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EAR ROUNDS

EAR ROUNDS. Clinical Clerks of Block 5A UP Medicine 2011 Department of Otorhinolaryngology, UP-PGH January 21, 2010. COMMON CAUSES OF HEARING LOSS AMONG NEONATES. INTRODUCTION. NEONATAL HEARING LOSS

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EAR ROUNDS

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  1. EAR ROUNDS Clinical Clerks of Block 5A UP Medicine 2011 Department of Otorhinolaryngology, UP-PGH January 21, 2010

  2. COMMON CAUSES OF HEARING LOSS AMONG NEONATES

  3. INTRODUCTION NEONATAL HEARING LOSS • A significant disability which any delay in early recognition, diagnosis and intervention will cause a significant detrimental impact on speech, language and cognitive abilities (Fakhim 2007). • Important to determine its prevalence, frequency, usual causes and significant risk factors

  4. Types of Hearing Loss

  5. Types of hearing loss CONDUCTIVE HEARING LOSS • Anything prevents the transmission of sound from the external environment to the cochlea cerumen neoplasm cholesteatoma

  6. Types of hearing loss SENSORINEURAL HEARING LOSS • disruptions in transmission after the cochlea • may be results of hair cell destruction or cochlear nerve damage

  7. Types of hearing loss MIXED HEARING LOSS

  8. Categories of Hearing Loss

  9. Categories of hearing loss American National Standards Institute defines hearing loss: • Slight hearing loss: 16-25 dB lost • Mild hearing loss: 26-40 dB lost • Moderate hearing loss: 41-55 dB lost • Severe hearing loss: 71-90 dB lost • Profound: > 90 dB lost

  10. Epidemiology of Hearing Loss

  11. EPIDEMIOLOGY • In the US, hearing loss occurs in about 10/1000 children • 1 / 1000 profound • 3-5 / 1000 mild-moderate • 10-20% acquired

  12. EPIDEMIOLOGY • Hearing loss requiring intervention among NICU admitted patients ranges from 1 to 4% • SNHL occurs in 9-27 per 1000 children worldwide • Though no sex predilection is noted, hereditary causes may occur more frequently in one sex than the other

  13. EPIDEMIOLOGY • Most hearing loss in children is congenital or acquired perinatally but may occur at any age • Approximately 10-20% of all cases are acquired postnatally

  14. EPIDEMIOLOGY • Prior to routine neonatal hearing screening, hearing loss was usually diagnosed at 2.5 years, now it improved to 14 months

  15. EPIDEMIOLOGY • Suspicion & Detection • Parents: 2/3 of the cases • Pediatricians: 10% • Other Healthcare providers: 15% • The mean time from suspicion to diagnosis is about 9 months

  16. High-Risk Criteria for HL in Neonates & Infants

  17. High risk criteria • The dissemination of high-risk criteria for neonates and infants in 1990 did not notably alter the mean age at diagnosis. • About 50% of children with SNHL do not meet any of the criteria listed, and only 10% of neonates have 1 or more of the high-risk criteria that prompt an evaluation.

  18. High risk criteria • These rates are among the reasons cited for the need for universal neonatal hearing screening. • The goals of such screening are to identify children who are deaf or hard of hearing and to start intervention by age 6 months.

  19. Neonates (birth to 28 d) • Family history of congenital or early SNHL • Congenital infection known to be associated with SNHL • Craniofacial anomalies • Birth weight of more than 1500 g (<3.3 lb) • Hyperbilirubinemia over the exchange level • Exposure to ototoxic medications • Bacterial meningitis • Low APGAR scores at birth • Prolonged mechanical ventilation • Findings of a syndrome associated with SNHL

  20. Infants (29 d to 2y) • Concern about hearing, speech, language, and/or developmental delay • Bacterial meningitis • Neonatal risk factors associated with SNHL • Head trauma, especially with fracture of the temporal bone • Findings of a syndrome associated with SNHL • Exposure to ototoxic medications • Neurodegenerative disorders • Infectious diseases associated with SNHL

  21. Causes of Neonatal Hearing Impairments • Genetic • Non-Genetic • Unknown

  22. I. Genetic • Accounts for 30-50% • May be autosomal dominant/recessive, x-linked or sporadic • Can be syndromic or non syndromic

  23. Syndromic • Typically present with involvement of other organ systems • May have specific characteristic manifestations • Cause approximately 38.5% of hearing loss present at birth (Billings, 1999)

  24. Syndromes associated with hearing loss

  25. Non syndromic • has isolated problem in the auditory system • gross physical abnormality of the structure or covert hearing impairment • estimated to represent more than half of the genetic cases • may account for most of the cases with no known cause

  26. II. Non Genetic • estimated to cause 28.6 % of hearing impairment in children (Billings, 1999) • classified into: • Prenatal - 5-10% of cases; classified as infectious (CMV, Herpes,etc) and non infectious (teratogens) • Perinatal - 5-15% of cases (LBW, low APGAR, etc) • Post natal - otitis media, ototoxicmedications,(aminoglycosides), infections (meningitis, mumps).

  27. III. Unknown Causes • Causes 20-30%; 31%(Billings, 1999) • Cases with no identifiable cause for hearing impairment • Hypothesized to be a result of denovo mutations or unidentified risk factors

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