3/20/2017. D. Richard Kang, MD, FACS, FAAP Pediatric Otolaryngology Director, ENT Institute Boys Town National Research Hospital

Transcription

1 D. Richard Kang, MD, FACS, FAAP Pediatric Otolaryngology Director, ENT Institute Boys Town National Research Hospital Pediatric providers have a reasonable chance to see a child with hearing loss in your pediatric practice Early intervention is critical and effective Early detection is necessary Almost all children with hearing loss can be helped to gain some hearing Sensorineural Hearing loss is a chronic illness Effects language, cognitive and social development There is treatment but no cure presently It is a lifetime commitment Learn about significant development that have changed the care of hearing loss Learn what are important steps in diagnosis and key therapeutic options for infants and children with hearing loss. 1

2 Grade Equivalents Language Age (yrs) 3/20/2017 Permanent hearing loss occurs more frequently than any other condition for which we can screen at birth Sources: American Academy of Pediatrics, 2002; National Institutes of Health, 2000, 2002; White, 2011, Age in Years Deaf Hearing Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press. Boys Town National Research Hospital Study of Earlier vs. Later 129 deaf and hard-of-hearing children assessed 2x each year. Assessments done by trained diagnostician as normal part of early intervention program Identified <6 mos (n = 25) Identified >6 mos (n = 104) Age (yrs) Moeller, M.P. (1997). Personal communication, moeller@boystown.org 2

3 Realized Universal Newborn Hearing Screen Otoacoutstic Emissions (OAE) Importance of early intervention Neuroplasticity Cochlear Implant Amplification (hearing aids) and assistive devices Waiting to be fully realized Genetics Failed Newborn screen Must have confirmation diagnostic ABR Consider CMV testing if seeing before 3 weeks rule Hearing Loss impacts at any level Mild, moderate, Severe and profound Unilateral Speech, cognitive and social Effects of Unilateral Hearing Loss Keller & Bundy (1980) (n = 26; age = 12 yrs) Peterson (1981) (n = 48; age = 7.5 yrs) Math Language Math Language Normal Hearing Unilateral Hearing Loss Bess & Thorpe (1984) (n = 50; age = 10 yrs) Social Blair, Peterson & Viehweg (1985) Math (n = 16; age = 7.5 yrs) Language Culbertson & Gilbert (1986) (n = 50; age = 10 yrs) Average Results Math = 30th percentile Language = 25th percentile Social = 32nd percentile Math Language Social 0th 10th 20th 30th 40th 50th 60th Percentile Rank 3

4 Data collection beginning 1999 More formal beginning 2004 Use of transient OtoAcoustic Emission (OAE) and Automated Auditory Brainstem Response (AABR). All states have program, 43 states mandated Early intervention is key If profound and has cochlea and nerve, cochlear implants by age 1 Auditory neuropathy/dys-synchrony Age can test behavioral Hearing aids Need for follow up Pediatric Audiologist Extrinsic vs Intrinsic Extrinsic NICU, infections, hypoxia, hypotension, toxic meds TORCH - congenital CMV infection Intrinsic Genetics Predominantly non syndromic autosomal recessive 4

5 From: Preciado: Otol Neurotol, Volume 26(4).July 2005 Kochhar et al, Genet Med 2007: 9(7) 393 In US, congenital CMV infection, the most common non-genetic cause of hearing loss in children May be the most common single cause of hearing loss in children overall (congenital and delayed included) 20 to 40,000 infants born with congenital infection, 90% no clinical abnormalites at birth, 10 to 15% have delayed hearing loss 5

6 Most nonsyndromic autosomal recessive Syndromes Usher s, Waardenburg, BOR, Pendreds Alports, Jervell Lange and Nielsen, CHARGE Diagnosis Knowledge and prognostic No effective genetic treatment option available (to reverse the hearing loss) at this time for humans. CT scan positive 6.8% to 31% in pediatric SNHL Best predictive factor of temporal bone anomaly is progressive HL and craniofacial anomalies EVA most common Risk of meningitis and progression Importance of pneumococcal vaccination Pediatric Otolaryngologist Pediatric audiologist Clinical geneticist Pediatrician Others Educator for deaf, Pediatric ophthalmologist Cost mostly social welfare system Over $1 million life time 6

7 Consider delayed onset hearing loss 15% preschool children with hearing loss Amplification Assistive Devices Speech Therapy Surgical Auditory Osseo-integrated Implants (AOI) Commonly known as BAHA Use in unilateral hearing loss Cochlear implants Approved for children 1990 Not a cure Resolution difference of 1000 fold compared to anatomical points The amazing ability of the brain Early surgery and speech therapy, able to achieve language close to typical ranage It is part of the available treatment options The only option that restores auditory function 7

8 Internal implant External speech processor 1.) Sounds picked up by microphone and converted to electrical signal. 2.) Speech processor breaks up the input signal into different frequency bands and compresses it to fit into the recipient s electrical dynamic range. Section V Filter Bank Analyzes incoming signal into component frequencies Low frequencies Apex dress High frequencies Base 8

9 3.) Electrical signal travels along cord to transmitting coil on headpiece. 4.) Internal receiver/ stimulator controls how much electrical current will be sent to which electrodes at what speed. 5.) Electrical current passes down the lead wires to specific electrodes in the cochlea. 6.) Position of electrodes within cochlea determines the frequency (pitch). Designed to mimic normal tonotopic organization. Low frequencies High frequencies 6.) Amount of electrical current determines loudness. Loud sounds Soft sounds 9

10 7.) Auditory nerve stimulation ascending neural pathways via normal conduction to auditory cortex, interpret as meaningful sound. Website sound demos: channel: original: 2-channel: 4-channel: 6-channel: 8-channel: BUT REMEMBER sound demos simply process the speech the way a speech processor would. They do not compensate for missing neurons. You are still listening to the demos with normal ears! Did not pass newborn hearing screening Auditory brainstem response test at age 1 month was no response, suggesting profound hearing loss, bilaterally Genetic testing showed mutation of gene for Connexin 26 (GJB2) 10

11 CI candidacy evaluations completed at age 7 months Cochlear implant for left ear at age 10 mos. Cochlear implant for right ear at age 12 mos. 11

12 % Correct 3/20/ Right CI Left CI Bilateral CI You have a reasonable chance to see a child with hearing loss in your pediatric practice Early intervention is critical and effective Early detection is necessary Almost all children with hearing loss can be helped to gain some hearing Sensorineural Hearing loss is a chronic illness Effects language, cognitive and social development There is treatment but no cure presently It is a lifetime commitment 12