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OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation.

Santarelli R, Rossi R, Scimemi P, Cama E, Valentino ML, La Morgia C, Caporali L, Liguori R, Magnavita V, Monteleone A, Biscaro A, Arslan E, Carelli V - Brain (2015)

Bottom Line: In this study we characterized the hearing dysfunction in OPA1-linked disorders and provided effective rehabilitative options to improve speech perception.The use of cochlear implant improved speech perception in all but one patient.Cochlear implantation improves speech perception and synchronous activation of auditory pathways by bypassing the site of lesion.

View Article: PubMed Central - PubMed

Affiliation: 1 Department of Neurosciences, University of Padova, Via Giustiniani 2, 35128 Padova, Italy 2 Audiology and Phoniatrics Service, Treviso Regional Hospital, Piazza Ospedale 1, 31100 Treviso, Italy rosamaria.santarelli@unipd.it.

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Electrically-evoked compound action potentials and ABRs from OPA1-M implanted patients. Electrically-evoked potentials from two representative subjects are displayed. In Subject 7 (bottom) both electrically-evoked compound action potentials (left) and electrically-evoked ABRs (middle and right) were recorded at all electrode locations; wave II was also identified in electrically-evoked ABR recordings in addition to wave V. No electrically-evoked compound action potentials were obtained from Subject 4, whereas electrically-evoked ABR wave V was recorded at all electrode locations. In both patients wave V was recorded with increasing latency from apical to basal electrodes (vertical dashed lines, middle). For a given electrode location, decreasing current levels resulted in increased latencies and attenuated wave V amplitudes (vertical dashed lines, right).
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awu378-F7: Electrically-evoked compound action potentials and ABRs from OPA1-M implanted patients. Electrically-evoked potentials from two representative subjects are displayed. In Subject 7 (bottom) both electrically-evoked compound action potentials (left) and electrically-evoked ABRs (middle and right) were recorded at all electrode locations; wave II was also identified in electrically-evoked ABR recordings in addition to wave V. No electrically-evoked compound action potentials were obtained from Subject 4, whereas electrically-evoked ABR wave V was recorded at all electrode locations. In both patients wave V was recorded with increasing latency from apical to basal electrodes (vertical dashed lines, middle). For a given electrode location, decreasing current levels resulted in increased latencies and attenuated wave V amplitudes (vertical dashed lines, right).

Mentions: Electrically-evoked compound action potentials were absent in all the implanted patients except for Subject 7, who showed the electrically-evoked neural response at each electrode location (Fig. 7).Figure 7


OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation.

Santarelli R, Rossi R, Scimemi P, Cama E, Valentino ML, La Morgia C, Caporali L, Liguori R, Magnavita V, Monteleone A, Biscaro A, Arslan E, Carelli V - Brain (2015)

Electrically-evoked compound action potentials and ABRs from OPA1-M implanted patients. Electrically-evoked potentials from two representative subjects are displayed. In Subject 7 (bottom) both electrically-evoked compound action potentials (left) and electrically-evoked ABRs (middle and right) were recorded at all electrode locations; wave II was also identified in electrically-evoked ABR recordings in addition to wave V. No electrically-evoked compound action potentials were obtained from Subject 4, whereas electrically-evoked ABR wave V was recorded at all electrode locations. In both patients wave V was recorded with increasing latency from apical to basal electrodes (vertical dashed lines, middle). For a given electrode location, decreasing current levels resulted in increased latencies and attenuated wave V amplitudes (vertical dashed lines, right).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4339771&req=5

awu378-F7: Electrically-evoked compound action potentials and ABRs from OPA1-M implanted patients. Electrically-evoked potentials from two representative subjects are displayed. In Subject 7 (bottom) both electrically-evoked compound action potentials (left) and electrically-evoked ABRs (middle and right) were recorded at all electrode locations; wave II was also identified in electrically-evoked ABR recordings in addition to wave V. No electrically-evoked compound action potentials were obtained from Subject 4, whereas electrically-evoked ABR wave V was recorded at all electrode locations. In both patients wave V was recorded with increasing latency from apical to basal electrodes (vertical dashed lines, middle). For a given electrode location, decreasing current levels resulted in increased latencies and attenuated wave V amplitudes (vertical dashed lines, right).
Mentions: Electrically-evoked compound action potentials were absent in all the implanted patients except for Subject 7, who showed the electrically-evoked neural response at each electrode location (Fig. 7).Figure 7

Bottom Line: In this study we characterized the hearing dysfunction in OPA1-linked disorders and provided effective rehabilitative options to improve speech perception.The use of cochlear implant improved speech perception in all but one patient.Cochlear implantation improves speech perception and synchronous activation of auditory pathways by bypassing the site of lesion.

View Article: PubMed Central - PubMed

Affiliation: 1 Department of Neurosciences, University of Padova, Via Giustiniani 2, 35128 Padova, Italy 2 Audiology and Phoniatrics Service, Treviso Regional Hospital, Piazza Ospedale 1, 31100 Treviso, Italy rosamaria.santarelli@unipd.it.

Show MeSH
Related in: MedlinePlus