Hearing Flashcards
How does otoacoustic emissions testing work
reliable screening test for middle and inner ear hearing loss
doesn’t test higher auditory pathways
What happens if you fail otoacoustic emissions testing
referred for follow up testing which utilises auditory brain stem responses
hearing test age 6-24 months
distraction test
hearing test age 6-30 months
Visual reinforcement audiometry
hearing test age 20 months to 5 years
play audiometry
Hearing test >4 years
pure tone audiometry
How do you assess middle ear function
tympanometry
What is the most common cause of autosomal recessive hearing loss
gap junction beta-2 protein GJP2 - connexin 26/Cx26
What is the most common cause of congenital hearing loss
CMV infection during pregnancy - 15% of infections lead to sensorineural hearing loss
How do you investigate the possibility of congenital CMV infection
- saliva/urine first 3 weeks of life
2. neonatal blood spot
What is the management of congenital CMV
IV ganciclovir oral valganciclovir they inhibit CMV replication monitoring for haematloigcal and hepatological side effect long term cochlear implant
What genetic mutation is associated with aminoglycoside (gentamicin) induced ototoxicity
m155A>G
What is Waardenburg syndrome type 1
syndromic hearing loss
autosomal dominant
mutation in Pax3 gene
how does Waardenburg syndrome present
bright blue eyes
monobrow
spaced inner canthi of eyes
