Profound Hearing Loss Patients Research Articles

DESIGN AND IMPLEMENTATION OF A PORTABLE AUTOMATED AUDIOMETER FOR HEARING CLASSIFICATION USING MACHINE LEARNING APPROACHES

Audiometric tests can identify the hearing loss at specific frequencies using the audiogram. The aim and objectives of the study were (i) to develop an automated audiometer for self-diagnosing the hearing ability of the patient; (ii) to extract the features from the acoustic signals and to classify the normal and profound hearing loss patients using different machine learning algorithms; (iii) to validate the hearing loss classification using six-frequency average (6-FA) method based on simple linear regression analysis and machine learning algorithms. The study is conducted among 150 patients, including 75 patients with normal hearing ability and 75 patients with profound hearing loss. The total population of 150 underwent audiometric test both in the soundproof audiometric room and in the normal field environment. Based on the patient response, the intensity and frequency are changed automatically, and the audiogram is plotted by the principle of Artificial Neural Network learning procedures. The overall accuracy produced by classification of normal and profound hearing loss patients using Support Vector Machine (SVM), k-Nearest Neighbor classifier, and Naïve Bayes classifier is 97%, 96%, and 95%, respectively. The results indicated that the SVM classifier outperforms the other two classifiers well. The preliminary audiometric test can be performed remotely and then consulted with an audiologist. Thus, the patient could operate the developed prototype independently and get a consultation from trained medical personnel.

Evaluating Neurotrophin Signaling Using MicroRNA Perilymph Profiling in Cochlear Implant Patients With and Without Residual Hearing.

MicroRNAs predicted to regulate neurotrophin signaling can be found in human perilymph. Animal and human temporal bone studies suggest that spiral ganglion health can affect cochlear implant (CI) outcomes. Neurotrophins have been identified as a key factor in the maintenance of spiral ganglion health. Changes in miRNAs may regulate neurotrophin signaling and may reflect neurotrophin expression levels. Perilymph sampling was carried out in 18 patients undergoing cochlear implantation or stapedotomy. Expression of miRNAs in perilymph was evaluated using an Agilent miRNA gene chip. Using ingenuity pathway analysis (IPA) software, miRNAs targeting neurotrophin signaling pathway genes present in a cochlear cDNA library were annotated. Expression levels of miRNAs in perilymph were correlated to the patients' preoperative pure-tone average. Expression of mRNAs coding for neurotrophins and their receptors were identified in tissue obtained from normal human cochlea during skull base surgery. We identified miRNAs predicted to regulate these signaling cascades, including miR-1207-5p, miR-4651, miR-103-3p, miR-100-5p, miR-221-3p, miR-200-3p. There was a correlation between poor preoperative hearing and lower expression of miR-1207 (predicted to regulate NTR3) and miR-4651 (predicted to regulate NTR2). Additionally, miR-3960, miR-4481, and miR-675 showed significant differences in expression level when comparing mild and profound hearing loss patients. Expression of some miRNAs that are predicted to regulate neurotrophin signaling in the perilymph of cochlear implant patients vary with the patient's level of residual hearing. These miRNAs may serve as biomarkers for changes in neurotrophin signaling.

Cochlear Implant Outcomes in Patients With Severe Compared With Profound Hearing Loss

To compare the speech perception outcomes for patients with preoperative severe versus profound hearing loss with a cochlear implant (CI). Retrospective patient review. Cochlear implant program. Cochlear implant adult recipients (16 yr and above) having surgery between 2008 and 2015 with speech perception results and four frequency averaged severe (70-89 dBHL) or profound (90 dBHL and above) hearing loss. Prelingual deaf adults were included in the data. Cochlear implant. Speech perception scores with CUNY sentences and monosyllabic (CNC/CVC) word scores at preoperative and 3, 6, and 12 months postoperatively testing. Mann-Whitney U test was performed to compare outcomes of the two groups. Interquartile comparisons were also made. The severe group had significantly better speech perception than the profound hearing loss group for CUNY sentences and CNC/CVC word scores preoperatively (p < 0.001) (p < 0.001), at 6 months (p < 0.001) (p < 0.001), and at 12 months (p < 0.01) (p < 0.001), respectively. At 3 months there was no significant difference. The number of patients in each severe or profound group at the different time points ranged from 92 to 367 patients for CUNY sentences and from 52 to 216 patients for the word scores. The 12 months' lower quartile score for CUNY sentences for severe and profound groups was 83% and 75% respectively. The lower quartile score for words was 32% and 26% respectively. Adult CI recipients showed marked improvements in speech perception with a CI. Those with severe hearing loss have significantly better outcomes compared with profound hearing loss patients. These outcomes can inform CI candidacy evaluation criteria.

Prognostic predictors of sudden sensorineural hearing loss in defibrinogenation therapy

Conclusions: Defibrinogenation therapy rather than corticosteroids therapy should be chosen for patients specifically with profound hearing loss and with initial high fibrinogen. Objectives: Corticosteroids therapy is the standard treatment for sudden sensorineural hearing loss (SSNHL) and prognostic factors by this therapy were reported. Defibrinogenation therapy is one of the treatment options for SSNHL. Aims of this study were to identify prognostic factors and correlative markers with hearing improvement in treating SSNHL by defibrinogenation therapy. Methods: During the early phase of the study, consecutive 61 patients were treated by defibrinogenation therapy with batroxobin (50 units), whereas corticosteroids (500 mg/day of hydrocortisone tapered by 9 days) were used for consecutive 64 patients during the late phase. Blood data that could predict a complete recovery were identified. Coagulation/fibrinolysis markers correlated with hearing improvement by defibrinogenation therapy were investigated. Results: Although there were no overall differences in hearing improvement between the two therapies, recovery rate in profound hearing loss patients was better in defibrinogenation therapy. In patients who showed complete recovery, serum fibrinogen level before treatment was significantly higher in the defibrinogenation group than the corticosteroid group. Responses of several fibrinolysis markers to defibrinogenation therapy evaluated by post-/pre-values were negatively correlated with hearing improvement.

Degree of satisfaction of patients fitted with hearing aids at a high complexity service

Some individuals with hearing loss choose to be fitted with hearing aids. Compliance is significantly affected by how satisfied patients are with their hearing aids. Patient satisfaction can be assessed through questionnaires and scales. ObjectiveTo assess the degree of satisfaction of patients fitted with hearing aids. MethodScale “Satisfaction With Amplification in Daily Life” (SADL) was applied to 180 patients fitted with hearing aids; results were categorized based on the mean values observed for global satisfaction scores and scores attained on each subscale. Patients were interviewed for additional information. ResultsMean global score was 5.6; 48.9% of the subjects were very satisfied, 47.2% were satisfied, and 3.9% were dissatisfied. The mean score on subscale Positive Effects was 5.6; the mean score for Service and Cost was 6.2; for Negative Factors the mean score was 4.9; and the mean score on subscale Personal Image was 5.8. Of the patients fitted with in-the-ear hearing aids, 83.3% were very satisfied. Fifteen percent of the individuals were dissatisfied with their Personal Image. Sensorineural and profound hearing loss patients were less satisfied, with 5.4% and 50.0% of the subjects revealing dissatisfaction with their hearing aids. ConclusionPatients were generally very satisfied with their hearing aids. Satisfaction rates were higher among patients fitted with in-the-ear hearing aids. Dissatisfaction was higher in subscale Personal Image. Lesser degrees of satisfaction were seen in patients with sensorineural and profound hearing loss.

Etiologic analysis of severe to profound hearing loss patients from Chifeng city in Inner Mongolia

To investigate the etiology of patients with severe to profound hearing loss and to identify the ratio of hereditary hearing loss in Chifeng area in Northern China. DNA were extracted from peripheral blood of 134 deaf patients from Chifeng special educational school and 100 normal hearing controls in Northern China. Audiology examinations showed that all patients had severe to profound bilateral sensorineural hearing impairment. Sequence analysis of the whole coding areas of GJB2, GJB3, GJB6, SLC26A4, mtDNA12SrRNA and mtDNAtRNASer(UCN) were performed. Individuals carrying SLC26A4 mutation were given further temporal bone CT scan. The ratio of hearing loss related to genetic factors in this population was 60.45% (81/134). About 33.58% (45/134) of the patients were given accurate genetic diagnosis. GJB2 mutations were responsible for approximately 17.16% of the cases in ChiFeng area. By screening SLC26A4 followed by temporal bone CT scan, we diagnosed 20 cases of enlarged vestibular aqueduct (EVA) and/or other inner ear malformation. SLC26A4 mutations account for about 14.93% of the cases. The aminoglycoside-related mtDNA 1555A>G mutation accounted for 0.76% of the cases in Chifeng area. In addition, another 13.43% (18/134) of the cases carried heterozygous GJB2 mutation and their hearing loss may be related to GJB2. 6.72% (9/134) of the cases carried heterozygous SLC26A4 mutation who were not found EVA by temporal bone CT or not took CT examination for some reasons. However, their hearing loss may also be SLC26A4-related. About 2.24% (3/134) of the cases carried mtDNA 12SrRNA 1095 T>C which may also be an aminoglycoside-related mutation and very likely be the cause of hearing loss. GJB3 might participate in the pathomechanism of hearing loss in 1.49% (2/134) of the patients. GJB6 mutation was not detected in this population. The ratio of hearing loss related to genetic factors in the sample drawing population from Chifeng was 60.45% (81 cases). GJB2 is the most common gene and SLC26A4 is the second common gene next to GJB2 that cause deafness in this area.

Usefulness of magnetic resonance imaging and Auditory Steady-State Response in the cochlear implant decision in a prelingually deaf child with a narrow internal auditory canal

difference in response to auditory stimulation between the normal group and profound SNHL patients. For the normal-hearing group, the primary auditory cortex, which is contralateral to the normal ear, shows strong activation and the activation of the secondary auditory cortex was seldom observed. However, in the case of profound SNHL patients, the broad activation of the secondary auditory cortex, rather than primary cortex, was observed. Our results therefore seem to suggest that the neural deficits, which were found mostly in SNHL patients, cause reorganization in the auditory pathway and resulted in the broad activation of the secondary auditory cortex, which is adjacent to the primary cortex. In conclusion, our results suggest that DTI and fMRI provide very useful functional information about the auditory pathway and auditory cortex, which will make a significant contribution to the evaluation of profound hearing loss patients.

An Acoustically Evoked Short Latency Negative Response in Profound Hearing Loss Patients

In our auditory brainstem response (ABR) tests, a peculiar V-shaped negative wave at around 3-4 ms latency was observed. At present, this acoustically evoked short latency negative response (ASNR) is poorly understood. In order to study its appearance and origin, the ABR data of 3104 tests during 1980-98 were reviewed. The ASNR was found only in profound hearing loss ears under intense stimuli (80-120 dB nHL). Out of the total 653 profound hearing loss patients (981 ears), the click-evoked ASNRs were present in 80 patients (12.3%), 117 ears (11.9%). The age range was from 8 months to 70 years. Demographic outcome revealed significant higher appearance rates in young subjects especially in the 20-30 years group. The ASNR was excluded from an artifact by its reproducibility over time, equipment and institutes. Moreover, it became absent after external auditory canal occlusion, which simply blocked the air conduction without any influence upon scalp potentials or equipment. It had neural response characteristics that the latency and amplitude shortened and increased respectively in response to the increase of stimulus intensity. Because the peculiar V-shaped waveform obviously differs from ABR, the ASNR was not interpreted as a potential generated from the conventional auditory pathway. On the other hand, the ASNR individuals were of good vestibular function in sharp contrast with their poor hearing. This suggests the probable relation between the ASNR and the vestibular system. The saccule and vestibular nucleus are hypothesized to be the sense organ and the origin of the response respectively.