Intro

ENT Updates for the General ENT Updates for the General Pediatric OfficePediatric Office

R. Christopher Miyamoto, M.D., FACS, FAAPR. Christopher Miyamoto, M.D., FACS, FAAP

Pediatric OtolaryngologyPediatric OtolaryngologyPeyton Manning Children’s Hospital Peyton Manning Children’s Hospital

at St. Vincent’sat St. Vincent’s

IntroIntro

Provide update on interesting ENT Provide update on interesting ENT developments for primary care Pediatric developments for primary care Pediatric officesoffices

Excellent New Text/Resource: Excellent New Text/Resource: Pediatric OtolaryngologyPediatric Otolaryngology, Schoem SR, , Schoem SR,

Darrow DH ed. AAP 2012Darrow DH ed. AAP 2012

Disclosure: I have no financial Disclosure: I have no financial interest/relationships with any interest/relationships with any biomedical companies, etc.biomedical companies, etc.

Congenital Hearing LossCongenital Hearing Losssignificancesignificance::

Hearing impairment one of the most common sensory Hearing impairment one of the most common sensory deficits in children & significant healthcare problemdeficits in children & significant healthcare problem

1 - 2 infants per 1000 births have significant hearing 1 - 2 infants per 1000 births have significant hearing loss [HL] loss [HL] Bilateral severe-profoundBilateral severe-profound

Up to 4/1000 births if mild-moderate or unilateral HL Up to 4/1000 births if mild-moderate or unilateral HL included included [compare to signif congenital heart disease [compare to signif congenital heart disease 4/1000, cleft lip 1/1000, cleft palate 1/2500]4/1000, cleft lip 1/1000, cleft palate 1/2500] Steel KP Steel KP Science 1998;279:1870-71Science 1998;279:1870-71

= = 40,000 infants born/year w/ significant HL;40,000 infants born/year w/ significant HL; = 4000 profoundly deaf= 4000 profoundly deaf

DevelopmentsDevelopments 1) Universal Newborn Hearing Screening1) Universal Newborn Hearing Screening

Healthcare providers must now screen & manage HL in infants— Healthcare providers must now screen & manage HL in infants— substantial pt population— substantial pt population— Goal 1 mosGoal 1 mos

Joint Committee on Infant Hearing Joint Committee on Infant Hearing and AAP:endorsed UNHS and AAP:endorsed UNHS revised guidelines 2000, revised guidelines 2000, Position update 2007Position update 2007 Confirm HL by 3 mos Confirm HL by 3 mos

Logistical issues:Logistical issues: Otolaryngology referral – timeOtolaryngology referral – time Ability of otolaryngologist to confirm:Ability of otolaryngologist to confirm:

Middle ear fluid, audiological capabilities [OAE, ABR]Middle ear fluid, audiological capabilities [OAE, ABR] Intraoperative vs office sedation ABR capabilities Intraoperative vs office sedation ABR capabilities

Intervention by 6 mosIntervention by 6 mos Increased receptive/expressive language quotientsIncreased receptive/expressive language quotients

EHDI / ISDH Physician tool kitEHDI / ISDH Physician tool kit

DevelopmentsDevelopments

2) 2) Molecular/geneticMolecular/genetic understanding of understanding of hereditary hearing loss vastly enhanced hereditary hearing loss vastly enhanced over last 10 yearsover last 10 years Genetic testing now integral for evaluation of Genetic testing now integral for evaluation of

hearing impairment in childrenhearing impairment in children Expertise of Medical Genetic specialists Expertise of Medical Genetic specialists

invaluableinvaluable 3) 3) Early intervention Early intervention [medical vs surgical] [medical vs surgical]

now standard of carenow standard of care

Importance?Importance? Significance: Significance: COST EFFECTIVE, POSITIVE QUALITY OUTCOME COST EFFECTIVE, POSITIVE QUALITY OUTCOME Early hearing diagnosis= early intervention at 6mos or Early hearing diagnosis= early intervention at 6mos or

earlierearlier = better speech/language development, school performance, = better speech/language development, school performance,

economic outcomeeconomic outcome = early identification of profound hearing loss requiring = early identification of profound hearing loss requiring

cochlear implantationcochlear implantation

NEJM 2008---Cochlear implantation one of few truly NEJM 2008---Cochlear implantation one of few truly cost effective interventions cost effective interventions

22

Hearing ScreeningHearing Screening

Per UNHS, must occur prior to d/cPer UNHS, must occur prior to d/c Automated auditory brainstem response Automated auditory brainstem response

[ABR] preferred[ABR] preferred +/- otoacoustic emissions [OAE]+/- otoacoustic emissions [OAE]

OAE alone can miss auditory neuropathyOAE alone can miss auditory neuropathy Failure [PC = “refer”] requires diagnostic Failure [PC = “refer”] requires diagnostic

audiology eval as outptaudiology eval as outpt = ABR, tympanograms, OAE= ABR, tympanograms, OAE

Hearing Loss BreakdownHearing Loss Breakdown Historically, infectious disorders [TORCH, meningitis], Historically, infectious disorders [TORCH, meningitis],

teratogens, ototoxic meds were primary causes of congenital & teratogens, ototoxic meds were primary causes of congenital & acquired HLacquired HL

Vaccines, abx, awareness of teratogens changed ddxVaccines, abx, awareness of teratogens changed ddx Hereditary causes account for 50% Hereditary causes account for 50% childhood deafnesschildhood deafness

Morton NE Morton NE Ann NY Acad Sci 1991;630:16-31Ann NY Acad Sci 1991;630:16-31 Over 150 loci [areas on genes] identifiedOver 150 loci [areas on genes] identified

70% hereditary hearing loss nonsyndromic70% hereditary hearing loss nonsyndromic 75% of this autosomal recessive75% of this autosomal recessive Important for evaluation processImportant for evaluation process Autosomal recessive hearing loss locus DFNB1 found on Autosomal recessive hearing loss locus DFNB1 found on

Chromosome 13q—contains GJB2 geneChromosome 13q—contains GJB2 gene Mutations in GJB2 responsible for up to 50% severe-Mutations in GJB2 responsible for up to 50% severe-

profound SNHL in autosomal recessive nonsyndromic HL in profound SNHL in autosomal recessive nonsyndromic HL in US & EuropeUS & Europe

Hearing Loss BreakdownHearing Loss Breakdown

The Rest: The Rest: 50% hearing loss NOT inherited50% hearing loss NOT inherited

Acquired—ototoxics, risk factors, othersAcquired—ototoxics, risk factors, others May have Genetic comp making susceptibleMay have Genetic comp making susceptible

30% Syndromic30% Syndromic 823 syndromes linked to hearing loss823 syndromes linked to hearing loss 150 gene loci linked to hearing loss150 gene loci linked to hearing loss

Importance of Medical Genetics EvaluationImportance of Medical Genetics Evaluation JCIH 2007 statement guidelinesJCIH 2007 statement guidelines

GJB2GJB2 Gene in DFNB1 locus on chromosome 13Gene in DFNB1 locus on chromosome 13 GJB2 gene codes for connexin 26 proteinGJB2 gene codes for connexin 26 protein

Membrane proteins that form gap junctionsMembrane proteins that form gap junctions Seem important in electrolyte, second messenger and metabolite Seem important in electrolyte, second messenger and metabolite

exchange in cochleaexchange in cochlea

Multiple mutations [60+ and counting] describedMultiple mutations [60+ and counting] described 35delG mutation especially common [15-40%] 35delG mutation especially common [15-40%]

[white/European descent, some Hispanic, Asian, african-[white/European descent, some Hispanic, Asian, african-american]—Connexin 26american]—Connexin 26

Many other genes involved with congenital hearing loss– Many other genes involved with congenital hearing loss– can screen with Chip technologycan screen with Chip technology 17-19 gene mutations 17-19 gene mutations screened screened

Hereditary Syndromic Hearing LossHereditary Syndromic Hearing Loss

Most are congenital and some acquiredMost are congenital and some acquired 400 - 800+ syndromes associated w/HL400 - 800+ syndromes associated w/HL Cause of HL: sensorineural, conductive, Cause of HL: sensorineural, conductive,

mixedmixed Craniofacial & other features associatedCraniofacial & other features associated Findings may be subtleFindings may be subtle Medical genetics evaluation helpful with Medical genetics evaluation helpful with

subtle phenotypessubtle phenotypes

Nonhereditary Congenital Hearing LossNonhereditary Congenital Hearing Loss TORCH:TORCH:

Toxoplasmosis, Rubella, CMV, HerpesToxoplasmosis, Rubella, CMV, Herpes SyphilisSyphilis CMV: most frequent cause nonhereditary HL in neonatesCMV: most frequent cause nonhereditary HL in neonates

40,000 CMV infected infants/yr; 4000 HL40,000 CMV infected infants/yr; 4000 HL 8-10% CMV-infected infants asx at birth can develop HL---need 8-10% CMV-infected infants asx at birth can develop HL---need

long-term f/ulong-term f/u

Cytomegalovirus [CMV]: 0.5 -2% live birthsCytomegalovirus [CMV]: 0.5 -2% live births Congenital CMV infection: Congenital CMV infection:

10% Symptomatic: 44% have HL by age 3yrs10% Symptomatic: 44% have HL by age 3yrs 21%are delayed onset21%are delayed onset

90% Asymptomatic: 7.4% have HL by 3yrs90% Asymptomatic: 7.4% have HL by 3yrs 33% delayed onset33% delayed onset

50% of both groups w/progressive loss50% of both groups w/progressive loss

Evaluation of Hearing Loss in Evaluation of Hearing Loss in Infants & Young ChildrenInfants & Young Children

Thorough History/physical examThorough History/physical exam Directed toward issues discussed earlierDirected toward issues discussed earlier Syndromic features—refer to Med GeneticsSyndromic features—refer to Med Genetics

Prenatal, perinatal,postnatal eventsPrenatal, perinatal,postnatal events AAP JCIH 2007 Risk Indicators AAP JCIH 2007 Risk Indicators

(congenital, delayed onset or progressive hearing loss(congenital, delayed onset or progressive hearing loss

PrematurityPrematurity TeratogensTeratogens Perinatal maternal infections: TORCHPerinatal maternal infections: TORCH Low birth wgt <1500 gLow birth wgt <1500 g Prolonged mechanical ventilation, hyperbilirubinemia, Prolonged mechanical ventilation, hyperbilirubinemia,

hypoxiahypoxia

Evaluation of Hearing Loss in Evaluation of Hearing Loss in Infants & Young Children Infants & Young Children

Prenatal, perinatal,postnatal eventsPrenatal, perinatal,postnatal events NICU graduatesNICU graduates

Persistent pulmonary hypertension of the Persistent pulmonary hypertension of the newbornnewborn

Extracorporeal membrane oxygenation Extracorporeal membrane oxygenation [ECMO]: [diaphragmatic hernia, heart [ECMO]: [diaphragmatic hernia, heart disease, etc]disease, etc]

20-25% with late-onset or progressive HL20-25% with late-onset or progressive HL = ABR at 6mos, audiogram 1 yr and annual = ABR at 6mos, audiogram 1 yr and annual

x3yrsx3yrs


Infections—neonatal meningitisInfections—neonatal meningitis Syncope [fainting]—Jervell & Lange-NielsenSyncope [fainting]—Jervell & Lange-Nielsen

Family members with syncopal or sudden Family members with syncopal or sudden cardiac death in teens, early adult yearscardiac death in teens, early adult years

Delayed walking/visual issues—UsherDelayed walking/visual issues—Usher Family historyFamily history


Physical Exam:Physical Exam: Check for craniofacial issues, subtle ear Check for craniofacial issues, subtle ear

deformitiesdeformities Check for ocular abnormalities: coloboma, Check for ocular abnormalities: coloboma,

hypertelorism, other abnormalitieshypertelorism, other abnormalities Up to 50% severe-profound hearing impaired kids Up to 50% severe-profound hearing impaired kids

have eye issueshave eye issues Armitage IM et al. Arch of Dis Childhood 1995;73(1);53Armitage IM et al. Arch of Dis Childhood 1995;73(1);53

Pediatric Ophthalmology evaluation for all hearing Pediatric Ophthalmology evaluation for all hearing impaired children recommendedimpaired children recommended

JCIH 2007 statementJCIH 2007 statement


Confirm Confirm with OAE with OAE / ABR/ ABR OAE alone can miss auditory neuropathyOAE alone can miss auditory neuropathy

If bilateral SNHL and diagnosis not If bilateral SNHL and diagnosis not apparent [identifiable syndrome, apparent [identifiable syndrome, meningitis, autosomal dominant meningitis, autosomal dominant SNHL,trauma]SNHL,trauma] genetic testing for genetic testing for hearing loss/genetic evaluationhearing loss/genetic evaluation

Auditory NeuropathyAuditory Neuropathy

PathophysiologyPathophysiology Hair cell death? Hair cell death?

HypoxiaHypoxia Temporal bone studiesTemporal bone studies

Genetics: Otoferlin geneGenetics: Otoferlin gene

Cochlear ImplantationCochlear Implantation

Currently the standard surgical treatment for Currently the standard surgical treatment for patients with hearing impairment & well-fit patients with hearing impairment & well-fit hearing aids that fail to permit effective oral hearing aids that fail to permit effective oral communicationcommunication

Designed to help the severe-profoundly deaf Designed to help the severe-profoundly deaf patientpatient Perceive environmental soundsPerceive environmental sounds Understand speech Understand speech Maximal benefit depends on patient and rehabMaximal benefit depends on patient and rehab

How it works: neural stimulationHow it works: neural stimulation

Implant with electrode Implant with electrode array placed array placed surgically in the surgically in the lumen of the cochlealumen of the cochlea Scala tympaniScala tympani near spiral ganglion near spiral ganglion

cell bodies/auditory cell bodies/auditory nervenerve

How it works: neural stimulationHow it works: neural stimulation External microphone picks up External microphone picks up

speech signalsspeech signals Signal processor transforms Signal processor transforms

into digital impulsesinto digital impulses Radio-frequency carrier Radio-frequency carrier

transmits percutaneously to transmits percutaneously to internal receiver-stimulator and internal receiver-stimulator and electrode arrayelectrode array

auditory nerve/cortex auditory nerve/cortex stimulated; perception of stimulated; perception of digitally processed info as digitally processed info as speechspeech

Current statusCurrent status

Technology reliable, sophisticated, tested over 20 years Technology reliable, sophisticated, tested over 20 years clinical useclinical use

200,000 + implants worked wide, ½ adult, ½ pediatric200,000 + implants worked wide, ½ adult, ½ pediatric 1 million potential U.S. candidates1 million potential U.S. candidates 3 companies manufacture: Cochlear, Med-EL, Advanced 3 companies manufacture: Cochlear, Med-EL, Advanced

BionicsBionics Outcomes similar among modelsOutcomes similar among models No single method for predicting better results among No single method for predicting better results among

devices—differ in processing strategies, slight technical/ devices—differ in processing strategies, slight technical/ surgical variationssurgical variations

Current statusCurrent status

Chief predictor of success = a short duration of Chief predictor of success = a short duration of hearing losshearing loss

Children: earlier implantation bestChildren: earlier implantation best Narrow window of opportunity for speech/language Narrow window of opportunity for speech/language

first 2 years life; best 18mos & underfirst 2 years life; best 18mos & under Adults: chronologic age itself not a factorAdults: chronologic age itself not a factor Requires detailed fitting, rehabilitation, practiceRequires detailed fitting, rehabilitation, practice

Special education in children without languageSpecial education in children without language Auditory/speech rehab the key element of Auditory/speech rehab the key element of

successsuccess

Selection criteria: pediatricsSelection criteria: pediatrics FDA minimum age recommendation = 12 mosFDA minimum age recommendation = 12 mos

CI at younger age may be advantageousCI at younger age may be advantageous Audiometric Pure Tone Averages [PTA]:Audiometric Pure Tone Averages [PTA]:

12- 18 mos: Profound loss >/=90 dbHL12- 18 mos: Profound loss >/=90 dbHL 18 mos and up: severe-profound loss 70-90 dbHL18 mos and up: severe-profound loss 70-90 dbHL

Speech Perception testing for infants [<24 mos]Speech Perception testing for infants [<24 mos] Lack of auditory progress measured on IT-MAIS [best aided condition]Lack of auditory progress measured on IT-MAIS [best aided condition]

No benefit/lack of progress with conventional No benefit/lack of progress with conventional amplificationamplification

Psychologically appropriate: no significant mental/neuro Psychologically appropriate: no significant mental/neuro disability*disability*

Enrollment in educational program emphasizing auditory Enrollment in educational program emphasizing auditory developmentdevelopment

Motivation to complete rehabMotivation to complete rehab

Selection criteria: pediatricsSelection criteria: pediatrics Older children--2 years-18 years:Older children--2 years-18 years:

Severe-profound loss both earsSevere-profound loss both ears Lack of progress with HA=Lack of progress with HA=

25 mos-4yrs,11 mos: Multisyllabic Lexical Neighborhood Test 25 mos-4yrs,11 mos: Multisyllabic Lexical Neighborhood Test [MLNT] <30% in better-aided ear[MLNT] <30% in better-aided ear

5 yrs – 17 yrs,11 mos: Lexical Neighborhood Test [LNT] <30%5 yrs – 17 yrs,11 mos: Lexical Neighborhood Test [LNT] <30% Other tests to evaluate: MAIS, HINT, WIPIOther tests to evaluate: MAIS, HINT, WIPI

High motivation, no medical contraindicationsHigh motivation, no medical contraindications Enrollment in educational program emphasizing auditory Enrollment in educational program emphasizing auditory

developmentdevelopment Careful selection of pts ESSENTIALCareful selection of pts ESSENTIAL

Do not want to implant pt who will do better with Hearing aidDo not want to implant pt who will do better with Hearing aid

Contraindications for implantationContraindications for implantation

Completely atretic VIII nerveCompletely atretic VIII nerve Small internal auditory canal syndromeSmall internal auditory canal syndrome

Agenesis of cochlea: Michel deformityAgenesis of cochlea: Michel deformity Active middle ear/mastoid infectionActive middle ear/mastoid infection Tympanic membrane perforationTympanic membrane perforation Severe organic brain dysfunctionSevere organic brain dysfunction Severe mental retardationSevere mental retardation Psychosis, unrealistic expectationsPsychosis, unrealistic expectations

Minimum expected benefitsMinimum expected benefits

Awareness of environment [warnings, others talking]Awareness of environment [warnings, others talking] Detection [not understanding] of sound in speech range Detection [not understanding] of sound in speech range Awareness of musicAwareness of music Improved speech reading ability with practiceImproved speech reading ability with practice Awareness of own voice (ability to monitor intensity and Awareness of own voice (ability to monitor intensity and

speech production)speech production) Potential for improvement in speech intelligibility (based Potential for improvement in speech intelligibility (based

on pt and therapy)on pt and therapy) Potential for telephone use (dependent on speech Potential for telephone use (dependent on speech

intelligibility)intelligibility)

Team approach for implantationTeam approach for implantation

Physician/Surgeon:Physician/Surgeon: Medical evaluation of candidatesMedical evaluation of candidates Responsible for all surgical care & complicationsResponsible for all surgical care & complications

Audiologists, speech-language pathologists, Audiologists, speech-language pathologists, psychologistspsychologists Vital/Key role in evaluation of candidacyVital/Key role in evaluation of candidacy Key for rehabilitation, learning use of deviceKey for rehabilitation, learning use of device Education, implant maintenanceEducation, implant maintenance

Cochlear Implants in InfantsCochlear Implants in Infants Universal Newborn Hearing ScreeningUniversal Newborn Hearing Screening Technological advances in CI systemsTechnological advances in CI systems Language delayLanguage delay Surgical feasibility:Surgical feasibility:

Pediatric Otolaryngology and Neurotologists: Pediatric Otolaryngology and Neurotologists: experienced with infants 1 to 12 mos of age in children’s experienced with infants 1 to 12 mos of age in children’s hospitals [pediatric anesthesiologists]hospitals [pediatric anesthesiologists]

routine airway interventions for premature routine airway interventions for premature and term infantsand term infants

Sublgottic /tracheal stenosis surgerySublgottic /tracheal stenosis surgery Choanal atresia surgeryChoanal atresia surgery Congenital masses of neck, vascular tumorsCongenital masses of neck, vascular tumors

SurgeonSurgeon: PMD referral, family preference : PMD referral, family preference

ComplicationsComplications Skin Flap breakdownSkin Flap breakdown Facial nerve stimulationFacial nerve stimulation Facial nerve injury/paralysisFacial nerve injury/paralysis CSF leak/gusherCSF leak/gusher Device failure : 3-6%, Device failure : 3-6%, Infection: otitis media, mastoiditis, implant pocketInfection: otitis media, mastoiditis, implant pocket Meningitis:Meningitis:

26 out of 4264 U.S. pediatric implants = 26 out of 4264 U.S. pediatric implants = 0.6 % 0.6 % Reefhuis J et al. NEJM 2003;349:435-45.Reefhuis J et al. NEJM 2003;349:435-45. 2 deaths2 deaths

Many cases occurred in pts with cochlear malformationMany cases occurred in pts with cochlear malformation Most cases involved device with spacerMost cases involved device with spacer Current protocol = Current protocol = pneumococcal & H. influenza vaccinationpneumococcal & H. influenza vaccination

Hib, Prevnar, PneumovaxHib, Prevnar, Pneumovax

ComplicationsComplications AnesthesiaAnesthesia: pertinent for all ENT operations: pertinent for all ENT operations Current anesthesia fatality rate 1:250,000 Current anesthesia fatality rate 1:250,000

for healthy individuals >1yrfor healthy individuals >1yr For perspective: activities with same risk of For perspective: activities with same risk of

death as anesthesia:death as anesthesia: 40 hours automobile driving [2,000 miles]40 hours automobile driving [2,000 miles] 40 hrs bicycle riding40 hrs bicycle riding 24 hrs commercial airline flying24 hrs commercial airline flying 7 hrs downhill skiing; 30 minutes rock climbing7 hrs downhill skiing; 30 minutes rock climbing 340 trips in passenger elevator340 trips in passenger elevator

Emerging IssuesEmerging Issues

Earlier implantation: prior to 1yearEarlier implantation: prior to 1year Bilateral implantationBilateral implantation

Why?Why? Psychoacoustic literaturePsychoacoustic literature Diminished function with only unilateral aiding of bilateral HLDiminished function with only unilateral aiding of bilateral HL

Improvement in speech intelligibilityImprovement in speech intelligibility 3 binaural mechanisms3 binaural mechanisms

Head shadow effect, Head shadow effect, Binaural squelch, SummationBinaural squelch, Summation Sound localization, listening in noisy environmentSound localization, listening in noisy environment Adult studies support, growing peds literatureAdult studies support, growing peds literature Sequential vs simultaneous implantationSequential vs simultaneous implantation

Risk/benefit ratio; insurance approvalRisk/benefit ratio; insurance approval Possible future alternative TX in future: stem cell,new implants---Possible future alternative TX in future: stem cell,new implants---

save one cochlea?save one cochlea?

CI Outcomes in Children: CI Outcomes in Children: Key FindingsKey Findings

Large individual differences; each child uniqueLarge individual differences; each child unique No preimplant predictors of outcomeNo preimplant predictors of outcome Abilities emerge after implantation Abilities emerge after implantation

Nature of early experience—Nature of early experience— Earlier implantation= better CI performance, better Earlier implantation= better CI performance, better

auditory/verbal communicationauditory/verbal communication

Implant must be worn every day, all day Implant must be worn every day, all day Environment with robust auditory input Environment with robust auditory input

necessary for maximal CI resultsnecessary for maximal CI results

CI Outcomes in Children: CI Outcomes in Children: Language 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 & GeersTomblin & Geers reading levels CI pts approaches hearing peers reading levels CI pts approaches hearing peers 15 yr Deaf ASL students: reading comprehension = 315 yr Deaf ASL students: reading comprehension = 3rdrd grade; grade; Some CI pts do not do as well [IQ, Oral commun, language skills]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 examineMany variables to further examine

Ref: Ref: Oto Clin N America: Feb 2012 Robbins A, Niparko JOto Clin N America: Feb 2012 Robbins A, Niparko J

Otitis MediaOtitis Media AAP 2004 AOM guidelinesAAP 2004 AOM guidelines AAO 2004 OME guidelinesAAO 2004 OME guidelines Cochrane reviews: OME, BMT, adenoidectomyCochrane reviews: OME, BMT, adenoidectomy Evidence-based medicine vs evidence based Evidence-based medicine vs evidence based

common sensecommon sense Smith and Pell BMJ 2003: Parachute use to Smith and Pell BMJ 2003: Parachute use to

prevent death and major trauma related to prevent death and major trauma related to gravitational challengegravitational challenge

AHRQ 2012: comparative effectiveness reviews AHRQ 2012: comparative effectiveness reviews for OME--pendingfor OME--pending

Nose and SinusNose and Sinus

Chronic sinusitis:Chronic sinusitis: Role of adenoidectomy, +/- maxillary Role of adenoidectomy, +/- maxillary

irrigationsirrigations Role of Role of balloon sinuplastyballoon sinuplasty: evidence based : evidence based

medicine support pending medicine support pending Useful tool for frontal sinus disease in our practiceUseful tool for frontal sinus disease in our practice Useful for maxillaryUseful for maxillary New hammer—many looking for usesNew hammer—many looking for uses Time will tellTime will tell

Nose and SinusNose and Sinus

Image Gently Image Gently campaigncampaign

selected imaging of selected imaging of sinus diseasesinus disease

In era of easy In era of easy antibiotic antibiotic availability, still availability, still mindful of sinusitis mindful of sinusitis complicationscomplications

::

Adenotonsillar Disease/OSAAdenotonsillar Disease/OSA

2011 American Academy of Otolaryngology-2011 American Academy of Otolaryngology-HNS adenotonsillectomy clinical guidelineHNS adenotonsillectomy clinical guideline Chronic tonsillitis: 7 per 1 yr, 5x2 yr, 3x3 yrsChronic tonsillitis: 7 per 1 yr, 5x2 yr, 3x3 yrs OSA sx, +/- PSGOSA sx, +/- PSG Admission >3yrsAdmission >3yrs PFAPAPFAPA

Recall prior data: recurrent tonsillitis in children: Recall prior data: recurrent tonsillitis in children: 80% resolution in 1 year80% resolution in 1 year


2011 American Academy of 2011 American Academy of Otolaryngology-HNS CPG: PSG for sleep-Otolaryngology-HNS CPG: PSG for sleep-disordered breathing prior to tonsillectomy disordered breathing prior to tonsillectomy in childrenin children for complex medical conditions preopfor complex medical conditions preop Discordance between tonsil size & SDB sxDiscordance between tonsil size & SDB sx Admit postop if <3yrs or severe OSA Admit postop if <3yrs or severe OSA

AHI>10, sats< 80%AHI>10, sats< 80%

Laboratory based PSG rather than home PSGLaboratory based PSG rather than home PSG


AAP 2012 : CPG Diagnosis/management of AAP 2012 : CPG Diagnosis/management of Obstructive sleep apnea syndromeObstructive sleep apnea syndrome [OSAS] [OSAS] 1) all children screened for snoring1) all children screened for snoring 2) PSG for snoring, OSAS2) PSG for snoring, OSAS 3) T&A primary treatment3) T&A primary treatment 4) high risk pts monitored inpt postop4) high risk pts monitored inpt postop

AHI>24, sat>80%, PCO2>60AHI>24, sat>80%, PCO2>60

5) intranasal steroids: indications5) intranasal steroids: indications 6) CPAP postop or if T&A not performed6) CPAP postop or if T&A not performed


Multiple publications on neurocognitive effectsMultiple publications on neurocognitive effects School aged children—even with negative PSGSchool aged children—even with negative PSG

ADHD behaviorADHD behavior Decreased cognitionDecreased cognition IQ testing changesIQ testing changes

Beebe DW: Persistent snoring in preschool children, Pediatrics sept Beebe DW: Persistent snoring in preschool children, Pediatrics sept 2012: --2012: --ages 2-3 yrs Cincinnatiages 2-3 yrs Cincinnati large prospective birth cohort study n=249 2 to 3 yrs oldlarge prospective birth cohort study n=249 2 to 3 yrs old Persistent loud snoring occurs 9% childrenPersistent loud snoring occurs 9% children = significantly higher behavior problems= significantly higher behavior problems

Hyperactivity, depression, inattentions, Hyperactivity, depression, inattentions, Worse cognitive developmentWorse cognitive development


Childhood Adenotonsillectomy Study Childhood Adenotonsillectomy Study [CHAT] 2012: [CHAT] 2012: First prospective, randomized controlled study, First prospective, randomized controlled study,

multicenter evaluated effectiveness of T&A or multicenter evaluated effectiveness of T&A or watchful waiting for OSAwatchful waiting for OSA

464 children, 5-9yrs with PSG proven mild-464 children, 5-9yrs with PSG proven mild-moderate OSA randomizedmoderate OSA randomized

Publication forthcomingPublication forthcoming PSG results, neurocognitive testing improvesPSG results, neurocognitive testing improves

Observation arm: may also show improvementObservation arm: may also show improvement

Hemangiomas/Vascular Hemangiomas/Vascular malformationsmalformations

Propranolol: Propranolol: Significant improvement in our care for Significant improvement in our care for

hemangiomas of airway, head/neckhemangiomas of airway, head/neck

Sclerotherapy vascular malformationsSclerotherapy vascular malformations

Neck Mass vs Lymph node vs Neck Mass vs Lymph node vs Cancer?Cancer?

Midline vs Lateral Neck MassMidline vs Lateral Neck Mass EvaluationEvaluation

HistoryHistory Growth 4-6 wks,+sx Growth 4-6 wks,+sx Abx no effectAbx no effect Cat, TB exposureCat, TB exposure Constitutional / Lymphoma symptomsConstitutional / Lymphoma symptoms

Ultrasound helpfulUltrasound helpful


Midline DDXMidline DDX Thyroglossal duct cystThyroglossal duct cyst Dermoid cystDermoid cyst Thyroid and Parathyroid massesThyroid and Parathyroid masses Vascular lesions/hemangiomaVascular lesions/hemangioma LaryngocelesLaryngoceles


Lateral Neck Masses:Lateral Neck Masses: Acute adenitisAcute adenitis Chronic adenitisChronic adenitis

Atypical mycobacterialAtypical mycobacterial Cat-ScratchCat-Scratch ToxoplasmosisToxoplasmosis HIVHIV

CongenitalCongenital Branchial CleftBranchial Cleft Pseudotumor infancy,Pseudotumor infancy, Thymus,Thymus, Vascular/Lymphatic Vascular/Lymphatic

Malignant Lateral Malignant Lateral Neck Masses;Neck Masses; LymphomaLymphoma SarcomaSarcoma NeuroblastomaNeuroblastoma Salivary glandSalivary gland


Inflammatory syndromesInflammatory syndromes Persistent, enlarging chronic adenopathyPersistent, enlarging chronic adenopathy Odd/usually rareOdd/usually rare

-history, exam give clues-history, exam give clues

SarcoidosisSarcoidosis

KawasakiKawasaki

Castleman’s disease, Castleman’s disease,

JRA, SLE, Rosai-Dorfman disease/histiocytosisJRA, SLE, Rosai-Dorfman disease/histiocytosis

AnkyloglossiaAnkyloglossia

To release or not to release?To release or not to release? Feeding/latching to breast/bottleFeeding/latching to breast/bottle

Can release in office or nursery 1Can release in office or nursery 1stst weeks life weeks life Studies support Studies support

Speech /articulation difficulties laterSpeech /articulation difficulties later Weigh vs anesthetic risksWeigh vs anesthetic risks

Intro

Documents

significant hearing

infant hearing

hearing screeningper

general pediatric office

cost effective

infants bornyear w significant

mos joint committee

mos logistical issues