Study of MenièRe’s Disease By Electrocochleography

Schmidt, P. H.; Eggermont, Jos J.; Odenthal, D. W.

doi:10.1080/16512251.1974.11675748

Cited by 63 publications

(23 citation statements)

References 3 publications

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“…Firstly, SP potentials recorded in controls show little sensitivity to a neural adaptation paradigm . Moreover, the duration of SP potentials in subjects with normal hearing (Eggermont and Odenthal 1974b) and patients affected by cochlear disorders (Schmidt et al 1974;Ferraro et al 1994) is typically restricted to the time of the stimulus when utilising tone burst stimulation (Sellick et al 2003).…”

Section: Discussionmentioning

confidence: 99%

Abnormal Cochlear Potentials from Deaf Patients with Mutations in the Otoferlin Gene

Santarelli

Castillo

Rodríguez-Ballesteros

et al. 2009

JARO

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Otoferlin is involved in neurotransmitter release at the synapse between inner hair cells (IHCs) and auditory nerve fibres, and mutations in the OTOF gene result in severe to profound hearing loss. Abnormal sound-evoked cochlear potentials were recorded with transtympanic electrocochleography from four children with otoferlin (OTOF) mutations to evaluate physiological effects in humans of abnormal neurotransmitter release from IHCs. The subjects were profoundly deaf with absent auditory brainstem responses and preserved otoacoustic emissions consistent with auditory neuropathy. Two children were compound heterozygotes for mutations c.2732_2735dupAGCT and p.Ala964Glu; one subject was homozygous for mutation p.Phe1795Cys, and one was compound heterozygote for two novel mutations c.1609delG in exon 16 and c.1966delC in exon 18. Cochlear potentials evoked by clicks from 60 to 120 dB peak equivalent sound pressure level were compared to recordings obtained from 16 normally hearing children. Cochlear microphonic (CM) was recorded with normal amplitudes from all but one ear. After cancelling CM, cochlear potentials were of negative polarity with reduced amplitude and prolonged duration compared to controls. These cochlear potentials were recorded as low as 50-90 dB below behavioural thresholds in contrast to the close correlation in controls between cochlear potentials and behavioural threshold. Summating potential was identified in five out of eight ears with normal latency whilst auditory nerve compound action potentials were either absent or of low amplitude. Stimulation at high rates reduced amplitude and duration of the prolonged potentials, consistent with neural generation. This study suggests that mechano-electrical transduction and cochlear amplification are normal in patients with OTOF mutations. The low-amplitude prolonged negative potentials are consistent with decreased neurotransmitter release resulting in abnormal dendritic activation and impairment of auditory nerve firing.

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Section: Discussionmentioning

confidence: 99%

Abnormal Cochlear Potentials from Deaf Patients with Mutations in the Otoferlin Gene

Santarelli

Castillo

Rodríguez-Ballesteros

et al. 2009

JARO

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“…Thus, various measures of evoked electrical activity, particularly electrocochleography studies, have focused on amplitude measures of the summating potential (SP) alone or on the ratio of the SP amplitude to compound action potential (AP) of the VIIIth cranial nerve (5)(6)(7)(8)(9)(10)(11). The SP/AP ratio has been studied for a number of years and the results have varied greatly.…”

Section: Hypothesis: Endolymphatic Hydrops In Patients Diagnosed Withmentioning

confidence: 99%

A Diagnostic Test for Ménière's Disease and Cochlear Hydrops: Impaired High-Pass Noise Masking of Auditory Brainstem Responses

Don¹,

Kwong²,

Tanaka³

2005

Otology &Amp; Neurotology

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“…In order to ensure that the recordings obtained in our laboratory represent true electrophysiological responses from the cochlea, it is necessary that the technique of the recordings fulfils the uniform standards for the measurements (7) and that the results correspond with the normative data obtained in other laboratories (9)(10)(11)(12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

Recording Techniques for Transtympanic Electrocochleography in Clinical Practice

Sass

Densert²,

Arlinger³

1998

Acta Oto-Laryngologica

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Enhancement of the signal-to-noise ratio is a central issue in electrically evoked response techniques. Transtympanic electrocochleography requires careful methodological considerations as responses from the cochlea may easily be affected by various sources of electromagnetic noise. The final electrophysiological waveforms are acquired by advanced signal processing which influences the content of the recordings. A standard method of transtympanic electrocohleography is presented here to increase the feasibility of the measurements in daily clinical use and to facilitate uniform interpretation of the recordings. The electrocochleographic recordings were carried out in 61 patients with a clinical diagnosis of Meniere's disease and in 15 ears of normal hearing subjects. The electrophysiological data are presented and set in relation to the results reported by other laboratories under equivalent recording conditions. Methodological factors of consequence for the quality of the recordings, such as testing environment, properties of the equipment, mode to produce and deliver stimuli and properties of the electrodes, are analysed.

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Study of MenièRe’s Disease By Electrocochleography

Cited by 63 publications

References 3 publications

Abnormal Cochlear Potentials from Deaf Patients with Mutations in the Otoferlin Gene

Abnormal Cochlear Potentials from Deaf Patients with Mutations in the Otoferlin Gene

A Diagnostic Test for Ménière's Disease and Cochlear Hydrops: Impaired High-Pass Noise Masking of Auditory Brainstem Responses

Recording Techniques for Transtympanic Electrocochleography in Clinical Practice

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