The management of patients characterised by the presence of otoacoustic emissions and/or cochlear microphonics suggesting normal outer hair cell function in conjunction with absent or grossly abnormal auditory brainstem responses is often associated with particularly poor response to amplification. Cochlear implantation has been shown to be an option in affected patients. Here, we report a case of successful cochlear implantation and preserved otoacoustic emissions in a child suffering from this hearing disorder.
