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
Anna K. Meyer, MD, FAAP Assistant Professor Division of Pediatric Otolarynoglogy University of California, San Francisco
February 16, 2013
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4 Riga, et al., 2005; Gulandi, 2003
– Severe to profound – autosomal recessive – bilateral
– Range of hearing loss – Unilateral, asymmetric – Progressive – Sudden SNHL – Autosomal dominant: syndromic and non-syndromic
keratitis-ichthyosis/hystrix-like ichthyosis-deafness
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– 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
and delayed in onset
– < 3 weeks: CMV viral culture on urine – After 3 weeks: dried blood spot
Deleenheer, 2011 8
– More common than all other diseases screened in neonates combined.
– >95%
– 55%
– 33-53%
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White, 2003
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– TORCH – Ototoxins – Diabetes
– Hypoxemia
ventilation, low Apgars – Hyperbilirubinemia – Sepsis – Ototoxins – Low birthweight/preemie – Syndromes
– Meningitis – Trauma – Progressive loss
– Undiagnosed syndromes
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– Infectious
– S. pneumo & N. meningitidis >> H. flu
– Delayed motor milestones – Syncope, arrhythmias
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– natural sleep ABR – sedated ABR – OAEs – Behavioral testing
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– Ophthalmologic evaluation
– HAs – Genetics – Imaging – Other
– HAs – Imaging – Genetics, other
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Math Language Math Language Social Math Language Math Language Social 0th 10th 20th 30th 40th 50th 60th Percentile Rank Normal Hearing Unilateral Hearing Loss
Keller & Bundy (1980) (n = 26; age = 12 yrs) Peterson (1981) (n = 48; age = 7.5 yrs) Bess & Thorpe (1984) (n = 50; age = 10 yrs) Blair, Peterson & Viehweg (1985) (n = 16; age = 7.5 yrs) Culbertson & Gilbert (1986) (n = 50; age = 10 yrs)
Average Results Math = 30th percentile Language = 25th percentile Social = 32nd percentile
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– 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
– More years at risk for cancer occurrence – 2001 and 2003 studies show small but significant increase in lifetime risk of fatal cancer
Brenner,et al., 2001; Slovis, 2003; Linet, et al., 2009
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Berrington de Gonzalez, A. et al. Arch Intern Med 2009;169:2071-2077.
Projected Number of Future Cancers That Could Be Related to CT Scans Performed in the United States in 2007, According to CT Scan Type
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Preciado, et al., 2005 *consider MRI
– 12 months or older – Unaided PTA > 90 dB HL – HA trial: 3-6 months – Lack of auditory skills development – Tolerance of HAs
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– Improved phonological skills – Superior speech understanding – Language skills growth rate similar to normal-hearing peers
– Total blood volume: 80mL per kg – Facial nerve position – Underdeveloped mastoid tip – Device migration/profile
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Niparko, et al., 2010
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– 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%
– Minimal trauma cochleostomy and insertion – As high as 60% retain residual hearing (Skarzynski, et al., 2002)
– Equivalent performance 10- and 24-electrode arrays
loss
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abnormal ABR
– -- refer for CI evaluation – CI may offer neural synchronization
– Comparable to SNHL in those without other medical/cognitive issues – Counseling is key
– may slow rate of rehabiliation post-CI – May require multiple modes of communication – Still improve QOL, communication, self-sufficiency and socialization
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– 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)
– 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
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– Some of our best performers – Resistant to proceeding with implantation
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be followed closely for reassessment of CI candidacy
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– Unilateral aidable hearing loss/contralateral deaf ear – Risk of progression?
– Similar benefits to unilateral aiding (Hassepass, et al., 2013)
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history/clinical findings
profound negative on GJB2 and CT imaging
genetic testing
be fully evaluated in the CI work-up
commitment is the key to success
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