Otoacoustic emissions in newborns evoked by 0.5kHz tone bursts

Jędrzejczak, Wiesław Wiktor; Konopka, Wiesław; Kochanek, Krzysztof; Skarżyńśki, Henryk

doi:10.1016/j.ijporl.2015.07.001

Cited by 6 publications

(6 citation statements)

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“…Stapedius reflexes were analyzed for the frequencies 500, 1000, 2000 and 4000 Hz in the ipsi and contralateral modes [ 26 ]. OAE were evoked by standard-click stimuli and 500 Hz tone bursts by using the ILO-292 system (Otodynamics Ltd, Hatfield, United Kingdom) [ 27 – 29 ]. ABRs were recorded using the Integrity V500 system (Vivosonic Inc., Toronto, Canada).…”

Section: Methodsmentioning

confidence: 99%

Novel neuro-audiological findings and further evidence for TWNK involvement in Perrault syndrome

et al. 2017

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BackgroundHearing loss and ovarian dysfunction are key features of Perrault syndrome (PRLTS) but the clinical and pathophysiological features of hearing impairment in PRLTS individuals have not been addressed. Mutations in one of five different genes HSD17B4, HARS2, LARS2, CLPP or TWNK (previous symbol C10orf2) cause the autosomal recessive disorder but they are found only in about half of the patients.MethodsWe report on two siblings with a clinical picture resembling a severe, neurological type of PRLTS. For an exhaustive characterisation of the phenotype neuroimaging with volumetric measurements and objective measures of cochlear hair cell and auditory nerve function (otoacustic emissions and auditory brainstem responses) were used. Whole exome sequencing was applied to identify the genetic cause of the disorder. Co-segregation of the detected mutations with the phenotype was confirmed by Sanger sequencing. In silico analysis including 3D protein structure modelling was used to predict the deleterious effects of the detected variants on protein function.ResultsWe found two rare biallelic mutations in TWNK, encoding Twinkle, an essential mitochondrial helicase. Mutation c.1196A>G (p.Asn399Ser) recurred for the first time in a patient with PRLTS and the second mutation c.1802G>A (p.Arg601Gln) was novel for the disorder. In both patients neuroimaging studies showed diminished cervical enlargement of the spinal cord and for the first time in PRLTS partial atrophy of the vestibulocochlear nerves and decreased grey and increased white matter volumes of the cerebellum. Morphological changes in the auditory nerves, their desynchronized activity and partial cochlear dysfunction underlay the complex mechanism of hearing impairment in the patients.ConclusionsOur study unveils novel features on the phenotypic landscape of PRLTS and provides further evidence that the newly identified for PRLTS TWNK gene is involved in its pathogenesis.Electronic supplementary materialThe online version of this article (doi:10.1186/s12967-017-1129-4) contains supplementary material, which is available to authorized users.

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Section: Methodsmentioning

confidence: 99%

Novel neuro-audiological findings and further evidence for TWNK involvement in Perrault syndrome

et al. 2017

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“…[4] Auditory brainstem response [ABR], otoacoustic emissions [OAEs], and automated ABR [AABR] testing have all been used in newborn hearing-screening programs for 20 years .OAEs are consider to be fast objective screening test, it can be used to evaluate the cochlear function for the 500-6000 Hz frequency range. The presence of evoked OAE responses indicating normal or close to normal hearing sensitivity [5] .…”

Section: Introductionmentioning

confidence: 99%

“…However, OAE is not an adequate screening tool in infants that at high risk for neuropathy. Infants were in the NICU or in the hospital for more than 5 days should undergo an ABR testing to insure that we don't miss the presence of neural hearing loss [5] .…”

Section: Introductionmentioning

confidence: 99%

Hearing Screening in Neonates of Pre-Eclamptic Mother

Amer

Beshr

Gaber

et al. 2020

International Journal of Medical Arts

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Background: The incidence of congenital sensorineural hearing loss in the newborn is higher than the combined incidence of all the metabolic conditions that we currently screen for with blood tests, the tool we are using, and the timing of screening are very important. Aim of the work: To evaluate the possible effects of pre-eclampsia on the neonate's hearing and determine the best timing to perform the screening. Patients and Methods: Seventy neonates were included, they were divided into two groups: control group included 40 neonates born to healthy mothers and study group included 30 neonates born to pre-eclamptic mothers. All neonates in this study subjected to the following: screening with handheld transient evoked otoacoustic emission [TEOAEs] within the first 48 hours after delivery. Infants who failed the first TEOAEs, were reexamined two weeks later. If failed, they were reexamined for the third time with TEOAEs two weeks later. Results: There were statistically significant difference between the study and the control group as regards the TEOAES results [p-value = 0.001] in the first and second screening tests. In the control group, 73.8% passed from the first time, versus 41.7% of the study group. In the second TEOAEs, 17.5% control versus 41.7% of the study group. Conclusion: Preeclampsia has some temporary effect on hearing in the newborns of pre-eclamptic mothers. So, it's better to postpone the first neonatal hearing screening of these babies, to be performed 2 weeks after delivery.

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“…In fact, it has subsequently been reported that all types of evoked OAEs are in some way affected by the presence of SOAEs, usually by showing higher amplitudes. This is the case for CEOAEs [ 21 , 25 – 27 ]; for DPOAEs [ 25 , 28 ]; for SFOAEs [ 29 ]; and for TBOAEs as well [ 30 , 31 , 32 , 33 ]. The dependence of OAE level on stimulus level–i.e.…”

Section: Introductionmentioning

confidence: 99%

“…The present study used a commercially available system, frequently used in research, in order to provide a standard baseline. Additional motivation came from previous TBOAE studies, especially of low-frequency (0.5 kHz) TBOAEs [ 33 ], where TBOAEs were affected by SSOAEs. A limitation of this previous study, however, was that the TBOAEs were evoked only by a single 80 dB SPL stimulus.…”

Section: Introductionmentioning

confidence: 99%

Otoacoustic emissions from ears with spontaneous activity behave differently to those without: Stronger responses to tone bursts as well as to clicks

2018

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It has been reported that both click-evoked otoacoustic emissions (CEOAEs) and distortion product otoacoustic emissions (DPOAEs) have higher amplitudes in ears that possess spontaneous otoacoustic emissions (SOAEs). The general aim of the present study was to investigate whether the presence of spontaneous activity in the cochlea affected tone-burst evoked otoacoustic emissions (TBOAEs). As a benchmark, the study also measured growth functions of CEOAEs. Spontaneous activity in the cochlea was measured by the level of synchronized spontaneous otoacoustic emissions (SSOAEs), an emission evoked by a click but closely related to spontaneous otoacoustic emissions (SOAEs, which are detectable without any stimulus). Measurements were made on a group of 15 adults whose ears were categorized as either having recordable SSOAEs or no SSOAEs. In each ear, CEOAEs and TBOAEs were registered at frequencies of 0.5, 1, 2, and 4 kHz, and input/output functions were measured at 40, 50, 60, 70, and 80 dB SPL. Global and half-octave-band values of response level and latency were estimated. Our main finding was that in ears with spontaneous activity, TBOAEs had higher levels than in ears without. The difference was more apparent for global values, but were also seen with half-octave-band analysis. Input/output functions had similar growth rates for ears with and without SSOAEs. There were no significant differences in latencies between TBOAEs from ears with and without SSOAEs, although latencies tended to be longer for lower stimulus levels and lower stimulus frequencies. When TBOAE levels were compared to CEOAE levels, the latter showed greater differences between recordings from ears with and without SSOAEs. Although TBOAEs reflect activity from a more restricted cochlear region than CEOAEs, at all stimulus frequencies their behavior still depends on whether SSOAEs are present or not.

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Otoacoustic emissions in newborns evoked by 0.5kHz tone bursts

Cited by 6 publications

References 30 publications

Novel neuro-audiological findings and further evidence for TWNK involvement in Perrault syndrome

Novel neuro-audiological findings and further evidence for TWNK involvement in Perrault syndrome

Hearing Screening in Neonates of Pre-Eclamptic Mother

Otoacoustic emissions from ears with spontaneous activity behave differently to those without: Stronger responses to tone bursts as well as to clicks

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