Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene
Auditory Neuropathy (AN) is characterized by disruption of temporal coding of acoustic signals in auditory nerve fibers resulting in alterations of auditory perceptions. Mutations in several genes have been associated to the most forms of AN. Underlying mechanisms include both pre-synaptic and post-...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-11-01
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| Series: | Audiology Research |
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| Online Access: | https://www.mdpi.com/2039-4349/11/4/59 |
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| author | Rosamaria Santarelli Pietro Scimemi Chiara La Morgia Elona Cama Ignacio del Castillo Valerio Carelli |
| author_facet | Rosamaria Santarelli Pietro Scimemi Chiara La Morgia Elona Cama Ignacio del Castillo Valerio Carelli |
| author_sort | Rosamaria Santarelli |
| collection | DOAJ |
| description | Auditory Neuropathy (AN) is characterized by disruption of temporal coding of acoustic signals in auditory nerve fibers resulting in alterations of auditory perceptions. Mutations in several genes have been associated to the most forms of AN. Underlying mechanisms include both pre-synaptic and post-synaptic damage involving inner hair cell (IHC) depolarization, neurotransmitter release, spike initiation in auditory nerve terminals, loss of auditory fibers and impaired conduction. In contrast, outer hair cell (OHC) activities (otoacoustic emissions [OAEs] and cochlear microphonic [CM]) are normal. Disordered synchrony of auditory nerve activity has been suggested as the basis of both the alterations of auditory brainstem responses (ABRs) and reduction of speech perception. We will review how electrocochleography (ECochG) recordings provide detailed information to help objectively define the sites of auditory neural dysfunction and their effect on receptor summating potential (SP) and neural compound action potential (CAP), the latter reflecting disorders of ribbon synapses and auditory nerve fibers. |
| first_indexed | 2024-03-10T04:35:31Z |
| format | Article |
| id | doaj.art-37e0df065ec847a69e75d200f3a1997c |
| institution | Directory Open Access Journal |
| issn | 2039-4349 |
| language | English |
| last_indexed | 2024-03-10T04:35:31Z |
| publishDate | 2021-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Audiology Research |
| spelling | doaj.art-37e0df065ec847a69e75d200f3a1997c2023-11-23T03:48:39ZengMDPI AGAudiology Research2039-43492021-11-0111463965210.3390/audiolres11040059Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 GeneRosamaria Santarelli0Pietro Scimemi1Chiara La Morgia2Elona Cama3Ignacio del Castillo4Valerio Carelli5Department of Neurosciences, University of Padova, Via Belzoni 160, 35121 Padova, ItalyDepartment of Neurosciences, University of Padova, Via Belzoni 160, 35121 Padova, ItalyDepartment of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Via Ugo Foscolo 7, 40123 Bologna, ItalyDepartment of Neurosciences, University of Padova, Via Belzoni 160, 35121 Padova, ItalyServicio de Genética, Hospital Universitario Ramón y Cajal, IRYCIS, 28034 Madrid, SpainDepartment of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Via Ugo Foscolo 7, 40123 Bologna, ItalyAuditory Neuropathy (AN) is characterized by disruption of temporal coding of acoustic signals in auditory nerve fibers resulting in alterations of auditory perceptions. Mutations in several genes have been associated to the most forms of AN. Underlying mechanisms include both pre-synaptic and post-synaptic damage involving inner hair cell (IHC) depolarization, neurotransmitter release, spike initiation in auditory nerve terminals, loss of auditory fibers and impaired conduction. In contrast, outer hair cell (OHC) activities (otoacoustic emissions [OAEs] and cochlear microphonic [CM]) are normal. Disordered synchrony of auditory nerve activity has been suggested as the basis of both the alterations of auditory brainstem responses (ABRs) and reduction of speech perception. We will review how electrocochleography (ECochG) recordings provide detailed information to help objectively define the sites of auditory neural dysfunction and their effect on receptor summating potential (SP) and neural compound action potential (CAP), the latter reflecting disorders of ribbon synapses and auditory nerve fibers.https://www.mdpi.com/2039-4349/11/4/59OPA1-related deafnessOTOF-related hearing losselectrocochleographycochlear implantsspeech perception |
| spellingShingle | Rosamaria Santarelli Pietro Scimemi Chiara La Morgia Elona Cama Ignacio del Castillo Valerio Carelli Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene Audiology Research OPA1-related deafness OTOF-related hearing loss electrocochleography cochlear implants speech perception |
| title | Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene |
| title_full | Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene |
| title_fullStr | Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene |
| title_full_unstemmed | Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene |
| title_short | Electrocochleography in Auditory Neuropathy Related to Mutations in the OTOF or OPA1 Gene |
| title_sort | electrocochleography in auditory neuropathy related to mutations in the otof or opa1 gene |
| topic | OPA1-related deafness OTOF-related hearing loss electrocochleography cochlear implants speech perception |
| url | https://www.mdpi.com/2039-4349/11/4/59 |
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