Hearing Features and Cochlear Implantation Outcomes in Patients With Pathogenic MYO15A Variants: a Multicenter Observational Study.

OBJECTIVES: Recessive variants in the MYO15A gene constitute an important cause of sensorineural hearing impairment (SNHI). However, the clinical features of MYO15A-related SNHI have not been systemically investigated. This study aimed to delineate the hearing features and outcomes in patients with pathogenic MYO15A variants. DESIGN: This study recruited 40 patients with biallelic MYO15A variants from 31 unrelated families. The patients were grouped based on the presence of N-terminal domain variants (N variants). The longitudinal audiological data and for those undergoing cochlear implantation, the auditory and speech performance with cochlear implants, were ascertained and compared between patients with different genotypes. RESULTS: At the first audiometric examination, 32 patients (80.0%) presented with severe to profound SNHI. Patients with at least one allele of the N variant exhibited significantly better hearing levels than those with biallelic non-N variants (78.2 ± 23.9 dBHL and 94.7 ± 22.8 dBHL, respectively) (p = 0.033). Progressive SNHI was observed in 82.4% of patients with non-profound SNHI, in whom the average progression rate of hearing loss was 6.3 ± 4.8 dBHL/year irrespective of the genotypes. Most of the 25 patients who underwent cochlear implantation exhibited favorable auditory and speech performances post-implantation. CONCLUSIONS: The hearing features of patients with biallelic pathogenic MYO15A variants are characterized by severe to profound SNHI, rapid hearing progression, and favorable outcomes with cochlear implants. Periodic auditory monitoring is warranted for these patients to enable early intervention.

Deep Learning-Based Noise Reduction Approach to Improve Speech Intelligibility for Cochlear Implant Recipients.

OBJECTIVE: We investigate the clinical effectiveness of a novel deep learning-based noise reduction (NR) approach under noisy conditions with challenging noise types at low signal to noise ratio (SNR) levels for Mandarin-speaking cochlear implant (CI) recipients. DESIGN: The deep learning-based NR approach used in this study consists of two modules: noise classifier (NC) and deep denoising autoencoder (DDAE), thus termed (NC + DDAE). In a series of comprehensive experiments, we conduct qualitative and quantitative analyses on the NC module and the overall NC + DDAE approach. Moreover, we evaluate the speech recognition performance of the NC + DDAE NR and classical single-microphone NR approaches for Mandarin-speaking CI recipients under different noisy conditions. The testing set contains Mandarin sentences corrupted by two types of maskers, two-talker babble noise, and a construction jackhammer noise, at 0 and 5 dB SNR levels. Two conventional NR techniques and the proposed deep learning-based approach are used to process the noisy utterances. We qualitatively compare the NR approaches by the amplitude envelope and spectrogram plots of the processed utterances. Quantitative objective measures include (1) normalized covariance measure to test the intelligibility of the utterances processed by each of the NR approaches; and (2) speech recognition tests conducted by nine Mandarin-speaking CI recipients. These nine CI recipients use their own clinical speech processors during testing. RESULTS: The experimental results of objective evaluation and listening test indicate that under challenging listening conditions, the proposed NC + DDAE NR approach yields higher intelligibility scores than the two compared classical NR techniques, under both matched and mismatched training-testing conditions. CONCLUSIONS: When compared to the two well-known conventional NR techniques under challenging listening condition, the proposed NC + DDAE NR approach has superior noise suppression capabilities and gives less distortion for the key speech envelope information, thus, improving speech recognition more effectively for Mandarin CI recipients. The results suggest that the proposed deep learning-based NR approach can potentially be integrated into existing CI signal processors to overcome the degradation of speech perception caused by noise.

Extractions of steady-state auditory evoked fields in normal subjects and tinnitus patients using complementary ensemble empirical mode decomposition.

BACKGROUND: Auditory steady-state response (ASSR) induced by repetitive auditory stimulus is commonly used for audiometric testing. ASSR can be measured using electro-encephalography (EEG) and magnetoencephalography (MEG), referred to as steady-state auditory evoked potential (SSAEP) and steady-state auditory evoked field (SSAEF), respectively. However, the signal level of SSAEP and SSAEF are weak so that signal processing technique is required to increase its signal-to-noise ratio. In this study, a complementary ensemble empirical mode decomposition (CEEMD)-based approach is proposed in MEG study and the extraction of SSAEF has been demonstrated in normal subjects and tinnitus patients. METHODS: The CEEMD utilizes noise assisted data analysis (NADA) approach by adding positive and negative noise to decompose MEG signals into complementary intrinsic mode functions (IMF). Ten subjects (five normal and five tinnitus patients) were studied. The auditory stimulus was designed as 1 kHz carrier frequency with 37 Hz modulation frequency. Two channels in the vicinities of right and left temporal areas were chosen as channel-of-interests (COI) and decomposed into IMFs. The spatial distribution of each IMF was correlated with a pair of left- and right-hemisphere spatial templates, designed from each subject's N100m responses in pure-tone auditory stimulation. IMFs with spatial distributions highly correlated with spatial templates were identified using K-means and those SSAEF-related IMFs were used to reconstruct noise-suppressed SSAEFs. RESULTS: The current strengths estimated from CEEMD processed SSAEF showed neural activities greater or comparable to those processed by conventional filtering method. Both the normal and tinnitus groups showed the phenomenon of right-hemisphere dominance. The mean current strengths of auditory-induced neural activities in tinnitus group were larger than the normal group. CONCLUSIONS: The present study proposes an effective method for SSAEF extraction. The enhanced SSAEF in tinnitus group echoes the decreased inhibition in tinnitus's central auditory structures as reported in previous studies.

Endoscopic Versus Microscopic Type I Tympanoplasty: An Updated Systematic Review and Meta-analysis.

OBJECTIVE: Our objective was to perform a systematic review and meta-analysis comparing the clinical outcomes after endoscopic and microscopic type I tympanoplasty. STUDY DESIGN: Randomized controlled trials, two-arm prospective studies, and retrospective studies were included. SETTING: Medline, Cochrane, EMBASE, and Google Scholar databases were searched until March 1, 2022 using the combinations of search terms: "endoscopic," "microscopic," and "tympanoplasty." METHODS: Two independent reviewers utilized the abovementioned search strategy to identify eligible studies. If any uncertainty existed regarding eligibility, a third reviewer was consulted. Primary outcome measures were graft success rate, air-bone gap (ABG) improvement, and operative time. Secondary outcomes were the rate of need for canalplasty, the proportion of self-rated excellent cosmetic results, and pain visual analog scale (VAS). RESULTS: Forty-three studies enrolled a total of 3712 patients who were undergoing type I tympanoplasty and were finally included. The pooled result showed endoscopic approach was significantly associated with shorter operative time (difference in means: -20.021, 95% confidence interval [CI]: -31.431 to -8.611), less need for canalplasty (odds ratio [OR]: 0.065, 95% CI: 0.026-0.164), more self-rated excellent cosmetic results (OR: 87.323, 95% CI: 26.750-285.063), and lower pain VAS (difference in means: -2.513, 95% CI: -4.737 to -0.228). No significant differences in graft success rate or ABG were observed between the two procedures. CONCLUSION: Endoscopic type I tympanoplasty provides a similar graft success rate, improvement in ABG, and reperforation rate to microscopic tympanoplasty with a shorter operative time, better self-rated cosmetic results, and less pain. Unless contraindicated, the endoscopic approach should be the procedure of choice in type I tympanoplasty.

Neuromagnetic index of hemispheric asymmetry predicting long-term outcome in sudden hearing loss.

The neuromagnetic index of hemispheric asymmetry in terms of ipsilateral/contralateral ratio at acute stage was previously revealed to prognosticate the 1-month hearing outcome of acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL), showing a dynamic relationship between top- and down-levels of auditory pathway. However, the prognostic effect of reorganization pattern for the long-term results remained elusive. This study aimed to probe the prognosticating relevance of hemispheric asymmetry to the hearing at chronic stage of ISSNHL. Using magnetoencephalography (MEG), inter-hemispheric differences in peak dipole of N100m responses to monaural tones were evaluated in 21 controls and 21 ISSNHL patients at initial and final (12 months later) stages. Predictive value of hemispheric asymmetry was assessed by correlating hearing level and ipsilateral/contralateral ratio (I/C) of N100m latency and amplitude. Healthy-side dominance of N100m was observed in ISSNHL initially, and remained in three final prognostic subgroups (complete, partial, and no recovery) of ISSNHL. The initial I/C(amplitude) on affected-ear stimulation strongly correlated with the hearing level of final stage in ISSNHL. However, there was no prognostic effect of hemispheric asymmetry pattern for the 12-month hearing improvement. The heterogeneity between neuromagnetic index and hearing levels possibly echoed different pathogeneses of ISSNHL. Since a restored hearing status did not necessarily lead toward a normal functional organization, the dynamics of hemispheric asymmetry could actually index a central resilient reorganization in the brain for sound processing in ISSNHL. Our finding showed not only a clinically relevant measure to predict final hearing of ISSNHL, but also a linkage between central plasticity and cochlear lesion. This finding suggests a new perspective, and perhaps new interventions, to diagnose and treat unilateral ISSNHL.

Association between exposure to road traffic noise and hearing impairment: a case-control study.

PURPOSE: Noise pollution in urban areas is increasing steadily, and the study of road traffic noises and their effects on the auditory system was rare. This study investigated the potential effects of road traffic noise on auditory systems and hearing. METHODS: A case-control study recruited outpatients from the Otolaryngology department. The case group (n = 41) had binaural hearing loss (HL) of standard pure-tone average(PTA) ≥ 25 dB or high frequency PTA ≥ 25 dB, while the control group (n = 39) had binaural hearing level of any frequency < 25 dB. Detailed otologic evaluations were performed. Between-group data were evaluated using logistic regression analysis. Case or control group was identified based on the audiogram. RESULTS: A total of 80 subjects were recruited, including 41 with hearing impairment and 39 as control. The mean exposure level of road traffic noise was significantly higher in the case group than the control group (p = 0.005). A crude OR of 5.78 showed an increased risk of greater than 70 dB of road traffic noise on hearing impairment and tinnitus (p < 0.001). The aOR of 9.24 (p = 0.002) from a multiple variate analysis suggested that road traffic noise levels greater than 70 dB may have a damaging effect on hearing. Damaging effects on hearing persisted even after adjusting for confounders in the full multivariate model (aOR of 9.24 [95% CI: 2.198-38.869]; p = 0.002). CONCLUSIONS: Exposing to road traffic noise greater than 70 dB showed an increased risk of damage to the auditory system. These results might help public health administrators and physicians to develop programs that address the health dangers of noise.

Screening for 22q11.2 deletion syndrome by two non-invasive prenatal testing methodologies: A case with discordant results.

OBJECTIVE: Non-invasive prenatal testing (NIPT) through the analysis of cell-free DNA in maternal plasma has bee expanded to include clinically-relevant microdeletions such as the 22q11.2 deletion syndrome (22q11.2DS). CASE REPORT: We present a pregnancy where the fetus was affected with 22q11.2DS based on chromosome microarray analysis. Discordant results were obtained through two different NIPT methodologies. The pregnancy was identified as high risk by a SNP-based approach but low risk using a genome-wide counting methodology. A review of the technical methods used for these tests provides insight into why they may provide conflicting results and emphasizes the importance of chromosome microarray studies for diagnostic confirmation and defining the deletion. CONCLUSION: Currently available NIPT for 22q11.2DS use different technologies that are not equivalent. The genome-wide counting methodology has the potential to detect deletions outside the critical 22q11.2 A-D region but current data suggests it may have a lower sensitivity for deletions within the critical region.

Ovarian abscess from Salmonella: a case report.

BACKGROUND: An ovarian site of Salmonella bacteremia is rare. The contents of a teratoma may hide the organisms easily. Clinicians should be alert to this possibility when the patient had a teratoma with Salmonella infection. CASE: A 19-year-old woman presented with a missed menstrual period. An adnexal mass was found during a routine gynecologic examination. The patient had had gastroenteritis 2 months earlier but did not complain of a gastrointestinal problem at presentation. Exploratory laparotomy was performed for a suspected ovarian tumor. An infective teratoma was considered, but the infection source was unknown until the culture report showed a Salmonella infection. CONCLUSION: Salmonella infection is a self-limiting, febrile disease and is unlikely to involve organs other than the gut. A nontyphoid ovarian abscess became a rare late complication of acute gastroenteritis. Clinicians should pay special attention to the differential diagnosis of ovarian tumor in patients with a history of Salmonella infection, especially those with such ovarian lesions as endometrioma or teratoma and with recent abdominal pain, as noted in this case.

Can pillar suturing promote efficacy of adenotonsillectomy for pediatric OSAS? A prospective randomized controlled trial.

OBJECTIVES/HYPOTHESIS: Pediatric obstructive sleep apnea syndrome (OSAS) is a common disorder with serious clinical implications if left untreated. The recommended initial treatment for pediatric patients with obstructive sleep apnea syndrome (OSAS) is adenotonsillectomy. However, recent reports have shown variable surgical results with adenotonsillectomy in the treatment of pediatric OSAS. STUDY DESIGN: Prospective, controlled study. METHODS: From April 2007 to August 2010, 24 participants were assigned alternatively to either adenotonsillectomy with pillar suturing (intervention group) or adenotonsillectomy alone (control group). RESULT: The average improvement in apnea hypopnea index (AHI) was 42.6% in the control group and 79.9% in the intervention group (P=0.037). The success rate was 50% in the control group and 91.6% in the intervention group (P=0.034). Six patients (50%) in the intervention group achieved complete resolution of pediatric OSAS, as defined by an AHI <1/hour, compared to 2 patients (16.7%) in the control group (P=0.097). CONCLUSION: Adenotonsillectomy with pillar suturing showed significant improvement in treating pediatric patients with OSAS compared to adenotonsillectomy alone. The procedure is simple and safe. LEVEL OF EVIDENCE: 4.

Peripheral neuropathology of the upper airway in obstructive sleep apnea syndrome.

Obstructive sleep apnea syndrome (OSAS) is a common sleep disorder that leads to significant morbidity and mortality without adequate treatment. Though much emphasis on the pathogenesis of OSAS has been placed on a narrow upper airway space and associated muscular factors, possible neuropathy of the upper airway has not been fully elucidated. Increasing peer reviewed evidence suggests involvement of neurologic lesions of the upper airway in OSAS patients. In this article, we review the etiology and pathophysiology of OSAS, the evidence and possible mechanisms leading to upper airway neuropathy, and the relationship between upper airway neuropathy and OSAS. Further studies should focus on the long term effects of the upper airway neuropathy as related to the duration and severity of snoring and or apnea, and also on the potential methods of prevention and management of the neuropathy in sleep disordered breathing.

Neuromagnetic index of hemispheric asymmetry prognosticating the outcome of sudden hearing loss.

The longitudinal relationship between central plastic changes and clinical presentations of peripheral hearing impairment remains unknown. Previously, we reported a unique plastic pattern of "healthy-side dominance" in acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL). This study aimed to explore whether such hemispheric asymmetry bears any prognostic relevance to ISSNHL along the disease course. Using magnetoencephalography (MEG), inter-hemispheric differences in peak dipole amplitude and latency of N100m to monaural tones were evaluated in 21 controls and 21 ISSNHL patients at two stages: initial and fixed stage (1 month later). Dynamics/Prognostication of hemispheric asymmetry were assessed by the interplay between hearing level/hearing gain and ipsilateral/contralateral ratio (I/C) of N100m latency and amplitude. Healthy-side dominance of N100m amplitude was observed in ISSNHL initially. The pattern changed with disease process. There is a strong correlation between the hearing level at the fixed stage and initial I/C(amplitude) on affected-ear stimulation in ISSNHL. The optimal cut-off value with the best prognostication effect for the hearing improvement at the fixed stage was an initial I/C(latency) on affected-ear stimulation of 1.34 (between subgroups of complete and partial recovery) and an initial I/C(latency) on healthy-ear stimulation of 0.76 (between subgroups of partial and no recovery), respectively. This study suggested that a dynamic process of central auditory plasticity can be induced by peripheral lesions. The hemispheric asymmetry at the initial stage bears an excellent prognostic potential for the treatment outcomes and hearing level at the fixed stage in ISSNHL. Our study demonstrated that such brain signature of central auditory plasticity in terms of both N100m latency and amplitude at defined time can serve as a prognostication predictor for ISSNHL. Further studies are needed to explore the long-term temporal scenario of auditory hemispheric asymmetry and to get better psychoacoustic correlates of pathological hemispheric asymmetry in ISSNHL.

Perinatal outcomes of polyhydramnios without associated congenital fetal anomalies after the gestational age of 20 weeks.

BACKGROUND: Polyhydramnios carries a high rate of complications during pregnancy and adverse perinatal outcomes. We could find no studies of this condition in a large Asian population. The aim of this investigation was to evaluate the risks of adverse perinatal outcomes in a large study population with polyhydramnios without associated fetal anomalies after the gestational age of 20 weeks in Taiwan. METHODS: We retrospectively reviewed the computerized records of women who had babies without associated fetal anomalies after the gestational age of 20 weeks at Chang Gung Memorial Hospital from July 1990 to December 2001. Possible confounding factors that could affect the occurrence of polyhydramnios were analyzed. We then investigated the relative risks of these events to adverse perinatal outcome by adjusting the variants. RESULTS: Significantly higher incidences of preeclampsia, placental abruption, placenta accreta, past history of fetal death or preterm delivery, multiple pregnancy, bodyweight gain > or = 20kg during pregnancy and primiparity were noted in patients with polyhydramnios than in patients without this condition. The presence of polyhydramnios significantly increased the rate of preterm delivery, low birth weight or very low birth weight, low one- and five-minute Apgar scores, fetal death, large for gestational age babies, meconium-stained amniotic fluid, Cesarean section, fetal distress in labor, NICU transfer and neonatal death. CONCLUSIONS: Polyhydramnios carried a higher incidence of adverse perinatal outcomes, such as low Apgar scores, fetal death, fetal distress in labor, NICU transfer and neonatal death, despite exclusion of congenital anomalies from the study population. Detailed antepartum fetal well-being surveillance, intensive intrapartum monitoring and further attention postpartum are warranted in patients with this condition.

Characteristics of women using a mental health clinic in a gynecologic out-patient setting.

A special mental health clinic was set up in the gynecologic out-patient setting of a medical center. The present study examined the demographic and clinical characteristics of patients seen at the clinic and evaluated the need for mental health services in a gynecologic setting. Participants were 136 consecutive patients who visited the clinic during a 6 month period. Their ages ranged from 20 to 74 years, with a mean age of 41.5 +/- 12.3 years. Twenty-three percent of women were referred by gynecologists. All subjects were interviewed by experienced psychiatrists using the structured Mini-International Neuropsychiatric Interview (MINI), supplemented by the DSM-IV criteria for premenstrual dysphoric disorder (PMDD) and other diagnoses. The most common diagnosis was major depressive disorder (36.0%), followed by generalized anxiety disorder (29.4%), PMDD (16.2%), dysthymic disorder (14.7%) and others. Patients were categorized into four major diagnostic categories: depressive disorders, anxiety disorders, PMDD and others. No significant differences were found in years of education, employment status, living situations or referral pattern among the four major diagnostic groups. Most patients with anxiety disorders were married. Our results suggest that the gynecologic department may be a good setting to help women with mental disorders.

Characteristics of women seeking treatment for premenstrual syndrome in Taiwan.

OBJECTIVE: To analyze the characteristics of 150 women who visited a premenstrual syndrome (PMS) clinic in a Taiwanese medical center staffed by both psychiatrists and gynecologists. METHOD: All subjects were interviewed and assessed for premenstrual dysphoric disorder (PMDD) using DSM-IV criteria, a PMS questionnaire, and the structured Mini-International Neuropsychiatric Interview (MINI). Where PMDD was suspected, subjects were followed using a daily symptom record. RESULTS: A total of 110 subjects (73%) met the ICD-10 criteria for PMS. The most common PMS symptoms were minor psychological discomfort, muscular tension, and aches or pains. For 129 subjects (86%), other concurrent psychiatric disorders were diagnosed using the MINI. Of these, 48 (37%) reported premenstrual exacerbation (PME) of a previously diagnosed psychiatric condition. CONCLUSION: The results of this study indicate that women who complain of PMS may be at a high risk of other psychiatric dysfunction, especially mood disorder. Further, the high proportion of PME cases determined in this study suggests that further investigation is required.