What are the two tests used in the newborn hearing screening programme?
Automated oto-acoustic emission (AOAE)
Automated auditory brainstem response (AABR).
What happens if a child fails the newborn hearing screening programme?
Stages >
- automated OAE
- repeat automated OAE - if no clear response is detected in the first test and
- automated ABR.
> referred to the local centre or hospital for further investigations, including
- tympanometry
- DIAGNOSTIC OAE
- DIAGNOSTIC ABR
Both of the these diagnostic tests give thresholds where as the automated ones just give a pass/fail
Causes of hearing impairment in child
Genetic factors
Congenital infections (TORCH: toxoplasmosis, rubella,
CMV, herpes simplex)
Meningitis
Intensive care unit for more than 48 hours
Craniofacial abnormalities.
What sound intensity level is required to elicit stapedial reflexes?
85dB
Stapedial reflex thresholds
Generally between 70-90 dB
Term for bat ears?
And why do they occur?
Prominent ears
Lack of antihelical fold,
Deep conchal bowl
Protruding lobule.
Management of prominent ears?
Conservative
Surgical- pinnaplasty
If neonate - can use ear splinting
Complications pinnaplasty
- Infection,
- Pinna h e m a t o m a
- Cartilage necrosis
- Asymmetry
- Telephone ear deformity
- Patient dissatisfaction.
- Keloid scarring
- Ear canal stenosis
- Recurrence
- Sensory changes
Small deformed ear called?
Total abscence pinna called?
Microtia
Anotia
What is associated with microtia?
CHL (due to canal atresia) (30-60dB)
Syndromes: ChaTGP (microtia syndromes)
-CHARGE
- Pierre Robin
- Goldenhar
- Treacher Collins
Treatment of microtia?
MDT approach
Treatment of CHL usually
- Bone conduction hearing aid (BCHA), eg BCHA on a soft band trial
- then a BAHA if BCHA trial succesful
Surgical recon of pinna
Or Prosthesis
Embriologically how does the pinnna form?
From the 6 hillocks of His which are dervied from the 1st & 2nd branchial arches.
How does a preauricular sinus form?
Incomplete fusion of the hillock of His
Described the procedure for surgically treating a preauricular sinus?
Elliptical incision
dissection down to temporalis fascia and cartilage of the root of the helix, which may be resected.
Methylene blue can be helpful.
Syndrome associated with pre-auricular sinuses?
Brachio-oto-renal syndrome
Pre-auricular sinuse
Additional discharging pit in the neck, Hearing loss
Kidney dysfunction.
Brachio-oto-renal syndrome presents as…
Brachio-oto-renal syndrome
Pre-auricular sinuse
Additional discharging pit in the neck, Hearing loss
Kidney dysfunction.
What are the parts of a cochlear implant (and describe their functions)
External:
Microphone(s): pick up sound from the environment
Speech processor: selectively filters sound to prioritize audible speech
Transmitter: sends power and the processed sound signals across the skin to the internal device by radio frequency transmission
Internal:
Receiver/stimulator: receives signals from the speech processor and converts into electric impulses
Electrode array embedded in the cochlea
Different coloured eyes + hearing loss.
Possible diagnosis?
Name of clinical signs of different coloured eyes?
Other sympotms to look out for?
Waardenburg syndrome (autosomal dominant)
Group of rare genetic conditions characterised by
- Degree of congenital hearing loss
- Pigmentation deficiencies,
e.g.
— Heterochromia
— or bright blue eyes
— White forelock
— Patches of light skin
Associated with congenital nonprogres-
sive hearing loss, either unilateral (70%) or bilateral (30%).
Waardenburg syndrome - symptoms
Group of rare genetic conditions characterised by
- Degree of congenital hearing loss
- Pigmentation deficiencies,
e.g.
— Heterochromia
— or bright blue eyes
— White forelock
— Patches of light skin
Associated with congenital nonprogres-
sive hearing loss, either unilateral (70%) or bilateral (30%).
Enlarged vestibular aqueduct with hearing loss - diagnosis and features
Pendred Syndrome (autosomal recessive)
- Progressive bilateral SNHL (due to enlarged vestibualr aqueducts)
- Goitre (75%)
- Can have mild hypothyroidism
Pendred Syndrome - features and advide to patients
Bilateral worsening SNHL due to enlarged vestibular aqueduct
Goitre in 70%
Some have mild hypothyroidism
Avoid trauma as worsens
aR
“Pendred, don’t Punch a Pendred - PendRed - aR”
Benign Paroxysmal Positional Vertigo
- Causes (2)
- Demographics
- Most common peripheral vestibular condition
- Usually occurs after a head injury or an URTI / ear infection
- Predominantly affects middle aged women
Describe the Dix-Hallpike manoeuvre
Ensure no cervical spine issues / RA / Atlanto-axial instability
- Patient positioned on bed
- Eyes open, facing forward - asked to keep eyes open
- Warn them it may bring on symptoms, you will look into their eyes, you will not let them fall
- Head held between examiners hands and turned 45 degrees to the right or left
- Patient rapidly laid backwards, head over edge of bed, 20 degree below the horizontal
- Ask patient if this provokes symptoms, eyes observed for rotary nystagmus (generally latency of 5 seconds in BPPV, no latency if central cause)
- If none after 30 seconds, return patient to upright position, again asked if symptoms brought on and eyes observed for nystagmus
If no symptoms, repeat on the opposite side
Provide post procedure advice
Avoid: driving, lying flat 48hrs, bending forwards, lying on affected side
Desciribe warnings to patient post Dix-Hallpike
Avoid driving,
Avoid lying flat for 48 hours
Avoid bending forwards
Avoid lying on the affected side.
