Perceptual separation of sensorineural hearing loss and auditory neuropathy spectrum disorder

2015 ◽  
Vol 126 (6) ◽  
pp. 1420-1425 ◽  
Author(s):  
Shuo Wang ◽  
Ruijuan Dong ◽  
Dongxin Liu ◽  
Yuan Wang ◽  
Yitao Mao ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Perceptual Separation

scholarly journals Cortical Neurophysiologic Correlates of Auditory Threshold in Adults and Children With Normal Hearing and Auditory Neuropathy Spectrum Disorder

2020 ◽  
pp. 1-15
Author(s):  
Garrett Cardon ◽  
Anu Sharma
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Normal Hearing ◽  
Auditory Neuropathy ◽  
Auditory Threshold ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Threshold Estimation ◽  
Auditory Thresholds ◽  
Auditory Neuropathy Spectrum Disorder

Purpose Auditory threshold estimation using the auditory brainstem response or auditory steady state response is limited in some populations (e.g., individuals with auditory neuropathy spectrum disorder [ANSD] or those who have difficulty remaining still during testing and cannot tolerate general anesthetic). However, cortical auditory evoked potentials (CAEPs) can be recorded in many such patients and have been employed in threshold approximation. Thus, we studied CAEP estimates of auditory thresholds in participants with normal hearing, sensorineural hearing loss, and ANSD. Method We recorded CAEPs at varying intensity levels to speech (i.e., /ba/) and tones (i.e., 1 kHz) to estimate auditory thresholds in normal-hearing adults ( n = 10) and children ( n = 10) and case studies of children with sensorineural hearing loss and ANSD. Results Results showed a pattern of CAEP amplitude decrease and latency increase as stimulus intensities declined until waveform components disappeared near auditory threshold levels. Overall, CAEP thresholds were within 10 dB HL of behavioral thresholds for both stimuli. Conclusions The above findings suggest that CAEPs may be clinically useful in estimating auditory threshold in populations for whom such a method does not currently exist. Physiologic threshold estimation in difficult-to-test clinical populations could lead to earlier intervention and improved outcomes.

scholarly journals Plasticity in the Developing Auditory Cortex: Evidence from Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder

2012 ◽  
Vol 23 (06) ◽  
pp. 396-411 ◽  
Author(s):  
Garrett Cardon ◽  
Julia Campbell ◽  
Anu Sharma
Keyword(s):  
Hearing Loss ◽  
Auditory Cortex ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Early Deprivation ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Cortical Input

The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for reorganizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: (1) input to the cortex and (2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants.

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 TMTC2variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad

2018 ◽  
Vol 6 (4) ◽  
pp. 653-659 ◽  
Author(s):  
Hector Guillen-Ahlers ◽  
Christy B. Erbe ◽  
Frédéric D. Chevalier ◽  
Maria J. Montoya ◽  
Kip D. Zimmerman ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy

2015 ◽  
Vol 42 (3) ◽  
pp. 249-253 ◽  
Author(s):  
Yukihide Maeda ◽  
Yuko Kataoka ◽  
Akiko Sugaya ◽  
Shin Kariya ◽  
Katsuhiro Kobayashi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Charcot Marie Tooth ◽  
Charcot Marie Tooth Disease ◽  
Steroid Dependent ◽  
Tooth Disease

scholarly journals Oto acoustic emissions in early detection of sensorineural hearing loss in high-risk neonates

2021 ◽  
Vol 7 (11) ◽  
pp. 1794
Author(s):  
J. Ramanjaneyulu ◽  
S. Rajesh Kumar ◽  
V. Krishna Chaitanya ◽  
A. Kusumanjali
Keyword(s):  
Hearing Loss ◽  
High Risk ◽  
Early Detection ◽  
Sensorineural Hearing Loss ◽  
Brain Stem ◽  
Sensitivity And Specificity ◽  
Acoustic Emissions ◽  
Otoacoustic Emission ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing

<p><strong>Background: </strong>Early identification of congenital hearing loss and early intervention ameliorated many adverse consequences. This study was performed to observe effectiveness of otoacoustic emission in screening of hearing loss in high-risk babies.</p><p><strong>Methods: </strong>Prospective study on 45 high-risk newborns delivered during period of 2013-2014. Selective newborn hearing performed with oto acoustic emissions (OAE) and auditory brain stem responses (ABR), in high-risk infants aged below 7 days, 15 days, after 45 days and after 90 days.</p><p><strong>Results: </strong>Study population comprised of 45 high-risk newborns. In 1<sup>st</sup> level screening, 28 (62%) babies showed recordable OAE, 17 (38%) babies failed. In 2<sup>nd</sup> level screening 31 (81%) passed and 7 (19%) failed and death occurred in 7 infants. In 3<sup>rd</sup> level screening both OAE and brain stem evoked response audiometry (BERA), was performed in 38 cases and positivity was reported in 37 cases. 4<sup>th</sup> level screening was similar to 3<sup>rd</sup> level screening where 3 babies failed ABR test. In our study incidence of sensorineural hearing loss found to be 78.91% (3/38×1000) per 1000 high-risk babies. Auditory neuropathy was observed in 2 (4.4%) patients. Sensitivity and specificity of OAE was 100% and 33.3% respectively. In high-risk low birth weight neonates’ sensitivity and specificity was 66.7% and 50.0%.</p><p><strong>Conclusions: </strong>In high-risk babies, appropriate time for screening with OAE is around 60 days of age. OAE are useful diagnostic tool in evaluation of high-risk neonates for early detection of sensorineural hearing loss.</p>

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