Is earlier always possible? A review of infants diagnosed with Unilateral ANSD

Ms Inge Kaltenbrunn1, Dr Robyn Cantle Moore2 

1Royal Institute For Deaf And Blind Children, North Rocks, Australia,

2RIDBC Renwick Centre/Macquarie University , North Rocks, Australia


Auditory Neuropathy Spectrum Disorder (ANSD) is a complex auditory condition. In the presence of unilateral ANSD, the audiological and medical assessment results critical to informing clinical management and hearing device options are often obtained at later stages than is possible for infants with bilateral ANSD. This delay is primarily due to the need to obtain additional audiological results to inform hearing device fitting and waiting lists for an MRI appointment to identify the integrity of the cochlear nerve. The incidence of cochlear nerve deficiency/aplasia is reported to be a significant cause of unilateral ANSD (Lui et al., 2012; Nakano, Arimoto & Matsunaga, 2013). Recommendations regarding hearing device options/cochlear implantation are therefore typically deferred until further audiological and MRI results are available. Electronic and archived records (2011-2018) were reviewed for 15 children with unilateral ANSD who receive services at the Royal Institute for Deaf and Blind Children (RIDBC). The data was examined in terms of age at diagnosis; age at enrolment in early intervention; age at completion of additional audiological assessments; age at MRI; integrity of the cochlear nerve; parent decision-making regarding hearing device fitting; range of RIDBC service accessed, and longitudinal speech and language outcomes.    The results are discussed in terms of the strengths and challenges of current practice. Recommendations are made to better inform future management pathways and enhance support for parents and families.


Inge Kaltenbrunn, BSp&Aud, MCommunication Pathology, LSLS Cert.AVT, is the Best Practice Lead: Early Intervention (Deaf/Hard of Hearing Children) at RIDBC in Sydney, Australia. She is a qualified speech pathologist, audiologist and LSLS Certified Auditory-Verbal Therapist with 23 years of experience in the field of hearing loss and cochlear implants. She has worked in early intervention programs and cochlear implant clinics for children with hearing loss in South Africa and Australia. She has significant experience in delivering family centred early intervention, cochlear implant (re)habilitation, program development, mentoring and professional training in the area of paediatric hearing loss.