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  1. Dipak S, Prepageran N, Sazila AS, Rahmat O, Raman R
    Med J Malaysia, 2005 Oct;60(4):489-91.
    PMID: 16570713
    Large Vestibular Aqueduct Syndrome is a congenital malformation of the temporal bone characterised by early onset of sensorineural hearing loss and vestibular disturbance. Familial large vestibular aqueduct syndrome suggests autosomal recessive or X-linked inheritance and accounts for non-syndromic sensorineural hearing loss in these patients.
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  2. Hossain ME, Jassim WA, Zilany MS
    PLoS One, 2016;11(3):e0150415.
    PMID: 26967160 DOI: 10.1371/journal.pone.0150415
    Sensorineural hearing loss occurs due to damage to the inner and outer hair cells of the peripheral auditory system. Hearing loss can cause decreases in audibility, dynamic range, frequency and temporal resolution of the auditory system, and all of these effects are known to affect speech intelligibility. In this study, a new reference-free speech intelligibility metric is proposed using 2-D neurograms constructed from the output of a computational model of the auditory periphery. The responses of the auditory-nerve fibers with a wide range of characteristic frequencies were simulated to construct neurograms. The features of the neurograms were extracted using third-order statistics referred to as bispectrum. The phase coupling of neurogram bispectrum provides a unique insight for the presence (or deficit) of supra-threshold nonlinearities beyond audibility for listeners with normal hearing (or hearing loss). The speech intelligibility scores predicted by the proposed method were compared to the behavioral scores for listeners with normal hearing and hearing loss both in quiet and under noisy background conditions. The results were also compared to the performance of some existing methods. The predicted results showed a good fit with a small error suggesting that the subjective scores can be estimated reliably using the proposed neural-response-based metric. The proposed metric also had a wide dynamic range, and the predicted scores were well-separated as a function of hearing loss. The proposed metric successfully captures the effects of hearing loss and supra-threshold nonlinearities on speech intelligibility. This metric could be applied to evaluate the performance of various speech-processing algorithms designed for hearing aids and cochlear implants.
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology*
  3. Maamor N, Billings CJ
    Neurosci Lett, 2017 01 01;636:258-264.
    PMID: 27838448 DOI: 10.1016/j.neulet.2016.11.020
    The purpose of this study was to determine the effects of noise type, signal-to-noise ratio (SNR), age, and hearing status on cortical auditory evoked potentials (CAEPs) to speech sounds. This helps to explain the hearing-in-noise difficulties often seen in the aging and hearing impaired population. Continuous, modulated, and babble noise types were presented at varying SNRs to 30 individuals divided into three groups according to age and hearing status. Significant main effects of noise type, SNR, and group were found. Interaction effects revealed that the SNR effect varies as a function of noise type and is most systematic for continuous noise. Effects of age and hearing loss were limited to CAEP latency and were differentially modulated by energetic and informational-like masking. It is clear that the spectrotemporal characteristics of signals and noises play an important role in determining the morphology of neural responses. Participant factors such as age and hearing status, also play an important role in determining the brain's response to complex auditory stimuli and contribute to the ability to listen in noise.
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology*
  4. Goh LC, Azman A, Siti HBK, Khoo WV, Muthukumarasamy PA, Thong MK, et al.
    Int J Pediatr Otorhinolaryngol, 2018 Jun;109:50-53.
    PMID: 29728184 DOI: 10.1016/j.ijporl.2018.03.010
    OBJECTIVE: To study the audiological outcome and early screening of pre-school going children with craniosynostosis under follow-up at the University of Malaya Medical Center(UMMC), Kuala Lumpur, Malaysia over a 10 year period.

    METHODS: A retrospective descriptive cohort study on the audiological findings detected during the first hearing assessment done on a child with craniosynostosis using otoacoustic emissions, pure tone audiometry or auditory brainstem response examination. The main aim of this study was to evaluate the type and severity of hearing loss when compared between syndromic and non-sydromic craniosynostosis, and other associated contributory factors.

    RESULTS: A total of 31 patients with 62 ears consisting of 14 male patients and 17 female patients were evaluated. Twenty two patients (71%) were syndromic and 9 (29%) were non-syndromic craniosynostosis. Amongst the syndromic craniosynostosis, 9 (41%) had Apert syndrome, 7 (32%) had Crouzon syndrome, 5 (23%) had Pfieffer syndrome and 1 (4%) had Shaethre Chotzen syndrome. Patients with syndromic craniosynostosis were more likely to present with all types and severity of hearing loss, including severe to profound sensorineural hearing loss while children with non-syndromic craniosynostosis were likely to present with normal hearing (p loss including sensorineural hearing loss is more likely to be present in a child with syndromic craniosynostosis (p loss, including that of a severe to profound degree compared to children with non-syndromic craniosynostosis. In addition to that, hearing loss is more likely to be detected when the first hearing test is done at a later age, and this can be an irreversible sensorineural hearing loss. We would like to advocate the need for early audiological screening and follow up in children with syndromic craniosynostosis.

    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  5. Saniasiaya J
    Ear Nose Throat J, 2021 Apr;100(2_suppl):152S-154S.
    PMID: 32755405 DOI: 10.1177/0145561320946902
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology*
  6. Ngui LX, Tang IP, Prepageran N, Lai ZW
    Int J Pediatr Otorhinolaryngol, 2019 May;120:184-188.
    PMID: 30844634 DOI: 10.1016/j.ijporl.2019.02.045
    INTRODUCTION: Congenital hearing loss is one of the commonest congenital anomalies. Neonatal hearing screening aims to detect congenital hearing loss early and provide prompt intervention for better speech and language development. The two recommended methods for neonatal hearing screening are otoacoustic emission (OAE) and automated auditory brainstem response (AABR).

    OBJECTIVE: To study the effectiveness of distortion product otoacoustic emission (DPOAE) and automated auditory brainstem response (AABR) as first screening tool among non-risk newborns in a hospital with high delivery rate.

    METHOD: A total of 722 non-risk newborns (1444 ears) were screened with both DPOAE and AABR prior to discharge within one month. Babies who failed AABR were rescreened with AABR ± diagnostic auditory brainstem response tests within one month of age.

    RESULTS: The pass rate for AABR (67.9%) was higher than DPOAE (50.1%). Both DPOAE and AABR pass rates improved significantly with increasing age (p-value<0.001). The highest pass rate for both DPOAE and AABR were between the age of 36-48 h, 73.1% and 84.2% respectively. The mean testing time for AABR (13.54 min ± 7.47) was significantly longer than DPOAE (3.52 min ± 1.87), with a p-value of <0.001.

    CONCLUSIONS: OAE test is faster and easier than AABR, but with higher false positive rate. The most ideal hearing screening protocol should be tailored according to different centre.

    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  7. Boo NY, Rohani AJ, Asma A
    Singapore Med J, 2008 Mar;49(3):209-14.
    PMID: 18363002
    This study was designed to compare the sensitivity and specificity of detecting sensorineural hearing loss (SNHL) using the transient-evoked otoacoustic emissions (OAE) machine (the Madsen TE Echoscreen) and automated auditory brainstem response (AABR) machine (the Sabre Compac portable AABR) in term neonates exposed to severe hyperbilirubinaemia.
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  8. Yeap LL, Lim KS, Lo YL, Bakar MZ, Tan CT
    Epileptic Disord, 2014 Sep;16(3):375-9.
    PMID: 25167568 DOI: 10.1684/epd.2014.0671
    Hearing loss has been reported with valproic acid (VPA) use. However, this is the first case of VPA-induced hearing loss that was tested and confirmed with a VPA rechallenge, supported by serial audiometry and pharmacokinetic modelling. A 39-year-old truck driver with temporal lobe epilepsy was treated with VPA at 400 mg, twice daily, and developed hearing loss after each dose, but recovered within three hours. Hearing loss fully resolved after VPA discontinuation. Audiometry performed five hours after VPA rechallenge showed significant improvement in hearing thresholds. Pharmacokinetic modelling during the VPA rechallenge showed that hearing loss occurred at a level below the therapeutic range. Brainstem auditory evoked potential at three months after VPA discontinuation showed bilateral conduction defect between the cochlear and superior olivary nucleus, supporting a pre-existing auditory deficit. VPA may cause temporary hearing threshold shift. Pre-existing auditory defect may be a risk factor for VPA-induced hearing loss. Caution should be taken while prescribing VPA to patients with pre-existing auditory deficit.
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  9. Sakina MS, Goh BS, Abdullah A, Zulfiqar MA, Saim L
    Int J Pediatr Otorhinolaryngol, 2006 Dec;70(12):2093-7.
    PMID: 16996619
    Sensorineural hearing loss (SNHL) due to internal auditory canal (IAC) stenosis with hypoplasia of the cochleovestibular nerve is a rare disorder. The diagnosis of the IAC stenosis requires both high resolution computed tomography scan (HRCT) and magnetic resonance imaging (MRI).
    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology
  10. Ibrahim IA, Ting HN, Moghavvemi M
    J Int Adv Otol, 2019 Apr;15(1):87-93.
    PMID: 30924771 DOI: 10.5152/iao.2019.4553
    OBJECTIVES: This study uses a new approach for classifying the human ethnicity according to the auditory brain responses (electroencephalography [EEG] signals) with a high level of accuracy. Moreover, the study presents three different algorithms used to classify the human ethnicity using auditory brain responses. The algorithms were tested on Malays and Chinese as a case study.

    MATERIALS AND METHODS: The EEG signal was used as a brain response signal, which was evoked by two auditory stimuli (Tones and Consonant Vowels stimulus). The study was carried out on Malaysians (Malay and Chinese) with normal hearing and with hearing loss. A ranking process for the subjects' EEG data and the nonlinear features was used to obtain the maximum classification accuracy.

    RESULTS: The study formulated the classification of Normal Hearing Ethnicity Index and Sensorineural Hearing Loss Ethnicity Index. These indices classified the human ethnicity according to brain auditory responses by using numerical values of response signal features. Three classification algorithms were used to verify the human ethnicity. Support Vector Machine (SVM) classified the human ethnicity with an accuracy of 90% in the cases of normal hearing and sensorineural hearing loss (SNHL); the SVM classified with an accuracy of 84%.

    CONCLUSION: The classification indices categorized or separated the human ethnicity in both hearing cases of normal hearing and SNHL with high accuracy. The SVM classifier provided a good accuracy in the classification of the auditory brain responses. The proposed indices might constitute valuable tools for the classification of the brain responses according to the human ethnicity.

    Matched MeSH terms: Hearing Loss, Sensorineural/physiopathology*
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