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ENT Updates for the General Pediatric Office R. Christopher Miyamoto, M.D., FACS, FAAP Pediatric Otolaryngology Peyton Manning Children’s Hospital at St.

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Presentation on theme: "ENT Updates for the General Pediatric Office R. Christopher Miyamoto, M.D., FACS, FAAP Pediatric Otolaryngology Peyton Manning Children’s Hospital at St."— Presentation transcript:

1 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

2 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.

3 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

4 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

5 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

6 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  2

7 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

8 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% severe- profound SNHL in autosomal recessive nonsyndromic HL in US & Europe

9 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

10 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

11 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

12 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

13 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

14 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

15 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

16 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

17 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

18 Auditory Neuropathy  Pathophysiology  Hair cell death?  Hypoxia  Temporal bone studies  Genetics: Otoferlin gene

19 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

20 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

21 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

22 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

23 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

24 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

25 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

26 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

27 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)

28 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

29 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

30 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

31 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

32 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?

33 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

34 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  Tomblin & Geers  Literacy: Tomblin & Geers  reading levels CI pts approaches hearing peers  15 yr Deaf ASL students: reading comprehension = 3 rd 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

35 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

36 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

37 Nose and Sinus  Image Gently campaign selected imaging of sinus disease  In era of easy antibiotic availability, still mindful of sinusitis complications ::::

38 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

39 Adenotonsillar Disease/OSA  2011 American Academy of Otolaryngology-HNS CPG: PSG for sleep- disordered 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 10, sats< 80%  Laboratory based PSG rather than home PSG

40 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

41 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

42 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 mild- moderate OSA randomized  Publication forthcoming  PSG results, neurocognitive testing improves  Observation arm: may also show improvement

43 Hemangiomas/Vascular malformations  Propranolol:  Significant improvement in our care for hemangiomas of airway, head/neck  Sclerotherapy vascular malformations

44 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

45 Neck Mass vs Lymph node vs Cancer?  Midline DDX  Thyroglossal duct cyst  Dermoid cyst  Thyroid and Parathyroid masses  Vascular lesions/hemangioma  Laryngoceles

46 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

47 Neck Mass vs Lymph node vs Cancer?  Inflammatory syndromes  Persistent, enlarging chronic adenopathy  Odd/usually rare -history, exam give clues SarcoidosisKawasaki Castleman’s disease, JRA, SLE, Rosai-Dorfman disease/histiocytosis

48 Ankyloglossia  To release or not to release?  Feeding/latching to breast/bottle  Can release in office or nursery 1 st weeks life  Studies support  Speech /articulation difficulties later  Weigh vs anesthetic risks


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