Patients with Auditory Neuropathy/Dys-synchrony Lack Efferent Suppression of Transient Evoked Otoacoustic Emissions

Hood, Linda J.; Berlín, Charles I.; Bordelon, Jill; Rose, Kelly

doi:10.1055/s-0040-1715746

Cited by 67 publications

(23 citation statements)

References 20 publications

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“…When suppression was measured within the 8-18 ms time window, as suggested by Hood et al [13,25], the mean suppression was 1.32 dB. This value was greater than the 0.9 dB obtained by Berlin et al [12] and 0.5 dB obtained by Tavartkiladze et al [29], but smaller than the 2.4 dB obtained by Hood et al [13].…”

Section: Discussionmentioning

confidence: 65%

“…Third, TEOAE suppression obtained by ipsilateral stimulation may be greater than that by contralateral stimulation. Hood et al [13] obtained mean TEOAE suppression of 2.4 and 1.5 dB in normal hearing adults by ipsilateral and contralateral stimulation, respectively.…”

Section: Introductionmentioning

confidence: 98%

“…The MOC reflex provides useful clinical information for understanding the auditory system and auditory disorders. Studies have reported reduced or no suppression of OAEs in patients with auditory neuropathy spectrum disorders (ANSD) [13], auditory processing disorders [14], unilateral acoustic neuroma with measurable OAEs [15], space-occupying lesions in the superior olivary complex of the brainstem [16], and unilateral tinnitus [17]. Individuals with hyperacusis may show large TEOAE suppression in the affected ear [18].…”

Section: Introductionmentioning

confidence: 99%

“…Hood et al [13] compared TEOAE suppression obtained from nine patients with bilateral ANSD and normally hearing adults matched for age and gender. As suppressor, 400 ms of wideband noise was presented either binaurally, ipsilaterally, or contralaterally in a forward-masking paradigm (again, 10 ms of silence separated the noise from the linear clicks).…”

Section: Introductionmentioning

confidence: 99%

“…There is also a lack of consensus on the amount of suppression considered normal in normally hearing adults. Findings from studies of ipsilateral suppression of TEOAEs in normally hearing adults range from 0.5-1 dB [12] to 2-4 dB [13]. Such variations can be attributed to subject differences, test protocols, and small sample sizes (<10).…”

Section: Introductionmentioning

confidence: 99%

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Ipsilateral Suppression of Transient Evoked Otoacoustic Emissions in Adults

et al. 2016

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Background: Measurement of the medial olivocochlear (MOC) reflex provides useful clinical information for understanding the function of the auditory system. Although transient evoked otoacoustic emission (TEOAE) suppression has been demonstrated to be an indicator of MOC activity, its full role and fine details of TEOAE suppression spectra are still not clear. The aim of this study was to investigate details of ipsilateral suppression of TEOAEs in normally hearing adults.Material and methods: Exactly 29 adults (13 males, 16 females, mean age 26.5 years, range 18-42 years), who passed a battery of tests including otoscopy, pure tone audiometry, immittance, and TEOAE tests, participated in the study. Suppression was evaluated by comparing TEOAEs obtained with and without an ipsilateral suppressor in a forward-masking paradigm.Results: In general, suppression was small -less than 1.4 dB at all frequencies studied. The spectrum of mean TEOAE suppression showed suppression was greatest between 586 and 3711 Hz (0.6-1.4 dB) but less than 0.6 dB between 3906 and 4883 Hz. Mean suppression increased with post-stimulus time from 0.1 dB in the 2-4 ms time window to a maximum of 2.26 dB in the 16-18 ms window; the mean suppression between 8 and 18 ms after noise stimulation was 1.32 dB (range=0.22-3.23 dB). There were no significant gender or ear-laterality effects. Noise levels as measured in the ear canal were found to have a significant effect on calculated suppression at some frequencies. Conclusions:The present study provides evidence of small ipsilateral TEOAE suppression by forward-masking noise in normally hearing adults. However, care is needed in interpreting the findings as noise in the ear canal can be a confounding factor during measurement of TEOAE suppression.

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Section: Discussionmentioning

confidence: 65%

Section: Introductionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Ipsilateral Suppression of Transient Evoked Otoacoustic Emissions in Adults

et al. 2016

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Clinical and Audiological Features in Auditory Neuropathy

Madden

Rutter²,

Hilbert³

et al. 2002

Arch Otolaryngol Head Neck Surg

201

172

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Management of AN in children requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. Prematurity, genetics, and hyperbilirubinemia appear to be significant factors in the development of AN; hyperbilirubinemia can be associated with spontaneous improvement of hearing thresholds. For those children not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.

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A homozygous SLITRK6 nonsense mutation is associated with progressive auditory neuropathy in humans

et al. 2013

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Objective SLITRK family proteins control neurite outgrowth and regulate synaptic development. In mice, Slitrk6 plays a role in the survival and innervation of sensory neurons in the inner ear, vestibular apparatus, and retina, and also influences axial eye length. We provide the first detailed description of the auditory phenotype in humans with recessive SLITRK6 deficiency. Study Design Prospective observational case study. Methods Nine closely related Amish subjects from an endogamous Amish community of Pennsylvania underwent audiologic and vestibular testing. Single nucleotide polymorphism microarrays were used to map the chromosome locus and Sanger sequencing or high-resolution melt analysis were used to confirm the allelic variant. Results All 9 subjects were homozygous for a novel nonsense variant of SLITRK6 (c.1240C>T, p.Gln414Ter). Adult patients had high myopia. The 4 oldest SLITRK6 c.1240C>T homozygotes had absent ipsilateral middle ear muscle reflexes (MEMRs). Distortion product otoacoustic emissions (DPOAEs) were absent in all ears tested and the cochlear microphonic (CM) was increased in amplitude and duration in young patients and absent in the two oldest subjects. Auditory brainstem responses (ABRs) were dys-synchronised bilaterally with no reproducible waves I, III or V at high intensities. Hearing loss and speech reception threshold deteriortated symmetrically with age, resulting in severe-to-profound hearing impairment by early adulthood. Vestibular evoked myogenic potentials were normal in three ears and absent in one. Conclusion Homozygous SLITRK6 c.1240C>T (p.Gln414Ter) nonsense mutations are associated with high myopia, cochlear dysfunction attributed to outer hair cell disease, and progressive auditory neuropathy.

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Patients with Auditory Neuropathy/Dys-synchrony Lack Efferent Suppression of Transient Evoked Otoacoustic Emissions

Cited by 67 publications

References 20 publications

Ipsilateral Suppression of Transient Evoked Otoacoustic Emissions in Adults

Ipsilateral Suppression of Transient Evoked Otoacoustic Emissions in Adults

Clinical and Audiological Features in Auditory Neuropathy

A homozygous SLITRK6 nonsense mutation is associated with progressive auditory neuropathy in humans

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