title: Auditory neuropathy in systemic sclerosis: a speech perception and evoked potential study before and after cochlear implantation.
abstract: We report the results of speech perception and electrophysiological evaluation of the auditory periphery performed before and after cochlear implantation in a 18-year-old girl with systemic sclerosis (SS) who presented the clinical picture of auditory neuropathy. Transtympanic electrocochleography (ECochG) in response to 0.1 ms clicks was recorded 1 month before cochlear implantation on both sides while the electrically evoked neural response was obtained intraoperatively in the right ear through cochlear implant stimulation. The ECochG recordings revealed the presence of the cochlear microphonic with normal amplitude and threshold on both sides. A compound action potential was only detected in the left ear at high stimulation intensity, while the electrically evoked neural response was clearly identifiable at all the recording sites during neural response telemetry. Standardized speech perception tests were performed 1 month before cochlear implantation and several times after cochlear implant connection. Speech perception scores were close to chance before cochlear implantation while they showed a remarkable improvement thereafter. The results of this study show that subjects affected by SS could present the clinical picture of auditory neuropathy which is possibly underlain by lesions involving the distal portion of auditory nerve fibers and/or synapses with inner hair cells. The restoration of synchronous neural discharge could be achieved by electrical stimulation through cochlear implant.
authors.affiliation: Department of Medical and Surgical Specialities, Audiology and Phoniatric Service, Treviso Hospital, University of Padova, Via Giustiniani 2, 35128 Padova, Italy. rosamaria.santarelli@unipd.it
authors.forename: Rosamaria
authors.initials: R
authors.lastname: Santarelli
authors.forename: Pietro
authors.initials: P
authors.lastname: Scimemi
authors.forename: Erica
authors.initials: E
authors.lastname: Dal Monte
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Genovese
authors.forename: Edoardo
authors.initials: E
authors.lastname: Arslan
mesh_terms: D000293:Adolescent; D001300:Audiometry, Evoked Response; D001301:Audiometry, Pure-Tone; D003051:Cochlea / Q000502:physiology; D019929:Cochlear Implantation / Q000379:methods*; D003056:Cochlear Nerve / Q000502:physiology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000628:therapy; D006801:Humans; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology; D012595:Scleroderma, Systemic / Q000503:physiopathology* / Q000628:therapy*; D013067:Speech Perception / Q000502:physiology*; D013069:Speech Reception Threshold Test; D014057:Tomography, X-Ray Computed