scholarly journals Neuropatia Auditiva: Avaliação Clínica e Abordagem Diagnóstica

2016 ◽  
Vol 29 (6) ◽  
pp. 353 ◽  
Author(s):  
Guilherme Machado Carvalho ◽  
Beatriz Prista Leão ◽  
Priscila Zonzini Ramos ◽  
Alexandre Caixeta Guimarães ◽  
Arthur Menino Castilho ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Case Series ◽  
Epidemiological Data ◽  
Auditory Pathway ◽  
Auditory Brainstem ◽  
Auditory Neuropathy ◽  
Auditory Brainstem Responses ◽  
Profound Deafness ◽  
Neuronal Transmission ◽  
Patients Experience

<p><strong>Introduction:</strong> Auditory neuropathy is a condition in which there is a change in the neuronal transmission of the auditory stimuli. Our objective was to describe the patients’ series within the clinical spectrum of auditory neuropathy. <br /><strong>Material and Methods:</strong> We designed a transversal, retrospective study, with a description of a consecutive case series. Auditory neuropathy was defined by the presence of acoustic otoemissions plus absent/abnormal auditory brainstem responses with cochlear microphonism. <br /><strong>Results:</strong> 34 patients with bilateral hearing loss, 23 males and 11 females, were included in the study. Eighty percent of the cases had congenital onset of hearing loss. Acoustic otoemissions were absent in 67% of them. Cochlear microfonism was present in 79% of all cases. Prenatal, perinatal or ambiental factors were present in 35.2% of the cases. <br /><strong>Discussion:</strong> Medical literature shows great variability in findings related to auditory neuropathy, both in its etiology and epidemiological data. <br /><strong>Conclusion:</strong> Auditory neuropathy presents a broad spectrum of changes that may result from mild to severe changes in the functioning of the auditory pathway, and in our sample we observed that 80% of Auditory neuropathy have congenital onset of hearing loss and/or with cochlear microphonism identified. 91% of patients experience significant hearing impairment and 53% suffer from severe or profound deafness.</p>

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.

Comparing the electrophysiological effects of traumatic noise exposure between rodents

2022 ◽  
Author(s):  
Lenneke Kiefer ◽  
Lisa Koch ◽  
Melisa Merdan-Desik ◽  
Bernhard H. Gaese ◽  
Manuela Nowotny
Keyword(s):  
Hearing Loss ◽  
Noise Exposure ◽  
Temporal Dynamics ◽  
Auditory Pathway ◽  
Auditory Brainstem ◽  
Individual Variability ◽  
Rodent Species ◽  
Waveform Analysis ◽  
Auditory Brainstem Responses ◽  
Noise Induced Hearing Loss

Noise-induced hearing deficits are important health problems in the industrialized world. As the underlying physiological dysfunctions are not well understood, research in suitable animal models is urgently needed. Three rodent species (Mongolian gerbil, rat and mouse) were studied to compare the temporal dynamics of noise-induced hearing loss after identical procedures of noise exposure. Auditory brainstem responses (ABRs) were measured before, during and up to eight weeks after noise exposure for threshold determination and ABR waveform analysis. Trauma induction with stepwise increasing sound pressure level was interrupted by five interspersed ABR measurements. Comparing short- and long-term dynamics underlying the following noise-induced hearing loss revealed diverging time courses between the three species. Hearing loss occurred early on during noise exposure in all three rodent species at or above trauma frequency. Initial noise level (105 dB SPL) was most effective in rats while the delayed level-increase to 115 dB SPL affected mice much stronger. Induced temporary threshold shifts in rats and mice were larger in animals with lower pre-trauma ABR thresholds. The increase in activity (gain) along the auditory pathway was derived by comparing the amplitudes of short- and long-latency ABR waveform components. Directly after trauma, significant effects were found for rats (decreasing gain) and mice (increasing gain) while gerbils revealed high individual variability in gain changes. Taken together, our comparative study revealed pronounced species-specific differences in the development of noise-induced hearing loss and the related processing along the auditory pathway.

Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation

2015 ◽  
Vol 129 (5) ◽  
pp. 504-508 ◽  
Author(s):  
R Chandran ◽  
M Alexander ◽  
P Naina ◽  
A Balraj
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Case Series ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Auditory Brainstem Responses ◽  
High Dose ◽  
Hearing Rehabilitation ◽  
Auditory Neuropathy Spectrum Disorder

AbstractBackground:Brown–Vialetto–Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.Case report:We examined the clinical and audiological profiles of a case series comprising four siblings with Brown–Vialetto–Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.Conclusion:The sensorineural hearing loss in Brown–Vialetto–Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy. However, further research is needed to confirm this. This report emphasises the need for a thorough neurological evaluation of auditory neuropathy spectrum disorder patients.

scholarly journals Monitoring of the auditory pathway maturation after early intervention during the first year of life in infants with sensorineural hearing loss

2020 ◽  
Author(s):  
F. Matin ◽  
S. Haumann ◽  
W. Roßberg ◽  
D. Mitovska ◽  
T. Lenarz ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Early Intervention ◽  
Hearing Aids ◽  
Hearing Threshold ◽  
Auditory Pathway ◽  
Auditory Brainstem Responses ◽  
First Year ◽  
Early Therapy ◽  
Hearing Thresholds ◽  
First Year Of Life

Abstract Purpose The objective of this study was to investigate the auditory pathway maturation monitored by auditory brainstem responses (ABR) in infants with hearing loss during the first year of life. ABR were used to estimate hearing thresholds and the effect of early intervention strategies using hearing aids (HA). Methods Click-evoked ABRs were measured in 102 infants aged from 0 to 12 months to determine their individual auditory threshold. Early therapy intervention was recommended before 12 months of age and analyzed. To evaluate the effect of hearing amplification on auditory maturation, different subgroups of infants with moderate hearing loss were analyzed and the auditory pathway maturation was determined based on IPL I–V shortening. Results Overall, 110 ears (54.0% of 204 ears) with mild to profound HL showed threshold changes of 10 dB up to 60 dB in the follow-up ABR testing. HA were prescribed at the age of 3.8 ± 3.9 months. Cochlear implantation (CI) was performed in cases of repeated profound HL at the age of 9.9 months ± 4.5 months. A significant shortening of IPL I–V in all subgroups of infants (with and without risk factors) who received HA was shown and assumed auditory pathway maturation. Conclusion An early intervention using optimally fitted HA influenced auditory pathway maturation and may lead to improvements of hearing thresholds during the first year of life in infants. This study underscores the importance of not only providing HAs to infants, but also controlling for hearing threshold changes ensuring that HAs provide the optimal level of intervention or CI is indicated.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

2021 ◽  
pp. 1-7
Author(s):  
Dario Ebode ◽  
Fleur Cohen-Aubart ◽  
Stéphanie Trunet ◽  
Evelyne Ferrary ◽  
Ghizlène Lahlou ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Corticosteroid Treatment ◽  
Auditory Neuropathy ◽  
Disease Exacerbation ◽  
Profound Hearing Loss ◽  
Hearing Recovery ◽  
Speech Performance

<b><i>Introduction:</i></b> Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. <b><i>Results:</i></b> Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. <b><i>Conclusion:</i></b> Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

Hearing recovery from deafness caused by bromate intoxication

2018 ◽  
Vol 132 (11) ◽  
pp. 1039-1041 ◽  
Author(s):  
J Suzuki ◽  
Y Takanashi ◽  
A Koyama ◽  
Y Katori
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Auditory Brainstem Responses ◽  
Common Symptom ◽  
Hearing Recovery ◽  
Continuous Haemodiafiltration

AbstractObjectivesSodium bromate is a strong oxidant, and bromate intoxication can cause irreversible severe-to-profound sensorineural hearing loss. This paper reports the first case in the English literature of bromate-induced hearing loss with hearing recovery measured by formal audiological assessment.Case reportA 72-year-old woman was admitted to hospital with complaints of profound hearing loss, nausea, diarrhoea and anuria after bromate ingestion in a suicide attempt. On admission, pure tone audiometry and auditory brainstem responses showed profound bilateral deafness. Under the diagnosis of bromate-induced acute renal failure and sensorineural hearing loss, continuous haemodiafiltration was performed. When dialysis was discontinued, pure tone audiometry and auditory brainstem responses showed partial threshold recovery from profound deafness.ConclusionSevere-to-profound sensorineural hearing loss is a common symptom of bromate intoxication. Bromate-induced hearing loss may be partially treated, and early application of continuous haemodiafiltration might be useful as a treatment for this intractable condition.

Auditory Brainstem Responses in a Case of High-Frequency Conductive Hearing Loss

1985 ◽  
Vol 50 (4) ◽  
pp. 346-350 ◽  
Author(s):  
Michael P. Gorga ◽  
Jan K. Reiland ◽  
Kathryn A. Beauchaine
Keyword(s):  
Hearing Loss ◽  
High Frequency ◽  
Conductive Hearing Loss ◽  
Auditory Brainstem ◽  
Intensity Function ◽  
Auditory Brainstem Responses ◽  
Normal Limits ◽  
Interpeak Latency ◽  
Lower Limits ◽  
Conductive Hearing

Click-evoked auditory brainstem responses were measured in a patient with high-frequency conductive hearing loss. As is typical in cases of conductive hearing loss, Wave I latency was prolonged beyond normal limits. Interpeak latency differences were just below the lower limits of the normal range. The Wave V latency-intensity function, however was abnormally steep. This pattern is explained by the hypothesis that the slope of the latency-intensity function is determined principally by the configuration of the hearing loss. In cases of high-frequency hearing loss (regardless of the etiology), the response may be dominated by more apical regions of the cochlea at lower intensities and thus have a longer latency.

scholarly journals Cochlear Synaptopathy: A Primary Factor Affecting Speech Recognition Performance in Presbycusis

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Zhe Chen ◽  
Yanmei Zhang ◽  
Junbo Zhang ◽  
Rui Zhou ◽  
Zhen Zhong ◽  
...  
Keyword(s):  
Speech Recognition ◽  
Action Potential ◽  
Animal Studies ◽  
Signal To Noise Ratio ◽  
Recognition Performance ◽  
Auditory Pathway ◽  
Auditory Brainstem ◽  
Auditory Brainstem Responses ◽  
Hearing Ability ◽  
Summating Potential

The results of recent animal studies have suggested that cochlear synaptopathy may be an important factor involved in presbycusis. Therefore, here, we aimed to examine whether cochlear synaptopathy frequently exists in patients with presbycusis and to describe the effect of cochlear synaptopathy on speech recognition in noise. Based on the medical history and an audiological examination, 94 elderly patients with bilateral, symmetrical, sensorineural hearing loss were diagnosed as presbycusis. An electrocochleogram, auditory brainstem responses, auditory cortical evoked potentials, and speech audiometry were recorded to access the function of the auditory pathway. First, 65 ears with hearing levels of 41-50 dB HL were grouped based on the summating potential/action potential (SP/AP) ratio, and the amplitudes of AP and SP were compared between the two resulting groups. Second, 188 ears were divided into two groups: the normal SP/AP and abnormal SP/AP groups. The speech recognition abilities in the two groups were compared. Finally, the relationship between abnormal electrocochleogram and poor speech recognition (signal-to-noise ratio loss ≥7 dB) was analyzed in 188 ears. The results of the present study showed: (1) a remarkable reduction in the action potential amplitude was observed in patients with abnormal SP/AP ratios; this suggests that cochlear synaptopathy was involved in presbycusis. (2) There was a large proportion of patients with poor speech recognition in the abnormal SP/AP group. Furthermore, a larger number of cases with abnormal SP/AP ratios were confirmed among patients with presbycusis and poor speech recognition. We concluded that cochlear synaptopathy is not uncommon among elderly individuals who have hearing ability deficits, and it may have a more pronounced effect on ears with declining auditory performance in noisy environments.

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