Update on Pediatric Hearing Loss & Cochlear Implantation Anna - PDF document

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 • Post-natal – TORCH – Meningitis – Ototoxins – Trauma – Diabetes – Progressive loss • Perinatal risks • FH – Hypoxemia • NICU admission, • PE ventilation, low Apgars – Hyperbilirubinemia – Undiagnosed syndromes – Sepsis – Ototoxins – Low birthweight/preemie – 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 Normal Hearing Unilateral Hearing Loss Math Keller & Bundy (1980) (n = 26; age = 12 yrs) Language Math Peterson (1981) (n = 48; age = 7.5 yrs) Language Social Bess & Thorpe (1984) (n = 50; age = 10 yrs) Math Blair, Peterson & Viehweg (1985) Language (n = 16; age = 7.5 yrs) Math Culbertson & Gilbert (1986) Language (n = 50; age = 10 yrs) Social Average Results 0th 10th 20th 30th 40th 50th 60th Math = 30th percentile Percentile Rank Language = 25th percentile Social = 32nd percentile 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