Deafness

Causes of Sensory Neural Hearing Loss

• Ototoxicity • Hereditary
• Meniere’s,disease • Noise induced
• Presbycusis • Syphilis
• Trauma • Diabetes mellitus
• Sudden Idiopathic HL • Multiple sclerosis
• Tumors
Normal PTA
Conductive Loss
Sensorineural Loss
Mixed Loss
BTE
ITE
ITC
CIC
TV Ears ®
Pediatric Hearing Loss
 Introduction
• Hearing loss is common

• 1 in 1000 babies have significant

hearing loss at birth

• 10 in 1000 children have significant

hearing loss at school (inc. mild and
moderate)

• 80% of all children will have at least

one middle ear infection
 Hearing loss: Impaired all Brain
Functions
• Speech and language development
• Cognitive functions and intellectual abilities
• Social activities
 Paediatric Conductive Loss
• Congenital
– Atresia of ear
– Congenital stapes fixation
– Cholesteatoma

• Acquired
– Acute and chronic otitis media
Microtia / Atresia
Congenital Cholesteatoma
 Paediatric Sensorineural Loss
 Sensorineural Hearing Loss (Nerve deafness)
– Genetic and Idiopathic (70%)
– Acquired (30%)
• Low birth weight/ NICU residents
• Hyperbilirubinaemia
• Ear and Head trauma
• Anoxia and Hypoxia
• Ototoxic Drugs and Chemicals
• Infectious Diseases
• Recurrent otitis media
Waardenburg Syndrome
Branchio-oto-renal Syndrome
Treacher Collins Syndrome
 Deafness - Genetic
• Otosclerosis
• Usher Syndrome with retinitis pigmentosa
• Pendred Syndrome with thyroid anomaly
• Jervell and Lange-Neilsen Syndrome
• Alport Syndrome with renal anomaly
 Diagnosis
• History
✦ Risk factors for SNHL
✦ Auditory milestones:
– 3 months: Startled by loud
sounds and calmed by familiar sounds
– 6 months: ability to localise
– 9 months: respond to name & mimic
environmental sounds
– 12 months: First meaningful words
– 18 months: Vocabulary of 20 words or more
– 24 months: Small sentences
Postnatal- NICU
 Acquired and later onset SNHL
• Acquired
– Meningitis – 10%, esp pneumococcal
– Head injury
– Measles and mumps
• Progressive
– Some genetic
– Congenital CMV
 Diagnosis
•SWISH (Statewide Infant Hearing
Screening Programme) aims to test
every child born in NSW and identify
significant hearing loss by the age of 3
months

•Parental (other carers) clues

•Family physician
 Diagnosis
• Tests if suspecting SNHL

– Imaging - CT, MRI (dysplasias,

large vestibular aqueduct)
– TFT (Pendred’s syndrome)

– Urinalysis (Alport’s)

• Liase with opthalmologist (Usher’s

Syndrome), geneticist (Connexin 26)
 Diagnosis
• Examination
– Local
• Auricle
• Canal
• Tympanic membrane
(pneumatic otoscopy)
– Regional
• Syndromic features
– Tuning Forks
• Difficult under 6 yrs
 Tests
• OAE
• Assesses the function of the outer hair cells
• Requires normal external and middle ears
• BERA
• Evaluates the “wiring” of auditory pathway
• Limited in range of frequencies evaluated
• Adult characteristics by 18-24 months of age
Auditory Response Cradle
 OTO ACOUSTIC EMISSION-(OAE)

 “Sound” generated by
the cochlea
 Outer hair cells
“amplify” the wave
sounds to the inner
hair cells
 Spontaneous
 Evoked
 Diagnosis
• Investigations
– Hearing test - audiologist with
children’s experience
 Auditory Pathway

• Eighth Nerve
• Cochlear Nucleus
• Olive superior
• Lateral leminiscus
• Inferior colliculus
• Medial geniculate body
• Auditory radiation
• Temporal Lobe
 Treatment for Paediatric
Sensorineural Hearing Loss
• Hearing assistive techniques
– Preferential seating
– Techniques to use at home

• Rehabilitation
– Hearing Aids
– FM systems

• Cochlear Implantation