UNLABELLED:Cornelia de Lange syndrome (CdLS) is a rare congenital multisystem syndrome characterized by a distinctive craniofacial appearance, developmental delay, intellectual disability, limb abnormalities, and hypertrichosis. Hearing loss is one of the common manifestations in CdLS. This study presents a 6-year-old child with CdLS, who exhibited developmental delay, intellectual disability, and displayed autistic features, and experienced challenges during right cochlear implant (CI) surgery at the age of 5 due to inner ear abnormality. After the CI, the sound field hearing test showed minimal response levels of 60-70 dB HL at post-CI 12 months. He obtained scores of 2%, 14%, 16%, and 27% on the PEACH rating scale at pre-CI, post-CI 3 months, post-CI 6 months, and post-CI 12 months, respectively. Nevertheless, for CdLS patients with severe-to-profound hearing loss, CI can offer potential improvements in auditory skills and social connections, even though the benefits may be limited. It remains a viable solution.

OBJECTIVE:To determine the hearing outcomes in patients with sudden sensorineural hearing loss (SSNHL) after exposure to different brands of vaccines against COVID-19. STUDY DESIGN:Cohort study. SETTING:Tertiary referral center. PATIENTS:Patients who met the International Classification of Disease, Tenth Revision code, medications, and procedure criteria for SSNHL in 2021. Patients were classified according to their vaccination status. A total of 128 patients (71 males and 57 females; mean age, 53.9 ± 16.5 yr; range, 7.6-82.1 yr) who met the criteria of idiopathic SSNHL were included. INTERVENTION:Exposure to COVID-19 vaccines in 2021. MAIN OUTCOME MEASUREMENTS:Hearing outcomes were classified according to the pure-tone audiometry hearing level, including complete recovery (CR), partial recovery (PR), and no recovery (NR). Time to recovery was defined as the point at which the patient achieved CR or PR. RESULTS:Among them, 35, 54, and 39 patients achieved CR, PR, and NR, respectively. The median time to recovery was 22 (interquartile range, 11-37) days. No significant differences were observed in hearing recovery in vaccinated or unvaccinated patients. CONCLUSION:Our preliminary study failed to show significant differences in hearing recovery among patients with SSNHL regardless of the vaccine exposure status. The administration of COVID-19 vaccines should not be influenced by their potential association with SSNHL, as our findings indicate no significant effect on hearing outcomes. However, as a preliminary study with limited statistical power, future large-scale studies are necessary to validate these results.

Hearing loss is often caused by genetic and environmental factors, with inherited mutations responsible for 50-60% of cases. The GJB2 gene, encoding connexin 26, is a major contributor to nonsyndromic sensorineural hearing loss (NSHL) due to its role in cellular communication critical for auditory function. In Taiwan, common deafness-associated genes include GJB2, SLC26A4, OTOF, MYO15A, and MTRNR1, which were similar to those found in other populations. The most common pathogenic genes is GJB2 mutations and the hearing level in children with GJB2 p.V37I/p.V37I or p.V37I/c.235delC was estimated to deteriorate at approximately 1 decibel hearing level (dB HL)/year. We found another common mutation in Taiwan Biobank, GJB2 p.I203T, which were identified in our data and individuals carrying this mutation experienced more severe hearing loss, suggesting a synergistic effect of these mutations on auditory impairment. We suggest GJB2 whole genetic screening is recommended for clinical management and prevention strategies in Taiwan. This study used data from the Taiwan Biobank to analyze allele frequencies of GJB2 gene variants. Predictive software (PolyPhen-2 version 2.2, SIFT for missense variants 6.2.1, MutationTaster Ensembl 112 and Alphamissense CC BY-NC-SA 4.0) assessed the pathogenicity of specific mutations. Additionally, 82 unrelated NSHL patients were screened for mutations in these genes using PCR and DNA sequencing. The study explored the correlation between genetic mutations and the severity of hearing loss in patients. Several common GJB2 mutation sites were identified from the Taiwan Biobank, including GJB2 p.V37I (7.7%), GJB2 p.I203T (6%), GJB2 p.V27I (31%), and GJB2 p.E114G (22%). Bioinformatics analysis classified GJB2 p.I203T as pathogenic, while GJB2 p.V27I and GJB2 p.E114G were considered polymorphisms. Patients with GJB2 p.I203T mutation experienced more severe hearing loss, emphasizing the potential interaction between the gene in auditory impairment. The mutation patterns of GJB2 in the Taiwanese population are similar to other East Asian regions. Although GJB2 mutations represent the predominant genetic cause of hereditary hearing loss, the corresponding mutant proteins exhibit detectable aggregation, particularly at cell-cell junctions, suggesting at least partial trafficking to the plasma membrane. Genetic screening for these mutations-especially GJB2 p.I203T (6%), GJB2 p.V27I (31%), and GJB2 p.E114G (22%)-is essential for the effective diagnosis and management of non-syndromic hearing loss (NSHL) in Taiwan. We found GJB2 p.I203T which were identified in our data and individuals carrying this mutation experienced more severe hearing loss, suggesting a synergistic effect of these mutations on auditory impairment. We suggest whole GJB2 gene sequencing in genetic screening is recommended for clinical management and prevention strategies in Taiwan. These findings have significant clinical and public health implications for the development of preventive and therapeutic strategies.

Background: Hearing loss is an important public health concern worldwide, especially because of the global trend of aging populations. Therefore, simple, effective, and efficient methods of hearing screening within communities are needed.Purpose: To develop and validate the effectiveness of a novel three-step hand rub hearing screening (3SHRHS) method to efficiently detect and categorize hearing loss severity.Research Design: This was a prospective study.Study Sample: The recruited participants were 470 adults (940 ears).Data Collection and Analysis: The 3SHRHS test involved the following three sequential steps: palm rub at 2 cm; finger rub at 2 cm; and finger rub at 70 cm. Pure-tone audiometry served as the reference standard for hearing loss classification.Results: The 3SHRHS method demonstrated high sensitivity and specificity for detecting different levels of hearing loss severity. In both quiet and noisy environments, step 1 demonstrated strong evidence of detecting levels more than 55 dB hearing level (HL), whereas step 2 showed moderate evidence of detecting levels exceeding 40 dB HL. However, step 3 exhibited weak evidence of detecting levels greater than 25 dB HL, specifically in quiet environments; therefore, it was considered unreliable for such detections in noisy environments.Conclusion: The 3SHRHS is a rapid and accessible method of detecting and categorizing hearing loss severity. This approach may improve the accessibility of hearing screenings and facilitate early interventions for individuals with hearing loss.

Background: Dominant deafness-onychodystrophy (DDOD) syndrome is a rare genetic disorder characterizedby sensorineural hearing loss and the absence or hypoplasia of nails, associated withdefects in the ATP6V1B2 gene. This gene defect significantly affects hearing function, leading to congenitalsevere-to-profound hearing loss. Purpose: We present the comprehensive cochlear implant (CI) outcome of a 5-year-old child withDDOD syndrome who received bilateral sequential CIs at the ages of 1 and 4 years. Research Design: Case study. Data Collection and Analysis: Retrospective chart review of aural-communication and languageperformance. Results: After the follow-up following the first CI, there was improvement in auditory, language, andcognitive abilities. At 41 months after the first CI, the child received the second CI. Although his languageability still lagged behind, his auditory and communication performance continued to improveafter bilateral CI surgery. He obtained 95 percent on the Parents’ Evaluation of Aural/Oral Performanceof Children scale at the last follow-up. Conclusions: Children with DDOD syndrome (ATP6V1B2 c.1516C > T) receiving bilateral CIs canenhance aural and communication skills.

Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high index of suspicion. Secondary AIED, which accompanies other autoimmune diseases, occurs in up to 30% of AIED cases. Secondary AIED is a rare manifestation of paraneoplastic syndrome, and the causal malignancy may be occult initially. Detecting and treating associated diseases, along with the use of immunosuppressants, are the mainstay for the management of AIED. Herein, we presented a woman with bilateral fluctuating and progressive SNHL, partially responding to steroid or immunosuppressant treatment. Her hearing eventually stabilized after completing the treatment for diffuse large B cell lymphoma, which was diagnosed 6 months after the onset of hearing loss. This case serves as a reminder to physicians to be aware of AIED in cases of fluctuating SNHL and the possibility of associated malignancy alongside autoimmune disturbance. Laryngoscope, 135:1496-1498, 2025.

BACKGROUND:With advances in cochlear implant (CI) technology, prelingual deaf adults may experience improved speech perception and quality of life (QoL). It is still a challenge for Mandarin-speaking CI user with tone recognition due to CI technology focused on intonation language.OBJECTIVES:To evaluate the long-term post-CI auditory performance and social-emotional benefits in prelingual deaf Mandarin-speaking adults and the difference between them and post-lingual deaf adults.MATERIAL AND METHODS:Fifty-five adult implanted ears were included (forty-six postlingual deaf group; nine prelingual deaf group). Post-CI long-term outcomes were using vowels, consonants, disyllabic words, Mandarin monosyllable words, categories of audiology performance, speech intelligibility rating, subjective social-emotional questionnaires.RESULTS:Post-CI auditory performance and speech intelligibility of prelingual deafness adults was significantly inferior to that of those with postlingual deafness. However, both groups presented improved social-emotional benefits, with no significant difference between both groups.CONCLUSIONS:Adult CI recipients who deaf before the age of 4 can experience benefits in social-emotional life functioning, regardless of their limited auditory performance and speech intelligibility. Therefore, prelingual Mandarin-speaking deaf adults, especially those using oral communication, can be considered as relative indications for cochlear implantation.SIGNIFICANCE:To clarify and validate the benefits among Mandarin-speaking prelingual deaf adult recipients.

PurposeTo evaluate monosyllable word recognition in noise under different conditions in bilateral sequential cochlear implants (CIs). Second, to assess subjective hearing benefits among bilateral sequential CIs, bimodal hearing, and CI-only groups. Lastly, to analyze the prognostic factors affecting CI outcomes in children with bilateral sequential CIs.MethodsSixty-five children with CI were enrolled. Mandarin monosyllable recognition tests in noise and the Speech, Spatial, and Qualities of Hearing Scale (SSQ) were used to assess post-CI outcomes. The SSQ benefits were compared among children with bilateral CIs, bimodal hearing, and CI-only.ResultsBilateral CIs significantly outperformed the first or second CI alone using in noise. The bilateral CI group had significantly better SSQ scores for speech, qualities, and total scores compared to the CI-only group. Additionally, 41% of the variance in speech perception of the second CI can be attributed to the inter-implantation interval between bilateral CIs.ConclusionBilateral sequential CIs can enhance speech perception in noise and daily life-functioning for children. Earlier implantation of bilateral sequential CIs results in better outcomes, while inter-implantation interval exceeding 9.6 years between bilateral CIs may lead to poor second CI performance in noise. Therefore, early bilateral sequential CIs should be encouraged.

Objective To evaluate the effects of related factors on long-term speech perception and quality-of-life (QoL) outcomes in postlingual deaf Mandarin-speaking adult cochlear implant (CI) recipients and to assess any differences between tone language and other intonation language CI adult users. Study Design Retrospective cases review Setting Tertiary referral center Patients Forty-five adult CI recipients (48 implanted ears). Main Outcome Measures Post-CI outcomes were evaluated after follow-ups of more than 24 months using speech perception tests and QoL questionnaires. We analyzed the related factors affecting CI outcomes. Results A shorter duration of profound deafness was predictive factors for better post-CI speech perception. Earlier implantation and better performance of vowel scores were predictive of better subjective improvements in social and emotional life. Post-CI vowel scores of over 56% indicated promising improvements in QoL. Conclusion Shorter duration of deafness could have better post-CI speech perception. Post-CI vowel scores instead of monosyllable words have the more potential to predict the QoL in Mandarin-speaking adult CI recipients.

Background Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a maternally inherited mitochondrial disease that affects various systems in the body, particularly the brain, nervous system, and muscles. Among these systems, sensorineural hearing loss is a common additional symptom. Methods A 42-year-old female patient with MELAS who experienced bilateral profound deafness and underwent bilateral sequential cochlear implantation (CIs). Speech recognition and subjective outcomes were evaluated. Results Following the first CI follow-up, the patient exhibited improved speech recognition ability and decided to undergo the implantation of the second ear just two months after the initial CI surgery. The second CI also demonstrated enhanced speech recognition ability. Subjective outcomes were satisfactory for bilateral CIs. Conclusions MELAS patients receiving bilateral CIs can attain satisfactory post-CI speech recognition, spatial hearing, and sound qualities.

This study aimed to investigate the role of hearing aid (HA) usage in language outcomes among preschool children aged 3–5 years with mild bilateral hearing loss (MBHL). The data were retrieved from a total of 52 children with MBHL and 30 children with normal hearing (NH). The association between demographical, audiological factors and language outcomes was examined. Analyses of variance were conducted to compare the language abilities of HA users, non-HA users, and their NH peers. Furthermore, regression analyses were performed to identify significant predictors of language outcomes. Aided better ear pure-tone average (BEPTA) was significantly correlated with language comprehension scores. Among children with MBHL, those who used HA outperformed the ones who did not use HA across all linguistic domains. The language skills of children with MBHL were comparable to those of their peers with NH. The degree of improvement in audibility in terms of aided BEPTA was a significant predictor of language comprehension. It is noteworthy that 50% of the parents expressed reluctance regarding HA use for their children with MBHL. The findings highlight the positive impact of HA usage on language development in this population. Professionals may therefore consider HAs as a viable treatment option for children with MBHL, especially when there is a potential risk of language delay due to hearing loss. It was observed that 25% of the children with MBHL had late-onset hearing loss. Consequently, the implementation of preschool screening or a listening performance checklist is recommended to facilitate early detection.

Purpose: Although previous studies have shown the efficacy of cochlear implants (CIs) in older adults, no study written in English has focused on Mandarin-speaking older recipients. Mandarin is a tonal language, it is hard to lip-read and tone recognition for CI users. This study aimed to evaluate the long-term post-CI outcomes in Mandarin-speaking older adults and the difference between them and younger recipients.Materials and methods: Forty-six post-lingually deafened adults were included. Speech perception tests (vowel, consonant, disyllable words, Mandarin monosyllable recognition test, and categories of audiology performance were evaluated) and psychosocial scale were evaluated.Results: There were no significant differences between older and younger recipients in post-CI open-set speech perception. However, older recipients had significantly lower social and total scores in the subjective questionnaire than younger recipients. In both duration of deafness less than seven years and hearing years in life over 92.6 %, older recipients had no less capable speech perception than in younger.Conclusion: Mandarin-speaking older recipients can improve not only speech perception but also psychosocial benefits. Well hearing experience may confer an advantage to older recipients, despite their older implanted age. These results can help provide pre-CI consultation guidelines for older Mandarin-speaking recipients.

OBJECTIVE:This investigation was to ascertain the performance of the UNHS in Taiwan.METHODS:The predefined questionnaire was delivered on the phone in 2016. The descriptive analysis was applied to the research data. 941 neonates in birth cohorts 2013-2014 who were documented as a bilateral referral in the national UNHS tracking system were targeted. The respondents were either caregivers or family members.RESULTS:40.3% of 941 children were lost to follow-up, and 66.24% of 363 children were diagnosed with SNHL. 45.15% of 163 children used hearing amplification device(s). 77.46% of hearing amplification device users and 7.51% of non-users participated in the auditory training courses. By six months of age, 38.51% and 22.58% of children diagnosed with bilateral SNHL commenced the hearing amplification device fitting and the auditory training courses, respectively.CONCLUSIONS:More efforts are needed to enhance the performance of the UNHS to achieve national goals stated in the 2014 Taiwan UNHS Revised Guidelines and the well-known benchmarks set by the JCIH in 2007. The development of an electronic tracking system for storing and sharing information on the follow-up on children with congenital hearing loss was as significant as the improvements in the understanding of early hearing detection and intervention of the public and stakeholders.

Mesenchymal stem cells (MSCs) can be isolated from different tissue origins, such as the bone marrow, the placenta, the umbilical cord, adipose tissues, and skin tissues. MSCs can secrete anti-inflammatory molecules and growth factors for tissue repair and remodeling. However, the ability of skin-derived MSCs (SMSCs) to repair cochlear damage and ameliorate hearing loss remains unclear. Cisplatin is a commonly used chemotherapeutic agent that has the side effect of ototoxicity due to inflammation and oxidative stress. This study investigated the effects of SMSCs on cisplatin-induced hearing loss in mice. Two independent experiments were designed for modeling cisplatin-induced hearing loss in mice, one for chronic toxicity (4 mg/kg intraperitoneal [IP] injection once per day for 5 consecutive days) and the other for acute toxicity (25 mg/kg IP injection once on day one). Three days after cisplatin injection, 1 × 106 or 3 × 106 SMSCs were injected through the tail vein. Data on auditory brain responses suggested that SMSCs could significantly reduce the hearing threshold of cisplatin-injected mice. Furthermore, immunohistochemical staining data suggested that SMSCs could significantly ameliorate the loss of cochlear hair cells, TUNEL-positive cells and cleaved caspase 3-positive cells in cisplatin-injected mice. Neuropathological gene analyses revealed that SMSCs treatment could downregulate the expression of cochlear genes involved in apoptosis, autophagy, chromatin modification, disease association, matrix remodeling, oxidative stress, tissue integrity, transcription, and splicing and unfolded protein responses. Additionally, SMSCs treatment could upregulate the expression of cochlear genes affecting the axon and dendrite structures, cytokines, trophic factors, the neuronal skeleton and those involved in carbohydrate metabolism, growth factor signaling, myelination, neural connectivity, neural transmitter release, neural transmitter response and reuptake, neural transmitter synthesis and storage, and vesicle trafficking. Results from TUNEL and caspase 3 staining further confirmed that cisplatin-induced apoptosis in cochlear tissues of cisplatin-injected mice could be reduced by SMSCs treatment. In conclusion, the evidence of the effects of SMSCs in favor of ameliorating ototoxicity-induced hearing loss suggests a potential clinical application.

Objectives: Recessive variants in theMYO15Agene constitute an important cause of sensorineural hearing impairment (SNHI). However, the clinical features ofMYO15A-related SNHI have not been systemically investigated. This study aimed to delineate the hearing features and outcomes in patients with pathogenicMYO15Avariants. Design: This study recruited 40 patients with biallelicMYO15Avariants 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 pathogenicMYO15Avariants 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.

Hearing loss, the most common sensory abnormality, is caused by the death of or damage to inner ear hair cells. Genetic mutations are the main cause of hearing loss. We used nex-generation sequencing data released by the Taiwan Biobank to investigate the GJB2 mutation spectrum in 1517 patients. We compared hearing function in Taiwanese patients with nonsyndromic hearing loss (NSHL) caused by the two most common GJB2 mutations c.109G>A (p.V37I) and c.235delC. We extracted DNA from the oral mucosa of patients with NSHL and performed Sanger sequencing to confirm the genotype. Of 240 patients with NSHL, we identified 25 with GJB2 c.109G>A and 9 with GJB2 c.235delC mutations, after excluding patients aged >10 years, in whom hearing loss may have been caused due to age-related degeneration. We investigated genotype–phenotype correlations in patients harboring GJB2 c.109G>A and c.235delC mutations. Furthermore, we described the GJB2 mutation spectrum in the Taiwanese population and identified the role of homozygous and heterozygous GJB2 mutations associated with hearing phenotypes in patients with NSHL. Thus, our study provides insights into the complexity of GJB2 genetics. Our data indicate that GJB2 c.109G>A heterozygotes had poorer hearing than did homozygotes. The mechanism underlying the more severe phenotype in heterozygotes and whether the phenotype is caused by GJB2 heterozygotes or compound heterozygotes warrant future investigation.

Cochlear implantation is a reliable and safe tool to rehabilitate patients with severe-to-profound sensorineural hearing loss, who get limited benefits from traditional hearing aids. Electrode misplacement is a rare but significant problem that fails to provide benefits and may cause injury to the nearby structures. We present a case that underwent mastoidectomy first to remove cholesteatoma, followed by cochlear implantation with electrode misplacement into the petrous area, with sustained hearing benefits and without any injury to the surrounding structures.

Three cochlear implant (CI) sound coding strategies were combined in the same signal processing path and compared for speech intelligibility with vocoded Mandarin sentences. The three CI coding strategies, biologically-inspired hearing aid algorithm (BioAid), envelope enhancement (EE), and fundamental frequency modulation (F0mod), were combined with the advanced combination encoder (ACE) strategy. Hence, four singular coding strategies and four combinational coding strategies were derived. Mandarin sentences with speech-shape noise were processed using these coding strategies. Speech understanding of vocoded Mandarin sentences was evaluated using short-time objective intelligibility (STOI) and subjective sentence recognition tests with normal-hearing listeners. For signal-to-noise ratios at 5 dB or above, the EE strategy had slightly higher average scores in both STOI and listening tests compared to ACE. The addition of EE to BioAid slightly increased the mean scores for BioAid+EE, which was the combination strategy with the highest scores in both objective and subjective speech intelligibility. The benefits of BioAid, F0mod, and the four combinational coding strategies were not observed in CI simulation. The findings of this study may be useful for the future design of coding strategies and related studies with Mandarin.

Umbilical cord-derived mesenchymal stromal cells (UCMSCs) have potential applications in regenerative medicine. UCMSCs have been demonstrated to repair tissue damage in many inflammatory and degenerative diseases. We have previously shown that UCMSC exosomes reduce nerve injury-induced pain in rats. In this study, we characterized UCMSC exosomes using RNA sequencing and proteomic analyses and investigated their protective effects on cisplatin-induced hearing loss in mice. Two independent experiments were designed to investigate the protective effects on cisplatin-induced hearing loss in mice: (i) chronic intraperitoneal cisplatin administration (4 mg/kg) once per day for 5 consecutive days and intraperitoneal UCMSC exosome (1.2 μg/μL) injection at the same time point; and (ii) UCMSC exosome (1.2 μg/μL) injection through a round window niche 3 days after chronic cisplatin administration. Our data suggest that UCMSC exosomes exert protective effects in vivo. The post-traumatic administration of UCMSC exosomes significantly improved hearing loss and rescued the loss of cochlear hair cells in mice receiving chronic cisplatin injection. Neuropathological gene panel analyses further revealed the UCMSC exosomes treatment led to beneficial changes in the expression levels of many genes in the cochlear tissues of cisplatin-injected mice. In conclusion, UCMSC exosomes exerted protective effects in treating ototoxicity-induced hearing loss by promoting tissue remodeling and repair.

Objective: CI (cochlear implantation) candidacy is somewhat controversial in severe hearing loss among tonal mandarin-speaking patients. To assess the relationship between pure tone audiometry (PTA) and speech recognition score (SRS), with and without hearing aid amplification, among patients who did not meet the NIH criteria of CI candidacy in tonal language mandarian-speaking countries, especially those with severe hearing loss (70 dB HL < 4FPTA(0.5, 1, 2, 4 KHz) ≤ 90 dB HL) Materials and Methods: A total of 414 patients with sensorineural hearing loss with 774 ears were reviewed retrospectively in a tertiary referral center. The Mandarin Monosyllable Recognition Test (MMRT) was used to evaluate the SRS of these ears. Results: 31% (10/32) of the 32 ears with severe hearing loss, 70-90 dB HL, still showed poor speech recognition (SRS<30%) after hearing aid amplification, while 71% (46/65) of the 65 ears with profound hearing loss, > 90 dB HL, showed poor speech recognition with hearing aid amplification. Conclusions: The speech audiometry with Mandarin Monosyllable Recognition Test (MMRT) helped identify those patients whose 4FPTA< 90 dB HL fell outside the CI candidacy criteria of NIH in tonal language mandarin-speaking countries but showed significantly poor (SRS< 30%) speech recognition performance.