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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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Cochlear microphonic potentials.Left: Cochlear microphonic potentials recorded at 120 dB SPL from one control with normal hearing, one hearing-impaired subject with cochlear hearing loss and one representative OPA1-M patient (Subject 7, left ear). Right: Mean cochlear microphonic amplitudes are reported as a function of stimulus intensity for the OPA1-M patients and for both normally-hearing and hearing-impaired subjects. Cochlear microphonic potentials recorded from OPA1-M patients are significantly larger compared to controls with normal hearing and hearing-impaired subjects with cochlear hearing loss (Cochlear HL).
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awu378-F3: Cochlear microphonic potentials.Left: Cochlear microphonic potentials recorded at 120 dB SPL from one control with normal hearing, one hearing-impaired subject with cochlear hearing loss and one representative OPA1-M patient (Subject 7, left ear). Right: Mean cochlear microphonic amplitudes are reported as a function of stimulus intensity for the OPA1-M patients and for both normally-hearing and hearing-impaired subjects. Cochlear microphonic potentials recorded from OPA1-M patients are significantly larger compared to controls with normal hearing and hearing-impaired subjects with cochlear hearing loss (Cochlear HL).

Mentions: Cochlear microphonic potentials were recorded from all the tested OPA1-M patients (Fig. 3). The responses proved to be significantly larger compared to both controls and subjects with cochlear hearing loss (Fig. 3). An enhancement of cochlear microphonic amplitude in patients with auditory neuropathy might result from decreased activity of the efferent system secondary to abnormal auditory nerve fibre activation (Santarelli and Arslan, 2002).Figure 3


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)

Cochlear microphonic potentials.Left: Cochlear microphonic potentials recorded at 120 dB SPL from one control with normal hearing, one hearing-impaired subject with cochlear hearing loss and one representative OPA1-M patient (Subject 7, left ear). Right: Mean cochlear microphonic amplitudes are reported as a function of stimulus intensity for the OPA1-M patients and for both normally-hearing and hearing-impaired subjects. Cochlear microphonic potentials recorded from OPA1-M patients are significantly larger compared to controls with normal hearing and hearing-impaired subjects with cochlear hearing loss (Cochlear HL).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

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

awu378-F3: Cochlear microphonic potentials.Left: Cochlear microphonic potentials recorded at 120 dB SPL from one control with normal hearing, one hearing-impaired subject with cochlear hearing loss and one representative OPA1-M patient (Subject 7, left ear). Right: Mean cochlear microphonic amplitudes are reported as a function of stimulus intensity for the OPA1-M patients and for both normally-hearing and hearing-impaired subjects. Cochlear microphonic potentials recorded from OPA1-M patients are significantly larger compared to controls with normal hearing and hearing-impaired subjects with cochlear hearing loss (Cochlear HL).
Mentions: Cochlear microphonic potentials were recorded from all the tested OPA1-M patients (Fig. 3). The responses proved to be significantly larger compared to both controls and subjects with cochlear hearing loss (Fig. 3). An enhancement of cochlear microphonic amplitude in patients with auditory neuropathy might result from decreased activity of the efferent system secondary to abnormal auditory nerve fibre activation (Santarelli and Arslan, 2002).Figure 3

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