Pediatric Hearing Loss Franklin L. Rimell, MD FACS


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Pediatric Hearing Loss Franklin L. Rimell, MD FACS

  1. 1. Pediatric Hearing Loss Franklin L. Rimell, MD FACS Associate Professor, Director Pediatric Otolaryngology / Head & Neck Surgery University of Minnesota
  2. 2. Prevalence and Incidence <ul><li>Every day, 33 babies (12,000 each year) are born in the United States with permanent hearing loss. </li></ul><ul><li>3/1000 newborns have hearing loss. 1 </li></ul>Boys Town National Research Hospital
  3. 3. Boys Town National Research Hospital <ul><li>Incidence increases by school age to 6/1000. </li></ul><ul><ul><li>late identification </li></ul></ul><ul><ul><li>late onset </li></ul></ul><ul><ul><li>progressive hearing losses </li></ul></ul>Prevalence and Incidence <ul><ul><li>930,000 children with mild to severe HL 6-19 years of age. 2 </li></ul></ul>
  4. 4. What Do We Know about Outcomes of Children Who are HH? <ul><li>Most studies have focused on children with severe to profound hearing loss </li></ul><ul><li>Children with unidentified mild to severe hearing loss are at risk for poorer: </li></ul><ul><ul><ul><li>Language </li></ul></ul></ul><ul><ul><ul><li>Academics </li></ul></ul></ul><ul><ul><ul><li>Social skills </li></ul></ul></ul><ul><ul><ul><li>Psychological outcomes </li></ul></ul></ul>Boys Town National Research Hospital
  5. 5. All degrees of HL place children at risk <ul><li>Children with a unilateral hearing loss are ten times as likely to be held back at least one grade. 3 </li></ul><ul><li>Children with minimal losses: </li></ul><ul><ul><li>37% fail one grade </li></ul></ul><ul><ul><li>8% don’t have skills at grade level </li></ul></ul><ul><ul><li>12-41% receive educational assistance </li></ul></ul>Boys Town National Research Hospital “ Minimal Is not inconsequential” ~ Fred Bess
  6. 6. Speech Production & Language Outcomes <ul><li>Phonemic and syllabic speech patterns are delayed even for children with mild to moderate HL 4-7 </li></ul><ul><li>Unidentified children are at risk for delayed: 8 </li></ul><ul><ul><li>vocabulary </li></ul></ul><ul><ul><li>word learning </li></ul></ul><ul><ul><li>advanced syntax </li></ul></ul><ul><ul><li>morphology </li></ul></ul><ul><ul><li>social use of language </li></ul></ul>Boys Town National Research Hospital
  7. 7. EHDI and the Medical Home Parent Groups Mental Health Hospital Screening Audiology Primary Provider Child/Family ENT Genetics EI Therapists 3rd Party Payors Deaf Community Interpreters Deaf Services
  8. 8. Hearing Loss <ul><li>Characterized broadly by degree , configuration and type , </li></ul><ul><ul><li>Degree: amount of hearing loss in relationship to normal auditory function </li></ul></ul><ul><ul><li>Configuration: overall ‘shape’ or pattern of the hearing loss as displayed on the conventional audiogram in dB HL as a function of frequency </li></ul></ul><ul><ul><li>Type: site (location) of the auditory disorder </li></ul></ul>
  9. 9. Degree: Categories of HL <ul><li>Normal </li></ul><ul><li>Borderline (Minimal) </li></ul><ul><li>Mild </li></ul><ul><li>Moderate </li></ul><ul><li>Moderate-severe </li></ul><ul><li>Severe </li></ul><ul><li>Profound </li></ul><ul><li>-10 to 15 dB HL </li></ul><ul><li>16 to 25 dB HL </li></ul><ul><li>26 to 40 dB HL </li></ul><ul><li>41 to 55 dB HL </li></ul><ul><li>56 to 70 dB HL </li></ul><ul><li>71 to 90 dB HL </li></ul><ul><li>> 90 dB HL </li></ul>New York State Department of Health
  10. 11. Types of Hearing Loss: Conductive <ul><li>Reduction of air-conductive sound delivered to the normal cochlea during transmission through a disordered outer ear and/or middle ear </li></ul><ul><li>Sound reaching cochlea attenuated to some degree (in OME or debris ~25 dB HL; in complete atresia, maximally ~60 dB HL) </li></ul><ul><li>Excellent speech perception when incoming acoustic signal is sufficiently intense </li></ul>
  11. 12. Causes of Conductive Loss <ul><li>Diseases of the ear canal </li></ul><ul><li>Diseases of the tympanic membrane </li></ul><ul><li>Diseases of the middle ear space </li></ul><ul><li>Diseases of the ossicles </li></ul><ul><li>Diseases of the eustachian tube </li></ul><ul><li>Diseases of the facial nerve </li></ul><ul><li>Diseases of the Jugular Vein or Carotid A. </li></ul>
  12. 13. Conductive Hearing Loss <ul><li>In Theory, all forms of conductive hearing loss are medically or surgically correctable </li></ul><ul><li>In practice, not all can be </li></ul>
  13. 15. Type of Hearing Loss: Sensory <ul><li>Damage to outer or outer and inner hair cells of the cochlea </li></ul><ul><li>Differing impact on speech perception depending on degree and configuration of hearing loss </li></ul><ul><li>Multiple audiometric configurations </li></ul><ul><li>Any degree of hearing loss </li></ul>
  14. 17. Sensorineural Hearing Loss <ul><li>In theory, all forms of sensorineural hearing loss can be addressed surgically if bilateral, child’s brain has no injury, and family chooses. </li></ul><ul><li>Timing is critical. </li></ul><ul><li>None can be treated medically to date. </li></ul>
  15. 18. Types of Hearing Loss: Mixed <ul><li>Both sensory component and overlying conductive component </li></ul><ul><li>Example: child with sensory loss who experiences OME </li></ul>
  16. 19. <ul><li>Bilateral </li></ul><ul><li>Unilateral (in one ear only) </li></ul>Hearing Loss: Characterized by Ear(s) Affected
  17. 20. Listening to Parental Concern About Delayed Language Development Gravel & Vohr
  18. 21. Listening to parent concern about language development <ul><li>Parent concerns about hearing, speech, language, or developmental delays are strong predictors of an actual problem. </li></ul><ul><li>Providers must avoid statements like “Babies develop at different rates. Lets take another look in about 6 months” </li></ul>
  19. 22. Myths about hearing and early speech language delay <ul><li>We don’t have to worry because: </li></ul><ul><li>His older brothers and sisters talk for him </li></ul><ul><li>Boys develop speech much later than girls </li></ul><ul><li>Twins always have language delays </li></ul><ul><li>Grandma says that her Dad did not speak until he was 3 years old. </li></ul><ul><li>She has great motor milestones </li></ul><ul><li>I know he hears because he gets upset every time I turn on the vacuum. </li></ul>
  20. 23. It is important to respond to concerns about language immediately ! <ul><li>Most children with delays of speech and language respond to appropriate medical, audiologic, and/or educational interventions. </li></ul><ul><li>A successful early screening, identification, and intervention program will ultimately permit every child with HL to develop to his/her potential. </li></ul>
  21. 24. Characteristics 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
  22. 25. Most Common Specific Environmental Risk Causes CMV > 1 risk factor Meningitis Perinatal Asphyxia Prematurity < 1500 g Ototoxic medications
  23. 26. Some Infants pass their hearing screen and have late onset hearing loss Some of these infants have a risk factor and some have no risk factor known to the family or physician.
  24. 27. Risk indicators from 29 d to 2 years of age <ul><li>Stigmata or Syndrome associated with HL </li></ul><ul><li>In-utero infections </li></ul><ul><li>Postnatal infections </li></ul><ul><li>Neonatal indicators such as ECMO </li></ul><ul><li>Neurodegenerative disorders </li></ul><ul><li>Head trauma </li></ul><ul><li>Recurrent or persistent OM </li></ul>
  25. 28. Risk Indicators obtained from the family <ul><li>Parent or caregiver concern regarding hearing, speech, language, or developmental delay (parent concern has been shown to be a good predictor). </li></ul><ul><li>Family history of permanent HL in first or second degree relatives with onset by 30y or age. </li></ul>
  26. 29. Causes of Permanent Hearing Loss in 100 Infants 50% Environmental 50 50% Genetic 30% syndromes (>300) 30 20% >75 genes ident 20 ½ are GJB2 – Connexin 26
  27. 30. Genetic Causes Single gene Connexin 26 Gene + environment Mitochondrial + ototoxic Gene + gene Gene + other gene
  28. 31. Early “Early Intervention” for Hearing Loss is Important because <ul><li>There are dramatic benefits associated with early identification and intervention for hearing loss before 6 months of age . </li></ul><ul><li>Children identified and receiving services < 6 m have larger vocabularies, better comprehension and better expressive language than children identified > 6 m. </li></ul>
  29. 32. The Medical Workup <ul><li>Complete prenatal & perinatal hx </li></ul><ul><li>Family Hx of onset of HL < age 30 </li></ul><ul><li>Physical for stigmata, ear tabs, cleft palate, cardiac, skeletal, microcephaly </li></ul><ul><li>Refer to ENT / CT of temporal bones and or MRI (sedation) </li></ul><ul><li>Refer to Genetics and Opthalmology </li></ul><ul><li>Other: CMV, EKG </li></ul>
  30. 33. Xrays <ul><li>CT scan of the ears </li></ul><ul><ul><li>Shows the outside shell of the cochlea </li></ul></ul><ul><ul><li>May need sedation </li></ul></ul><ul><li>MRI of the inner ear </li></ul><ul><ul><li>Shows the nerve </li></ul></ul><ul><ul><li>Always needs sedation </li></ul></ul><ul><ul><li>Experimental (shows inside the cochlea) </li></ul></ul>
  31. 34. What are some of the questions to ask ? <ul><li>Antenatal history- maternal illness during the pregnancy or delivery </li></ul><ul><li>Neonatal complications, prematurity, jaundice, asphyxia, assisted ventilation, ECMO </li></ul>
  32. 35. Examination for Causes <ul><li>Evaluate for dysmorphic features, minor and major stigmata and syndromes </li></ul><ul><li>Other anomalies – visual, facial, endocrine, cardiac, kidney, hair, and skin </li></ul><ul><li>Particular attention to the head and neck. HL may be associated with abn. pinna, atresia or stenosis of the ear canal, ear tags, and bony growths in the ear canal. </li></ul><ul><li>Cleft lip and palate may have middle ear fluid </li></ul>
  33. 36. What to ask about family history ? <ul><li>Is there a family hx of onset of permanent HL < 30 years of age ( over 3 generations) </li></ul><ul><li>Are there other family members with hearing loss, syndromes or anomalies ? </li></ul>
  34. 37. When to refer to Ophthalmology <ul><li>First- Follow periodicity schedule for all patients </li></ul><ul><li>Some syndromes with permanent HL have specific eye findings such as heterochromia in Wardenburgs. </li></ul><ul><li>In Ushers the child is at risk of late onset vision loss secondary to retinitis pigmentosa. </li></ul>
  35. 38. When to get an EKG ? <ul><li>This is ordered to rule out long QT ( Jervell and Lange-Nielsen )syndrome. </li></ul><ul><li>This syndrome may manifest itself with apnea, passing out episodes, or a history of sudden death in a relative. </li></ul>
  36. 39. Considerations in Determining the Appropriateness of Cochlear Implantation including Risks, Benefits & Timing
  37. 40. 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.
  38. 41. What are the Candidacy Criteria for a Cochlear Implant? <ul><li>Limited benefit from conventional amplification following a minimum of 3-6 months use </li></ul><ul><ul><li>May be sooner following deafness from meningitis </li></ul></ul><ul><li>Profound hearing loss: (sensorineural loss only) </li></ul><ul><ul><li>12-18 months </li></ul></ul><ul><li>Severe-to-Profound hearing loss </li></ul><ul><ul><li>>18 months </li></ul></ul><ul><li>Motivated, involved family </li></ul><ul><li>Educational support to develop auditory skills </li></ul>From:
  39. 42. Who is a Candidate for a Cochlear Implant? Range of hearing for a cochlear implant candidate.
  40. 43. <ul><li>Adults (18 years+) </li></ul><ul><li>Severe to Profound, bilateral sensorineural hearing loss </li></ul><ul><li>Less than 50% speech recognition with hearing aids on open-set sentence recognition </li></ul><ul><li>Children (12 months - 2 years) </li></ul><ul><li>Profound, bilateral sensorineural deafness ( > 90 dB HL) </li></ul><ul><li>Little or no benefit from hearing aids </li></ul><ul><li>Children (2 years - 17 years) </li></ul><ul><li>Severe to Profound, bilateral sensorineural deafness </li></ul><ul><li>Little or no benefit from hearing aids </li></ul>Who is a Candidate for a Cochlear Implant?
  41. 44. Cochlear Implants <ul><li>Acoustic signal picked up by microphone located in headset worn at ear level </li></ul><ul><li>Cord carries sound from microphone to a speech processor </li></ul><ul><li>Speech processor digitizes sound into coded signals </li></ul><ul><li>Coded signals sent up to the transmitting coil </li></ul><ul><li>Coil sends coded signals as FM radio signals to CI under the skin </li></ul><ul><li>CI delivers electrical energy to the electrode array inserted into the cochlea </li></ul><ul><li>Electrodes along the array stimulate remaining nerve fibers in cochlea </li></ul>Cochlear Corp. 2002
  42. 45. Selecting a Cochlear Implant Center <ul><li>Experienced cochlear implant team </li></ul><ul><ul><li>Audiologist, speech-language pathologist, surgeon </li></ul></ul><ul><ul><li>Others: educator of the deaf, psychologist, social worker </li></ul></ul><ul><li>Comprehensive program covering eligibility, surgery, device activation, and long-term habilitation. </li></ul><ul><ul><li>Multi-disciplinary, family centered approach. </li></ul></ul><ul><ul><li>Knowledgeable regarding deafness, child development, and speech, language and auditory development </li></ul></ul><ul><ul><li>Offers intervention program and continued </li></ul></ul><ul><ul><li>Follow-up for changes in cochlear implant mapping </li></ul></ul>
  43. 46. Benefits <ul><li>Similar to early amplification provision, children implanted at early ages with more experience tend to do better developing auditory skills and speech than older children who receive implants after greater period of deafness </li></ul>
  44. 47. <ul><li>Sound waves enter through the microphone. </li></ul><ul><li>The sound processor converts the sound into a distinctive digital code. </li></ul><ul><li>The electrically coded signal is transmitted across the skin through the headpiece to the internal portion of the device. </li></ul><ul><li>The internal device delivers the sound to the electrodes. </li></ul><ul><li>The electrodes stimulate the hearing nerve. </li></ul><ul><li>The hearing nerve sends the signal to the brain for processing. </li></ul>How Does a Cochlear Implant Work?
  45. 49. Electrode Array 3 turn gold wire coil Internal Electronics Removable Magnet Internal Equipment HiRes ™ 90K
  46. 51. <ul><li>Cochlear Implants </li></ul>Amplification Options for Hearing Loss Hearing Aids
  47. 52. Team Approach Psychologist SLP, if speech chosen Audiologist Care Givers Surgeon & Pediatrician Teacher of The Deaf Child
  48. 53. Medical Evaluation <ul><li>Determine cause of hearing loss </li></ul><ul><li>Assess status of middle ear & cochlea </li></ul><ul><li>CT scan/MRI </li></ul><ul><li>Counseling </li></ul><ul><ul><li>Hearing loss </li></ul></ul><ul><ul><li>The surgical procedure </li></ul></ul><ul><ul><ul><li>Typically out-patient and performed by an otolaryngologist (ENT) or otologist (ear specialist) </li></ul></ul></ul><ul><ul><li>Post surgical considerations </li></ul></ul>
  49. 54. <ul><li>Bilateral Implants </li></ul><ul><ul><li>Industry trend </li></ul></ul><ul><ul><li>Simultaneous vs. Sequential </li></ul></ul><ul><ul><li>Benefits: </li></ul></ul><ul><ul><ul><li>Improved directionality </li></ul></ul></ul><ul><ul><ul><li>Improved listening in noise </li></ul></ul></ul><ul><ul><ul><li>Clarity of speech </li></ul></ul></ul><ul><ul><ul><li>Developmental </li></ul></ul></ul>Bilateral Cochlear Implants
  50. 55. Risks of Implant Surgery <ul><li>Facial nerve injury </li></ul><ul><li>Device infection </li></ul><ul><li>Device failure </li></ul><ul><li>Menningits </li></ul><ul><li>Poor insertion </li></ul><ul><li>Does not work </li></ul><ul><li>Can I go back to a hearing aid </li></ul>
  51. 56. About Surgery <ul><li>Surgery takes 3 to 4 hours </li></ul><ul><li>Incision is 2” behind the ear and 3” long </li></ul><ul><li>Bilateral simultaneous surgery 5 to 6 hours </li></ul><ul><li>Minimal bleeding </li></ul><ul><li>Risk of CSF leak </li></ul><ul><li>Facial nerve </li></ul>
  52. 57. <ul><li>Audiology </li></ul><ul><li>Rehabilitation </li></ul><ul><li>Family Commitment </li></ul><ul><li>School Support </li></ul>Pediatric Support
  53. 58. Unilateral Implant vs. Bilateral <ul><li>Sequential: Should do second implant with in 2 to 3 years </li></ul><ul><li>Simultaneous </li></ul>
  54. 59. Having Binaural Hearing? <ul><li>“ I could appreciate music better.” </li></ul><ul><li>“ I could identify the talker in a group better and easier.” </li></ul><ul><li>“ I felt more confident in classes and meeting.” </li></ul><ul><li>“ I liked the quality of my own voice.” </li></ul><ul><li>“ The other peoples’ voices sounded more natural.” </li></ul>
  55. 60. Unilateral Deafness ? <ul><li>What about school </li></ul><ul><li>What can be done ? </li></ul>
  56. 67. Bottom Line <ul><li>Children who are deaf and hard of hearing need to be identified as early as possible in order to have access to language development. </li></ul><ul><li>Family choice is supported and respected </li></ul><ul><li>For families choosing auditory/oral communication component, ENT care can provide assistance with: </li></ul><ul><ul><li>Amplification </li></ul></ul><ul><ul><li>Medical decisions </li></ul></ul><ul><ul><li>Surgical options </li></ul></ul>
  57. 68. Question-and-Answer