Download Pediatric Considerations in Cochlear Implantation R. Christopher

ENT Updates for the General
Pediatric Office
R. Christopher Miyamoto, M.D., FACS, FAAP
Pediatric Otolaryngology
Peyton Manning Children’s Hospital
at St. Vincent’s
Intro
 Provide update on interesting ENT
developments for primary care Pediatric
offices
 Excellent New Text/Resource:
 Pediatric Otolaryngology, Schoem SR,
Darrow DH ed. AAP 2012
 Disclosure: I have no financial
interest/relationships with any
biomedical companies, etc.
Congenital Hearing Loss
significance:
 Hearing impairment one of the most common sensory
deficits in children & significant healthcare problem
 1 - 2 infants per 1000 births have significant hearing
loss [HL]
 Bilateral severe-profound
 Up to 4/1000 births if mild-moderate or unilateral HL
included [compare to signif congenital heart disease
4/1000, cleft lip 1/1000, cleft palate 1/2500]
 Steel KP Science 1998;279:1870-71
 = 40,000 infants born/year w/ significant HL;
 = 4000 profoundly deaf
Developments
 1) Universal Newborn Hearing Screening
 Healthcare providers must now screen & manage HL in infants—
substantial pt population— Goal 1 mos
 Joint Committee on Infant Hearing and AAP:endorsed UNHS
revised guidelines 2000, Position update 2007
 Confirm HL by 3 mos
 Logistical issues:
 Otolaryngology referral – time
 Ability of otolaryngologist to confirm:
 Middle ear fluid, audiological capabilities [OAE, ABR]
 Intraoperative vs office sedation ABR capabilities
 Intervention by 6 mos
 Increased receptive/expressive language quotients
 EHDI / ISDH Physician tool kit
Developments
 2) Molecular/genetic understanding of
hereditary hearing loss vastly enhanced
over last 10 years
 Genetic testing now integral for evaluation of
hearing impairment in children
 Expertise of Medical Genetic specialists
invaluable
 3) Early intervention [medical vs surgical]
now standard of care
Importance?
 Significance:
 COST EFFECTIVE, POSITIVE QUALITY
OUTCOME
 Early hearing diagnosis= early intervention
at 6mos or earlier
 = better speech/language development,
school performance, economic outcome
 = early identification of profound hearing loss
requiring cochlear implantation
 NEJM 2008---Cochlear implantation one of
few truly cost effective interventions
Hearing Screening
 Per UNHS, must occur prior to d/c
 Automated auditory brainstem response
[ABR] preferred
 +/- otoacoustic emissions [OAE]
 OAE alone can miss auditory neuropathy
 Failure [PC = “refer”] requires diagnostic
audiology eval as outpt
 = ABR, tympanograms, OAE
Hearing Loss Breakdown
 Historically, infectious disorders [TORCH, meningitis],
teratogens, ototoxic meds were primary causes of
congenital & acquired HL
 Vaccines, abx, awareness of teratogens changed ddx
 Hereditary causes account for 50% childhood deafness
 Morton NE Ann NY Acad Sci 1991;630:16-31
 Over 150 loci [areas on genes] identified
 70% hereditary hearing loss nonsyndromic
 75% of this autosomal recessive
 Important for evaluation process
 Autosomal recessive hearing loss locus DFNB1
found on Chromosome 13q—contains GJB2 gene
 Mutations in GJB2 responsible for up to 50% severeprofound SNHL in autosomal recessive nonsyndromic
HL in US & Europe
Hearing Loss Breakdown
 The Rest:
 50% hearing loss NOT inherited
 Acquired—ototoxics, risk factors, others
 May have Genetic comp making susceptible
 30% Syndromic
 823 syndromes linked to hearing loss
 150 gene loci linked to hearing loss
 Importance of Medical Genetics Evaluation
 JCIH 2007 statement guidelines
GJB2
 Gene in DFNB1 locus on chromosome 13
 GJB2 gene codes for connexin 26 protein
 Membrane proteins that form gap junctions
 Seem important in electrolyte, second messenger and
metabolite exchange in cochlea
 Multiple mutations [60+ and counting] described
 35delG mutation especially common [15-40%]
[white/European descent, some Hispanic, Asian,
african-american]—Connexin 26
 Many other genes involved with congenital
hearing loss– can screen with Chip technology
17-19 gene mutations screened
Hereditary Syndromic Hearing Loss
 Most are congenital and some acquired
 400 - 800+ syndromes associated w/HL
 Cause of HL: sensorineural, conductive,
mixed
 Craniofacial & other features associated
 Findings may be subtle
 Medical genetics evaluation helpful with
subtle phenotypes
Nonhereditary Congenital Hearing Loss
 TORCH:
 Toxoplasmosis, Rubella, CMV, Herpes
 Syphilis
 CMV: most frequent cause nonhereditary HL in
neonates
 40,000 CMV infected infants/yr; 4000 HL
 8-10% CMV-infected infants asx at birth can develop
HL---need long-term f/u
 Cytomegalovirus [CMV]: 0.5 -2% live births
 Congenital CMV infection:
 10% Symptomatic: 44% have HL by age 3yrs
 21%are delayed onset
 90% Asymptomatic: 7.4% have HL by 3yrs
 33% delayed onset
 50% of both groups w/progressive loss
Evaluation of Hearing Loss in
Infants & Young Children
 Thorough History/physical exam
 Directed toward issues discussed earlier
 Syndromic features—refer to Med Genetics
 Prenatal, perinatal,postnatal events

AAP JCIH 2007 Risk Indicators
(congenital, delayed onset or progressive hearing loss





Prematurity
Teratogens
Perinatal maternal infections: TORCH
Low birth wgt <1500 g
Prolonged mechanical ventilation, hyperbilirubinemia,
hypoxia
Evaluation of Hearing Loss in
Infants & Young Children
 Prenatal, perinatal,postnatal events
 NICU graduates
 Persistent pulmonary hypertension of the
newborn
 Extracorporeal membrane oxygenation
[ECMO]: [diaphragmatic hernia, heart
disease, etc]
 20-25% with late-onset or progressive HL
 = ABR at 6mos, audiogram 1 yr and annual
x3yrs
Evaluation of Hearing Loss in
Infants & Young Children
 Infections—neonatal meningitis
 Syncope [fainting]—Jervell & Lange-Nielsen
 Family members with syncopal or sudden
cardiac death in teens, early adult years
 Delayed walking/visual issues—Usher
 Family history
Evaluation of Hearing Loss in
Infants & Young Children
 Physical Exam:
 Check for craniofacial issues, subtle ear
deformities
 Check for ocular abnormalities: coloboma,
hypertelorism, other abnormalities
 Up to 50% severe-profound hearing impaired kids
have eye issues
 Armitage IM et al. Arch of Dis Childhood 1995;73(1);53
 Pediatric Ophthalmology evaluation for all hearing
impaired children recommended
 JCIH 2007 statement
Evaluation of Hearing Loss in
Infants & Young Children
 Confirm with OAE / ABR
 OAE alone can miss auditory neuropathy
 If bilateral SNHL and diagnosis not
apparent [identifiable syndrome,
meningitis, autosomal dominant
SNHL,trauma] genetic testing for
hearing loss/genetic evaluation
Auditory Neuropathy
 Pathophysiology
 Hair cell death?
 Hypoxia
 Temporal bone studies
 Genetics: Otoferlin gene
Cochlear Implantation
 Currently the standard surgical treatment for
patients with hearing impairment & well-fit
hearing aids that fail to permit effective oral
communication
 Designed to help the severe-profoundly deaf
patient
 Perceive environmental sounds
 Understand speech
 Maximal benefit depends on patient and rehab
How it works: neural stimulation
 Implant with electrode
array placed
surgically in the
lumen of the cochlea
 Scala tympani
 near spiral ganglion
cell bodies/auditory
nerve
How it works: neural stimulation
 External microphone picks up
speech signals
 Signal processor transforms
into digital impulses
 Radio-frequency carrier
transmits percutaneously to
internal receiver-stimulator and
electrode array
 auditory nerve/cortex
stimulated; perception of
digitally processed info as
speech
Current status
 Technology reliable, sophisticated, tested over 20 years





clinical use
200,000 + implants worked wide, ½ adult, ½ pediatric
1 million potential U.S. candidates
3 companies manufacture: Cochlear, Med-EL, Advanced
Bionics
Outcomes similar among models
No single method for predicting better results among
devices—differ in processing strategies, slight technical/
surgical variations
Current status
 Chief predictor of success = a short duration of
hearing loss
 Children: earlier implantation best
 Narrow window of opportunity for speech/language
first 2 years life; best 18mos & under
 Adults: chronologic age itself not a factor
 Requires detailed fitting, rehabilitation, practice
 Special education in children without language
 Auditory/speech rehab the key element of
success
Selection criteria: pediatrics
 FDA minimum age recommendation = 12 mos
 CI at younger age may be advantageous
 Audiometric Pure Tone Averages [PTA]:
 12- 18 mos: Profound loss >/=90 dbHL
 18 mos and up: severe-profound loss 70-90 dbHL
 Speech Perception testing for infants [<24 mos]
 Lack of auditory progress measured on IT-MAIS [best aided condition]
 No benefit/lack of progress with conventional
amplification
 Psychologically appropriate: no significant mental/neuro
disability*
 Enrollment in educational program emphasizing auditory
development
 Motivation to complete rehab
Selection criteria: pediatrics
 Older children--2 years-18 years:
 Severe-profound loss both ears
 Lack of progress with HA=
 25 mos-4yrs,11 mos: Multisyllabic Lexical Neighborhood
Test [MLNT] <30% in better-aided ear
 5 yrs – 17 yrs,11 mos: Lexical Neighborhood Test [LNT]
<30%
 Other tests to evaluate: MAIS, HINT, WIPI
 High motivation, no medical contraindications
 Enrollment in educational program emphasizing
auditory development
 Careful selection of pts ESSENTIAL
 Do not want to implant pt who will do better with Hearing aid
Contraindications for implantation
 Completely atretic VIII nerve
 Small internal auditory canal syndrome






Agenesis of cochlea: Michel deformity
Active middle ear/mastoid infection
Tympanic membrane perforation
Severe organic brain dysfunction
Severe mental retardation
Psychosis, unrealistic expectations
Minimum expected benefits





Awareness of environment [warnings, others talking]
Detection [not understanding] of sound in speech range
Awareness of music
Improved speech reading ability with practice
Awareness of own voice (ability to monitor intensity and
speech production)
 Potential for improvement in speech intelligibility (based
on pt and therapy)
 Potential for telephone use (dependent on speech
intelligibility)
Team approach for implantation
 Physician/Surgeon:
 Medical evaluation of candidates
 Responsible for all surgical care & complications
 Audiologists, speech-language pathologists,
psychologists
 Vital/Key role in evaluation of candidacy
 Key for rehabilitation, learning use of device
 Education, implant maintenance




Cochlear Implants in Infants
Universal Newborn Hearing Screening
Technological advances in CI systems
Language delay
Surgical feasibility:
 Pediatric Otolaryngology and Neurotologists:
experienced with infants 1 to 12 mos of age in children’s
hospitals [pediatric anesthesiologists]
 routine airway interventions for premature
and term infants
 Sublgottic /tracheal stenosis surgery
 Choanal atresia surgery
 Congenital masses of neck, vascular tumors
 Surgeon: PMD referral, family preference
Complications







Skin Flap breakdown
Facial nerve stimulation
Facial nerve injury/paralysis
CSF leak/gusher
Device failure : 3-6%,
Infection: otitis media, mastoiditis, implant pocket
Meningitis:
 26 out of 4264 U.S. pediatric implants = 0.6 %
 Reefhuis J et al. NEJM 2003;349:435-45.
 2 deaths
 Many cases occurred in pts with cochlear malformation
 Most cases involved device with spacer
 Current protocol = pneumococcal & H. influenza vaccination
 Hib, Prevnar, Pneumovax
Complications
 Anesthesia: pertinent for all ENT operations
 Current anesthesia fatality rate 1:250,000
for healthy individuals >1yr
 For perspective: activities with same risk of
death as anesthesia:





40 hours automobile driving [2,000 miles]
40 hrs bicycle riding
24 hrs commercial airline flying
7 hrs downhill skiing; 30 minutes rock climbing
340 trips in passenger elevator
Emerging Issues
 Earlier implantation: prior to 1year
 Bilateral implantation
 Why?
 Psychoacoustic literature
 Diminished function with only unilateral aiding of bilateral HL
 Improvement in speech intelligibility
 3 binaural mechanisms
 Head shadow effect, Binaural squelch, Summation
 Sound localization, listening in noisy environment
 Adult studies support, growing peds literature
 Sequential vs simultaneous implantation
 Risk/benefit ratio; insurance approval
 Possible future alternative TX in future: stem cell,new
implants---save one cochlea?
CI Outcomes in Children:
Key Findings
 Large individual differences; each child
unique
 No preimplant predictors of outcome
 Abilities emerge after implantation
 Nature of early experience—
 Earlier implantation= better CI performance, better
auditory/verbal communication
 Implant must be worn every day, all day
 Environment with robust auditory input
necessary for maximal CI results
CI Outcomes in Children:
Language
 Children with CIs Outperform their Profoundly Deaf Peers Who Use
Hearing Aids


Faster rates of language learning and higher overall language achievement levels in CI
pts vs unimplanted Deaf children
Literacy: Tomblin & Geers
 reading levels CI pts approaches hearing peers
 15 yr Deaf ASL students: reading comprehension = 3rd grade;
 Some CI pts do not do as well [IQ, Oral commun, language skills]
 Children Using both Oral and Total Communication Improve in their
Language Skills After CI; but as a group Oral Communication Users
Outperform Those TC
 Many variables to further examine
 Ref: Oto Clin N America: Feb 2012 Robbins A, Niparko J
Otitis Media
 AAP 2004 AOM guidelines
 AAO 2004 OME guidelines
 Cochrane reviews: OME, BMT,
adenoidectomy
 Evidence-based medicine vs evidence
based common sense
 Smith and Pell BMJ 2003: Parachute use to
prevent death and major trauma related to
gravitational challenge
 AHRQ 2012: comparative effectiveness
reviews for OME--pending
Nose and Sinus
 Chronic sinusitis:
 Role of adenoidectomy, +/- maxillary
irrigations
 Role of balloon sinuplasty: evidence based
medicine support pending
 Useful tool for frontal sinus disease in our practice
 Useful for maxillary
 New hammer—many looking for uses
 Time will tell
Nose and Sinus
 Image Gently
campaign
selected imaging of
sinus disease
 In era of easy
antibiotic
availability, still
mindful of sinusitis
complications
:
Adenotonsillar Disease/OSA
 2011 American Academy of
Otolaryngology-HNS adenotonsillectomy
clinical guideline




Chronic tonsillitis: 7 per 1 yr, 5x2 yr, 3x3 yrs
OSA sx, +/- PSG
Admission >3yrs
PFAPA
 Recall prior data: recurrent tonsillitis in
children: 80% resolution in 1 year
Adenotonsillar Disease/OSA
 2011 American Academy of
Otolaryngology-HNS CPG: PSG for sleepdisordered breathing prior to tonsillectomy
in children
 for complex medical conditions preop
 Discordance between tonsil size & SDB sx
 Admit postop if <3yrs or severe OSA
 AHI>10, sats< 80%
 Laboratory based PSG rather than home PSG
Adenotonsillar Disease/OSA
 AAP 2012 : CPG Diagnosis/management
of Obstructive sleep apnea syndrome
[OSAS]




1) all children screened for snoring
2) PSG for snoring, OSAS
3) T&A primary treatment
4) high risk pts monitored inpt postop
 AHI>24, sat>80%, PCO2>60
 5) intranasal steroids: indications
 6) CPAP postop or if T&A not performed
Adenotonsillar Disease/OSA
 Multiple publications on neurocognitive effects
 School aged children—even with negative PSG
 ADHD behavior
 Decreased cognition
 IQ testing changes
Beebe DW: Persistent snoring in preschool children,
Pediatrics sept 2012: --ages 2-3 yrs Cincinnati
 large prospective birth cohort study n=249 2 to 3 yrs old
 Persistent loud snoring occurs 9% children
 = significantly higher behavior problems
 Hyperactivity, depression, inattentions,
 Worse cognitive development
Adenotonsillar Disease/OSA
 Childhood Adenotonsillectomy Study
[CHAT] 2012:
 First prospective, randomized controlled
study, multicenter evaluated effectiveness of
T&A or watchful waiting for OSA
 464 children, 5-9yrs with PSG proven mildmoderate OSA randomized
 Publication forthcoming
 PSG results, neurocognitive testing improves
 Observation arm: may also show improvement
Hemangiomas/Vascular
malformations
 Propranolol:
 Significant improvement in our care for
hemangiomas of airway, head/neck
 Sclerotherapy vascular malformations
Neck Mass vs Lymph node vs
Cancer?
 Midline vs Lateral Neck Mass
 Evaluation
 History
 Growth 4-6 wks,+sx
 Abx no effect
 Cat, TB exposure
 Constitutional / Lymphoma symptoms
 Ultrasound helpful
Neck Mass vs Lymph node vs
Cancer?
 Midline DDX





Thyroglossal duct cyst
Dermoid cyst
Thyroid and Parathyroid masses
Vascular lesions/hemangioma
Laryngoceles
Neck Mass vs Lymph node vs
Cancer?
 Lateral Neck Masses:
 Acute adenitis
 Chronic adenitis
 Atypical mycobacterial
 Cat-Scratch
 Toxoplasmosis
 HIV
 Congenital
 Branchial Cleft
 Pseudotumor infancy,
 Thymus,
 Vascular/Lymphatic
 Malignant Lateral
Neck Masses;




Lymphoma
Sarcoma
Neuroblastoma
Salivary gland
Neck Mass vs Lymph node vs
Cancer?
 Inflammatory syndromes
 Persistent, enlarging chronic adenopathy
 Odd/usually rare
-history, exam give clues
Sarcoidosis
Kawasaki
Castleman’s disease,
JRA, SLE, Rosai-Dorfman disease/histiocytosis
Ankyloglossia
 To release or not to release?
 Feeding/latching to breast/bottle
 Can release in office or nursery 1st weeks life
 Studies support
 Speech /articulation difficulties later
 Weigh vs anesthetic risks