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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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Articulation gain curves from patients with DOA. Articulation curves obtained from OPA1-H and OPA1-M groups have been superimposed on the mean articulation functions (dashed lines) with 95% confidence limits (shadowed areas) calculated for controls at corresponding PTA values. These were defined by minimum and maximum (10–14 dB) PTA values in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (15–28 dB, 35–50 dB, 58–73 dB). Mean function is displayed for the OPA1-H group, whereas articulation curves from individual ears have been considered for the OPA1-M patients due to the high variability of scores. Speech intelligibility was within normal limits in OPA1-H patients, whereas a remarkable decrease in reception scores compared to controls was found for all but one of the OPA1-M subjects. R = right, L = left.
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awu378-F1: Articulation gain curves from patients with DOA. Articulation curves obtained from OPA1-H and OPA1-M groups have been superimposed on the mean articulation functions (dashed lines) with 95% confidence limits (shadowed areas) calculated for controls at corresponding PTA values. These were defined by minimum and maximum (10–14 dB) PTA values in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (15–28 dB, 35–50 dB, 58–73 dB). Mean function is displayed for the OPA1-H group, whereas articulation curves from individual ears have been considered for the OPA1-M patients due to the high variability of scores. Speech intelligibility was within normal limits in OPA1-H patients, whereas a remarkable decrease in reception scores compared to controls was found for all but one of the OPA1-M subjects. R = right, L = left.

Mentions: Articulation-gain curves were obtained from all subjects. Ears were pooled into different classes of PTA. These were defined by minimum and maximum PTA levels in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (Fig. 1). Subjects with profound hearing loss were not included. Because of the high variability of scores, in the case of the OPA1-M patients, the articulation curves obtained from individual ears were considered.Figure 1


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)

Articulation gain curves from patients with DOA. Articulation curves obtained from OPA1-H and OPA1-M groups have been superimposed on the mean articulation functions (dashed lines) with 95% confidence limits (shadowed areas) calculated for controls at corresponding PTA values. These were defined by minimum and maximum (10–14 dB) PTA values in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (15–28 dB, 35–50 dB, 58–73 dB). Mean function is displayed for the OPA1-H group, whereas articulation curves from individual ears have been considered for the OPA1-M patients due to the high variability of scores. Speech intelligibility was within normal limits in OPA1-H patients, whereas a remarkable decrease in reception scores compared to controls was found for all but one of the OPA1-M subjects. R = right, L = left.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

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

awu378-F1: Articulation gain curves from patients with DOA. Articulation curves obtained from OPA1-H and OPA1-M groups have been superimposed on the mean articulation functions (dashed lines) with 95% confidence limits (shadowed areas) calculated for controls at corresponding PTA values. These were defined by minimum and maximum (10–14 dB) PTA values in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (15–28 dB, 35–50 dB, 58–73 dB). Mean function is displayed for the OPA1-H group, whereas articulation curves from individual ears have been considered for the OPA1-M patients due to the high variability of scores. Speech intelligibility was within normal limits in OPA1-H patients, whereas a remarkable decrease in reception scores compared to controls was found for all but one of the OPA1-M subjects. R = right, L = left.
Mentions: Articulation-gain curves were obtained from all subjects. Ears were pooled into different classes of PTA. These were defined by minimum and maximum PTA levels in the OPA1-H group, whereas the OPA1-M subjects were pooled into three classes characterized by increasing PTA values (Fig. 1). Subjects with profound hearing loss were not included. Because of the high variability of scores, in the case of the OPA1-M patients, the articulation curves obtained from individual ears were considered.Figure 1

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