Paediatric Hearing Loss Dr Nirmal Patel MBBS (Hons)(Syd) FRACS (OHNS) MS (UNSW) Associate Professor of Surgery (Macquarie University) Director, Kolling Deafness Research Centre (University of Sydney)

Summary ∗ Introduction ∗ Conductive Hearing Loss ∗ Sensorineural Hearing Loss ∗ Diagnosis ∗ Treatment

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

And Mild Unilateral Hearing Loss has an Impact... And Mild Unilateral Hearing Loss has an Impact... ∗ Significant reduction in language acquisition and speech with prolonged mild unilateral hearing loss. hl/Mild_Uni_2005%20Workshop_Proceedings.pdf

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 residentsLow birth weight/ NICU residents HyperbilirubinaemiaHyperbilirubinaemia Ear and Head traumaEar and Head trauma Anoxia and HypoxiaAnoxia and Hypoxia Ototoxic Drugs and ChemicalsOtotoxic Drugs and Chemicals Infectious DiseasesInfectious Diseases Recurrent otitis mediaRecurrent otitis media

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 ∗ 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

Diagnosis ∗ Examination Local Auricle Canal Tympanic membrane (pneumatic otoscopy) Regional Syndromic features Tuning Forks Difficult under 6 yrs

Diagnosis ∗ Investigations Hearing test - refer to audiologist with children’s experience

Diagnosis Refer to ENT if concerned 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) Hart CK, Choo DI Laryngoscope April 2013

Treatment for Conductive Hearing Loss - Chronic Otitis Media Observation - minimise day care exposure and passive smoking, reflux disease (prevalence 48%) Autoinflation/ otovent - low cost no adverse effects, reasonable to consider; cochrane review small studies no significant benefit. Nasal Steroids, antihistamines,antibiotics - no evidence to support use. Largest effect when used for 4 weeks to 3 months. American Academy Otolaryngology, Pediatrics & Family Physicians Consensus 2005 Miura et al OHNS March 2012 Cochrane Review Oct 2010, Sept 2011 and Sept 2012

Treatment for Conductive Hearing Loss - Chronic Otitis Media Middle Ear Ventilation Tubes (Grommets) - effect is small is normal speech and development. Likely significant effect in either speech/ hearing delayed child or developmental delayed child. Measures of disease specific quality of life improve in all studies. Children <24 months untreated have a higher risk of speech and language delay. Hearing Aids - poor compliance, fluctuating pathology therefore difficult to fit, damage. American Academy Otolaryngology, Pediatrics & Family Physicians Consensus 2005 Miura et al OHNS March 2012 Cochrane Review Oct 2010, Sept 2011 and Sept 2012

Bone Anchoured Hearing Aids for Conductive Hearing Loss

Treatment for Paediatric Sensorineural Hearing Loss ∗ Hearing assistive techniques Preferential seating Techniques to use at home ∗ Rehabilitation Hearing Aids FM systems ∗ Cochlear Implantation

Cochlear Implantation Operation Video

Summary ∗ Hearing loss in children is common and significantly impacts speech and development even in mild unilateral loss. ∗ Early diagnosis is the key to treatment success.