Pediatric Hearing Loss Pediatric Hearing Loss

Franklin L. Rimell, MD FACSFranklin L. Rimell, MD FACS

Associate Professor, DirectorAssociate Professor, Director

Pediatric Otolaryngology / Head & Neck SurgeryPediatric Otolaryngology / Head & Neck Surgery

University of MinnesotaUniversity of Minnesota

Boys Town National Research Hospital

Prevalence and IncidencePrevalence and Incidence

• Every day, 33 babies (12,000 each year) are born in the Every day, 33 babies (12,000 each year) are born in the United States with permanent hearing loss. United States with permanent hearing loss.

• 3/1000 newborns have hearing loss. 3/1000 newborns have hearing loss. 11

0

5

10

15

20

25

30

35

Hearing loss Cleft lip orpalate

Downsyndrome

Limb defects Spina bifida Sickle cellanemia

PKU

Congenital Condition Type

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Boys Town National Research Hospital

• Incidence increases by school age to 6/1000.– late identification

– late onset – progressive hearing losses

Prevalence and Incidence

• 930,000 children with mild to severe HL 6-19

years of age. 2

Boys Town National Research Hospital

What Do We Know about Outcomes of What Do We Know about Outcomes of Children Who are HH?Children Who are HH?

• Most studies have focused on children with Most studies have focused on children with severe to profound hearing losssevere to profound hearing loss

• Children with unidentified mild to severe Children with unidentified mild to severe hearing loss are at risk for poorer:hearing loss are at risk for poorer:

• LanguageLanguage

• AcademicsAcademics

• Social skillsSocial skills

• Psychological outcomesPsychological outcomes

Boys Town National Research Hospital

All degrees of HL place children at riskAll degrees of HL place children at risk

• Children with a Children with a unilateral hearing loss unilateral hearing loss

are are ten timesten times as as likely to be held back likely to be held back at least one grade.at least one grade. 33

• Children with minimal Children with minimal losses:losses:– 37% fail one grade37% fail one grade

– 8% don’t have skills at grade 8% don’t have skills at grade levellevel

– 12-41% receive educational 12-41% receive educational assistanceassistance

“Minimal Is not inconsequential”

~Fred Bess

Boys Town National Research Hospital

Speech Production & Language Speech Production & Language OutcomesOutcomes

• Phonemic and syllabic speech patterns are Phonemic and syllabic speech patterns are delayed even for children with mild to delayed even for children with mild to moderate HL moderate HL 4-74-7

• Unidentified children are at risk for delayed: Unidentified children are at risk for delayed: 88 – vocabulary vocabulary – word learningword learning– advanced syntaxadvanced syntax– morphology morphology – social use of languagesocial use of language

EHDI and the Medical Home

Parent Groups Mental Health

Hospital Screening

Audiology

Primary Provider

Child/Family

ENT

GeneticsEI Therapists

3rd Party Payors

Deaf Community

Interpreters Deaf Services

Hearing LossHearing Loss

Characterized broadly by Characterized broadly by degreedegree, , configuration configuration and and typetype, , – Degree: amount of hearing loss in relationship Degree: amount of hearing loss in relationship

to normal auditory functionto normal auditory function– Configuration: overall ‘shape’ or pattern of the Configuration: overall ‘shape’ or pattern of the

hearing loss as displayed on the conventional hearing loss as displayed on the conventional audiogram in dB HL as a function of frequencyaudiogram in dB HL as a function of frequency

– Type: site (location) of the auditory disorderType: site (location) of the auditory disorder

Degree: Categories of HLDegree: Categories of HL

• NormalNormal• Borderline (Minimal)Borderline (Minimal)• MildMild• ModerateModerate• Moderate-severeModerate-severe• SevereSevere• ProfoundProfound

• -10 to 15 dB HL-10 to 15 dB HL• 16 to 25 dB HL16 to 25 dB HL• 26 to 40 dB HL26 to 40 dB HL• 41 to 55 dB HL41 to 55 dB HL• 56 to 70 dB HL56 to 70 dB HL• 71 to 90 dB HL71 to 90 dB HL• > 90 dB HL> 90 dB HL

New York State Department of Health

Types of Hearing Loss:Types of Hearing Loss:ConductiveConductive

• Reduction of air-conductive sound delivered to the Reduction of air-conductive sound delivered to the normal cochlea during transmission through a normal cochlea during transmission through a disordered disordered outer earouter ear and/or and/or middle earmiddle ear

• Sound reaching cochlea Sound reaching cochlea attenuated attenuated to some degree to some degree (in OME or debris ~25 dB HL; in complete (in OME or debris ~25 dB HL; in complete atresia, maximally ~60 dB HL)atresia, maximally ~60 dB HL)

• Excellent speech perception when incoming Excellent speech perception when incoming acoustic signal is sufficiently intenseacoustic signal is sufficiently intense

Causes of Conductive LossCauses of Conductive Loss

• Diseases of the ear canalDiseases of the ear canal

• Diseases of the tympanic membraneDiseases of the tympanic membrane

• Diseases of the middle ear spaceDiseases of the middle ear space

• Diseases of the ossiclesDiseases of the ossicles

• Diseases of the eustachian tubeDiseases of the eustachian tube

• Diseases of the facial nerveDiseases of the facial nerve

• Diseases of the Jugular Vein or Carotid A.Diseases of the Jugular Vein or Carotid A.

Conductive Hearing LossConductive Hearing Loss

• In Theory, all forms of conductive hearing In Theory, all forms of conductive hearing loss are medically or surgically correctableloss are medically or surgically correctable

• In practice, not all can beIn practice, not all can be

Type of Hearing Loss: Type of Hearing Loss: SensorySensory

• Damage to outer or outer and inner hair cells Damage to outer or outer and inner hair cells of the cochleaof the cochlea

• Differing impact on speech perception Differing impact on speech perception depending on degree and configuration of depending on degree and configuration of hearing losshearing loss

• Multiple audiometric configurations Multiple audiometric configurations

• Any degree of hearing lossAny degree of hearing loss

Sensorineural Hearing LossSensorineural Hearing Loss

• In theory, all forms of sensorineural hearing In theory, all forms of sensorineural hearing loss can be addressed surgically if bilateral, loss can be addressed surgically if bilateral, child’s brain has no injury, and family child’s brain has no injury, and family chooses. chooses.

• Timing is critical.Timing is critical.

• None can be treated medically to date.None can be treated medically to date.

Types of Hearing Loss:Types of Hearing Loss:MixedMixed

• BothBoth sensorysensory component and overlying component and overlying conductiveconductive component component

• Example: child with sensory loss who Example: child with sensory loss who experiences OMEexperiences OME

• BilateralBilateral

• Unilateral (in one ear only)Unilateral (in one ear only)

Hearing Loss: Hearing Loss: Characterized by Ear(s) AffectedCharacterized by Ear(s) Affected

Listening to Parental Concern About Listening to Parental Concern About Delayed Language DevelopmentDelayed Language Development

Gravel & VohrGravel & Vohr

Listening to parent concern about Listening to parent concern about language developmentlanguage development

• Parent concerns about hearing, speech, Parent concerns about hearing, speech, language, or developmental delays are language, or developmental delays are strong predictors of an actual problem.strong predictors of an actual problem.

• Providers must avoid statements like Providers must avoid statements like “Babies develop at different rates. Lets take “Babies develop at different rates. Lets take another look in about 6 months”another look in about 6 months”

Myths about hearing and early speech Myths about hearing and early speech language delaylanguage delay

• We don’t have to worry because:We don’t have to worry because:• His older brothers and sisters talk for himHis older brothers and sisters talk for him• Boys develop speech much later than girlsBoys develop speech much later than girls• Twins always have language delaysTwins always have language delays• Grandma says that her Dad did not speak until Grandma says that her Dad did not speak until

he was 3 years old.he was 3 years old.• She has great motor milestonesShe has great motor milestones• I know he hears because he gets upset every I know he hears because he gets upset every

time I turn on the vacuum.time I turn on the vacuum.

It is important to respond to concerns about language It is important to respond to concerns about language immediately !immediately !

Most children with delays of speech and Most children with delays of speech and language respond to appropriate medical, language respond to appropriate medical, audiologic, and/or educational audiologic, and/or educational interventions.interventions.

A successful early screening, identification, A successful early screening, identification, and intervention program will ultimately and intervention program will ultimately permit every child with HL to develop to permit every child with HL to develop to his/her potential.his/her potential.

Characteristics of Children with Hearing LossCharacteristics of Children with Hearing Loss

Site Rate

Well Baby Nursery 1 per 1000

NICU 10 per 1000

Total population 2-3 per 1000

# infants ident annually US 8,000-16,000

Average career pediatrician 12 patients

Most Common Specific Environmental Risk CausesMost Common Specific Environmental Risk Causes

CMV > 1 risk factor

Meningitis Perinatal Asphyxia

Prematurity < 1500 g Ototoxic medications

Some Infants pass their hearing screen and have Some Infants pass their hearing screen and have late onset hearing losslate onset hearing loss

Some of these infants have a risk Some of these infants have a risk factor and some have no risk factor factor and some have no risk factor known to the family or physician.known to the family or physician.

Risk indicators from 29 d to 2 years of ageRisk indicators from 29 d to 2 years of age

• Stigmata or Syndrome associated with HLStigmata or Syndrome associated with HL

• In-utero infectionsIn-utero infections

• Postnatal infectionsPostnatal infections

• Neonatal indicators such as ECMONeonatal indicators such as ECMO

• Neurodegenerative disordersNeurodegenerative disorders

• Head traumaHead trauma

• Recurrent or persistent OMRecurrent or persistent OM

Risk Indicators obtained from the familyRisk Indicators obtained from the family

• Parent or caregiver concern regarding hearing, Parent or caregiver concern regarding hearing, speech, language, or developmental delay (parent speech, language, or developmental delay (parent concern has been shown to be a good predictor).concern has been shown to be a good predictor).

• Family history of permanent HL in first or second Family history of permanent HL in first or second degree relatives with onset by 30y or age.degree relatives with onset by 30y or age.

Causes of Permanent Hearing Loss in 100 Causes of Permanent Hearing Loss in 100 Infants Infants

50% Environmental 50

50% Genetic

30% syndromes (>300) 30

20% >75 genes ident 20

½ are GJB2 – Connexin 26

Genetic CausesGenetic Causes

Single gene Connexin 26

Gene + environment Mitochondrial + ototoxic

Gene + gene Gene + other gene

Early “Early Intervention” for Hearing Early “Early Intervention” for Hearing Loss is Important becauseLoss is Important because

• There are dramatic benefits associated with early There are dramatic benefits associated with early identification and intervention for hearing loss identification and intervention for hearing loss before 6 months of agebefore 6 months of age..

• Children identified and receiving services < 6 m Children identified and receiving services < 6 m have larger vocabularies, better comprehension have larger vocabularies, better comprehension and better expressive language than children and better expressive language than children identified > 6 m.identified > 6 m.

The Medical WorkupThe Medical Workup

• Complete prenatal & perinatal hxComplete prenatal & perinatal hx

• Family Hx of onset of HL < age 30Family Hx of onset of HL < age 30

• Physical for stigmata, ear tabs, cleft Physical for stigmata, ear tabs, cleft palate, cardiac, skeletal, microcephalypalate, cardiac, skeletal, microcephaly

• Refer to ENT / CT of temporal bones Refer to ENT / CT of temporal bones and or MRI (sedation)and or MRI (sedation)

• Refer to Genetics and OpthalmologyRefer to Genetics and Opthalmology

• Other: CMV, EKG Other: CMV, EKG

XraysXrays

• CT scan of the earsCT scan of the ears– Shows the outside shell of the cochleaShows the outside shell of the cochlea– May need sedationMay need sedation

• MRI of the inner earMRI of the inner ear– Shows the nerveShows the nerve– Always needs sedationAlways needs sedation– Experimental (shows inside the cochlea)Experimental (shows inside the cochlea)

What are some of the questions to ask ?What are some of the questions to ask ?

• Antenatal history- maternal illness during Antenatal history- maternal illness during the pregnancy or deliverythe pregnancy or delivery

• Neonatal complications, prematurity, Neonatal complications, prematurity, jaundice, asphyxia, assisted ventilation, jaundice, asphyxia, assisted ventilation, ECMOECMO

Examination for CausesExamination for Causes

• Evaluate for dysmorphic features, minor and Evaluate for dysmorphic features, minor and major stigmata and syndromesmajor stigmata and syndromes

• Other anomalies – visual, facial, endocrine, Other anomalies – visual, facial, endocrine, cardiac, kidney, hair, and skincardiac, kidney, hair, and skin

• Particular attention to the head and neck. HL Particular attention to the head and neck. HL may be associated with abn. pinna, atresia or may be associated with abn. pinna, atresia or stenosis of the ear canal, ear tags, and bony stenosis of the ear canal, ear tags, and bony growths in the ear canal.growths in the ear canal.

• Cleft lip and palate may have middle ear fluidCleft lip and palate may have middle ear fluid

What to ask about family history ?What to ask about family history ?

• Is there a family hx of onset of permanent Is there a family hx of onset of permanent HL HL << 30 years of age ( over 3 generations) 30 years of age ( over 3 generations)

• Are there other family members with Are there other family members with hearing loss, syndromes or anomalies ?hearing loss, syndromes or anomalies ?

When to refer to OphthalmologyWhen to refer to Ophthalmology

• First- Follow periodicity schedule for all patientsFirst- Follow periodicity schedule for all patients

• Some syndromes with permanent HL have Some syndromes with permanent HL have specific eye findings such as heterochromia in specific eye findings such as heterochromia in Wardenburgs.Wardenburgs.

• In Ushers the child is at risk of late onset vision In Ushers the child is at risk of late onset vision loss secondary to retinitis pigmentosa. loss secondary to retinitis pigmentosa.

When to get an EKG ?When to get an EKG ?

• This is ordered to rule out long QT ( Jervell This is ordered to rule out long QT ( Jervell and Lange-Nielsen )syndrome.and Lange-Nielsen )syndrome.

• This syndrome may manifest itself with This syndrome may manifest itself with apnea, passing out episodes, or a history of apnea, passing out episodes, or a history of sudden death in a relative.sudden death in a relative.

Considerations in Determining the Considerations in Determining the Appropriateness of Cochlear Appropriateness of Cochlear

Implantation including Risks, Benefits & Implantation including Risks, Benefits & TimingTiming

How is a Cochlear Implant Different From a Hearing Aid?

Hearing Aid Cochlear Implant

Acoustically amplify sound.

Convert sound into electrical signals.

Rely on the responsiveness of healthy inner ear sensory cells.

Bypass the inner ear sensory cells and stimulate the hearing nerve directly.

What are the Candidacy Criteria for a What are the Candidacy Criteria for a Cochlear Implant?Cochlear Implant?

• Limited benefit from conventional amplification Limited benefit from conventional amplification following a minimum of 3-6 months use following a minimum of 3-6 months use – May be sooner following deafness from meningitisMay be sooner following deafness from meningitis

• Profound hearing loss: (sensorineural loss only)Profound hearing loss: (sensorineural loss only)– 12-18 months 12-18 months

• Severe-to-Profound hearing lossSevere-to-Profound hearing loss– >18 months>18 months

• Motivated, involved family Motivated, involved family • Educational support to develop auditory skillsEducational support to develop auditory skills

From: www.babyhearing.org

Who is a Candidate for a Cochlear Implant?

Range of hearing for a

cochlear implant

candidate.

Adults (18 years+)• Severe to Profound, bilateral sensorineural hearing loss• Less than 50% speech recognition with hearing aids on

open-set sentence recognition

Children (12 months - 2 years)• Profound, bilateral sensorineural deafness (> 90 dB HL)

• Little or no benefit from hearing aids

Children (2 years - 17 years)• Severe to Profound, bilateral sensorineural deafness

• Little or no benefit from hearing aids

Who is a Candidate for a Cochlear Implant?

Cochlear ImplantsCochlear Implants

• Acoustic signal picked up by microphone located in headset Acoustic signal picked up by microphone located in headset worn at ear levelworn at ear level

• Cord carries sound from microphone to a speech processor Cord carries sound from microphone to a speech processor • Speech processor digitizes sound into coded signalsSpeech processor digitizes sound into coded signals• Coded signals sent up to the transmitting coilCoded signals sent up to the transmitting coil• Coil sends coded signals as FM radio signals to CI under the Coil sends coded signals as FM radio signals to CI under the

skinskin• CI delivers electrical energy to the electrode array inserted into CI delivers electrical energy to the electrode array inserted into

the cochleathe cochlea• Electrodes along the array stimulate remaining nerve fibers in Electrodes along the array stimulate remaining nerve fibers in

cochleacochlea

Cochlear Corp. 2002

Selecting a Cochlear Implant CenterSelecting a Cochlear Implant Center• Experienced cochlear implant teamExperienced cochlear implant team

– Audiologist, speech-language pathologist, surgeonAudiologist, speech-language pathologist, surgeon– Others: educator of the deaf, psychologist, social workerOthers: educator of the deaf, psychologist, social worker

• Comprehensive program covering eligibility, surgery, Comprehensive program covering eligibility, surgery, device activation, and long-term habilitation.device activation, and long-term habilitation.– Multi-disciplinary, family centered approach. Multi-disciplinary, family centered approach. – Knowledgeable regarding deafness, child development, Knowledgeable regarding deafness, child development,

and speech, language and auditory developmentand speech, language and auditory development– Offers intervention program and continued Offers intervention program and continued – Follow-up for changes in cochlear implant mappingFollow-up for changes in cochlear implant mapping

BenefitsBenefits

• Similar to early amplification provision, Similar to early amplification provision, children implanted at early ages with more children implanted at early ages with more experience tend to do better developing experience tend to do better developing auditory skills and speech than older auditory skills and speech than older children who receive implants after greater children who receive implants after greater period of deafness period of deafness

• Sound waves enter through the microphone.

• The sound processor converts the sound into a distinctive digital code.

• The electrically coded signal is transmitted across the skin through the headpiece to the

internal portion of the device.

• The internal device delivers the sound to the electrodes.

• The electrodes stimulate the hearing nerve.

• The hearing nerve sends the signal to the brain for processing.

How Does a Cochlear Implant Work?

Electrode Array

3 turn gold wire coil

Internal Electronics

Removable Magnet

Internal EquipmentHiRes™ 90K

Cochlear ImplantsCochlear Implants

Amplification Options for Hearing Loss

Hearing Aids

Team Approach

Psychologist

SLP, if speech chosen

Audiologist

Care Givers

Surgeon &Pediatrician

Teacher ofThe Deaf

Child

Medical EvaluationMedical Evaluation• Determine cause of hearing loss Determine cause of hearing loss

• Assess status of middle ear & cochleaAssess status of middle ear & cochlea

• CT scan/MRICT scan/MRI

• CounselingCounseling– Hearing lossHearing loss

– The surgical procedureThe surgical procedure

• Typically out-patient and performed by an otolaryngologist Typically out-patient and performed by an otolaryngologist (ENT) or otologist (ear specialist)(ENT) or otologist (ear specialist)

– Post surgical considerationsPost surgical considerations

• Bilateral ImplantsBilateral Implants

– Industry trendIndustry trend

– Simultaneous vs. SequentialSimultaneous vs. Sequential

– Benefits:Benefits:• Improved directionalityImproved directionality

• Improved listening in noiseImproved listening in noise

• Clarity of speechClarity of speech

• DevelopmentalDevelopmental

Bilateral Cochlear ImplantsBilateral Cochlear Implants

Risks of Implant SurgeryRisks of Implant Surgery

• Facial nerve injuryFacial nerve injury

• Device infectionDevice infection

• Device failureDevice failure

• Menningits Menningits

• Poor insertionPoor insertion

• Does not workDoes not work

• Can I go back to a hearing aidCan I go back to a hearing aid

About SurgeryAbout Surgery

• Surgery takes 3 to 4 hoursSurgery takes 3 to 4 hours

• Incision is 2” behind the ear and 3” longIncision is 2” behind the ear and 3” long

• Bilateral simultaneous surgery 5 to 6 hoursBilateral simultaneous surgery 5 to 6 hours

• Minimal bleedingMinimal bleeding

• Risk of CSF leakRisk of CSF leak

• Facial nerveFacial nerve

• Audiology Audiology

• RehabilitationRehabilitation

• Family CommitmentFamily Commitment

• School SupportSchool Support

Pediatric Support

Unilateral Implant vs. BilateralUnilateral Implant vs. Bilateral

• Sequential: Should do second implant with Sequential: Should do second implant with in 2 to 3 yearsin 2 to 3 years

• SimultaneousSimultaneous

Having Binaural Hearing?Having Binaural Hearing?

““I could appreciate music better.”I could appreciate music better.”

““I could identify the talker in a group better I could identify the talker in a group better and easier.”and easier.”

““I felt more confident in classes and meeting.”I felt more confident in classes and meeting.”

““I liked the quality of my own voice.”I liked the quality of my own voice.”

““The other peoples’ voices sounded more The other peoples’ voices sounded more natural.”natural.”

Unilateral Deafness ?Unilateral Deafness ?

• What about schoolWhat about school

• What can be done ?What can be done ?

Bottom LineBottom Line

• Children who are deaf and hard of hearing need to be Children who are deaf and hard of hearing need to be identified as early as possible in order to have access to identified as early as possible in order to have access to language development.language development.

• Family choice is supported and respectedFamily choice is supported and respected

• For families choosing auditory/oral communication For families choosing auditory/oral communication component, ENT care can provide assistance with:component, ENT care can provide assistance with:

– AmplificationAmplification

– Medical decisionsMedical decisions

– Surgical optionsSurgical options

Question-and-AnswerQuestion-and-Answer