develop sensorineural hearing loss.
• 33-50% of sensorineural hearing loss due to
congenital CMV will have delayed onset hearing
loss.
• 21% of all congenital hearing loss is CMV
• 25% of hearing loss by age 4 yrs, is likely related
to CMV
Hearing Loss in Children with CMV
Configuration and degree of hearing loss
– Unilateral or Bilateral
– Mild to profound degree of hearing loss
• Mild hearing loss at birth may progress to profound hearing loss
– Unpredictable configuration (i.e. rising, sloping, or flat)
Hearing Loss in Children with CMV
Stable or progressive
• 50% of children with sensorineural hearing loss related to
CMV will have progressive hearing loss.
• Recommendation- hearing evaluations at minimum every 6
months. Every 3 months during times when hearing is
changing.
Family History
• A family member with a congenital hearing loss congenital or hearing loss
acquired during childhood.
• Family history of hearing loss is the most common risk indicator found in
healthy newborns (Hall 2007).
Extended NICU stay
• National Perinatal Research Center (NPIC) (Quality Analytic Services
(QAS) ~ made the recommendation regarding NICU stay for JCIH 2007
– Approximately 25% of NICU infants are considered “LOW” risk and
discharged by 5 days old.
– The remaining approximately 75% of NICU infants, who are
hospitalized for greater than 5 days, are considered the “TARGET”
population to rule out neural hearing loss.
Other neonatal high risk indicators
• Low birth weight (<1,500 grams)
• Ototoxic drugs –recommends monitoring for any amount
» A1555G mutation
» Most common Aminoglycosides: Gentamycin, Tobramycin,
Viomycin, Vancomycin, Neomycin, Kanamycin, Amikacin,
Streptomycin
• Loop diuretics (furosemide/Lasix)
• Hyperbilirubinemia requiring transfusion
Syndromes associated with hearing loss
•
•
•
•
•
Waardenburg syndrome
• Congenital sensorineural hearing
loss
Branchio-Oto-Renal (BOR) syndrome
• Mild-to-profound conductive,
sensorineural or mixed hearing
loss
Stickler syndrome
• Hearing loss variable (sometimes
progressive)
CHARGE syndrome
• Ear anomalies and/or deafness
(mixed sensorineural and
conductive)
Neurofibromatosis Type II
• Hearing loss, tinnitus, balance
disorders
•
•
•
•
•
Downs syndrome
• Conductive hearing loss
Treacher Collins syndrome
• Conductive hearing loss
Usher syndrome
• Congenital bilateral mild-toprofound hearing loss or
progressive hearing loss
Pendred syndrome
• Congenital or late onset.
Hearing loss can be
progressive. Possible
vestibular dysfunction
Alport syndrome
• Hearing loss is never
congenital but can be
detected in late childhood or
early adulthood
Data collected by referral forms
Prevalence of risk indicators
Class A risk indicators (n= 153)
In utero & postnatal infections (n =18)
Syndromes (n=13)
Craniofacial anomalies (n=114)
Craniofacial anomalies with hearing loss
• n =12 (11%)
• 7 infants with cleft lip/palate
– 6 passed newborn hearing screening
– 1 referred newborn hearing screening
• 5 infants with tags, pits or microtia
PEDIATRIC AUDIOLOGIST ROLE IN
NEWBORN HEARING SCREENING AND
RISK INDICATOR MONITORING FOR
DELAYED ONSET HEARING LOSS
When a baby fails newborn hearing screening…
• “Comprehensive audiological evaluation of newborn and
young infants who fail newborn hearing screening should
be performed by audiologists experienced in pediatric
hearing assessment.”
• “A comprehensive assessment should be performed on
BOTH EARS even if only 1 ear failed the screening test.”
JCIH 2007 Position Statement
What about children who have risk indicators…
“Infants with risk factors for hearing loss should have
at least one diagnostic evaluation by 24-30 months
of age.”
JCIH 2007 Position Statement
Pediatric Audiology Assessment
(Birth to 6 months)
• Child and family history
• Frequency specific ABR using tone bursts
• Click evoked ABR testing using condensation and
rarefaction single-polarity stimulus
• Otoacoustic Emissions
• 1000 Hz tympanometry
• “Behavioral observation alone is not adequate in this
age group, and it is not adequate for the fitting of
amplification.”
Pediatric Audiology Assessment
(6 months and older)
•
•
•
•
•
•
•
•
Child and family history
Parental report of auditory and visual behaviors
Behavioral audiometry (VRA or CPA)
Speech audiometry (detection and recognition)
Otoacoustic Emissions
Tympanometry
Acoustic middle ear muscle reflexes (MEMR)
If behavioral testing is not reliable, ABR testing is
recommended
OAE vs. ABR
Otoacoustic Emissions
(OAE)
• The hair cells of the
cochlea response to
incoming sounds with
movement
• Part of ear tested:
Cochlea
Auditory Brainstem
Response (ABR)
•The electrodes measure
the neural synchrony of
the auditory nerve using
EEG activity in response
to sound.
•Parts of ear tested:
Cochlea, Auditory Nerve,
lower brainstem
If hearing loss is diagnosed…
• Child should be referred back to medical home for
recommendations, specialist referrals and additional
testing
– Urine CMV viral culture
• Child should return for audiological testing at
minimum every 3 months in the first year of life
• Family should be given appropriate intervention
options by Pediatric Audiologist based on the
family’s communication choice
PHYSICIAN ROLE IN NEWBORN
HEARING SCREENING AND RISK
INDICATOR MONITORING FOR
DELAYED ONSET HEARING LOSS
“Medical Home”
• Defined as “a philosophy of care that
emphasizes the role of the primary care
physician in the care of all children, including
children who have special needs.”
–
–
–
–
Primary medical care
Family support
Coordination of specialty medical care
Referrals for various services
2012 Idaho Physician Survey
regarding newborn hearing screening
36%
43%
33%
36%
60%
40%
81%
95%
86%
93%
67%
80%
%
100%
100
Which conditions are risk indicators
for delayed-onset hearing loss?
20%
0%
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Who to refer a child with confirmed
permanent hearing loss?
Specialist
2012
ENT/Otolaryngology*
89.5%
Geneticist*
Ophthalmologist*
Audiologist
0.0%
2.6%
60.5%
SLP
28.9%
Occupational Therapist
7.9%
Child Dev./ EI
15.8%
Neurologist
2.6%
Social Worker
0.0%
How confident are you talking to
parents about…..
Very
Confident
Somewhat
Confident
Not
Confident
Causes of HL
19.0%
73.8%
7.1%
Sign language, A/O modes
9.5%
35.7%
54.8%
Unilateral, Mild HL Consequences
14.3%
66.7%
19.0%
Bilateral Moderate- Profound HL
Consequences
9.5%
66.7%
23.8%
Candidates for cochlear implants
9.8%
26.8%
63.4%
What to do after diagnosis
38.1%
47.6%
14.3%
Role of the medical home
• Review results of newborn hearing screening (and risk
indicators for delayed onset hearing loss) for every child
• Encourage families to follow-up with pediatric audiologist for
diagnostic testing
• Provide families with specialist referrals and additional testing
– Urine CMV viral culture
Concluding points
• Importance of early detection and intervention of
childhood hearing loss
• Importance of audiological monitoring for children
with risk indicators for delayed onset hearing loss
• Understand the pediatric audiologist role in newborn
hearing screening and risk indicator monitoring for
delayed onset hearing loss
• Understand the physician role in newborn hearing
screening and risk indicator monitoring for delayed
onset hearing loss
References
• Cone-Wesson et. al. (2000). Identification of neonatal hearing impairment: Infants with
hearing impairment. Ear and Hearing, 21, 488-507.
• Fligor, B. (2008). Hearing outcomes in the most critically ill neonate population. Audiology
Today, 20 (5), 9-16.
• Hall (2007). New Handbook of Auditory Evoked Potentials.
• Hi-Track data from Idaho Sound Beginnings Program (2007-2011).
• Joint Committee on Infant Hearing (2007). Year 2007 Position Statement: Principles and
Guidelines for Early Hearing Detection and Invention Programs. Pediatrics, 120, 898-921.
• NCHAM eBook (2013). A Resource guide for Early Hearing Detection & Intervention. http:
//www.ncham.org
• Van Riper & Kileny (2002). ABR hearing screening for high-risk infants. Neonatal Intensive
Care, 15. 47-54.