UNLABELLED: To present a case with IP-II that had unilaterally refer result in newborn hearing screening and had progressive hearing loss by demonstrating the diagnosis and intervention processes. DESIGN: Case Report. SETTING: A University Hospital. SUBJECTS: A 6-year-old boy with bilateral IP-II who has a history of failed newborn hearing screening on one side. METHODS: Pure tone hearing thresholds, Auditory Brainstem Response and Magnetic Resonance Imaging results investigated. A progressive hearing loss and IP-II were noted. The developmental characteristics, diagnosis process including radiological evaluation, hearing intervention and follow-up were presented. This case report revealed the importance of follow-up of referred children in newborn hearing screening, the importance of radiological imaging in the diagnosis and intervention process of hearing loss, the approach to progressive hearing loss and the necessity of auditory intervention due to the hearing needs.