Return to Diagnosis ListShow Learning Points most relevant to Phase 1:

Congenital hearing loss

Clinical Discipline(s)/Organ System(s)
Nervous System, Otorhinolaryngology, Paediatrics
Progress Test Topic(s)
Child health
Description
A 4 month old Māori baby is sent to paediatric outpatients as he failed his newborn audiology screen and missed his planned repeat testing. His mother is concerned that he does not hear and he is not babbling or laughing.
Progress Test-Type Questions:   Question 1 | Question 2
Applied Science for Medicine 
   - Anatomy, physiology and embryologic development of the ear and auditory system
   - Understand the methods of testing for hearing loss at different ages (including the tests used for neonatal hearing screening)
   - Understand the difference between conductive and sensorineural hearing loss
   - Understand age appropriate developmental milestones for hearing and speech and language
   - Knowledge of the prevalence of congenital hearing loss
   - Knowledge of the aetiology/risk factors for sensorineural hearing loss
   - Genetics of hearing loss (autosomal dominant, autosomal recessive and X-linked deafness)
   - Outline the pharmacology of aminoglycosides and their ototoxic side effects
   - Microbiology and pathogenesis of otitis media
Clinical and Communication Skills 
   - Obtain an adequate history for potential risk factors/causes of hearing loss (including family history of hearing loss)
   - Obtain, document and interpret assessment of child with hearing loss including developmental assessment
   - Know how to examine the ear and tympanic membrane
   - Examine for important clinical signs such as neurocutaneous stigmata, or congenital abnormalities including normal growth parameters, head circumference and development
   - Know the indications for performing audiologic testing and what tests are appropriate/possible at different ages (audiograms, otoacoustic emissions, automated brainstem responses)
   - Be able to interpret audiology tests
   - Management of hearing loss
Personal and Professional Skills 
   - Empathise with the parent/caregiver
   - Breaking bad news to parents
Hauora Māori 
   - Appropriate engagement and consultation with whānau
   - Accessing cultural support in life-changing scenarios
   - Ability to cater for differential health literacy needs of Māori patients and whānau
Population Health 
   - Knowledge of the Newborn Hearing Screening Programme
   - Community and national services available for people with hearing impairment including hearing aids and cochlear implants
Conditions to be considered relating to this scenario
Common
genetic hearing loss, congenital infection particularly cytomegalovirus, recurrent acute otitis media, otitis media with effusion
Less common but 'important not to miss'
cleft palate, meningitis and sequelae including hearing loss and hydrocephalus
Uncommon
ototoxic drugs (including herbal remedies), prematurity, hyperbilirubinaemia, cerebral palsy, cochlear malformation, aplasia