Update on Pediatric Hearing Loss & Cochlear Implantation Anna K. Meyer, MD, FAAP Assistant Professor Division of Pediatric Otolarynoglogy University of California, San Francisco February 16, 2013 No disclosures 2 1
Objectives Common Etiologies Candidacy Evaluation Expanding Criteria 3 Causes of Hearing Loss Hereditary 40 to 50% Acquired 15-40% Unknown 15-44% Riga, et al., 2005; Gulandi, 2003 4 2
Connexin 26 and 30 Historical Severe to profound autosomal recessive bilateral Current Range of hearing loss Unilateral, asymmetric Progressive Sudden SNHL Autosomal dominant: syndromic and non-syndromic palmoplantar keratoderma, Vohwinkel syndrome and keratitis-ichthyosis/hystrix-like ichthyosis-deafness 5 Connexin 26 Genotype-Phenotype 6 3
CMV: the ongoing mystery! Estimated 1% of all neonates infected. Most no apparent signs of infection at birth. ~5% with asymptomatic congenital CMV infection present hearing loss before 3 months of age. mostly sensorineural, profound can be progressive, fluctuating, asymmetric/unilateral and delayed in onset Diagnosis < 3 weeks: CMV viral culture on urine After 3 weeks: dried blood spot Deleenheer, 2011 Enlarged Vestibular Aqueduct Most common imaging finding High risk for progression Variable hearing at birth from normal to profound 8 4
UNHS 1-3/1000 births with hearing loss More common than all other diseases screened in neonates combined. Screen by 1 month >95% Identify HL by 3 months 55% Intervention by 6 months 33-53% Birth to kindergarten:???? By school age incidence 6/1000 White, 2003 9 10 5
Hearing Loss Evaluation Prenatal exposures TORCH Ototoxins Diabetes Perinatal risks Hypoxemia NICU admission, ventilation, low Apgars Hyperbilirubinemia Sepsis Ototoxins Low birthweight/preemie Syndromes Post-natal FH PE Meningitis Trauma Progressive loss Undiagnosed syndromes 11 Hearing Loss Evaluation Postnatal Infectious Bacterial meningitis S. pneumo & N. meningitidis >> H. flu Viral: mumps, measles Check vaccination record Delayed motor milestones Syncope, arrhythmias FH, SH, Medications 12 6
Audiologic Confirmation Screen: automated ABR: max two! Diagnosis: natural sleep ABR sedated ABR OAEs Behavioral testing 13 Etiology Work-up All HL Ophthalmologic evaluation Severe to Profound SNHL: EKG Bilateral Loss HAs Genetics Imaging Other Unilateral Loss HAs Imaging Genetics, other 14 7
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) Blair, Peterson & Viehweg (1985) (n = 16; age = 7.5 yrs) Social Math 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 15 16 8
Should we limit CT scans? CT in U.S. Up to 7 million children annually in 2009 Head is common region for pediatric CT Younger patients receive higher radiation dose/unit Children are more sensitive to radiation 10 fold neoplastic potential More years at risk for cancer occurrence 2001 and 2003 studies show small but significant increase in lifetime risk of fatal cancer 1/1000 children with a single CT scan 0.35% increase > baseline Brenner,et al., 2001; Slovis, 2003; Linet, et al., 2009 17 Projected Number of Future Cancers That Could Be Related to CT Scans Performed in the United States in 2007, According to CT Scan Type Berrington de Gonzalez, A. et al. Arch Intern Med 2009;169:2071-2077. 18 9
CT-limiting Algorithm? *consider MRI Preciado, et al., 2005 19 Candidacy FDA pediatric guidelines 12 months or older Unaided PTA > 90 db HL HA trial: 3-6 months Lack of auditory skills development Tolerance of HAs 20 10
Pediatric CI Criteria? Severe SNHL Asymmetric Hearing Loss Unilateral HL Progressive HL Auditory Neuropathy with good pure tones Apparently absent cochlear nerves on MRI 6 month old with confirmed profound SNHL Severely developmentally delayed children Adolescent with long-term severe hearing loss 21 Expanding Criteria <12 months Improved phonological skills Superior speech understanding Language skills growth rate similar to normal-hearing peers Risks Total blood volume: 80mL per kg Facial nerve position Underdeveloped mastoid tip Device migration/profile 22 11
Will Older Children Catch Up? Niparko, et al., 2010 23 Severity of SNHL 24 12
Patients with Residual Hearing More likely to be good performers Children with pure tone thresholds 70 db and good HA benefit (Mondain, et l., 2002) Improved speech perception post-ci Pre-implant 21%, post-implant 84% Preservation of residual hearing Minimal trauma cochleostomy and insertion As high as 60% retain residual hearing (Skarzynski, et al., 2002) Bilateral CI: Standard + Hybrid (Gantz, et al., 2010) Equivalent performance 10- and 24-electrode arrays Caveat: children at higher risk for progressive loss 25 Auditory Neuropathy Present OAEs/cochlear microphonic with SNHL or abnormal ABR No progress with auditory or language skills -- refer for CI evaluation CI may offer neural synchronization Outcomes are variable Comparable to SNHL in those without other medical/cognitive issues Counseling is key 26 13
Developmental Delay Cognitive and developmental delay may slow rate of rehabiliation post-ci May require multiple modes of communication Still improve QOL, communication, self-sufficiency and socialization Counseling is key 27 Bilateral Implants The potential benefits of bilateral implants Insures that the best ear is implanted May allow some of the benefits of binaural hearing May avoid the effects of auditory deprivation on the unimplanted ear Signifiantly more audition and vocalization 1 year post-implantation (Tait, et al., 2010) Improved early complex expressive and receptive language (Wie, 2010) 28 14
Adolescents with Long-term Hearing Loss Highly variable outcome Congenital Deafness: Some able to convert to oral communication Improvements in open set recognition 1/3 good performers Factors: length of deafness, length of device use, mode of communication, degree of hearing loss, cause of hearing loss, use of speech therapy Kos, et. Al., 2009; Heman-Ackah, et al., 2012 29 Adolescents Progressive Hearing Loss Some of our best performers Resistant to proceeding with implantation larger HA Stigma Used to compensation 30 15
Progressive HL Children with progressive hearing loss should be followed closely for reassessment of CI candidacy 31 Unilateral loss Asymmetric hearing loss Unilateral aidable hearing loss/contralateral deaf ear Risk of progression? Unilateral SNHL Similar benefits to unilateral aiding (Hassepass, et al., 2013) 32 16
Success 33 Summary Selective additional testing based on history/clinical findings Ophthalmologic and ECG in all severe-to profound negative on GJB2 and CT imaging Genetic counseling for all patients undergoing genetic testing Children may have complex issues that need to be fully evaluated in the CI work-up Early referral of children for CI is best Counseling families on expectations and commitment is the key to success 34 17
35 Thank you! 18
References available upon request 37 19