Week 7.2 Pediatric Candidacy Flashcards
when did the FDA 1st approve CI’s for children
june 1990
AB audiometric threshold requirements for children
severe to profound bilateral sensorineural hearing loss (>70 dB HL)
AB auditory skills and speech recognition in younger children
<4 years of age: failure to reah developmentally appropriate auditory milestones (such as spontaneous response to name in quiet or to environmental sounds) measured using the infant-toddler meaningful auditory integration scale or meaningful auditory integration scale; or <20% correct on a simple open-set word recognition test (multisyllabic lexical neighborhood test (MLNT)) administered using monitored live voice (70 dB SPL)
AB auditory skills and speech recognition in older children
> 4 years of age: <12% on a difficult open-set word recognition (phonetically balanced kindergarten test) or <30% on an open-set sentences (hearing in noise test for children) administered using recorded materials in the sound field (70 dB SPL)
AB amplification requirements for children
use of appropriately fitted hearing aids for at least 6 months in children 2-17 years, or at least 3 months in children 12-23 months of age. The minimum duration of hearing aid use is waived in the presence of ossification concerns
cochlear audiometric threshold requirements in children
- 12-23 months: bilateral profound SNHL
* 24 months-18yrs: bilateral severe-to-profound SNHL
Cochlear auditory skills and speech recognition in younger children
lack of progress in the development of simple auditory skills as quantified an a measure such as the Meaningful Auditory Integration Scale or the Early Speech Perception test
cochlear auditory skills and speech recognition in older children
= 30% correct on MLNT or lexical neighborhood test (LNT), depending on the child’s cognitive and linguistic skills
cochlear amplification requirements for children
use of appropriate amplification and participation in intensive aural habilitation over a 3 to 6 month period
med-el audiometric threshold requirements
profound bilateral SNHL with thresholds of 90 dB HL or greater at 1000 Hz
med-el auditory skills and speech recognition in younger children
lack of progress in the development of simple auditory skills in conjunction with appropriate amplification
med-el auditory skills and speech recognition in older children
<20% correct on the MLNT or LNT depending on cognitive ability and linguistic skills
Mel-el amplification requirements for children
use of appropriate amplification and participation in intensive aural habilitation over a 3 to 6 month perios
*radiologic evidence of cochlear ossification may justify a shorter trial with amplification
when can you use conditioned play audiometry
24-30 months up to 6 yrs
CI criteria for children (audiometric thresholds)
- 12-24 months: bilateral profound SNHL
- > 24 months
- –cochlear= bilateral severe to profound SNHL
- –AB and Mel-el= bilateral profound SNHL
- can implant earlier then 12 months by decision of the CI team
different ways to assess hearing status of children
- objective testing
- –case history
- –tymps
- –acoustic reflex
- –OAE
- –ABR
- –EcochG (will tell you pre synaptic or post synaptic lesion)
- speech recognition testing
- accurate diagnosis (VRA or play audiometry is really helpful to know what is really going on, this is preferable)
when to use evoked potential testing for candadicy for children
- use on your itty bittys who cant give behavioral responses
* can also use to try and find the site of lesion
about what percent of children with HL have auditory neuropathy and what is the significance of this
- about 9% of kids with HL have AN
- this is important to know because it would affect the recommendations depending on type 1 or type 2
- –type 2 is more of damage to the synapse of the IHC
hearing aids eval in CI candidate for children
- testing conditions: the best-aided condition
- HA verification process
- –PMM or test box verification with pt specific real ear to coupler difference (RECD) corrections
- –DSL m[i/o] should be used
- –verify target at speech-input levels corresponsing to soft, average, and loud, such as 50, 60, and 70 dB SPL
speech recognition testing for children basic
materials and presentation level
- speech testing material
- –recorded is necessary for older children
- –some children only respond to MLV, but really need to do recorded
- —–use the multisyllabic lexical neighborhood test (MLNT) and monosyllabic lexical neighborhood test (LNT)- both are recorded
- presentation level
- –use 60 dB A
- amplification trial period
- –importance
- –6 months
- –shorter trial
speech recognition testing in younger children
- mainly looking at auditory skills development via questionnaires
- closed set tests are recommended for younger kiddos
- –Nu-chips
- –WIPI
- –CRISP Jr
NU-CHIPS
northwestern university children’s perception of speech
- picture booklet with 4 pics per page (25% chance)
- vocab level is 2.5-5 years
WIPI
word identification picture intelligibility
- picture booklet with 6 pictures per page (16.7% chance)
- vocab level is 3-5 years
CRISP Jr
children’s realistic intelligibility of speech perception (computer based)
- 16 words that are body parts or names of objects
- age group is 2.5-5 years
FDA speech recognition testing in older children in order of developmentally appropriate progression
- early speech perception (ESP) test
- multisyllabic lexical neighborhood test (MLNT)
- lexical neighborhood test (LNT)
- phonetically balanced kindergarten (PBK) word recognition test
- BabyBio sentences for children
- CRISP (childrens realistic intelligibility of speech perception (computer based)
ESP
early speech perception test
- closed-set (36 words, 3 lists)
- ages 2-6 years
MLNT
multisyllabic lexical neighborhood test
- open-set, 2 lists of 12 lexically easy and 2 lists of 12 hard mutisyllabic words (level 3-5 years)
- words and phonemes correct
LNT
lexical neighborhood test
*2 lists of 25 lexically easy and 2 lists of 25 hard monosyllabic words (level 3-5 years)
PBK test
phonetically balance kindergarten word recognition test
- minimum 4 years
- ope-set, 50 words-phonemes and word correct
BabyBio sentences for children
- at least 5 years old
* 16 lists, each has 20 lists of 3-12 words
CRISP
children’s realistic intelligibility of speech perception (computer based)
- 25 spondees
- age group is 4-7 years
FDA labeled ped CI candidacy for speech for AB
- young=<20% on MLNT
* older= <12% on open set (PBK) or <30% on HINT-C
FDA labeled ped CI candidacy for speech for cochlear
- young= lack of progress
* older=<30% on MLNT or LNT
FDA labeled ped CI candidacy for speech for med-el
- young= lack of progress
* older=<20% on MLNT or LNT
evaluating progress of auditory skill for kids 0-3 years
- looking to see auditory skills and progress during the HA trial
- must be done via parental history and validated questionnaires:
- –infant-toddler version of the Meaningful Auditory Integration Scale (IT-MAiS)
- —–0-3 yrs
- —–10 item parental interview
- —–open-ended questions with scores ranging from 0 (never) to 4 (always)
- –the LittlEars Questionnaire
- —–0-24 months in normals (or up to 24 months following implantation)
- —–35 yes/no questions
- —–questions are hierarchical, so ceiling at 6 consecutive no answers
- –the auditory skills checklist (ASC)
- ——0-3 yrs
- —–parental interview style with 35 items assessing detection, discrimination, identification, and comprehension
- —–soreing 0= child does not have the skill; 1= child has skill developing; 2= child demonstrated the skill
- –functioning after pediatric cochlear implantation (FAPCI) questionnaire
- —–2-5 yrs
- —–23 items does not require parental interview
- —–provides baseline (pre-op)
- –functional auditory performance indicators (FAPI)
- —–assess sound awareness, meaningful sounds, auditory feedback,sound localization, discrimination, short-term auditory memory and linguistic auditory processing
- —–scoring: emerging (0-35%); in process (36-79%); acquired (80-100%)
- –3mo until all acquired
- –early language milestone scale (ELM)
- —–0-3 yrs
- —–43 items designed to assess language development (expressive, receptive,a and visual language–parental interview
evaluating progress of auditory skills for preschool to school-age children
- common questionnaires
- –the meaningful auditory integration scale (MAIS)
- —–3-5 yrs
- —–10 item parental interview style questionnaire
- –the parents’ evaluation of aural/oral performance in children (PEACH)
- —–3-7 yrs
- —–13 items designed for parental completion
- —–parent reflect on the child’s listening behavior over the previous week and assigns a numerical value ranging from 0 (never) to 4 (always)
SLP’s role in CI
- critical role in CI candidate selection
* at least SLP eval during candidacy selection process should take place during the HA trial (after verification)
social worker and physiologist involvement in CI candidate selection
- often helpful for families struggling with the diagnosis
- help to determine family dynamics and level of dedication to the recommended post-op therapy schedule
- social workers can provide counseling and support in financial options
medical and surgical issues with ped CI candidacy
- medical team ensures that candidates are current on recommended immunizations
- a pre-op medical eval anesthesia) and other medical specialties as needed
- imaging: CT and MRI
vestibular considerations for ped CI candidacy
- vestib impairment is the single most common associated feature of SNHL
- –many pts with HL experience dizziness and/or imbalance
- balance system: detection and integration from the visual, proprioceptive, and vestib system
- –vestib system is at the biggest risk during cochlear implantation
- 5 vestibular end organs within each ear:
- –3 SSC that detect and encode angular accelerations
- —–info detected here triggers the vestibulo-ocular reflex, leading to maintenance of stable vision during movement
- —–2 remaining= otolith (utricle and saccule)- linear movements such as gravity and head tilts
- ———important for postural stability through vestibulo-colic and vestibulo-spinal reflexes
- ———important for gross motor skill development (learning to sit, stand, and walk)
vestibular function and HL
- 72% of children and adults with significant SNHL have some level of vestib dysfunction-type depends on underlying inner ear pathology
- –for cases evaled for CI, up to 50% have SCC abnormalities, and up to 44% have abnormal otolith function
- genetic:
- –Usher syndrome (Type I)
- –pendred syndrome
- –waardensburg syndrome
- –cogan syndrome
- –brachio-oto-renal syndrome
- –CHARGE
- –connexin 26
- –inner ear malformations
- –enlarged vestibular aqueduct
- –charcot-marie-tooth disease
- Acquired:
- –prematurity
- –anoxia
- –fetal alcohol syndrome
- –meningitis
- –CMV
- –ototoxicity
- –aging
- –menieres
- –labyrinthitis
- –autoimmune inner ear disease
- –trauma
pediatric vestibular considerations
- children with severe to profound HL are likely to demonstrate additional vestibular impairment
- children with congenital SNHL demonstrate semicircular canal (41%) and otolith (42%) dysfunction
- vestibular function in children is commonly overloooked. but congenital and early-onset vestib dysfunction may lead to significant delays in motor skill development
- developmental milestones (upper limit)
- –response to tilt (>36 months)-should right within seconds
- –head control- 4 months
- –sitting unsupported-9 months
- –walk 18 months
- motor function (standing on one leg)
- –2.5 yrs= 1 sec
- –3 yrs= 2 sec
- –4 yrs=5 sec
- –>/= 5 yrs= 8 sec (eyes open), 4 sec (eyes closed)
when to refer kid for vestib eval
- delayed motor development
- delayed walking
- loss of postural control= falls
- episodes where pallor/vomiting
- investigating etiology of HL +/- balance symptoms
barriers to ped CI
- lack of pt awareness
- finances
- –SEC
- –insurance
- lack of professional knowledge
- lack of referrals
infants under 12 months and CI
- FDA candidacy is 12 months
- no greater anesthetic risks for infants <12 months
- surgical issues:
- –intraoperative blood loss
- –facial nerve damage
- –skull thickness
- –fixation of the internal device
- –thin skin flap
- –skull growth causing migration
SSD and amplification options
- CROS
- osseointegrated bone conduction hearing device
- remote mic system
- CI
- –improve speech in quiet and noise
- –better spatial hearing
- –tinnitus suppression in unilateral hearing loss
SSD in peds
- cochlear nerve deficiency: as many as 50% of children with SSD have cochlear nerve deficiency- CRUCIAL to get an MRI
- –likely to reject CI
- CMV-most common environmental cause of genetic HL
- –some children with SSD due to CMV will make limited progress with implant due to cognitive/neural deficits associated with CMV
- bacterial meningitis-if ossification has occurred, unlikely that a CI will be possible
- enlarged vestibular aqueduct- relatively common cause of SSD; highly successful with Ci if duration of deafness prior to implantation is short
SSD in adults
- menieres
- -CI with simultaneous labyrinthectomy (if vertigo) is generally successful
- more successful if implanted after a short period of deafness (shorter the better)
- adults should be highly motivated to be implanted
children with global developmental delay and CI
- no professional consensus on whether children with compromised cognition/severe global developmental delay should be implanted
- difficulty in obtaining reliable behavioral estimated of hearing
- expectations need to be managed with counseling
neurofibromatosis Type 2
and CI
- autosomal dominant disorder
- outcomes from cochlear implantation is variable but generally better than patients with an ABI
- patient counseling
- ***i think this really meant they do better with ABI
