3/27/2014
Pediatric Hearing Loss and Cochlear Implant Update
David H. Chi, MD Children’s Hospital of Pittsburgh Medical Director, Hearing Center April 25, 2014
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Disclosure
• Nothing to disclose
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Learning Objectives
1. Review etiologies and various presentations of pediatric hearing loss.
2. Select appropriate workup and recognize findings that lead to a decision for cochlear implant.
3. Discuss surgery, plan of care, and the PA/NP role in followup for pediatric CI patients.
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Why is early detection and treatment of sensorineural hearing loss so important? • Hearing loss is the most frequent birth condition.
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Incidence per 10,000 of Congenital Defects/Diseases
40 30 30
20 12 11 10 6 5 2 1 0
Epidemiology
• 1‐3 of 1,000 live births with moderate to severe hearing loss • Approximately 12,000 infants born annually in the U.S. with SNHI – 33 babies / day • 90% of deaf genetic kids / hearing parents
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Why is early detection and treatment of sensorineural hearing loss so important?
• Hearing loss is the most frequent birth condition • Undetected hearing loss has important negative consequences.
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Reading Comprehension Scores of Hearing and Deaf Students
10.0 9.0 8.0 7.0 6.0 Deaf 5.0 Hearing 4.0 3.0 2.0 1.0 Grade Equivalents 8 9 10 11 12 13 14 15 16 17 18 Age in Years Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Why is early detection and treatment of sensorineural hearing loss so important?
• Hearing loss is the most frequent birth condition • Undetected hearing loss has important negative consequences. • Early intervention has significant benefits.
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Benefits of intervention
• Yoshinaga‐Itano (1998)
72 kids 78 kids Diagnosis: birth –6 months vs Diagnosis after 6 months
Same intervention Conclusion Children diagnosed by 6 mths: Better receptive, expressive language skills Independent of age, gender, SES, ethnicity
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Universal newborn hearing screen
• States with newborn hearing screen – Goal is : 1‐3‐6 – Screen by 1 MONTH – Audiological diagnosis by 3 MONTHS – Early intervention by 6 MONTHS
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Etiology
Acquired 25%
Genetic 50% Unknown 25%
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Syndromic Etiologies
• More than 500 1/3 syndromic syndromes
2/3 nonsyndromic
Pendred
• Autosomal recessive • Described by Pendred in 1896 – Irish family with 2/10 kids deaf and with goiter • Goiter, not always present • Associated with Mondini and LVA
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Pendred
• Chromosome 7q21‐34 • Mutation in SLC26A4 (or PDS gene) • Encodes protein, pendrin – Transports chloride and iodide • Diagnosis: Perchlorate discharge test
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Pendred
• Phenotype – Bilateral, prelingual – more severe high frequency • Phelps, 1998 – 31 of 40 with LVA – 8 of 40 with Mondini • Recommend: PDS or SLC26A4 testing in patients with LVA or Mondini on CT
QTc: >470 ms, asymptomatic Atrophic stria, degenerated or > 460 ms with hx organ of Corti
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Jervell and Lange‐Nielson
• Meisnner (1856): “Patient was a deaf‐mute girl in the Leipzig Institute for the deaf, who collapsed and died while being publicly admonished by the director for a misdemeanor.” • Prolonged QT interval: syncope and sudden death‐ Jervell and Lange‐Nielson, 1957 • Chromosome 11 • Once identified, mortality rate <6%
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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SNHL with blindness
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Usher Syndrome
• Four types • Retinitis pigmentosa • Dx: electroretinography or electronystagmography
Type Gene location Hearing loss Vestibular 1A 14q Profound congenital Absent B 11q13 C 11p13
21q41Mod‐Severe congenital Nl or dec 3 3q Progressive Absent 4x linked Mod‐severe congenital nl or dec
• MYO7A: implicated in type 1A
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
RP with Usher
• Onset is not at birth • Affects Rods and Cones – Rods: night vision • First affected, therefore have poor night vision – Cones: bright light and fine details/color • Affected second
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Waardenburg Syndrome
• Dystopia canthorum • Flat nasal root • Confluent eyebrow • Heterochromic irides • White Forelock • Pigmentary changes • Diminished vestibular function • Cleft lip and palate
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Waardenburg Syndrome
• Autosomal dominant • Type I: with dystopia canthorum, 2p • Type 2:without dystopia, 3p • Type 3: with dystopia/ upper limb abnormalities • Type 4: No dystopia/ Hirschprung disease • 2‐5% of infants with congenital deafness • SNHL in 20% cases
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
What is dystopia canthorum???
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Alport Syndrome
• X‐linked (80%) or autosomal recessive and dominant • Progressive glomerulonephritis and SNHL • Dx: (3 of 4) – Family hx: hematuria +/‐ RF – EM evidence of glomerulonephritis – High frequency SNHL – Ophthalmologic signs: eg. retinal flecks • Associated with COL4A3, COL4A4,COL4A5 – Collagen genes
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Branchiootorenal Syndrome
• Autosomal dominant, 8q • Features – Branchial cleft fistula or cysts – Malformed pinnae – Preauricular pits/ sinuses – Hearing loss • 30% CHL • 20% SNHL • 50% mixed – Renal abnormalities • EYA1: gene for development of kidneys and inner ear
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Syndromes
Pendred Aut Recessive Goiter Perchlorate SLC26A4 (PDS) LVA/ Mondini on CT discharge test
Jervell and Lange Aut Recessive Syncope EKG prolonged QT KCNE1, KCNQ1 Nielson
Usher Syndrome Aut Recessive Blindness Electroretinography MYO7A Retinitis Pigmentosa 8 others
Branchio-Oto-Renal Aut Dominant Branchial anomalies Clinical BOR Auricular cupping/ pits Renal anomalies
Waardenburg Aut Dominant White forelock Clinical PAX3 Dystopia canthorum Synophrys Heterochromic irides
Alport Aut Dominant Hematuria Clinical COL4A3, X-linked Glomerulonephritis COL4A4,COL4A5
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Etiology: Non syndromic What is the most common inheritance?
1. Autosomal recessive 2. Autosomal dominant
2/3 3. X‐linked nonsyndromic 4. mitochondrial
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Etiology: Non syndromic
• Autosomal recessive 60‐80%
• Autosomal dominant 15‐20%
2/3 nonsyndromic • X‐linked 2‐3%
• mitochondrial <0.5%
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Autosomal dominant
• One parent usually has hearing loss • Parent with 50% risk of transmitting to child • In general, milder hearing loss • Starts in 2nd‐3rd decades of life – Allows for normal speech development
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Autosomal dominant
• DFNA loci – DFN = deafness – A = dominant • > 54 loci – www.uia.ac.be/dnalab/hh h
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Autosomal recessive
• Require inheritance from each parent • Parent usually does not have hearing loss – Parents are carriers • 25% offspring of these parents will be affected
Autosomal recessive
• DFNB loci – DFN: deafness – B=recessive • > 67 loci identified
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Connexin 26
• DFNB1 mapped to chromosome 13q12‐13 • Mutation of gene GJB2 • GJB2 encodes for connexin protein Cx26 • Mutation associated with 50% of autosomal recessive hearing loss – Carrier rate in midwest US: 2.5%
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Connexin
• Transmembrane protein that oligomerizes – 6 connexin = connexon • Gap junction protein stria vascularis, basement membrane, limbus, spiral prominence of cochlea • Inability to recirculate potassium in the inner ear – Resultant decrease of the endocochlear potential and deafness
Connexin 26
• 35delG mutation predominates • One Base‐pair deletion: Frame shift and premature protein truncation • Over 60 other non‐ 35 del G mutations
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Other mutations in GJB2 • 35 delG – 68.7% • 167 del T – most common in Ashkenazi Jews, 4% carrier • 235 del C – in Asians, 1% • V37I Missense mutation • Del342 kB
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Phenotype of GJB2 hearing loss • Typically, prelingual bilateral nonprogressive loss • Varies from mild to profound – Severe to profound most common (80%) – Small fraction only mild (<2%) – Cryns K et al, March 2004 • Homozygote 35delG had more impairment than 35delG/non‐35delG mutation
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Connexin 26 Phenotype
• Usually congenital – Reports of homozygote 35 delG passed newborn screen, later developed profound SNHL • Usually symmetric, > 20 dB in less than ¼ • Nonfluctuating, nonprogressive • No comorbidities • Normal vestibular function • Normal CT, but….
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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X‐linked Recessive
• DFN • Prevalence: <2% • 5 mapped loci • Hallmark: No male to male transmission • 50% of her sons : express the trait • 50% of her daughters : carriers for the trait
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
X linked recessive mixed deafness with perilymphatic gusher • Mixed HL • Risk of gusher with stapes • CT: lateral aspect of IAC may be bulbous • POU3F4
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Mitochondrial
• 2 to 10 copies of a small ring‐shaped piece of nonnuclear DNA, each with 16 kb • Inherited maternally • Mutations impact energy production, tissues with high energy requirements (e.g., muscle, cochlea) are particularly vulnerable
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Mitochondrial Hearing loss
• Usually associated with syndromes • With aminoglycoside ototoxic hearing loss – A1555G mutation in the 12S ribosomal RNA gene – Maternally transmitted predisposition to aminoglycoside ototoxicity
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Acquired Hearing Loss
• Infection • Ototoxins • Prematurity 25% • Kernicterus Acquired • Birth Anoxia
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Infection • CMV – Most common intrauterine infection associated with SNHL • CMV+ in 1/100‐200 newborns – 10‐15% of babies with congenital CMV asymptomatic • 5‐10% develop SNHL – DX: • Infant’s urine and saliva : PCR test • PCR on cord blood
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Ototoxins
• Aminoglycoside • Loop diuretics • Cisplatin: dose dependent • Salicylates: High dose, reversible • Ethyl Alcohol • Cocaine
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Prematurity
• Most significant perinatal factor • 20 fold increase in SNHL • 5% of NICU grads have SNHL • Risk factors of preemies – Anoxia – Low birth weight – Acidosis – Hyperbilirubinemia – Meds – Meningitis
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
What is the appropriate workup for hearing loss? • Previous to genetic tests: – History and Physical • 22‐35% of cases, may identify cause – Shotgun approach • Laboratories • CT scans
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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History
• Prenatal – environmental teratogens – maternal infections – drugs – metabolic and nutritional disturbances
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
History
• Perinatal Intrauterine hypoxia secondary to placental insufficiency Placenta previa or abruptio placenta Prolonged labor with evidence of fetal distress Prematurity Low Apgar scores at birth Birth trauma Neonatal jaundice Neonatal infection
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
History
Postnatal Infections Speech and language problems • Family history – Hearing aid before age 30 – Stigmata of syndromes – Consanguinity
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Physical exam
• Ears: shape, location, EAC • Eyes: Synophrys, Dystopia canthorum, Color of eyes, retinal pigmentation – ½ of children with severe‐profound HL with ocular abnormalities (Leguire LE, 1992) – 20% of children with ophthalmologic abnormality • Sharma, et al Archives 2009 • Face: shape, symmetry • Skin: texture, pigmentation, cafe au lait • Extremities: shape of toes, fingers
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Diagnostic Tests
• Laboratories • Genetic screen • EKG • CT/MRI
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Laboratory tests
• CBC • Chemistries • TSH • ESR • FTA/ MHA‐TP • TORCH
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Results of Laboratory Tests and CT scans
Study N Lab Tests CT Dx test ____Dx CT
Ohlms 86 N/A 70 0 14
Billings 301 N/A 156 N/A 23
Lalwani 114 789 97 0 38
Preciado 650 2676 616 2 169 ______
Totals 1151 3465 939 2(0.06%) 244(26%)
Recommend: 1) laboratory tests (eg. UA, TSH, ESR) based on history 2) Imaging every child with SNHL
Radiographic anomalies
• Incidence of CT anomalies with hearing loss: 6.8‐39% • Most common is LVA
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Inner ear abnormalities • Michel’s • Mondini’s • Scheibe’s More dysplasia • Alexander’s Less dysplasia
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Large Vestibular Aqueduct Syndrome
• Inheritance not defined • Usually bilateral • Risk of hearing loss progression with head trauma – Transmission of intracranial pressure to inner ear? • Counselling of avoiding contact sports >1.5 mm in middle third >2 mm anywhere
Michel aplasia
• Autosomal dominant • Complete aplasia of inner ear • Etio: arrest of development of the inner ear at 4th week • Contraindication for cochlear implant
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Mondini aplasia
• Described in 1791 – Cochlea 1.5 turns – Large vestibule – Large vestibular aqueduct
• Insult during 6th week • Variable severity • May be implantable
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Narrowed IAC
• May signal absent 8th nerve • Relative contraindication for implant
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
CT or MR
Pros Cons
Easier to obtain Limited information of Less sedation need cochleovestibular nerve
Cheaper CT Great bony detail: trauma
Most ENT more familiar with CT Great soft tissue/CNS Higher need for sedation evaluation More expensive Preoperative evaluation MR
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Should we use CT’s in Children?
• Cumultative dose of 50‐60 mGy – 3 fold increased risk of Brain tumor – 3 fold increased risk of leukemia
Pearce, et al. Lancet. June 2012 • 50‐60 mGy reached with – 3‐5 head CT: Brain – 5‐10 head CT: marrow Radiation Risks and Pediatric Computed Tomography (CT): A Guide for Health Care Providers. National Cancer Institute. June 7. 2012
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Algorithm for work up
• Shotgun – Order all tests all the time • Staged – Connexin first • If negative, then CT – Preciado, et al
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
What is everyone else doing?
• Duncan, et al. Archives, Mar 2007 – Questionnaire 63 of 250 ASPO members
• 70% use connexin testing • 70% use CT/ MRI • 45% answered correctly recurrence risk
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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CHP workup April 2014
Diagnosis certain Appropriate Rx
Eye exam Early intervention History/Physical Hearing Aid/CI Audiogram Follow up
Unilateral
Diagnosis unclear Genetic Studies and MRI and CT
Bilateral
OtoScope
• AUTOSOMAL RECESSIVE GENES: • CDH23, CLDN14, COL11A2, ESPN, ESSRB, GIPC3,GJB2, GJB3, GJB6, GPSM2, GRXCR1, HGF, ILDR1,LHFPL5, LOXHD1, LRTOMT, MARVELD2, MYO3A,MYO6, MYO7A, MYO15A, OTOA, OTOF, PCDH15, • PJVK, PTPRQ, RDX, SLC26A4, SLC26A5, STRC,TECTA, TMC1, TMIE, TMPRSS3, TPRN, TRIOBP,USH1C, WHRN • AUTOSOMAL DOMINANT GENES: • ACTG1, CCDC50, COCH, COL11A2, CRYM, DFNA5,DIAPH1, DSPP, EYA4, GJB2, GJB3, GJB6, GRHL2,KCNQ4, MYH14, MYH9, MYO1A, MYO6, MYO7A,POU4F3, SLC17A3, TECTA, TMC1, TJP2, WFS1 • X‐LINKED GENES:POU3F4, PRPS1 • Micro ‐RNAs and mitoc hondrial genes : • miR‐96, miR‐182, miR‐183, MT‐RNR1, MT‐TS1 • Pendred Syndrome genes:SLC26A4 • USHER SYNDROME GENES: • CDH23, CLRN1, GPR98, MYO7A, PCDH15, USH1C, USH1G, USH2A, WHRN
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
10 year old girl with severe hearing loss
Spring is my favorite season. The sun shines bright. The flowers begin to grow. I like spring.
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Normal hearing
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Degrees of hearing loss
• May range from mild to profound • Hearing aids are an option
• Severe to profound loss: – Children may not benefit from hearing aids alone
Early experience with electrical stimulation
• In 1790 Volta experienced “a boom in the head” followed by the sound of “boiling soup” when running current through metal rods held to his ears • “The disagreeable sensation, which I apprehended might be dangerous, prevented me from repeating the experiment.”
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Electrical stimulation of the ear
• Quack electrogadgetry – 19th century – Electro‐magnetic head cap • “revive the nerves of the ear”
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
• Duchenne, 1855 – Electrode in warm saline in ear canal – Sounds like crackling parchment paper – When current strength was increased, loudness increased
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
First implant
• Djourno and Eyries, 1957
– Implanted induction coil into stump of cochlear nerve in temporal bone resection patient – Transcutaneous stimulus via external induction coil
Andre Djourno Charles Eyries
Eisen Otol Neurotol 2003
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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First experiments
• Djourno and Eyries, 1957 – Implanted induction coil into stump of cochlear nerve in temporal bone resection patient – Transcutaneous stimulus via external induction coil
Eisen Otol Neurotol 2003
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
First experiments
• Djourno and Eyries, 1957 – Pt perceived environmental sounds; could distinguish high and low frequencies and several words – Could not understand speech – Paper had limited recognition because written in French
Eisen Otol Neurotol 2003
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
First experiments
• House, Los Angeles 1960’s – Work of Djourno and Eyries brought to William House by a patient – Collaborated with neurosurgeon John Doyle and engineer James Doyle to create single‐channel device – Short‐lived due to lack of biocompatible materials
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
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Pioneers
• Simmons, Stanford 1960’s – Implanted patients with a six channel device – Demonstrated that separate electrodes could stimulate tonotopically Blair Simmons
• Michelson 1970’s – Implanted 2 channel device – Congenitally deaf patient able to hum a melody after presentation of synthesized sound – Reports of outcomes a watershed for the scientific community Robin Michelson
First experiments
• House, 1970’s – House continued work with engineer Jack Urban – Collaborated with 3M to create William House House/3M unit, FDA approved for adults in 1984 • Clarke, Melbourne 1970’s – Multichannel device (eventually Cochlear Corp. Nucleus 22)
Graeme Clark
1990s to current
• Miniaturization of components • Multichannel Nucleus 22 device FDA approved • 1990‐ FDA lowered approved age to 2 • 1998: FDA approved for children 18 months Nucleus 22 device ca 1985 • 2002: FDA approves for babies of 12 months
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How does a cochlear implant work?
• Replace the hair cell function of the cochlea – Acoustic signal – Electric signal – Processed – Signal delivered to auditory nerve
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Components of CI
• External component (outside the body) – Small microphone – Speech processor – Decoding unit – Coil • Internal component – AKA Receiver‐Stimulator – Signal is transmitted via electrodes the cochlea
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
How does a cochlear implant work?
Digitized signals are transmitted from the coil to the internal receiver
The receiver sends the Sound is received signal to stimulate the by the microphone nerve ending
Sound is digitized by the speech processor
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What is it like to hear with a CI?
• Initially robotic or mechanical
• Over time, the sound perception is “natural”
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Who are the Candidates?
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Selection Criteria
• Age 24 months to 17 years – Severe to profound bilateral hearing loss – Lack of auditory development and minimal benefit from hearing aid – No medical contraindications – High motivation and realistic expectations
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Selection Criteria for Kids
• Age 12 to 24 months – Profound bilateral sensorineural hearing loss – Minimal hearing aid benefit – No medical contraindications – High motivation and realistic expectations
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Who are the non candidates?
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Who are the non candidates?
• No cochlea
• No auditory nerve
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Non‐Candidates: Those that don’t desire hearing
• “deafness” = hearing impairment, usually profound – ‐includes all persons with hearing loss • “D”eaf = a member of Deaf World – Separate culture from mainstream hearing society – Minority culture with unique customs, values, language
Deaf culture
• Deaf World – In the US, ASL is defining language – Deafness as disability rejected – Deafness is a culturally defining characteristic – Deafness is highly valuable to the Deaf Culture
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Deaf culture
• Deaf World – Deaf World has a moral claim to the well‐being of deaf children born to hearing parents • A child born deaf is a member of the Deaf world – Cochlear implantation is antithetical to the principles of Deaf culture – www.cochlearwar.com
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How about younger than 12 months?
• Becoming possible because of UNHS
• Considered based on extrapolated data from Hearing Aids – Goal of amplification by 6 months
• Feasible because cochlea adult size by birth
Typical scenario
• A 3 month old girl diagnosed with newborn screen • Bilateral profound loss • No response to Hearing aids • Connexin positive • Parents read on‐line that implants are done before 12 months
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Is it safe to implant at less than 12 months?
• Roland JT (Laryngoscope, Nov 2009) – 50 infants, ages 5‐11 months – No anesthesia problems
• Blood volume: 80 ml/kg in infants <12 months – 10% loss can lead to hypovolemia
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Is there a benefit to implant before 12 months?
• Schauwers (Otol and Neurotol, 2004) – 5 children – Ages 5‐10 months – Approach normal for babbling and CAP scores • Colletti (Laryngoscope, 2005) – 10 children – Age 4 –11 months – Approached normal age for babbling (6‐11 mths)
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Follow up data
• Colletti (Acta Otolaryngologica, 2009) – 4‐9 year follow up of initial group – Compared group implanted 4‐11 months to those implanted 12‐23 months and 24‐36 months. – Children implanted early had quicker outcomes to reach normal – At 5 years, • 0 children in special schools • 30% implanted age 12‐23 months • 60% implanted age 24‐36 months
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Expanding Indications
• Hybrid cochlear implants
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Bilateral Cochlear Implants
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Drawbacks of Bilateral Cochlear Implantation • Future Technology – The bilateral implant may prevent the use of other technologies in the future • Hair Cell Regeneration • Stem cells • Drug delivery systems • Better cochlear implant
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Hair Cell Regeneration
• Gene Atoh1 injected
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Despite these risks….
• New technologies are not likely to be available for 20 years. • Benefits – Sound localization – Hearing in noise • While waiting, there may be negative consequences…
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Changes in the contralateral unstimulated ear • Leake , 1999 – Deafened kittens – Measured SGC survival – Ipsilateral stimulated ear: 50% – Contralateral unstimulated ear: 30%
What about for unilateral hearing loss?
• Questions – How does brain adapt to acoustic signal in one ear and electric signal in another? – Benefits? – Risk? – Cost?
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Unilateral hearing loss
• Adults (primarily European studies) – Improved speech recognition scores – Suppression of tinnitus – Improved balance – Improved sound localization • Better than compared with BAHA or CROS aid
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Costs of CI
• K‐12 education per hearing child‐ $9000 • K‐12 education per mainstreamed deaf child‐ $44,000 • K‐12 education per child in residential school for the deaf‐ $429,000 (Johnson et. al. Seminars in Hearing 1996)
• $60,228 lifetime cost for medical care and associated services • $113,426 lifetime reduction in expenses from decreased educational costs and increased expected earnings • Net expected lifetime savings of $53,198 for CI vs. no CI
(Cheng et. al. 2000)
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
A Look to the Future
• Totally implanted CI
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Role of PA/NP in Hearing Center
• Jennifer Hanselman, PA‐C
• Coordinate visits • Manage database of Hearing Center patients • Participate in the evaluation of patients
Fourth Annual ENT for the PA-C | April 24-27, 2014 | Pittsburgh, PA
Conclusion
• Hearing loss is the most common birth condition, occurring in 12,000 US births per year
• Cochlear implants stimulate the auditory nerve directly
• Cochlear implants allow children with severe to profound hearing loss to develop speech and language
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