Bilateral Evoked Otoacoustic Emissions in a Child with Bilateral Profound Hearing Loss

1996 ◽  
Vol 105 (4) ◽  
pp. 286-288 ◽  
Author(s):  
Laurence Laccourreye ◽  
Esther Tran BA Huy ◽  
Martine François ◽  
Philippe Narcy
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Brain Stem ◽  
Inner Ear ◽  
Otoacoustic Emissions ◽  
Review Of The Literature ◽  
Profound Hearing Loss ◽  
Normal Limits

Recording of bilateral evoked otoacoustic emissions in a 3-year-old girl with bilateral profound hearing loss is presented. No component of auditory-evoked brain stem potentials was recorded. Findings on computed tomography of the inner ear were within normal limits. At retest 3 months later, these results were unchanged. Explanations for this particular condition are presented together with a review of the literature.

Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Alaa Nasser Hussain Zaher ◽  
Tougan Taha Abd El Aziz ◽  
Ahmed Samy Abdelrahman
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Inner Ear ◽  
Resonance Imaging ◽  
High Resolution Computed Tomography ◽  
Vestibular Aqueduct ◽  
Incomplete Partition ◽  
Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

scholarly journals The Use of Frequency Lowering Technology in the Treatment of Severe-to-Profound Hearing Loss: A Review of the Literature and Candidacy Considerations for Clinical Application

2018 ◽  
Vol 39 (04) ◽  
pp. 377-389 ◽  
Author(s):  
Susan Scollie ◽  
Danielle Glista
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Clinical Application ◽  
Current Literature ◽  
High Frequency ◽  
Hearing Aid ◽  
Verification And Validation ◽  
Review Of The Literature ◽  
Profound Hearing Loss

AbstractThis article provides a review of the current literature on the topic of frequency lowering hearing aid technology specific to the treatment of severe and profound levels of hearing impairment in child and adult listeners. Factors to consider when assessing listener candidacy for frequency lowering technology are discussed. These include factors related to audiometric assessment, the listener, the type of hearing aid technology, and the verification and validation procedures that can assist in determining candidacy for frequency lowering technology. An individualized candidacy assessment including the use of real-ear verification measures and carefully chosen validation tools are recommended for listeners requiring greater audibility of high-frequency sounds, when compared with amplification via conventional hearing aid technology.

scholarly journals Hearing Screening of Newborns using Distortion Product Otoacoustic Emissions

2021 ◽  
Vol 29 (2) ◽  
pp. 189-195
Author(s):  
Vybhavi MK ◽  
Srinivas V
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Screening Tool ◽  
Hearing Screening ◽  
Distortion Product Otoacoustic Emissions ◽  
Profound Hearing Loss ◽  
Distortion Product ◽  
Lost To Follow Up ◽  
Evoked Response Audiometry

Introduction  The present study was devised to estimate the prevalence of neonatal hearing loss and document the importance of using DPOAE as a screening tool for identifying hearing loss in newborns. Materials and Methods This hospital based descriptive study was conducted from August 2018 to August 2019. A total of 928 newborn babies were included in the study. These newborn babies were subjected to hearing screening by distortion product otoacoustic emissions (DPOAE) at 24-72 hrs after birth. For pass cases, no further testing was done. For refer cases, repeat testing with DPOAE was done within 15-30 days. Newborns with refer result on repeat DPOAE testing were subjected to Brainstem evoked response audiometry (BERA) within 3 months to confirm hearing loss. Results Nine hundred and twenty eight newborn babies were screened by DPOAE. 851 newborns passed the first DPOAE hearing screening and 77 newborns gave refer result. 21 newborns were lost to follow-up. 56 newborns underwent repeat DPOAE testing and 5 newborns were referred for BERA. Amongst the 5 newborns who underwent BERA testing, one newborn was diagnosed with bilateral profound hearing loss. Hence, the prevalence of hearing loss of 1.08 per thousand newborn babies was estimated in this study. Conclusion Hearing screening of newborns using DPOAE followed by BERA in refer cases to confirm hearing loss is useful for early detection followed by timely intervention and rehabilitation.

scholarly journals Evidence for a dynorphin-mediated inner ear immune/inflammatory response and glutamate-induced neural excitotoxicity: an updated analysis

2019 ◽  
Vol 122 (4) ◽  
pp. 1421-1460
Author(s):  
Tony L. Sahley ◽  
David J. Anderson ◽  
Michael D. Hammonds ◽  
Karthik Chandu ◽  
Frank E. Musiek
Keyword(s):  
Hearing Loss ◽  
Brain Stem ◽  
Inner Ear ◽  
Hair Cells ◽  
Oxidative Stress Response ◽  
Neural Systems ◽  
Type I ◽  
Excitatory Neurotransmitter ◽  
Axon Terminals ◽  
Cochlear Hearing Loss

Acoustic overstimulation (AOS) is defined as the stressful overexposure to high-intensity sounds. AOS is a precipitating factor that leads to a glutamate (GLU)-induced Type I auditory neural excitotoxicity and an activation of an immune/inflammatory/oxidative stress response within the inner ear, often resulting in cochlear hearing loss. The dendrites of the Type I auditory neural neurons that innervate the inner hair cells (IHCs), and respond to the IHC release of the excitatory neurotransmitter GLU, are themselves directly innervated by the dynorphin (DYN)-bearing axon terminals of the descending brain stem lateral olivocochlear (LOC) system. DYNs are known to increase GLU availability, potentiate GLU excitotoxicity, and induce superoxide production. DYNs also increase the production of proinflammatory cytokines by modulating immune/inflammatory signal transduction pathways. Evidence is provided supporting the possibility that the GLU-mediated Type I auditory neural dendritic swelling, inflammation, excitotoxicity, and cochlear hearing loss that follow AOS may be part of a brain stem-activated, DYN-mediated cascade of inflammatory events subsequent to a LOC release of DYNs into the cochlea. In support of a DYN-mediated cascade of events are established investigations linking DYNs to the immune/inflammatory/excitotoxic response in other neural systems.

scholarly journals High incidence of cisplatin-induced ototoxicity in paediatric patients in the Western Cape, South Africa

2018 ◽  
Vol 2 ◽  
Author(s):  
Mukovhe Phanguphangu ◽  
Lebogang Ramma
Keyword(s):  
Hearing Loss ◽  
Otoacoustic Emissions ◽  
Negative Impact ◽  
Paediatric Oncology ◽  
Cancer Drug ◽  
Oncology Patients ◽  
Profound Hearing Loss ◽  
Distortion Product ◽  
High Incidence

Background: Fourteen million new cancer cases are reported annually, and up to 10% of those involve children below 15 years. Cisplatin, a commonly used anti-cancer drug for its high success rate, is associated with ototoxicity. Cisplatin-induced ototoxicity is characterised by permanent bilateral severe-to-profound hearing loss. Hearing loss, when occurring during childhood, can impact negatively communication development, scholastic performance and quality of life.Aim: To determine the incidence of cisplatin-induced ototoxicity in paediatric oncology.Setting: A retrospective records review of paediatric oncology patients who underwent cisplatin-based chemotherapy and had ototoxicity monitoring from January 2015 to December 2017 at a children’s hospital.Method: Data collected included demographic, cisplatin treatment and audiometric information. The data were analysed using descriptive and inferential statistics.Results: A total of 49 records meeting the inclusion criteria were reviewed. Ototoxic hearing loss was found in 39 (80%) of the patients whose records were reviewed and the majority (56%) presented with a bilateral moderate-to-severe sensorineural hearing loss. Distortion product otoacoustic emissions were absent in 32 (67%) patients. Cumulative dose (> 200 mg/m2) was associated with higher incidences of ototoxicity (odds ratio [OR]: 1.81; 95% confidence interval [CI]: 0.67–17.34; p = 0.044). Younger patients (< 10 years) had higher odds of developing ototoxicity, but this was not statistically significant (OR: 4.00; 95% CI: 0.82–19.46; p = 0.085).Conclusion: This study found a high incidence of cisplatin-induced ototoxicity in paediatric oncology patients. This is concerning because hearing loss during this age can have long-term negative impact on a child’s development and overall quality of life. Early identification of ototoxicity-induced hearing loss and appropriate intervention are highly recommended in this patient group.

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.

scholarly journals Bilateral Sudden Sensorineural Hearing Loss in Waldenström’s Macroglobulinemia: Case Report and Review of the Literature

2019 ◽  
Vol 2 (02) ◽  
pp. 81-84
Author(s):  
Nael M. Shoman ◽  
Thamir Aldahmashi
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Review Of The Literature ◽  
Waldenström’S Macroglobulinemia ◽  
Waldenstrom's Macroglobulinemia ◽  
Waldenstrom’S Macroglobulinemia ◽  
Waldenström's Macroglobulinemia

Abstract Objective Waldenström’s macroglobulinemia (WM) is a rare indolent B-cell lymphoproliferative disorder, representing 1 to 2% of all hematological malignancies. Involvement of the inner ear is rare with only case reports published over the past six decades. Methods The occurrence of bilateral sudden sensorineural hearing loss is exceeding rare, with limited published data in the literature. We present a case of a 62-year-old man diagnosed with WM who presented with bilateral sequential sudden sensorineural hearing loss. Results A few months following his WM diagnosis, he experienced sudden hearing loss in his left ear. He was treated with a course of oral steroids with no improvement. Three months following this incident, he experienced a similar sudden loss of hearing in his right ear. Treatment for WM was initiated. A repeat hearing test, done 1 week later, did not show any significant improvement in his right hearing. Conclusion The clinical course of WM is highly variable, with relatively infrequent involvement of lymph nodes, spleen, or liver. The inner ear is rarely involved. In this article, otologic clinical presentation is discussed, along with a review of the literature on hearing loss in WM.

Enlarged vestibular aqueducts and other inner-ear abnormalities in patients with Down syndrome

2016 ◽  
Vol 131 (4) ◽  
pp. 298-302 ◽  
Author(s):  
C M Clark ◽  
H H Patel ◽  
S G Kanekar ◽  
H Isildak
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Down Syndrome ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Sensorineural Hearing ◽  
Vestibular Aqueduct ◽  
Computed Tomography Images ◽  
Temporal Bones

AbstractBackground:Histopathological anomalies of inner-ear structures in individuals with Down syndrome have been well documented; however, few studies have examined the radiological features.Methods:A retrospective study was conducted of temporal bone computed tomography images in 38 individuals (75 ears) with Down syndrome to evaluate the prevalence of inner-ear abnormalities and assess vestibular aqueduct widths.Results:Inner-ear anomalies were identified in 20 of the 38 individuals (52.6 per cent). Seven of the 75 temporal bones (9.3 per cent) were found to have higher than previously reported. A dilated internal auditory canal and vestibule were more common among the present study group, while prior studies have demonstrated internal auditory canal stenosis and decreased vestibule size.Conclusion:Down syndrome patients exhibit a high prevalence of dysplastic inner-ear features that confer substantial risk of sensorineural hearing loss. Computed tomography is a useful screening aid to detect inner-ear abnormalities, particularly enlarged vestibular aqueducts, which cause preventable sensorineural hearing loss in this population.

Prognosis of patients with idiopathic sudden hearing loss: role of vestibular assessment

2010 ◽  
Vol 125 (3) ◽  
pp. 251-257 ◽  
Author(s):  
S Korres ◽  
G A Stamatiou ◽  
E Gkoritsa ◽  
M Riga ◽  
J Xenelis
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Sudden Hearing Loss ◽  
Caloric Test ◽  
Test Results ◽  
Vestibular Evoked Myogenic Potential ◽  
Early Recovery ◽  
Profound Hearing Loss ◽  
Myogenic Potential ◽  
Vestibular Assessment

AbstractObjective:To evaluate the correlation between caloric and vestibular evoked myogenic potential test results, initial audiogram data, and early hearing recovery, in patients with idiopathic sudden hearing loss.Materials and methods:One hundred and four patients with unilateral idiopathic sudden hearing loss underwent complete neurotological evaluation. Results for vestibular evoked myogenic potential and caloric testing were compared with patients' initial and final audiograms.Results:Overall, abnormal vestibular evoked myogenic potential responses occurred in 28.8 per cent of patients, whereas abnormal caloric test results occurred in 50 per cent. A statistically significant relationship was found between the type of inner ear lesion and the incidence of profound hearing loss. Moreover, a negative correlation was found between the extent of the inner ear lesion and the likelihood of early recovery.Conclusion:In patients with idiopathic sudden hearing loss, the extent of the inner ear lesion tends to correlate with the severity of cochlear damage. Vestibular assessment may be valuable in predicting the final outcome.

scholarly journals Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience

2020 ◽  
Vol 36 (7) ◽  
Author(s):  
Jawwad Ahmed ◽  
Ghulam Saqulain ◽  
Muhammad Iqbal javed Khan ◽  
Mobeen Kausar
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Demographic Data ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Creative Commons ◽  
Inner Ear Malformations ◽  
Ear Malformations

Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and left ears was noted with inner ear malformations. The most common anomaly noted were CLA, CH-III and CH-II in decreasing order of frequency in both ears. Conclusion: The prevalence of IEM’s was found to be 48(10%). Commonest anomalies noted were CLA, CH-III and CH-II. No significant association of gender and age of presentation was noted with type of anomaly in both ears. doi: https://doi.org/10.12669/pjms.36.7.3134 How to cite this:Ahmed J, Saqulain G, Khan MIJ, Kausar M. Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience . Pak J Med Sci. 2020;36(7):---------.  doi: https://doi.org/10.12669/pjms.36.7.3134 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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