Australian guidelines on investigations for childhood hearing loss

Dr Valerie Sung1, Prof David Amor1,2, A/Prof Catherine Birman3, Dr Wei Wei Chan4, Dr Carolyn Cottier5, Dr Lilian Downie1,2, Dr  Alison Harris6, Dr Matthew Hunter7,8, Dr Karen Liddle6, A/Prof Georgia Paxton1, Dr Kenneth Peacock9, Dr Elizabeth Peadon9, Dr Laurence Roddick10, Dr Elizabeth Rose11, Dr Kerryn Saunders12,8, . On behalf of CHAMP Network

 1Murdoch Children’s Research Institute, Royal Children’s Hospital, University of Melbourne Department of Paediatrics

2Victorian Clinical Genetics Service, Melbourne

3Children’s Hospital at Westmead, Sydney Cochlear Implant Centre, Sydney University, Macquarie University, Sydney

 4Gold Coast University Hospital, Gold Coast, Australia,

 5Sydney Children’s Hospital, Randwick, University of New South Wales, Sydney, Australia,

6Lady Cilento Children’s Hospital, University of Queensland, Brisbane, Australia,

7Monash Genetics, Monash Health, Melbourne, Australia,

8Department of Paediatrics, Monash University, Melbourne, Australia,

 9Deafness Centre, The Children’s Hospital at Westmead, Sydney, Australia,

10John Hunter Children’s Hospital, Newcastle, Australia,

 11Royal Children’s Hospital, Murdoch Children’s Research Institute, University of Melbourne Department of Otolaryngology, Royal Victorian Eye and Ear Hospital, Melbourne, Australia,

12Paediatric Hearing Services, Monash Children’s Hospital, Melbourne, Australia

 

Purpose of presentation

To describe consensus recommendations on medical management of childhood hearing loss in Australia

Nature and scope of topic

The Childhood Hearing Australasian Medical Professionals (CHAMP) Network is a group of paediatricians, otolaryngologists and geneticists whose goal is to facilitate and improve care of hearing-impaired children in Australasia. These consensus guidelines stem from a workshop at the 2017 ANHS conference.

Issue or problem under consideration

Australia currently has a high quality universal hearing screening program. However, there are no existing guidelines beyond the screening process for the clinical management of infants with newly diagnosed hearing loss.

Outcome or conclusion reached

Saliva swab for cytomegalovirus (CMV) polymeraise chain reaction (PCR) should be taken where possible within 3 weeks of birth for all infants with newly diagnosed hearing loss. Early magnetic resonance imaging (MRI) of the brain and parasagittal sections of the internal acoustic canal is recommended to all infants with sensorineural hearing loss or auditory neuropathy where resources are available. Genetic testing should be offered based on parental desire after adequate genetic counseling for those with bilateral hearing loss. Connexin testing is the recommended first line genetic test, followed by microarray and gene panels/whole exome sequencing where available. The role of genetic testing in unilateral loss is limited. Audiology testing of first degree family members and ophthalmology examination is also recommended for all.

All other investigations should be offered according to clinical presentation. ECG should be offered to those with bilateral severe/profound hearing loss or those with a positive family history of sudden death. Older children without known etiology should be offered urine dipstick at around 10 years of age to exclude Alport syndrome.


Biography:

Valerie is a paediatrician at Melbourne’s Royal Children’s Hospital, NHMRC Early Career Fellow at the Murdoch Children’s Research Institute and Senior Honorary Fellow at the University of Melbourne Department of Paediatrics. She leads a clinical and research program on childhood hearing loss. Her research aims to capture population-level outcomes of universal hearing screening for the full spectrum of hearing loss, optimise childhood hearing loss management, in particular mild losses, and identify treatments to potentially reverse hearing loss progression. She founded CHAMP, a group of Australasian paediatric specialists, to optimise medical management of childhood hearing loss.

About ANHSC

The Australasian Newborn Hearing Screening Committee aims to foster the establishment, maintenance and evaluation of high quality screening programs for the early detection of permanent childhood hearing impairment throughout Australia and New Zealand.

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