Long-term analysis of ABI auditory performance in patients with neurofibromatosis type 2-related schwannomatosis.

PURPOSE: This retrospective monocentric study aimed to evaluate long-term auditory brainstem implant (ABI) function in patients with neurofibromatosis type 2, and to investigate the prognostic factors for ABI use. METHODS: Between 1997 and 2022, 27 patients with at least five years of follow-up underwent implantation with 32 ABIs. At 1- and 5-years post-implantation and at last follow-up, ABIs were classified as used or non-used and the size of the ipsilateral tumor was recorded. For patients who used their ABIs, we assessed speech perception (disyllabic words, MBAA sentences) in quiet conditions with the ABI only, by lip-reading (LR), and with a combination of the two (ABI + LR). Hearing improvement was calculated as Δ ABI = (ABI + LR)-LR scores. Predictive factors for ABI use were analyzed. RESULTS: One year post-implantation, 74% patients were ABI-users and 66% of the ABIs were used. Two of these patients were non-users at five years, and another two at last follow-up (14 ± 5.2 years); 54% of the patients were ABI-users at last follow-up. Δ ABI revealed a hearing improvement of 32-41% (disyllabic words) and 28-37% (MBAA sentences). Among 16 ABIs with at least LR improvement at 1-year post-implantation, 4 decreased their performance, coinciding with a large growing ipsilateral tumor in 3/4 ABIs. We identified no significant prognostic factors for ABI use. CONCLUSIONS: ABIs are indicated in case of bilateral deafness with a non-functional cochlear nerve. Half the patients with ABIs used their implants and auditory performance remained stable over time, except in cases of ipsilateral tumor growth.

Evaluation of the Brain Stem Auditory Evoked Potential Response among New Borns Post-Recovery after Hyper-Bilirubinemia: An Original Research.

Introduction: Neurological harm from neonatal hyperbilirubinemia includes loss of hearing and encephalopathy. The current research used the "Brainstem Evoked Response Audiometry (BERA)" test to screen for as well as assess hearing loss in newborns who had recovered from hyperbilirubinemia. Materials and Procedures: A cross-sectional comparative investigation was conducted at a tertiary care center. Fifty neonates were included out of which 25 were healthy and 25 received treatment for the increased bilirubin. Prior to BERA testing, the subjects' ears were examined for any obstruction. Following a conventional lab procedure described, the BERA recordings were carried out after the neonate fell asleep on its own. The data collected were compared for the significance using the ANOVA, keeping P < 0.05 as significant. Results: In comparison to the controls, a large proportion of neonates in cases had BERA wave latencies that were delayed (I-R = 80, L = 84; III- R = 76, L = 84; V- R = 84, L = 88 percentages latencies). The percentage of subjects in whom the latencies was noted for the healthy neonates was lesser than the case group (I-R = 8, L = 24; III- R = 8, L = 8; V- R = 4, L = 12 percentages latencies). Comparable numbers of infants in each group had inter-peak latencies that were lengthy. Subjects in the case group showed that the threshold hearing as per the WHO grade was mild (R = 32, L = 36) and moderate (R = 32, L = 28). Conclusion: Elevated serum bilirubin may cause damage to hearing capability. After hyperbilirubinemia has been completely treated, BERA can detect even the slightest degree of hearing damage. BERA is therefore a useful technique for the quick recognition of hearing impairment in newborns. Early treatment helps in the prognosis so that the neurosensory systems can fully mature, and the patient can lead a quality life.

Protocol for Evaluation and Management of Hearing Loss After Meningitis.

Bacterial meningitis is the most common cause of post-natal acquired hearing loss in children. Although cochlear implantation helps in improving the hearing in these patients, the fibrosis and ossification of the cochlear lumen that occurs as a result of bacterial meningitis, limits the chances of successful implantation. In developing countries like India, the reduced awareness, limited resources, and financial constraints warrant judicial use of radiological and audiological tests to increase the rate of successful cochlear implantation. The present paper is a review of the literature and a proposed protocol for follow-up of post-meningitis patients to help clinicians diagnose and hence, intervene early when profound hearing loss occurs. Every patient who has had an episode of bacterial meningitis must be followed up for atleast 2 years for possible hearing loss with frequent audiological and radiological evaluation, as required. Cochlear implantation must be done as early as possible when profound hearing loss is detected.

Can Auditory and Vestibular Findings Differentiate Vestibular Migraine and Meniere's Disease?

BACKGROUND AND OBJECTIVES: Besides evaluating the auditory and vestibular systems of patients with vestibular migraine (VM) and Meniere's disease (MD), this study aimed to examine the clinical overlaps between these two conditions by detailed evaluation of the patient's symptoms. Subjects and. METHODS: The ears of the patients with VM and MD were evaluated and patients' vestibular and auditory complaints were questioned particularly. Pure tone audiometry, vestibular evoked myogenic potential (VEMP) responses, and caloric test results were evaluated for objective measurements. RESULTS: The VM group had better air-conduction and boneconduction threshold and speech reception threshold and speech discrimination score test values (p<0.05). Regarding the interaural N1-P1 asymmetry ratio, the cervical VEMP between the groups had significant differences (p=0.019). The MD group had more unilateral tinnitus and ear fullness complaints and canal paresis results (p<0.01). The VM group had more motion sickness complaints (p<0.01). CONCLUSIONS: If only ears with hearing loss are evaluated; there was no significant difference between VM and MD, but regardless of hearing level or only the patients with normal hearing were evaluated, the VM group had better hearing levels. It should be considered that patients with VM may have VM-independent hearing loss, and patient complaints should be sufficiently detailed to make an accurate distinction from MD.

Intracochlear signal in FIESTA-C and hearings of patients with cerebellopontine angle schwannoma.

BACKGROUND: Hearing loss in patients with cerebellopontine angle (CPA) schwannoma, is thought to be caused by the damage to the cochlea and the cochlear nerve. AIM: This study aimed to examine the relationships between the intracochlear signal in magnetic resonance imaging (MRI) and hearing in patients with CPA schwannoma. MATERIAL AND METHOD: In 79 patients with CPA schwannoma, we retrospectively examined the signal in the cochlea on the affected side was compared with that on the unaffected side to determine signal degradation in fast imaging reagents steady-state acquisition with cycle phases (FIESTA-C) MRI. For hearing evaluation, pure tone audiometry (PTA), speech audiometry, distortion product otoacoustic emissions (DPOAE), and auditory brainstem response (ABR) were used. For each parameter, we examined the differences between the groups with and without signal degradation. RESULTS: In the hearing test results, the I-wave latency of ABR was significantly longer in the group with signal degradation in FIESTA-C (1.84 ± 0.35 msec vs. 2.04 ± 0.37 msec, p = 0.048). There was no statistically significant difference in other tests. CONCLUSION: The MRI signal changes in the cochlear were related to the I-wave latency of ABR and reflected cochlear function. SIGNIFICANCE: We suggested the cochlear signal changes in CPA schwannoma patients related the hearing.

Is cortical automatic threshold estimation a feasible alternative for hearing threshold estimation with adults with dementia living in aged care?

Objective: This study explored the feasibility of cortical automatic threshold estimation (CATE), a fully automated late auditory evoked potential (AEP) test, as an alternative to pure-tone audiometry for hearing threshold estimation for adults with dementia living in aged care.Design: A single group cross-sectional study was conducted. Participants' dementia severity was determined through the Clinical Dementia Rating scale. Hearing thresholds were obtained for four audiometric frequencies in at least one ear by using both pure-tone audiometry and CATE.Study sample: Sixteen participants enrolled in the study, of which 14 completed at least one of the hearing tests. Twelve ears, from six participants, were included in the final correlation analysis.Results: Pearson correlation coefficients were significant between CATE and pure-tone audiometry for all frequencies: r2 = 0.52 (p = 0.008) for 500 Hz, r2 = 0.79 (p = 0.0001) for 1000 Hz, r2 = 0.71 (p = 0.0005) for 2000 Hz, and r2 = 0.92 (p < 0.0001) for 4000 Hz. Cortical thresholds were within 10 dB of behavioural thresholds for all four frequencies.Conclusions: Findings are encouraging for the feasibility of CATE as an alternative diagnostic test to pure-tone audiometry for adults living with dementia in aged care.

Prevalence of Hearing Loss among School-Age Children in the North of Iran.

INTRODUCTION: The present study aimed to investigate the audiological profiles of elementary school-age children in Rasht, Iran, and estimate the prevalence of hearing impairments in this population. MATERIALS AND METHODS: In this cross-sectional descriptive-analytical study, the hearing threshold was screened using pure tone audiometry (PTA). Hearing impairment was defined as equal to or higher than 20 dB HL. Results of the hearing thresholds were separately reported in the left or right ears and better or worse ears. Logistic regression tests were used to investigate the association between hearing loss and possible risk factors. In this study, all the analyses were conducted using SPSS software (version 21). RESULTS: The present study was carried out on a total of 2019 children. Mean age of the participants was reported as 9.66±1.66 years. Based on low-frequency pure-tone average, the prevalence rates of hearing loss > 15 dB in the right and left ears were reported as 1.94% and 1.68%, respectively. The high-frequency hearing loss > 15 dB in the right and left ears was obtained at 1.14% and 1.04%, respectively. Prevalence rate of hearing loss (in all frequencies) in boys was higher than that in girls. There was a strong association between a history of otitis media and sensorineural or conductive hearing loss (adjusted odds ratio reported as 12.2 and 8.1, respectively). CONCLUSION: In this study, the rate of hearing loss in the participants was approximately 2%. It was concluded that the screening of hearing loss in children is necessary for the identification and management of these children as early as possible. It is recommended to perform further trials to investigate the impact of different causes on childhood hearing impairment.

Frequency-specific auditory brainstem response testing with age-appropriate sedation.

OBJECTIVE: Auditory brainstem response (ABR) testing is the gold-standard procedure for hearing evaluation in pediatric patients who cannot complete a behavioral hearing test. The amount of audiological information obtained depends on the quality of the patient's sleep during the test. In this retrospective database review, we aimed to assess the amount and the characteristics of the audiological information obtained in ABR testing in pediatric patients with age-appropriate sedation. METHODS: A retrospective chart review was conducted on 501 consecutive ABR sedation sessions performed between January 2014 and June 2016 at the Tel Aviv Medical Center. Oral triclofos was used for the sedation of younger patients (3-24 months) and intravenous propofol for older patients (>24 months). The dataset included 370 triclofos sessions (in 337 patients) and 131 propofol sessions (in 126 patients). RESULTS: None of the children developed complications, and all were discharged on the same day of the evaluation. Among the hearing-impaired children, a mean of 10 (1.8 SD) ABR threshold measurements was obtained from propofol-sedated patients and 9.4 (2.8 SD) measurements from those sedated with triclofos (P = 0.039). The major characteristics of the hearing loss, including its degree, type, and configuration, were obtained from all propofol-sedated patients and from 95% of those sedated with triclofos. CONCLUSIONS: A comprehensive evaluation of hearing status can be obtained in ABR testing with age-appropriate sedation. An average number of ∼10 threshold measurements were obtained during ABR testing with age-appropriate sedation, thus allowing for the evaluation of the degree, type and configuration of the hearing loss.

Common Misconceptions Regarding Pediatric Auditory Processing Disorder.

Pediatric hearing evaluation based on pure tone audiometry does not always reflect how a child hears in everyday life. This practice is inappropriate when evaluating the difficulties children experiencing auditory processing disorder (APD) in school or on the playground. Despite the marked increase in research on pediatric APD, there remains limited access to proper evaluation worldwide. This perspective article presents five common misconceptions of APD that contribute to inappropriate or limited management in children experiencing these deficits. The misconceptions discussed are (1) the disorder cannot be diagnosed due to the lack of a gold standard diagnostic test; (2) making generalizations based on profiles of children suspected of APD and not diagnosed with the disorder; (3) it is best to discard an APD diagnosis when another disorder is present; (4) arguing that the known link between auditory perception and higher cognition function precludes the validity of APD as a clinical entity; and (5) APD is not a clinical entity. These five misconceptions are described and rebutted using published data as well as critical thinking on current available knowledge on APD.