Objective:Toevaluatetheauditoryfunctionofaninpidualwithgeneticallyconfirmedhemochromatosis.Methods:A57yearoldmalewithmildlyimpairedsounddetectionthresholdsunderwentarangeofbehavioural,electroacousticandelectrophysiologicassessments.Theseincludedtherecordingofotoacousticemissionsandauditorybrainstemresponses,measurementofmonauraltemporalresolutionandevaluationofbinauralspeechprocessing.Findingsforthispatientweresubsequentlycomparedwiththoseof80healthycontrolswithsimilaraudiometricthresholds.Results:Thepatientshowedthethreecardinalfeaturesofauditoryneuropathy,presentingwithevidenceofnormalcochlearouterhaircellfunction,disruptedneuralactivityintheauditorynerve/brainstemandimpairedtemporalprocessing.Hisfunctionalhearingability(speechperception)wassignificantlyaffectedandsuggestedareducedcapacitytouselocalizationcuestosegregatesignalsinthepresenceofbackgroundnoise.Conclusion:Wepresentthefirstcaseofaninpidualwithhemochromatosisandauditoryneuropathy.Thefindingsforthispatienthighlighttheneedforcarefulevaluationofauditoryfunctionininpidualswiththedisorder.
