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  1. Fulltext Reference-Free Assessment of Speech Intelligibility Using Bispectrum of an Auditory Neurogram
    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*
  2. Fulltext The Prevalence of Sensorineural Hearing Loss in β-thalassaemia patient treated with Desferrioxamine
    Kong MH, Goh BS, Hamidah A, Zarina AL
    Med J Malaysia, 2014 Feb;69(1):9-12.
    PMID: 24814621 MyJurnal
    OBJECTIVE: This study aimed to evaluate the prevalence of sensorineural hearing loss (SNHL) in β-thalassaemia patients treated with Desferrioxamine (DFO) and determine the correlation of SNHL with average daily DFO dosage, serum ferritin level and Therapeutic index (T.I).

    METHODS: This is a cross sectional descriptive study carried out for a period of 14 months and 54 patients were recruited. The recruited patients are transfusion dependant β- thalassaemia patient aged 3 years and above treated with DFO. An interview, clinical examination and hearing assessment, which included tympanogram, and Pure Tone Audiometry (PTA) or behaviour alaudiometry were performed. The data on age started on DFO, average daily DFO, duration of DFO intake, serum ferritin past 1 year and Therapeutic Index (T.I) were obtained from patients' case notes.

    RESULTS: The prevalence of SNHL was 57.4% and majority has mild hearing loss (93.6%). Fourteen patients (25.9%) have bilateral ear involvement and as many as 17 patients (31.5%) have SNHL in either ear. A total of 23 patients (42.6%) have normal hearing level. Although the prevalence of SNHL was 57.4%, only a small percentage of the patient noticed and complained of hearing loss (11.1%). There is no association between age started on DFO, average daily DFO and duration of DFO intake with normal hearing group and those patients with SNHL. Positive correlation was seen between average daily DFO with 2000 and 4000Hz on PTA in the left ear and between serum ferritin level past 1 year with 4000 and 8000Hz in the right ear and 8000Hz in the left ear. No significant correlation was seen between T.I on PTA.

    CONCLUSION: The prevalence of SNHL from hearing assessment is high in β-thalassaemia patients in this study. However, it is manifested clinically in a smaller percentage. We suggest a baseline hearing assessment should be carried on all β-thalassaemia patients prior to DFO chelation therapy.
    Matched MeSH terms: Hearing Loss, Sensorineural
  3. Vancomycin administration in continuous ambulatory peritoneal dialysis: the risk of ototoxicity
    Gendeh BS, Gibb AG, Aziz NS, Kong N, Zahir ZM
    Otolaryngol Head Neck Surg, 1998 Apr;118(4):551-8.
    PMID: 9560111
    A prospective study was undertaken in 16 patients with chronic renal failure on continuous ambulatory peritoneal dialysis, with 22 episodes of peritonitis treated with vancomycin, a known ototoxic agent. Twelve patients had one episode each, and four had recurrent peritonitis. Each treatment course consisted of two infusions of vancomycin (30 mg/kg body weight) in 2 L of peritoneal dialysate administered at 6-day intervals. Serum vancomycin analyzed by enzyme immunoassay showed a mean trough level of 11.00 microg/ml on day 6 and mean serum levels of 33.8 and 38.6 microg/ml about 12 hours after administration on days 1 and 7, respectively. Similar levels, well within the therapeutic range, were encountered with repeated vancomycin therapy for recurrent episodes of peritonitis, suggesting that no changes occurred in the pharmacokinetic profile of the drug. Pure-tone audiometry, electronystagmography, and clinical assessment performed during each course of treatment showed no evidence of ototoxicity even on repeated courses of vancomycin therapy. The results suggest that vancomycin therapy when given in appropriate concentrations as a single therapeutic agent is both effective and safe. We believe, however, that vancomycin administered in combination with an aminoglycoside may produce ototoxic effects that may be greatly aggravated, possibly because of synergism.
    Matched MeSH terms: Hearing Loss, Sensorineural/blood; Hearing Loss, Sensorineural/chemically induced*; Hearing Loss, Sensorineural/diagnosis
  4. Fulltext Auditory neuropathy: three cases among a group with sensorineural hearing loss
    Mohd Khairi MD, Normastura AR, Wan Zaharah AW
    Singapore Med J, 2009 Sep;50(9):e324-5.
    PMID: 19787161
    The prevalence of auditory neuropathy is not known, although the majority of cases are felt to lie within the population of neonatal intensive care unit graduates. We report three cases of auditory neuropathy, out of 211 children with sensorineural hearing loss, seen at our audiology clinic from April 1, 1999 to December 31, 2003. Two patients did not have a risk factor for hearing impairment. Screening policies based solely on transient evoked otoacoustic emissions testing will not detect auditory neuropathy effectively, and may falsely reassure parents and professionals unaware of this condition.
    Study site: Audiology clinic, Hospital Universiti Sains Malaysia, Kelantan, Malaysia
    Matched MeSH terms: Hearing Loss, Sensorineural/diagnosis*; Hearing Loss, Sensorineural/etiology*
  5. Fulltext Cervical vestibular evoked myogenic potential (cVEMP) findings in adults with sensorineural hearing loss (SNHL): comparisons between 500 Hz tone burst and narrowband CE-Chirp stimuli
    Zakaria MN, Salim R, Abdul Wahat NH, Md Daud MK, Wan Mohamad WN
    Sci Rep, 2023 Dec 21;13(1):22842.
    PMID: 38129442 DOI: 10.1038/s41598-023-48810-1
    There has been a growing interest in studying the usefulness of chirp stimuli in recording cervical vestibular evoked myogenic potential (cVEMP) waveforms. Nevertheless, the study outcomes are debatable and require verification. In view of this, the aim of the present study was to compare cVEMP results when elicited by 500 Hz tone burst and narrowband (NB) CE-Chirp stimuli in adults with sensorineural hearing loss (SNHL). Fifty adults with bilateral SNHL (aged 20-65 years) underwent the cVEMP testing based on the established protocol. The 500 Hz tone burst and NB CE-Chirp (centred at 500 Hz) stimuli were presented to each ear at an intensity level of 120.5 dB peSPL. P1 latency, N1 latency, and P1-N1 amplitude values were analysed accordingly. The NB CE-Chirp stimulus produced significantly shorter P1 and N1 latencies (p  0.80). In contrast, both stimuli elicited cVEMP responses with P1-N1 amplitude values that were not statistically different from one another (p = 0.157, d = 0.15). Additionally, age and hearing level were found to be significantly correlated (r = 0.56, p 
    Matched MeSH terms: Hearing Loss, Sensorineural*
  6. Fulltext The association between GJB2 mutation and GJB6 gene in non syndromic hearing loss school children
    Asma A, Ashwaq A, Norzana AG, Atmadini AM, Ruszymah BH, Saim L, et al.
    Med J Malaysia, 2011 Jun;66(2):124-8.
    PMID: 22106692 MyJurnal
    Recently, molecular testing for GJB2 mutations has become the standard of care for the diagnosis of patients with non syndromic hearing impairment of unknown cause. The aims of this study are to determine the association between GJB2 mutation and GJB6 and to report the variation of mutations in deaf students who have heterozygous GJB2. This retrospective study was conducted at Universiti Kebangsaan Malaysia Medical Center (UKMMC). Data was collected from previous files and records from Tissue Engineering and Human Genetic Research Group Laboratory. Approval from Ethical Committee was obtained prior to the study. A total of 138 students have been screened in previous studies in UKMMC for the presence of GJB2 mutations as a cause for hearing loss. Thirty four of the 138 subjects have GJB2 mutations; 2 showed homozygous mutations whereas another 32 were heterozygous for GJB2 gene mutation. Only 31 DNA samples of students presented with sensorineural hearing loss with heterozygous mutation in GJB2 gene were included in this study. The sequencing results obtained were analyzed. The degree of hearing loss of those students with association between GJB2 mutation and GJB6 mutation will be discussed. Five out of 31 subjects (16.2%) have mutations in their GJB6 gene, suggesting a digenic inheritance of GJB2/GJB6 mutation. In total, four novel mutations were identified; E137D (n=1), R32Q (n=1), E101K (n=1) and Y156H (n=1) and one mutation deletion; 366delT (n=1). All students with association GJB2 mutation and GJB6 showed severe to profound hearing loss in both ears. Interestingly this study not detected the large deletion of 342 kb in GJB6 gene suggesting that the mutation is very rare in this region compared to certain parts of the world.
    Matched MeSH terms: Hearing Loss, Sensorineural/ethnology; Hearing Loss, Sensorineural/genetics*
  7. Therapeutic approach in managing patients with large vestibular aqueduct syndrome (LVAS)
    Asma A, Anouk H, Luc VH, Brokx JP, Cila U, Van De Heyning P
    Int J Pediatr Otorhinolaryngol, 2010 May;74(5):474-81.
    PMID: 20189254 DOI: 10.1016/j.ijporl.2010.01.023
    To discuss the clinical approach in managing patients with large vestibular aqueduct syndrome.
    Matched MeSH terms: Hearing Loss, Sensorineural/etiology; Hearing Loss, Sensorineural/surgery*
  8. Fulltext Prognostic indicators of management of sudden sensorineural hearing loss in an Asian hospital
    Tiong TS
    Singapore Med J, 2007 Jan;48(1):45-9.
    PMID: 17245516
    INTRODUCTION: This retrospective review evaluates the prognostic indicators in the management of sudden sensorineural hearing loss (SSNHL) in otorhinolaryngology patients admitted to Brunei RIPAS Hospital.
    METHODS: The review covered data collected from 1996 to 1998, and included patients who were treated with naftidrofuryl and dexamethasone. Their hearing improvement (more than 20 dB) in relation to pure tone audiogram patterns, hearing loss severity, vertigo, age of the patients and duration from symptom onset to time of treatment, were evaluated.
    RESULTS: After exclusion of three patients with known causes of hearing loss, 50 patients with idiopathic SSNHL were reviewed. Six audiogram patterns were observed with good prognostic indicators in patterns 1 and 4, where respectively, 95 percent and 100 percent of the patients improved. The hearing improvement was noted in the majority of the patients with mild or moderate degree of hearing loss, especially noticeable in audiogram patterns 1 and 4. Initial vertigo gave poor prognosis in hearing (more than 33 percent patients with vertigo improved versus 88 percent patients without vertigo improved). Patients aged between 21 and 60 years appear to have better prognosis of hearing improvement compared to those who are outside of this age range. 92 percent patients whose treatment started within the first week of onset of hearing loss experienced hearing improvement compared to 84 percent patients whose treatment started in the second week after onset.
    CONCLUSION: Fairly consistent with the previously-reported prognostic indicators are hearing loss severity, vertigo, age and duration of onset to treatment, though there was reasonably good prognosis when the patients were treated in the second week from hearing loss onset. Further findings of note were the six audiogram patterns and the good prognostic indicators in patterns 1 and 4.
    Matched MeSH terms: Hearing Loss, Sensorineural/drug therapy; Hearing Loss, Sensorineural/epidemiology*
  9. Valproate-induced reversible sensorineural hearing loss: a case report with serial audiometry and pharmacokinetic modelling during a valproate rechallenge
    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/chemically induced*; Hearing Loss, Sensorineural/physiopathology
  10. Fulltext A novel de novo mutation in COL2A1 gene associated with fetal skeletal dysplasia
    Chu FC, Hii LY, Hung TH, Lo LM, Hsieh TT, Shaw SW
    Taiwan J Obstet Gynecol, 2021 Mar;60(2):359-362.
    PMID: 33678343 DOI: 10.1016/j.tjog.2021.01.017
    OBJECTIVE: Skeletal dysplasias, caused by genetic mutations, are a heterogenous group of heritable disorders affecting bone development during fetal life. Stickler syndrome, one of the skeletal dysplasias, is an autosomal dominant connective tissue disorder caused by abnormal collagen synthesis owing to a genetic mutation in COL2A1.

    CASE REPORT: We present the case of a 38-year-old multipara woman whose first trimester screening showed a normal karyotype. However, the bilateral femur and humerus length symmetrically shortened after 20 weeks. Next-generation sequencing for mutations in potential genes leading to skeletal dysplasia detected a novel de novo mutation (c.1438G > A, p.Gly480Arg) in COL2A1, causing Stickler syndrome type 1. This pathogenic mutation might impair or destabilize the collagen structure, leading to collagen type II, IX, and XI dysfunction.

    CONCLUSION: We identified a novel de novo mutation in COL2A1 related to the STL1 syndrome and delineated the extent of the skeletal dysplasia disease spectrum.

    Matched MeSH terms: Hearing Loss, Sensorineural/diagnosis*; Hearing Loss, Sensorineural/embryology; Hearing Loss, Sensorineural/genetics*
  11. Evaluation of real-world preferences and performance of hearing aids fitted according to the NAL-NL1 and DSL v5 procedures in children with moderately severe to profound hearing loss
    Quar TK, Ching TY, Newall P, Sharma M
    Int J Audiol, 2013 May;52(5):322-32.
    PMID: 23570290 DOI: 10.3109/14992027.2012.755740
    The study aims to compare the performance of hearing aids fitted according to the NAL-NL1 and DSL v5 prescriptive procedure for children.
    Matched MeSH terms: Hearing Loss, Sensorineural/diagnosis; Hearing Loss, Sensorineural/psychology; Hearing Loss, Sensorineural/rehabilitation*
  12. Fulltext Hearing loss screening tool (COBRA score) for newborns in primary care setting
    Poonual W, Navacharoen N, Kangsanarak J, Namwongprom S, Saokaew S
    Korean J Pediatr, 2017 Nov;60(11):353-358.
    PMID: 29234358 DOI: 10.3345/kjp.2017.60.11.353
    Purpose: To develop and evaluate a simple screening tool to assess hearing loss in newborns. A derived score was compared with the standard clinical practice tool.

    Methods: This cohort study was designed to screen the hearing of newborns using transiently evoked otoacoustic emission and auditory brain stem response, and to determine the risk factors associated with hearing loss of newborns in 3 tertiary hospitals in Northern Thailand. Data were prospectively collected from November 1, 2010 to May 31, 2012. To develop the risk score, clinical-risk indicators were measured by Poisson risk regression. The regression coefficients were transformed into item scores dividing each regression-coefficient with the smallest coefficient in the model, rounding the number to its nearest integer, and adding up to a total score.

    Results: Five clinical risk factors (Craniofacial anomaly, Ototoxicity, Birth weight, family history [Relative] of congenital sensorineural hearing loss, and Apgar score) were included in our COBRA score. The screening tool detected, by area under the receiver operating characteristic curve, more than 80% of existing hearing loss. The positive-likelihood ratio of hearing loss in patients with scores of 4, 6, and 8 were 25.21 (95% confidence interval [CI], 14.69-43.26), 58.52 (95% CI, 36.26-94.44), and 51.56 (95% CI, 33.74-78.82), respectively. This result was similar to the standard tool (The Joint Committee on Infant Hearing) of 26.72 (95% CI, 20.59-34.66).

    Conclusion: A simple screening tool of five predictors provides good prediction indices for newborn hearing loss, which may motivate parents to bring children for further appropriate testing and investigations.

    Matched MeSH terms: Hearing Loss, Sensorineural
  13. Fulltext Hearing Loss in SARS-CoV-2: What Do We Know?
    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/etiology; Hearing Loss, Sensorineural/physiopathology*
  14. Fulltext Sudden sensorineural hearing loss: A missed opportunity for treatment
    Ali AH, Salahuddin Z, Salim R
    Malays Fam Physician, 2018;13(3):29-30.
    PMID: 30800231 MyJurnal
    Sudden sensorineural hearing loss (SSNHL) is an otology emergency and carries significant morbidity if the diagnosis is missed. It can present to any specialty but in our local setting the patient usually presents to primary care as it is easily accessible. We present a case of SSNHL that was initially presented to a primary care centre and the patient was reassured without any investigation being carried out. SSNHL has many causes thus making diagnosis difficult. However, with knowledge of its possible, a diagnosis can be made and appropriate management can be advocated to the patient. Hence, we discuss the three main causes of SSNHL, while emphasizing the immune system-mediated mechanism as the main cause in this case.
    Matched MeSH terms: Hearing Loss, Sensorineural
  15. Internal auditory canal stenosis in congenital sensorineural hearing loss
    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/congenital*; Hearing Loss, Sensorineural/diagnosis; Hearing Loss, Sensorineural/pathology*; Hearing Loss, Sensorineural/physiopathology
  16. Fulltext The noise exposed factory workers: the prevalence of sensori-neural hearing loss and their use of personal hearing protection devices
    Maisarah SZ, Said H
    Med J Malaysia, 1993 Sep;48(3):280-5.
    PMID: 8183139
    A total of 524 industrial workers were studied. They consisted of 442 noise exposed and 82 non-noise exposed workers. The purpose was to compare the prevalence of sensori-neural hearing loss among the noise exposed and the non-noise exposed workers, to study their knowledge on the hazard of noise to hearing and the workers' attitude towards the hearing protection devices. The prevalence of sensori-neural hearing loss was significantly higher among the noise exposed workers, i.e., 83% versus 31.7% (p < 0.01). However, the prevalence of hearing impairment was much lower for both groups, being 30.1% for the noise exposed and 3.7% for the non-noise exposed group. Although hearing protection devices were provided to 80.5% of the workers, only 5.1% were wearing them regularly. The possibility of developing hearing loss due to exposure to excessive noise was only known by 35.5% of the noise exposed workers. This awareness was found to have a positive correlation with the workers' compliance to the hearing protection devices. Our findings highlight the need for workers to be educated on the hazards of excessive noise exposure to hearing.
    Matched MeSH terms: Hearing Loss, Sensorineural/epidemiology*; Hearing Loss, Sensorineural/prevention & control
  17. Fulltext Rheumatoid pachymeningitis
    Tan HJ, Raymond AA, Phadke PP, Rozman Z
    Singapore Med J, 2004 Jul;45(7):337-9.
    PMID: 15221051
    Symptomatic rheumatoid pachymeningitis is a rare extra-articular manifestation of rheumatoid arthritis. Clinical symptoms are non-specific and diagnosis is frequently made by exclusion. We present a 61-year-old woman with a 9-year history of rheumatoid arthritis presenting with deafness and progressive disability over a two month duration. She was diagnosed as having rheumatoid pachymeningitis based on the cerebral magnetic resonance imaging findings.
    Matched MeSH terms: Hearing Loss, Sensorineural/etiology
  18. Fulltext Large vestibular aqueduct syndrome
    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/congenital*; Hearing Loss, Sensorineural/physiopathology
  19. Fulltext Idiopathic sudden sensorineural deafness--an approach to the problem.
    V K Khanijow VK, Raman R
    Singapore Med J, 1988 Feb;29(1):76-7.
    PMID: 3406777
    Matched MeSH terms: Hearing Loss, Sensorineural*
  20. Fulltext Hypertensive retinopathy and sensorineural hearing loss
    Tan TY, Rahmat O, Prepageran N, Fauzi A, Noran NH, Raman R
    Indian J Otolaryngol Head Neck Surg, 2009 Dec;61(4):275-9.
    PMID: 23120650 DOI: 10.1007/s12070-009-0082-9
    To determine the correlation between hypertensive retinopathy (which is the end-organ damage of the vessels due to chronic hypertension) with sensorineural hearing loss.
    Matched MeSH terms: Hearing Loss, Sensorineural
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