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
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AAP JCIH 2007 Risk Indicators
(congenital, delayed onset or progressive hearing loss
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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
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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
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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
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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
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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
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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:
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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
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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
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Adenotonsillar Disease/OSA
 2011 American Academy of
Otolaryngology-HNS adenotonsillectomy
clinical guideline
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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]
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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
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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;
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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