[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, synapsis or dendrites of the ganglion cells of the auditory nerve result in specific audiometric findings. Due to the normal function of the outer hair cells otoacoustic emissions can be registered, ABR and ECochG show at least elevated thresholds or are absent.


METHODS
We demonstrate 5 cases with these audiological findings described in the literature as Auditory Neuropathy.


RESULTS
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 understanding but one prelingually adult, also implanted, has only sound identification.


CONCLUSIONS
Hypoxia, carboplatin, ototoxicity and metabolic disorders are possible etiologies for damage to the inner hair cells or synapsis. The results will be discussed with reference to the localisation of the pathology and the definition as Auditory Neuropathy.
Lesinski-Schiedat
A
A
Medizinische Hochschule Hannover, Klinik und Poliklinik für Hals-, Nasen-, Ohrenheilkunde.
Frohne
C
C
Hemmaouil
I
I
Battmer
R D
RD
Lenarz
T
T
D000328:Adult; D001299:Audiometry; D002648:Child; D003054:Cochlear Implants; D003056:Cochlear Nerve; D003638:Deafness / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006199:Hair Cells, Auditory, Inner; D006801:Humans; D008297:Male
Funktionelle Taubheit bei peri-synaptischer Audiopathie - Isolierte Störungen der inneren Haarzellen?