Auditory neuropathy in a patient with hemochromatosis

Abstract

Objective To evaluate the auditory function of an individual with genetically confirmed hemochromatosis. Methods A 57 year old male with mildly impaired sound detection thresholds underwent a range of behavioural, electroacoustic and electrophysiologic assessments. These included the recording of otoacoustic emissions and auditory brainstem responses, measurement of monaural temporal resolution and evaluation of binaural speech processing. Findings for this patient were subsequently compared with those of 80 healthy controls with similar audiometric thresholds. Results The patient showed the three cardinal features of auditory neuropathy, presenting with evidence of normal cochlear outer hai..