When the diagnosis of deafness is wrong.

OBJECTIVE: To describe three children in whom there had been major errors in the diagnosis of hearing loss. CLINICAL FEATURES: In three children (two developmentally delayed, one not developmentally delayed) hearing thresholds obtained by behavioural testing were later proven wrong. This resulted in significant family distress and inappropriate educational approaches. INTERVENTION AND OUTCOME: Electrocochleography and brainstem audiometry were performed, demonstrating normal cochlear function. Simultaneous microinspection of the ears gave information about current or old middle ear disease and the likelihood of past conductive hearing loss. In each case hearing aids could be discarded, enabling parents and teachers to concentrate on one rather than multiple problems. CONCLUSION: Electrocochleography and brainstem audiometry should be used more frequently to check the diagnosis of hearing loss in children who are developmentally delayed, hyperactive or autistic and who do not give consistent responses to behavioural testing. It should also be considered if parents are firmly convinced that the diagnosis of deafness is wrong.