An auditory profile of sclerosteosis

2014 ◽  
Vol 128 (4) ◽  
pp. 336-344 ◽  
Author(s):  
J M Potgieter ◽  
D W Swanepoel ◽  
B M Heinze ◽  
L M Hofmeyr ◽  
A A S Burger ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Bone Growth ◽  
Round Window ◽  
Internal Auditory Canal ◽  
Auditory Brainstem ◽  
Oval Window ◽  
Speech Discrimination ◽  
Cross Sectional ◽  
Brainstem Response

AbstractObjective:To characterise auditory involvement secondary to excessive craniotubular bone growth in individuals with sclerosteosis in South Africa.Methods:This cross-sectional study assessed the auditory profile of 10 participants with sclerosteosis. An auditory test battery was used and results for each ear were recorded using descriptive and comparative analyses.Results:All participants presented with bilateral, mixed hearing losses. Of the 20 ears, hearing loss was moderate in 5 per cent (n = 1), severe in 55 per cent (n = 11) and profound in 40 per cent (n = 8). Air–bone gaps were smaller in older participants, although the difference was not statistically significant (p > 0.05). Computed tomography scans indicated pervasive abnormalities of the external auditory canal, tympanic membrane, middle-ear space, ossicles, oval window, round window and internal auditory canal. Narrowed internal auditory canals corresponded to poor speech discrimination, indicative of retrocochlear pathology and absent auditory brainstem response waves.Conclusion:Progressive abnormal bone formation in sclerosteosis involves the middle ear, the round and oval windows of the cochlea, and the internal auditory canal. The condition compromises conductive, sensory and neural auditory pathways, which results in moderate to profound, mixed hearing loss.

scholarly journals Diagnostic Yield of MRI for Sensorineural Hearing Loss – An Audit

2020 ◽  
Vol 47 (5) ◽  
pp. 656-660
Author(s):  
Helen Wong ◽  
Yaw Amoako-Tuffour ◽  
Khunsa Faiz ◽  
Jai Jai Shiva Shankar
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Diagnostic Yield ◽  
Internal Auditory Canal ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Vestibular Evoked Myogenic Potentials ◽  
Clinical Indication ◽  
Presenting Symptoms ◽  
Brainstem Response

ABSTRACT:Purpose:Contrast-enhanced magnetic resonance imaging (CEMRI) of the head is frequently employed in investigations of sensorineural hearing loss (SNHL). The yield of these studies is perceptibly low and seemingly at odds with the aims of wise resource allocation and risk reduction within the Canadian healthcare system. The purpose of our study was to audit the use and diagnostic yield of CEMRI for the clinical indication of SNHL in our institution and to identify characteristics that may be leveraged to improve yield and optimize resource utilization.Materials and methods:The charts of 500 consecutive patients who underwent CEMRI of internal auditory canal for SNHL were categorized as cases with relevant positive findings on CEMRI and those without relevant findings. Demographics, presenting symptoms, interventions and responses, ordering physicians, and investigations performed prior to CEMRI testing were recorded. Chi-squared test and t-test were used to compare proportions and means, respectively.Results:CEMRI studies revealed relevant findings in 20 (6.2%) of 324 subjects meeting the inclusion criteria. Pre-CEMRI testing beyond audiometry was conducted in 35% of those with relevant positive findings compared to 7.3% of those without (p < 0.001). Auditory brainstem response/vestibular-evoked myogenic potentials were abnormal in 35% of those with relevant CEMRI findings compared to 6.3% of those without (p < 0.001).Conclusion:CEMRI is a valuable tool for assessing potential causes of SNHL, but small diagnostic yield at present needs justification for contrast injection for this indication. Our findings suggest preferred referral from otolaryngologists exclusively, and implementation of a non-contrast MRI for SNHL may be a better diagnostic tool.

scholarly journals The design of a lumped parameter model considering the stimulus path of round window

2021 ◽  
Vol 29 ◽  
pp. 49-56
Author(s):  
Jong Hoon Kim ◽  
Min Gyu Park ◽  
Qun Wei ◽  
Ki Woong Seong ◽  
Jyung Hyun Lee
Keyword(s):  
Guinea Pigs ◽  
Round Window ◽  
Animal Experiments ◽  
Hearing Threshold ◽  
Auditory Brainstem ◽  
Oval Window ◽  
Lumped Parameter Model ◽  
Piezo Actuator ◽  
Lumped Parameter ◽  
Brainstem Response

BACKGROUND: Sound normally enters the ear canal, passes through the middle ear, and stimulates the cochlea through the oval window. Alternatively, the cochlea can be stimulated in a reverse manner, namely round window stimulation. The reverse stimulation is not well understood, partly because in classic lumped-parameter models the path of reverse drive during the round window stimulation is usually not considered. OBJECTIVE: The study goal is to gain a better understanding of the hearing mechanism during round window stimulation. METHODS: A piezo actuator was coupled to the oval and round window of the guinea pigs. The auditory brainstem response produced by the forward and reverse stimulation at four frequencies was recorded. RESULTS: The results show that the input voltage of the actuator required at the hearing threshold in the round window drive was higher than that in the oval window drive. In order to understand the data, we designed a lumped-parameter cochlear model that can simulate both forward and reverse drive. The model-predicted results were consistent with the experimental results. CONCLUSIONS: The response of the auditory system to stimulus of oval window and round window was quantified through animal experimentation, and guinea pigs were used as experimental animals. When the same stimulus was applied to the oval window and round window of the cochlea, the ABR signals were compared. A lumped parameter model was designed to incorporate the sound transmission paths in both oval and round window stimulation. The simulated results are consistent with those of animal experiments. This model will be useful in understanding the inner-ear response in round window.

scholarly journals Study of effect of chronic suppurative otitis media on auditory brainstem response latency

2020 ◽  
Vol 6 (4) ◽  
pp. 690
Author(s):  
Hanumanth Prasad Muniyappa ◽  
Ravi Dudda ◽  
Balaji Nagavara Kalegowda ◽  
Vandana Basvaraj
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Cross Sectional Study ◽  
Chronic Suppurative Otitis Media ◽  
Cross Sectional ◽  
Suppurative Otitis Media ◽  
Brainstem Response ◽  
Latency Shift

<p class="abstract"><strong>Background:</strong> Chronic suppurative otitis media (CSOM) emerging as common hearing problems in the recent years with high prevalence requiring immediate attention. Hence, our study includes assessment of adult patients with CSOM using electrophysiological test, auditory brainstem response (ABR) and analysing the latency of ABR peaks in order to find the effect of CSOM on ABR latency with respect to shift in latency.</p><p class="abstract"><strong>Methods:</strong> The study followed cross sectional study design where data collected from March to September 2019 were used. A total of 50 subjects with unilateral CSOM were analysed. Descriptive statistics and paired t-test was used for statistical analysis of the data.  </p><p class="abstract"><strong>Results:</strong> The data was divided into 3 groups based on degree of hearing loss (mild, moderate and moderately severe). The Mean ABR peak latency was analysed and subjects showed a significant latency shift. Also, it was found that the magnitude of latency shift increased with increase in degree of hearing loss.</p><p class="abstract"><strong>Conclusions:</strong> Hence the study concludes that as the amount of conductive component increases the pure tone threshold deteriorates and ABR latency gets affected. Also, the morphology of ABR peaks on comparison to the normal hearing ear gets affected due to constant conductive pathology in the pathological ear.</p><p class="abstract"> </p>

scholarly journals Hearing Loss after Round Window Surgery in Mice Is due to Middle Ear Effusion

2016 ◽  
Vol 21 (6) ◽  
pp. 356-364 ◽  
Author(s):  
Bovey Z. Zhu ◽  
Jasmine Saleh ◽  
Kevin T. Isgrig ◽  
Lisa L. Cunningham ◽  
Wade W. Chien
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Hair Cells ◽  
Round Window ◽  
Auditory Brainstem ◽  
Auditory Brainstem Responses ◽  
Middle Ear Effusion ◽  
Injection Methods ◽  
Highly Correlated ◽  
Ear Effusion

Background: Delivery of therapeutic agents directly through the round window (RW) offers promise for treating sensorineural hearing loss. However, hearing loss can result from the surgical approach itself, and the reasons for this are poorly understood. We examined the hearing loss following the 3 major steps involved with the RW approach to access the mouse cochlea: bullostomy, RW puncture, and RW injection. Methods: Twenty-one adult CBA/J mice underwent bullostomy alone, 10 underwent RW puncture, and 8 underwent RW injection with PBS with 5% glycerol. Auditory brainstem responses (ABR) and otoscopy were performed preoperatively and up to 6 weeks postoperatively. Hair cells were stained, and survival was assessed using immunofluorescence. Results: One week postoperatively, mice in all groups showed significant threshold shifts. Otoscopy revealed approximately half of all mice had middle ear effusion (MEE), with a higher incidence of effusion in the RW puncture and RW injection groups. Those with MEE had significant ABR threshold shifts, whereas those without MEE had minimal hearing loss. MEE persisted through 6 weeks in a majority of cases, but in those mice with MEE resolution, there was at least partial improvement in hearing. Immunohistochemistry showed minimal loss of hair cells in all animals. Conclusion: MEE is highly correlated with hearing loss in mice undergoing RW surgery. Otoscopy is an important adjunct to consider after ear surgery in mice, as MEE may contribute to postsurgical hearing loss.

Effects of Conductive Hearing Loss on Auditory Brainstem Response

1982 ◽  
Vol 91 (3) ◽  
pp. 304-309 ◽  
Author(s):  
Therese J. McGee ◽  
Jack D. Clemis
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Auditory Brainstem Response ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Auditory Brainstem ◽  
Middle Ear Effusion ◽  
Brainstem Response ◽  
Conductive Hearing ◽  
Ear Effusion

The purpose of this paper is not to propose that auditory brainstem response (ABR) be utilized for the assessment of conductive losses, but to define the effects of conductive hearing loss on the ABR when such a complication occurs. Conductive losses attenuate cochlear stimulation. Since wave V latency is inversely related to stimulus intensity, the magnitude of the conductive loss should be a predictor of the wave V latency delay. In this study, ABR wave V latencies from patients with known conductive losses due to canal occlusion, middle ear effusion, ossicular fixation and chain interruption were compared with latency values calculated from the magnitude of the loss. In those patients with occlusion of the external auditory canal and middle ear effusion, the shift of the wave V latency-intensity function correlated well with the air-bone gap. This correlation was poor for patients with ossicular chain disorders. In mixed hearing losses, the increased wave V latency due to the conductive component may totally mask an increase in latency caused by a retrocochlear component.

Cochlear Prostheses: Stimulation-Induced Damage

1983 ◽  
Vol 92 (6) ◽  
pp. 599-609 ◽  
Author(s):  
Josef M. Miller ◽  
Larry G. Duckert ◽  
Bryan E. Pfingst ◽  
Mark A. Malone
Keyword(s):  
Auditory Brainstem Response ◽  
Bone Growth ◽  
Round Window ◽  
Auditory Brainstem ◽  
Current Level ◽  
Stimulation Site ◽  
Scala Tympani ◽  
Stimulus Current ◽  
Brainstem Response ◽  
Stimulation Of

The effects of 4 weekly, three-hour exposures to continuous sinusoidal (1 kHz) electrical stimulation of the inner ear at various current levels were assessed in the chronically implanted guinea pig. With scala tympani stimulation, histopathological damage, including new bone growth, was observed for currents at and above 100 μA rms. No changes were observed in similarly implanted, but not stimulated cochleas. At equal current levels, less damage was found in subjects stimulated via electrodes placed on the round window and promontory, as compared to the scala tympani. Consistent reversible changes in threshold and suprathreshold features of the electrically evoked auditory brainstem response (EABR) were found. The magnitude of EABR change was directly related to exposure stimulus current level and to cochlear stimulation site. Suprathreshold features of the EABR were more sensitive to continuous stimulation exposures than threshold measures. Reversible EABR changes were found in the presence and absence of stimulation-induced histopathology.

scholarly journals Prevalence of hearing loss among preschool children in Hanoi, Vietnam

2019 ◽  
Vol 6 (4) ◽  
pp. 1501
Author(s):  
Nguyen Tuyet Xuong ◽  
Van Dinh Tran
Keyword(s):  
Hearing Loss ◽  
Preschool Children ◽  
School Health ◽  
Acoustic Emissions ◽  
Auditory Brainstem ◽  
Primary School Children ◽  
School Age Children ◽  
Cross Sectional ◽  
Brainstem Response ◽  
School Health Programs

Background: Hearing loss in children is a common entity worldwide. This study examined the prevalence of hearing loss among preschool children in Hanoi, Vietnam.Methods: A cross sectional was conducted among pre-schoolers aged 2-5 years in Hanoi, Vietnam to determine the prevalence of hearing loss according to the method recommended by the Joint Committee on Infant Hearing (JCIH): a two-step Automated Oto-Acoustic Emissions (AOAE) program, completed by an Auditory Brainstem Response (ABR) for the positive diagnosis of hearing impairment.Results: In total, there were 7,191 preschoolers and kindergarten screened. Three hundred and thirty-seven (4.7%) of children screened failed and were referred for further testing. The percentage of children with true hearing loss was 4.4% confirmed by ABR test. Majority of the hearing loss children was conductive hearing loss (70.4%). Mild hearing loss (21–≤40 dB) accounted for almost half of the children with hearing loss.Conclusions: This study provides the first estimates of audiometrically measured hearing loss prevalence among preschool children in Hanoi, Vietnam. The study found that hearing loss is common among pre-primary school children in the country. Routine hearing screening of school-age children should be included in annual school health programs in Vietnam.

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