Identification of Late-Onset Hearing Loss

1
Identification of Late-Onset Hearing Loss

• As a supplement to Universal Newborn Hearing
  Screening Programs.

2
PRESENTERS

• Yusnita Weirather Kapiolani Medical
  Center, Hawaii
• Karl White Utah State University.

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Child A DOB 05-22-1998

• Age of identification 5 years and 4 months
• Failed behavioral hearing screening at
  pediatricians 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

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Audiological findings
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Late onset vs Progressive Hearing Loss

• PROGRESSIVE HL
• Onset time at birth or after birth

• LATE ONSET HL
• Onset time 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

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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

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Lack of clarity of infants 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.

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Etiology of Hearing Loss
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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

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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

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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.

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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

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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)

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JCIH 2000 indicators for late onset or
progressive HLcontinued .

• Neonatal indicatorshyperbilirubinemia at a
  serum level requiring exchange transfusion,
  persistent pulmonary hypertension associated with
  mechanical ventilation, and conditions requiring
  the use of extracorporeal membrane oxygenation
  (ECMO).

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of children with hearing loss who have the
following risk factors
Cone-Wesson et al. (2000) taken from Fortnum
Davis, 1997 and the NIH multicenter study (Norton
et al., 2000)
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Reported Etiology of HL by onset, for the
estimated population of Deaf and HH students in
The US, 1992-1993 (N 48,300)
CMV Rubella Hereditary Birth complica-tion Other causes Un- known
At birth 1.3 2.1 13 8.7 4.5 17.8
OM Trauma Infection Meningitis Other causes Un- known
After birth 3.7 0.6 4 8.1 1.5 5.3
Onset not known 29.4 Onset not known 29.4 Onset not known 29.4 Onset not known 29.4 Onset not known 29.4 Onset not known 29.4 Onset not known 29.4
1992-1993 Annual survey of hearing impaired
children and youth, Center for Assessment and
Demographic Studies, Gallaudet University.
17
Gallaudet Research Institute Reported Etiology of
HL, Jan 2003 (N 42,361)

• Genetic / Hereditary / Familial 20.8
• Cause can not be determined / DNA 52.4
• Pregnancy related
• Maternal rubella 0.8
• CMV 1.8
• Prematurity 4.2
• Trauma at birth / complications 5.3
• Other causes 1.3
• Post Birth Disease / injury
• OM 5.6
• Meningitis 4.7
• Other infections 1.7
• Medications 1.3
• Trauma 0.9
• Other 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
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Examples how states are implementing
surveillance for LOHL

• Provide parents with a list of risk indicators
• Send NHS results to the childs 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
  childrens annual physicals.

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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.

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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

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Child C
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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

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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.

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Possible referral sources for detecting LOHL

• NHS program
• Parents
• Physician
• School
• Other health professional

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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

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Parental education during NHS

• More than just leaving a brochure in the infants
  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

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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

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Complete recording of medical history

• Pre and postnatal history
• Primary and secondary diagnosis
• Medical management
• Screening results
• Family history

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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

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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,
Friedreichs 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
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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

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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

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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

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Hawaii Data
1998 1999 2000 2001
of birth 11,997 15,009 14,989 14,979
need f/u 154 326 159 124
received f/u 59 71 51 44
confirmed 7 5 2 4
confirmed loss by NHS 62 59 65 64
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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 ( ECMO1, PPHN3,
  hyperbilirubinemia3, mechanical ventilation2)
• 1 with Ototoxic medication 1

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Hawaii Data (continued)
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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, ????)