Subjective deafness in case of peri-synaptic audiopathy. Isolated defects of the inner haircells?

Background: Damage to or functional impair of the inner hair cells, synapsi s or dendrites of the ganglion cells of the auditory nerve result in specif ic audiometric findings. Due to the normal function of the outer hair cells otoacoustic emissions can be registered, ABR and ECochG show at least elev ated thresholds or are absent. Patients: We demonstrate 5 cases with these audiological findings described in the literature as Auditory Neuropathy. R esults: All patients have profound to severe hearing loss with poor speech understanding under best aided conditions with conventional hearing aids. 3 patients, which were implanted with a cochlear implant have speech underst anding but one prelingually adult, also implanted, has only sound identific ation. Conclusions: Hypoxia, carboplatin, ototoxicity and metabolic disorde rs are possible etiologies for damage to the inner hair cells or synapsis. The results will be discussed with reference to the localisation of the pat hology and the definition as Auditory Neuropathy.