Auditory Neuropathy with Preserved Cochlear Microphonics and Secondary Loss of Otoacoustic Emissions

Deltenre, Paul; Mansbach, Anne-Laure; Bozet, C.; Christiaens, Florence; Barthélémy, Pierre; Paulissen, D.; Renglet, Thierry

doi:10.3109/00206099909073022

Cited by 109 publications

(126 citation statements)

References 33 publications

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“…Acoustic reflexes are also absent. 1 This electrophysiologic profile suggests an abnormality of the hearing apparatus proximal to the outer hair cell that may include the synapses between the cochlear inner hair cells, neurons of the spiral ganglion and the entire auditory nerve. Clinically, the hearing loss is variable with an unsynchronized auditory signal, and often fluctuating with particular difficulty in speech perception in the presence of background noise.…”

Section: Introductionmentioning

confidence: 97%

Extremely low birth weight infants are at high risk for auditory neuropathy

et al. 2007

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Auditory neuropathy (AN) is a condition in which transmission of sound to the brain is abnormal. This is reflected as an electrophysiologic profile of normal otoacoustic emissions (OAE), with abnormal auditory brainstem evoked responses (ABR). Functionally speech perception is impaired and management strategies remain controversial. AN can be missed if high-risk newborns are screened for hearing loss with only OAE testing. The rate of sensorineural hearing loss (SNHL) in high-risk nursery infants is 10 times greater compared with normal term newborns. Therefore, we hypothesize that infants from the neonatal intensive care unit (NICU) are at significantly higher risk for AN than normal term infants.Objective: The objective of this study is to establish a prevalence rate and characterize risk factors for NICU graduates who demonstrate the AN electrophysiologic profile.Study Design: This retrospective study examined infants admitted to the NICU at Kapi'olani Medical Center for Women and Children in Honolulu, HI from 1999 through 2003. Infants were screened with automated ABR. Diagnostic testing and OAE were performed before discharge if the ABR was abnormal. Hospital courses of 24 AN, 71 SNHL and 95 gestational age (GA)-matched control infants with normal hearing were reviewed.Result: With a SNHL prevalence of 16.7/1000, the rate for AN was 5.6/1000 NICU infants. Compared to infants with SNHL, infants with AN were significantly younger (GA 28.3±4.8 AN vs 32.9±5.2 weeks SNHL, P<0.0001) and smaller (BW 1318±894 AN vs 1968±1006 g SNHL).Nearly two-thirds of the AN infants were ELBW and had significantly longer hospital stays compared to SNHL infants of the same birth weight group. Exposure to furosemide, aminoglycosides, vancomycin or dexamethasone was associated with increased AN but not SNHL. Peak bilirubin level correlated with SNHL but not AN.Conclusion: Low birth weight NICU infants are at significant risk for AN. ELBW infants are at significantly higher risk for both AN and SNHL.Infants admitted to the NICU should be routinely screened by automated ABR and if abnormal, further evaluation should be started before hospital discharge. Early identification of AN will result in better understanding of this disorder and lead to the development of appropriate intervention strategies.

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Section: Introductionmentioning

confidence: 97%

Extremely low birth weight infants are at high risk for auditory neuropathy

et al. 2007

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“…In such cases, the presence of CMs becomes the determinant finding in the differential diagnosis of this condition. 1,20,21 Some authors have proposed that AN/AD be defined as a condition with absent or abnormal ABEPs including wave I, associated with present OAEs and/or CMs. 20 In the past 10 years, many papers on auditory neuropathy have underlined the role of CMs, which are now analyzed with greater care in AN/AD patients.…”

Section: Introductionmentioning

confidence: 99%

“…1,20,21 Some authors have proposed that AN/AD be defined as a condition with absent or abnormal ABEPs including wave I, associated with present OAEs and/or CMs. 20 In the past 10 years, many papers on auditory neuropathy have underlined the role of CMs, which are now analyzed with greater care in AN/AD patients. Reports in the literature have shown that CMs in these patients are more prominent, having an abnormally increased amplitude, [23][24][25][26] and persist for up to 4 to 6 milliseconds after click stimulation, 22 contrary to what is found in normal subjects.…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, CMs may be identified by recording auditory evoked potentials using negative and positive polarity acoustic stimuli in which results may be compared, given that CMs are affected by the stimulus polarity, contrary to ABEPs. 1,20,25,34 To confirm the presence of CMs, some researchers block the insertion phone plastic tube to guarantee that recordings are auditory neurophysiological responses that truly reflect acoustic stimulation, rather than electrical artifacts. 3,20,24 Although the literature has suggested that cochlear function is normal in AN/AD, 3,13,34 it is important to analyze CMs in these cases; a number of findings about this specific cochlear potential in AN/AD patients appear to show that the cochlea may be dysfunctional.…”

Section: Introductionmentioning

confidence: 99%

“…1,20,25,34 To confirm the presence of CMs, some researchers block the insertion phone plastic tube to guarantee that recordings are auditory neurophysiological responses that truly reflect acoustic stimulation, rather than electrical artifacts. 3,20,24 Although the literature has suggested that cochlear function is normal in AN/AD, 3,13,34 it is important to analyze CMs in these cases; a number of findings about this specific cochlear potential in AN/AD patients appear to show that the cochlea may be dysfunctional. [23][24][25][26] The use of ABEP testing of CMs seems inadequate, as CMs cannot be carefully analyzed by this method.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Eletrococleografia extratimpânica na neuropatia/dessincronia auditiva

Anastásio

Alvarenga

Filho

2008

Rev. Bras. Otorrinolaringol.

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The brainstem auditory evoked potential (BAEP) is being extensively used as a method for the evaluation of cochlear function in individuals with diagnosis of auditory neuropathy/auditory dyssynchrony (AN/AD). In the absence of otoacoustic emissions, many cases of AN/AD have been diagnosed by the presence of CM identified in the BAEP. Aim: to demonstrate the clinical applicability of extratympanic electrocochleography (ET-Ecochg) in the differential diagnosis of AN/AD compared to the BAEP. Method: a 4-year-old child with a diagnosis of AN/AD seen at the Audiological Research Center was submitted to ET-Ecochg with a 2000 Hz tone burst in rarefaction and condensation polarities. Results: the ET-Ecochg exam was illustrated. Using an appropriate protocol, it was possible to demonstrate CM and to confirm it in the Ecochg, with a recording quality superior to that obtained in the BAEP. Conclusion: ET-Ecochg permitted a more detailed analysis of CM compared to the BAEP, thus showing clinical applicability for the investigation of cochlear function in AN/AD.

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Connexin 26 variants and auditory neuropathy/dys‐synchrony among children in schools for the deaf

Cheng

Brashears

et al. 2005

American J of Med Genetics Pt A

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Genetic and auditory studies of 731 children with severe-to-profound hearing loss in US schools for the deaf and 46 additional children receiving clinical services for hearing loss ranging from moderate to profound demonstrated that mutations in the connexin 26 (GJB2) and connexin 30 (GJB6) genes explain at least 12% of those with nonsyndromic sensorineural deafness. Otoacoustic emissions (OAEs) testing to detect functional outer hair cells indicated that 76 of the children had emissions and therefore may have (as yet unconfirmed) auditory neuropathy/dys-synchrony (AN/AD). Five of these children with OAEs were GJB2 homozygotes or compound heterozygotes with the genotypes 35delG/35delG, W77X/W77X, 35delG/360delGAG, 35delG/V95M, and V84M/M34T. In particular, unilateral AN/AD was confirmed in a child with moderate hearing loss and the 35delG/V95M genotype. Detecting OAEs in individuals with GJB2 mutations suggests that lack of functional gap junctions as a result of GJB2 mutations does not necessarily destroy all outer hair cell function.

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Auditory Neuropathy with Preserved Cochlear Microphonics and Secondary Loss of Otoacoustic Emissions

Cited by 109 publications

References 33 publications

Extremely low birth weight infants are at high risk for auditory neuropathy

Extremely low birth weight infants are at high risk for auditory neuropathy

Eletrococleografia extratimpânica na neuropatia/dessincronia auditiva

Connexin 26 variants and auditory neuropathy/dys‐synchrony among children in schools for the deaf

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