Late-Onset Unilateral Auditory Neuropathy/Dysynchrony: A Case Study

2009 ◽  
Vol 20 (03) ◽  
pp. 172-179 ◽  
Author(s):  
Andrew Stuart ◽  
Kristal N. Mills
Keyword(s):  
Otoacoustic Emissions ◽  
Late Onset ◽  
Internal Auditory Canal ◽  
Auditory Neuropathy ◽  
Auditory Brain Stem Response ◽  
Affected Side ◽  
Auditory Brain Stem ◽  
Long Latency ◽  
Acoustic Reflexes

Background: Auditory neuropathy/dysynchrony (AN/AD) typically develops early in life and is bilateral in nature. Purpose: Herein, we describe an unusual finding of late-onset unilateral AN/AD based on reported case history and audiometric findings. Research Design: A 64-year-old female presented with a complaint of a progressive unilateral hearing loss that had developed over the past two–three years. She underwent an extensive behavioral/electrophysiological test battery. Results: Magnetic resonance imaging was negative for internal auditory canal mass or lesion. A unilateral notched loss centered at 1000 Hz and other findings were consistent with late-onset unilateral AN/AD: observable bilateral otoacoustic emissions and cochlear microphonics, absent middle acoustic reflexes with stimulation on the affected side, abnormal auditory brain stem response on the affected side, and poorer speech recognition than would be predicted by the audiogram. Middle-latency and long-latency evoked responses were present bilaterally, although with lower amplitudes on the affected side.

Electrical Stimulation of the Auditory Nerve via Cochlear Implants in Patients with Auditory Neuropathy

2002 ◽  
Vol 111 (5_suppl) ◽  
pp. 29-31 ◽  
Author(s):  
Yvonne S. Sininger ◽  
Patricia Trautwein
Keyword(s):  
Electrical Stimulation ◽  
Cochlear Implants ◽  
Auditory Nerve ◽  
Otoacoustic Emissions ◽  
Outer Hair Cell ◽  
Cell Function ◽  
Auditory Neuropathy ◽  
Auditory Brain Stem Response ◽  
Average Performance ◽  
Auditory Brain Stem

Auditory neuropathy (AN) is a term used to describe an auditory disorder in which there is evidence of normal outer hair cell function (otoacoustic emissions and/or cochlear microphonics) and poor function of the auditory nerve (absent or highly distorted auditory brain stem response starting with wave I). Many of these patients have evidence of generalized peripheral nerve disease, leading to an assumption that the peripheral portion of the auditory nerve is the most likely site of lesion. A small group of these patients has received cochlear implants, and the majority of them achieve average to above-average performance. Although this outcome may seem incongruous with neural disease, average performance by patients with AN may be a result of the reintroduction of neural synchrony by electrical stimulation and/or the fact that most deaf patients have poor nerve survival. Although cochlear implants are promising for deaf patients with AN, more study of the disorder is needed.

Gender Differences in Audiological Findings and Hearing Aid Benefit in 255 Individuals with Auditory Neuropathy Spectrum Disorder: A Retrospective Study

2016 ◽  
Vol 27 (10) ◽  
pp. 839-845 ◽  
Author(s):  
Vijaya Kumar Narne ◽  
Prashanth Prabhu ◽  
Hunsur S. Chandan ◽  
Mahadeva Deepthi
Keyword(s):  
Gender Differences ◽  
Retrospective Study ◽  
Hearing Aids ◽  
Otoacoustic Emissions ◽  
Hearing Aid ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Acoustic Reflexes ◽  
Hearing Aid Benefit

Background: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. Purpose: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit. Research Design: A retrospective study. Study Sample: A total of 255 individuals diagnosed with ANSD were selected for the study. The study included 137 females and 88 males. Data Collection and Analysis: The demographic details, results of diagnostic audiological testing, and hearing aid benefit were analyzed retrospectively. The differences in findings across gender were compared. Results: The study shows that females have a relatively higher degree of hearing loss and that the majority of females show a rising type of audiometric configuration. The study shows that females have poorer speech perception abilities and experience limited benefits from hearing aids compared to males. Conclusions: The results of the study show that there are gender differences in audiological findings and hearing aid benefits in individuals with ANSD. However, well-controlled prospective studies are essential to confirm the results obtained and to identify the possible mechanisms underlying the gender differences.

scholarly journals Auditory neuropathy in a patient exposed to xylene: case report

2008 ◽  
Vol 123 (4) ◽  
pp. 462-465 ◽  
Author(s):  
T H J Draper ◽  
D-E Bamiou
Keyword(s):  
Case Report ◽  
Otoacoustic Emissions ◽  
Auditory Pathway ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Auditory Brainstem Responses ◽  
Test Results ◽  
Solvent Exposure ◽  
Complex Sounds ◽  
Acoustic Reflexes

AbstractObjective:To report the case of an adult patient who developed auditory complaints following xylene exposure, and to review the literature on the effects of solvent exposure on hearing.Case report:The patient presented with a gradual deterioration in his ability to hear in difficult acoustic environments and also to hear complex sounds such as music, over a 40-year period. His symptoms began following exposure to the solvent xylene, and in the absence of any other risk factor. Our audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and absent acoustic reflexes in both ears, consistent with a diagnosis of bilateral auditory neuropathy. Central test results were also abnormal, indicating possible involvement of the central auditory pathway.Conclusions:To our knowledge, this is the first report of retrocochlear hearing loss following xylene exposure. The test results may provide some insight into the effect of xylene as an isolated agent on the human auditory pathway.

Incidence of Cochlear Involvement in Hyperbilirubinemic Deafness

2002 ◽  
Vol 111 (11) ◽  
pp. 1021-1025 ◽  
Author(s):  
Cagatay Oysu ◽  
Arif Ulubil ◽  
Ismet Aslan ◽  
Nermin Baserer
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Significant Risk ◽  
Neonatal Hyperbilirubinemia ◽  
Neural Pathways ◽  
Auditory Brain Stem Response ◽  
Childhood Deafness ◽  
Identifiable Risk Factor ◽  
Auditory Brain Stem ◽  
Severe Hyperbilirubinemia

Neonatal hyperbilirubinemia remains an important cause of childhood deafness, especially in developing countries. After neonatal hyperbilirubinemia, the auditory neural pathways, cochlea, or both may be affected. In this study, we aimed to determine the incidence of cochlear impairment and the appropriate means of hearing screening in hyperbilirubinemic neonates. A retrospective review of 1,032 pediatric patients with hearing loss revealed 67 cases (6.5%) of severe hyperbilirubinemia in the neonatal period. Thirty of these patients had neonatal hyperbilirubinemia as the single identifiable risk factor for hearing loss. In 26 of 30 cases (87%), otoacoustic emissions (OAEs) were absent, whereas in the remaining 4 cases (13%), robust emissions were detected despite an absent auditory brain stem response (ABR). Auditory screening of newborns with jaundice by OAEs possesses a significant risk of undiagnosed deafness. On the other hand, if the ABR is used as the single means of screening, auditory neuropathic conditions will probably be underlooked. Therefore, we recommend dual screening of hearing by ABR and OAEs in hyperbilirubinemic newborns.

Neonatal Hearing Screening with Otoscopy, Auditory Brain Stem Response, and Otoacoustic Emissions

1995 ◽  
Vol 113 (2) ◽  
pp. P151-P151
Author(s):  
Karen Jo Doyle ◽  
Barbara Burggraaff ◽  
Sharon Fujikawa ◽  
Ju Kim ◽  
Carol MacArthur
Keyword(s):  
Brain Stem ◽  
Otoacoustic Emissions ◽  
Hearing Screening ◽  
Auditory Brain Stem Response ◽  
Neonatal Hearing Screening ◽  
Auditory Brain Stem ◽  
Brain Stem Response

Audiogram, Acoustic Reflexes, and Evoked Otoacoustic Emissions

2016 ◽  
pp. 851-859
Author(s):  
Christopher D. Bauch ◽  
Wayne O. Olsen
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Bone Conduction ◽  
Outer Hair Cells ◽  
Auditory Neuropathy ◽  
Screening Tests ◽  
Acoustic Reflex ◽  
Acoustic Reflexes ◽  
External Canal ◽  
Peripheral Auditory System

Pure-tone air-conduction and bone-conduction evaluations separate hearing loss into conductive, sensorineural, or mixed categories, and also indicate the degree of hearing loss and attendant communication difficulties. The inclusion of specific types of speech tests assess the ability of the patient to hear and understand speech. Acoustic reflex threshold and reflex decay evaluations evaluate a complex neural network, including afferent pathways to and through the lower brainstem, decussating brainstem pathways, and efferent innervation of CN VII to the stapedius muscle in the middle ear. Evoked otoacoustic emissions provide objective measurement of the peripheral auditory system coursing from the external canal to the cochlear outer hair cells. They are implemented widely in screening tests for hearing in infants, for patients suspected of auditory neuropathy spectrum disorder, and for patients suspected of pseudohypacusis; that is, feigning or exaggerated hearing loss.

Changes in Otoacoustic Emissions and Auditory Brain Stem Response after C/s-Platinum Exposure in Gerbils

1997 ◽  
Vol 116 (6) ◽  
pp. 585-592 ◽  
Author(s):  
Kathleen C. Y. Sie ◽  
Susan J. Norton
Keyword(s):  
Hearing Loss ◽  
Brain Stem ◽  
Otoacoustic Emissions ◽  
Outer Hair Cell ◽  
Outer Hair Cells ◽  
Distortion Product Otoacoustic Emissions ◽  
Auditory Brain Stem Response ◽  
Distortion Product ◽  
Auditory Brain Stem ◽  
Significant Difference

Ototoxicity associated with cis-platinum administration commonly presents as hearing loss and tinnitus. The hearing loss is usually an irreversible, high-frequency sensorineural loss. Histologic studies in humans and animals suggest that the outer hair cells (OHCs) are most susceptible to cis-platinum. Evoked otoacoustic emissions (EOAE), as a measure of outer hair cell function, are potentially useful in following ototoxic insults involving OHCs. Distortion-product otoacoustic emissions (DPOAE) test frequency-specific regions of the cochlea and therefore may be particularly well suited for monitoring ototoxic injuries. We measured distortion product otoacoustic emissions, at f2 = 2, 4, 6, 8, 10, and 12 kHz, in gerbils after a single large dose of cis-platinum. Animals treated with saline served as controls. The findings were compared to auditory brain stem evoked response (ABR) thresholds, using tone pips of the same frequencies. The DPOAE and ABR thresholds were measured before treatment and again 2, 5, and 14 days after drug administration. The changes in DPOAE were compared with the changes in ABR. No treatment effect was noted in the 2-day group. Animals treated with c/s-platinum demonstrated significant elevation of DPOAE and ABR thresholds compared with control animals at 5 and 14 days. There was no significant difference between the threshold changes in the 5-and 14-day groups.

Cochlear Endoscopy with Preservation of Hearing in Guinea Pigs

1994 ◽  
Vol 111 (4) ◽  
pp. 439-445 ◽  
Author(s):  
Thomas Balkany ◽  
Annelle V. Hodges ◽  
Martin Whitehead ◽  
Faramarz Memari ◽  
Glen K. Martin
Keyword(s):  
Guinea Pig ◽  
Brain Stem ◽  
Guinea Pigs ◽  
Otoacoustic Emissions ◽  
Round Window ◽  
Distortion Product Otoacoustic Emission ◽  
Auditory Brain Stem Response ◽  
Distortion Product ◽  
Guinea Pig Cochlea ◽  
Auditory Brain Stem

Advances in fiberoptic technology have revolutionized the way many disorders are treated by otolaryngologists. We have previously described our experiences with cochlear endoscopy during cochlear Implantation. However, endoscopy of the functioning cochlea has not previously been reported. To test the hypothesis that endoscopy of the guinea pig cochlea is possible without catastrophic loss of auditory function, we subjected 20 ears of 10 Hartley-strain albino guinea pigs to limited endoscopy of the cochlea through the round window with evaluation of distortion product otoacoustic emissions and auditory brain stem responses. Insertion of the endoscope caused measurable changes in auditory brain stem response latency and amplitude. Distortion product otoacoustic emission amplitudes were reduced an average of 6 dB with greater loss close to the round window. Frequencies above 18 kHz, corresponding to the region of endoscopy, were not evaluated in this preliminary study and are suspected to have sustained more damage. Results demonstrate that endoscopy of the guinea pig cochlea is possible without major loss of the above physiologic measures in the regions tested. If endoscopy of the cochlea is to become a tool with clinical and basic science applications, refinements in techniques to avoid damage are necessary.

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