Electrocochleography in children with auditory synaptopathy/neuropathy: Diagnostic findings and characteristic parameters

Stuermer, Konrad Johannes; Beutner, Dirk; Foerst, Astrid; Hahn, Moritz; Lang‐Roth, Ruth; Walger, Martin

doi:10.1016/j.ijporl.2014.11.025

Cited by 24 publications

(16 citation statements)

References 23 publications

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“…Furthermore, in both ANSD and non-ANSD groups, when a large CAP did exist the morphology of the SP was distinctly different, being close to zero in most cases with no preference for polarity. These findings of a relatively reduced CAP and enhanced SP closely parallel those reported previously for ANSD subjects using high frequency stimuli such as clicks and 8 kHz tone bursts (McMahon et al, 2008; Santarelli et al, 2008; Stuermer et al, 2015). …”

Section: Discussionsupporting

confidence: 87%

“…Most previous studies of ECochG in ANSD children undergoing cochlear implantation used 8 kHz tone pips or clicks as stimuli (Gibson and Sanli, 2007; McMahon et al, 2008; Santarelli et al, 2008; Stuermer et al, 2015). These are primarily high frequency stimuli, which is an appropriate choice for many ANSD subjects who typically have good responses to high frequencies.…”

Section: Discussionmentioning

confidence: 99%

“…Many subjects with ANSD have hearing loss and/or speech perception deficits severe enough that treatment with a cochlear implant (CI) is indicated. A number of studies of the electrocochleography (ECochG) of ANSD subjects receiving CIs have been done, however these studies used acoustic stimuli specialized for this group such as high frequency 8 kHz tone pips or clicks (McMahon et al, 2008; Santarelli et al, 2008; Santarelli, 2010; Stuermer et al, 2015). While high frequencies may be useful in diagnosis, most of the ECochG responses in CI subjects, in both children and adults, are in fact to low frequencies (Fitzpatrick et al, 2014; McClellan et al, 2014; Formeister et al, 2015).…”

Section: Introductionmentioning

confidence: 99%

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Intraoperative Electrocochleographic Characteristics of Auditory Neuropathy Spectrum Disorder in Cochlear Implant Subjects

et al. 2017

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Auditory neuropathy spectrum disorder (ANSD) is characterized by an apparent discrepancy between measures of cochlear and neural function based on auditory brainstem response (ABR) testing. Clinical indicators of ANSD are a present cochlear microphonic (CM) with small or absent wave V. Many identified ANSD patients have speech impairment severe enough that cochlear implantation (CI) is indicated. To better understand the cochleae identified with ANSD that lead to a CI, we performed intraoperative round window electrocochleography (ECochG) to tone bursts in children (n = 167) and adults (n = 163). Magnitudes of the responses to tones of different frequencies were summed to measure the “total response” (ECochG-TR), a metric often dominated by hair cell activity, and auditory nerve activity was estimated visually from the compound action potential (CAP) and auditory nerve neurophonic (ANN) as a ranked “Nerve Score”. Subjects identified as ANSD (45 ears in children, 3 in adults) had higher values of ECochG-TR than adult and pediatric subjects also receiving CIs not identified as ANSD. However, nerve scores of the ANSD group were similar to the other cohorts, although dominated by the ANN to low frequencies more than in the non-ANSD groups. To high frequencies, the common morphology of ANSD cases was a large CM and summating potential, and small or absent CAP. Common morphologies in other groups were either only a CM, or a combination of CM and CAP. These results indicate that responses to high frequencies, derived primarily from hair cells, are the main source of the CM used to evaluate ANSD in the clinical setting. However, the clinical tests do not capture the wide range of neural activity seen to low frequency sounds.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Intraoperative Electrocochleographic Characteristics of Auditory Neuropathy Spectrum Disorder in Cochlear Implant Subjects

et al. 2017

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“…Moreover, the VsEP is a neural response, and should not be used as a definitive indicator of vestibular HCs function. Auditory neuropathy spectrum disorder is an example pathology of a pathology which affects peripheral nerve but not HCs function (Stuermer et al, 2015; Kim et al, 2016). Lastly, precisely which HCs and neurones are responsible for generating the VsEP is still not entirely clear.…”

Section: Utility Of Evestg In Researchmentioning

confidence: 99%

Electrophysiological Measurements of Peripheral Vestibular Function—A Review of Electrovestibulography

Brown

Pastras

Curthoys

2017

Front. Syst. Neurosci.

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Electrocochleography (EcochG), incorporating the Cochlear Microphonic (CM), the Summating Potential (SP), and the cochlear Compound Action Potential (CAP), has been used to study cochlear function in humans and experimental animals since the 1930s, providing a simple objective tool to assess both hair cell (HC) and nerve sensitivity. The vestibular equivalent of ECochG, termed here Electrovestibulography (EVestG), incorporates responses of the vestibular HCs and nerve. Few research groups have utilized EVestG to study vestibular function. Arguably, this is because stimulating the cochlea in isolation with sound is a trivial matter, whereas stimulating the vestibular system in isolation requires significantly more technical effort. That is, the vestibular system is sensitive to both high-level sound and bone-conducted vibrations, but so is the cochlea, and gross electrical responses of the inner ear to such stimuli can be difficult to interpret. Fortunately, several simple techniques can be employed to isolate vestibular electrical responses. Here, we review the literature underpinning gross vestibular nerve and HC responses, and we discuss the nomenclature used in this field. We also discuss techniques for recording EVestG in experimental animals and humans and highlight how EVestG is furthering our understanding of the vestibular system.

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“…Acoustic reflexes ipsi-and contralateral are absent [Berlin et al, 2010;Doyle et al, 1998;He et al, 2013;Manchaiah et al, 2011;Moser et al, 2006;Starr et al, 1996]. The most sensitive but invasive diagnostic tool to verify the diagnosis of AS/AN is transtympanic electrocochleography; it enables the differentiation of pre-and postsynaptic pathologies [McMahon et al, 2008;Santarelli and Arslan, 2002;Stuermer et al, 2015].…”

mentioning

confidence: 99%

AUNA2: A Novel Type of Non-Syndromic Slowly Progressive Auditory Synaptopathy/Auditory Neuropathy with Autosomal-Dominant Inheritance

Lang‐Roth

Fischer-Krall²,

Kornblum³

et al. 2017

Audiol Neurotol

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Background: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative. Materials and Methods: Here, we report detailed clinical data on a large German AS/AN family with slowly progressive postlingual hearing loss. Affected family members developed their first symptoms in their second decade. Moderate hearing loss in the fourth decade then progressed to profound hearing impairment in older family members. Comprehensive audiological and neurological tests were performed in the affected family members. Genetic testing comprised linkage analyses with polymorphic markers and a genome-wide linkage analysis using the Affymetrix GeneChip® Human Mapping 250K. Results and Conclusion: We identified a large family with autosomal-dominant AS/AN. By means of linkage analyses, the AUNA1 locus was excluded, and putatively linked regions on chromosomal bands 12q24 and 13q34 were identified as likely carrying the second locus for autosomal-dominant AS/AN (AUNA2). AUNA2 is associated with a slowly progressive postlingual hearing loss without any evidence for additional symptoms in other organ systems.

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Electrocochleography in children with auditory synaptopathy/neuropathy: Diagnostic findings and characteristic parameters

Cited by 24 publications

References 23 publications

Intraoperative Electrocochleographic Characteristics of Auditory Neuropathy Spectrum Disorder in Cochlear Implant Subjects

Intraoperative Electrocochleographic Characteristics of Auditory Neuropathy Spectrum Disorder in Cochlear Implant Subjects

Electrophysiological Measurements of Peripheral Vestibular Function—A Review of Electrovestibulography

AUNA2: A Novel Type of Non-Syndromic Slowly Progressive Auditory Synaptopathy/Auditory Neuropathy with Autosomal-Dominant Inheritance

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