Speech Intelligibility Rating Research Articles

Outcomes Using the Optimized Pitch and Language Strategy Versus the Advanced Combination Encoder Strategy in Mandarin-Speaking Cochlear Implant Recipients.

The experimental Optimized Pitch and Language (OPAL) strategy enhances coding of fundamental frequency (F0) information in the temporal envelope of electrical signals delivered to channels of a cochlear implant (CI). Previous studies with OPAL have explored performance on speech and lexical tone perception in Mandarin- and English-speaking CI recipients. However, it was not clear which cues to lexical tone (primary and/or secondary) were used by the Mandarin CI listeners. The primary aim of the present study was to investigate whether OPAL provides improved recognition of Mandarin lexical tones in both quiet and noisy environments compared with the Advanced Combination Encoder (ACE) strategy. A secondary aim was to investigate whether, and to what extent, removal of secondary (duration and intensity envelope) cues to lexical tone affected Mandarin tone perception. Thirty-two CI recipients with an average age of 24 (range 7 to 57) years were enrolled in the study. All recipients had at least 1 year of experience using ACE. Each subject attended two testing sessions, the first to measure baseline performance, and the second to evaluate the effect of strategy after provision of some take-home experience using OPAL. A minimum take-home duration of approximately 4 weeks was prescribed in which subjects were requested to use OPAL as much as possible but were allowed to also use ACE when needed. The evaluation tests included recognition of Mandarin lexical tones in quiet and in noise (signal to noise ratio [SNR] +5 dB) using naturally produced tones and duration/intensity envelope normalized versions of the tones; Mandarin sentence in adaptive noise; Mandarin monosyllabic and disyllabic word in quiet; a subset of Speech, Spatial, and Qualities of hearing questionnaire (SSQ, speech hearing scale); and subjective preference for strategy in quiet and noise. For both the natural and normalized lexical tone tests, mean scores for OPAL were significantly higher than ACE in quiet by 2.7 and 2.9%-points, respectively, and in noise by 7.4 and 7.2%-points, respectively. Monosyllabic word recognition in quiet using OPAL was significantly higher than ACE by approximately 7.5% points. Average SSQ ratings for OPAL were significantly higher than ACE by approximately 0.5 points on a 10-point scale. In quiet conditions, 14 subjects preferred OPAL, 7 expressed a preference for ACE, and 9 reported no preference. Compared with quiet, in noisy situations, there was a stronger preference for OPAL (19 recipients), a similar preference for ACE (7 recipients), while fewer expressed no preference. Average daily take-home use of ACE and OPAL was 4.9 and 7.1 hr, respectively. For Mandarin-speaking CI recipients, OPAL provided significant improvements to lexical tone perception for natural and normalized tones in quiet and noise, monosyllabic word recognition in quiet, and subjective ratings of speech intelligibility. Subjects accessed both primary and secondary cues to lexical tone for perception in quiet and noise conditions. The benefits of lexical tone recognition were attributed to enhanced F0 rate cues encoded by OPAL, especially in a noisy environment. The OPAL strategy was well accepted by many of the Mandarin-speaking CI recipients.

Cochlear reimplantation outcomes over 20 years: Expertise in reimplantation surgery and auditory-speech rehabilitation

ObjectivesThe aim of this study was to present an institution's experience with cochlear reimplantation (CRI), to assess surgical challenges and post-operative outcomes and to increase the success rate of CRI. Study designRetrospective single-institution study. SettingTertiary medical center. MethodsWe retrospectively evaluated data from 76 reimplantation cases treated in a tertiary center between 2001 and 2022. Clinical features including etiology of hearing loss, type of failure, surgical issues, and auditory speech performance were analyzed. Categorical Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to evaluate pre- and post-CRI outcomes. ResultsThe CRI population comprises of 7 patients from our institute,69 referred patients from other centers. Device failure was the most common reason (68/76, 89.5 %) for CRI; in addition, there were 7 medical failures and 1 had both soft device failure. Medical failures included flap rupture and device extrusion, magnet migration, auditory neuropathy, leukoencephalopathy, foreign-body residue and meningitis. In 21/76 patients, the electrode technology was upgraded. The mean time to failure was 0.58–13 years, with a mean of 4.97 years. The mean (± SD) CAP and SIR scores before and after CRI were 5.2 ± 1.2 versus 5.5 ± 1.1 and 3.4 ± 1.1 versus 3.5 ± 1.1, respectively. Performance was poor in six patients with severe cochlear malformation, auditory nerve dysplasia, leukoencephalopathy, and epilepsy. ConclusionCRI surgery is a challenging but relatively safe procedure, and most reimplanted patients experience favorable postoperative outcomes. Medical complications and intracochlear damage are the main causes of poor postoperative results. Therefore, adequate preoperative preparation and atraumatic CRI should be carried out for optimal results.

Early auditory processing of speech in prelingually-deafened children with cochlear implants based on event-related potentials

Objective: To investigate the early auditory discrimination of vowels, consonants and lexical tones in prelingually-deafened children with cochlear implants (CI) using auditory event-related potentials. Methods: Nineteen prelingually-deafened CI children and 19 normal hearing (NH) children were recruited in this study. A multi-deviant oddball paradigm was constructed using the monosyllable/ta1/as the standard stimulus and monosyllables/tu1/,/te1/, /da1/,/ra1/,/ta4/and/ta2/as the deviant stimuli. The event-related potentials evoked by vowel, consonant and lexical tone contrasts were recorded and analyzed in the two groups. Results: NH children showed robust mismatch negativities (MMNs) to vowel, consonant and lexical tone contrasts (P<0.05), whereas CI children only showed positive mismatch responses (pMMRs) and P3a responses to the vowel (P<0.05) and consonant contrasts (P<0.05) and no significant event-related potential to the lexical tone contrasts (P>0.05). The longer pMMR and P3a peak latencies (P<0.01) but similar amplitudes (P>0.05) were found in CI children than in NH children. CI children showed weaker phase synchronization of θ oscillations than NH children (P<0.05). The duration of CI use was positively correlated with the scores of Categories of Auditory Performance (CAP) (P=0.004), Speech Intelligibility Rate (SIR) (P=0.044) and Meaningful Auditory Integration Scale (MAIS) (P=0.001) in CI children. Conclusions: Prelingually-deafened CI children can process vowels and consonants at an early stage. However, their ability of processing speech, especially lexical tones, is still more immature compared with their NH peers. The event-related potentials could be objective electrophysiological indicators reflecting the maturity of CI children's auditory speech functions. Long-term CI use is beneficial for prelingually-deafened children to improve auditory and speech performance.

Criterion Validity of Infant-Toddler Meaningful Auditory Integration Scale for Persian Children with Cochlear Implantation

Introduction: The infant-toddler meaningful auditory integration scale (IT-MAIS) can evaluate toddlers and infants’ early auditory development in aspects of detection, discrimination, and identification of sounds. The present study aims to evaluate the reliability and criterion validity of the Persian version of the IT-MAIS (IT-MAIS-P) for children with hearing loss (HL). Materials and Methods: In this study, due to the limitations of COVID-19 and the lack of access to samples, 23 available children with HL were sampled. The cause of participants› HL was 43.5% congenital, 30.4% acquired, and 26.1% unknown. The Mean±SD of the age of these children was 21.6±6.3 months. To evaluate the predictive validity and concurrent validity, the speech intelligibility rating (SIR) and categories of auditory performance (CAP) were used, respectively. Also, to assess the inter-rater reliability, both parents of each child completed the IT-MAIS-P. Results: A significant correlation (concurrent validity) was observed between the IT-MAIS-P and the CAP scores (r=0.87, P&lt;0.001). Predictive validity was not confirmed by comparing it with the SIR (P&gt;0.05). Also, the inter-rater reliability (kappa=0.44, P&lt;0.001) of the IT-MAIS-P was confirmed. Conclusion: The IT-MAIS-P is a valid tool to investigate the progress of auditory skills in infants, although the predictive validity was not confirmed.

Cochlear Implantation Outcomes: A 10-Year Single-Surgeon Experience.

Cochlear implantation (CI) is an appropriate management strategy for prelingual hearing loss. Rehabilitation programs are essential in enabling children to cope with devices and acquire language skills. Objectives: This study aimed to assess the hearing outcomes of CIs performed by a single surgeon in Erbil, Kurdistan region of Iraq, and compare them with the results of other CI centers worldwide. This is a prospective study implemented in Erbil from November 1, 2013, to October 30, 2023, on a convenience sample of 161 patients with prelingual hearing loss (HL) who underwent CI by a single surgeon. The following were collected: age at implantation, use of hearing aids before implantation, parent's educational level, and duration of rehabilitation. The effect of the previous variables on Categories of Auditory Performance (CAP) score and speech intelligibility rating (SIR) on the auditory levels of children was assessed by the researcher six months and one year following surgery. Implantation age showed significant associations with the CAP score examined at six months and 12 months post implantation (p-value 0.001). Speech intelligibility rating was also significantly linked to implantation age (p-value 0.001).

THE BENEFITS OF EARLY COCHLEAR IMPLANTATION FOR SPEECH DEVELOPMENT IN CHILDREN WITH USHER SYNDROME: LITERATURE REVIEW

IntroductionUsher syndrome (USH) is a rare autosomal recessive genetic disorder characterized by sensorineural hearing loss (SNHL), vision loss (retinitis pigmentosa), and occasional balance impairment. Depending on the severity and onset of hearing loss and coexisting vestibular dysfunction, USH is divided into three clinical types – USH1, USH2, USH3 – as well as atypical USH which combines features of all these three. The purpose of this review is to present the impact of cochlear implantation on speech development in children diagnosed with all types of Usher syndrome.Material and methodsAll relevant publications published in from 2013 to 2023 were retrieved from PubMed based on the keywords &lt;i&gt;Usher syndrome&lt;/i&gt;, &lt;i&gt;Usher syndrome diagnostics&lt;/i&gt;, &lt;i&gt;Usher syndrome hearing loss&lt;/i&gt;, &lt;i&gt;Usher syndrome cochlear&lt;/i&gt;, and &lt;i&gt;Usher syndrome speech&lt;/i&gt;. Exactly 67 papers were selected.ResultsBilateral cochlear implantation in children with Usher syndrome is beneficial for audiological and verbal development provided that hearing loss is detected early and implantation done promptly. In USH1 the preferred age is before 3 in the case of severe to profound congenital SNHL; in USH2 and USH3 the optimal time for implantation is hampered by the difficulty of estimating when hearing loss occurred and its rate of progression. After bilateral cochlear implantation, studies showed improvements in the categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), meaningful use of speech scale (MUSS), and speech reception score (SRS), together with good development of speech perception and verbal communication.ConclusionsEarly diagnosis (before the onset of vision loss) and early bilateral cochlear implantation in children who have suffered severe to profound SNHL due to Usher syndrome reduces disability and maximizes auditory–oral communication skills, significantly increasing their quality of life.

Effects of SPEAK OUT! & LOUD Crowd on Functional Speech Measures in Parkinson's Disease.

This study investigated the effects of the SPEAK OUT! & LOUD Crowd therapy program on speaking rate, percent pause time, intelligibility, naturalness, and communicative participation in individuals with Parkinson's disease (PD). Six adults with PD completed 12 individual SPEAK OUT! sessions across four consecutive weeks followed by group-based LOUD Crowd sessions for five consecutive weeks. Most therapy sessions were conducted via telehealth, with two participants completing the SPEAK OUT! portion in person. Speech samples were recorded at six time points: three baseline time points prior to SPEAK OUT!, two post-SPEAK OUT! time points, and one post-LOUD Crowd time point. Acoustic measures of speaking rate and percent pause time and listener ratings of speech intelligibility and naturalness were obtained for each time point. Participant self-ratings of communicative participation were also collected at pre- and posttreatment time points. Results showed significant improvement in communicative participation scores at a group level following completion of the SPEAK OUT! & LOUD Crowd treatment program. Two participants showed a significant decrease in speaking rate and increase in percent pause time following treatment. Changes in intelligibility and naturalness were not statistically significant. These findings provide preliminary support for the effectiveness of the SPEAK OUT! & LOUD Crowd treatment program in improving communicative participation for people with mild-to-moderate hypokinetic dysarthria secondary to PD. This study is also the first to demonstrate positive effects of this treatment program for people receiving the therapy via telehealth.

Clinical characteristics and hearing loss etiology of cochlear implantees undergoing surgery in their teens, 20s, and 30s.

This study aimed to investigate the etiology of hearing loss, including genetic variants, in individuals who underwent cochlear implantation (CI) in their teens to thirties. It also sought to analyze post-CI speech performance and identify prognostic factors affecting CI outcomes in this age group. We conducted a retrospective review of 421 cochlear implant patients at Seoul National University Bundang Hospital, focusing on 63 subjects aged 10-39years who underwent their first CI by a single surgeon between July 2018 and June 2022. The study included audiologic evaluation, molecular genetic testing, and analysis of speech performance post-CI. Statistical analyses were performed using SPSS 25 and GraphPad Prism 7. Among 63 participants (M:F, 24:39), nine underwent CI in their teens, 24 in their 20s, and 30 in their 30s. Most of them (40, 63.5%) had postlingual deafness. The study found that 65.2% (40/63) of subjects received a genetic diagnosis, with DFNB4 being the most common etiology (37.5%, 15/40). Post-CI speech evaluation showed an average sentence score of 80% across all subjects. Factors such as the onset of hearing loss, duration of deafness (DoD), and preoperative Speech Intelligibility Rating (SIR) significantly influenced CI outcomes. Notably, longer DoD was associated with poorer CI outcomes, but this did not affect individuals with postlingual hearing loss as much. The study concludes that in individuals aged 10-39 undergoing CI, the onset of hearing loss and preoperative SIR are critical predictors of postoperative outcomes. CI is recommended for those with postlingual hearing loss in this age group, irrespective of the DoD. The study highlights the importance of genetic factors especially DFNB4 in hearing loss etiology and underscores the value of the relatively easy-to-evaluate factor, preoperative SIR in predicting CI outcomes.

Autism's Impact on Cochlear Implantation Surgery Outcomes in Deaf Children.

Cochlear implants (CI) provide a hearing sense for severe to profound hearing-impaired patients, both adults and children, and they are a broadly effective and accepted therapeutic method for those patients. Also, Deaf children with comorbidities, including autism spectrum disorders (ASDs), undergo cochlear implantation. ASDs are a group of developing disorders characterized by abnormalities in social interaction and communication with limited repetitive patterns of behavior. This study aimed to assess the effect of Autism on CI surgery outcomes in Deaf Children. We followed 12 autistic patients with cochlear implantation and 12 non-autistic cochlear-implanted patients for two years. The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to assess 6, 12, and 24 months after cochlear implantation surgery. During the 24-month follow-up, the CAP means scores increased in both groups, and SIR and CAP progresses were considerably greater in non-ASD children (P<0.001). However, in ASD children, the progress of CAP and SIR variables were significant, with 99% and 95% confidence, respectively, at 24 months after surgery. Although the CIs could improve hearing performance in autistic patients, speech development after CIs in autistic children could affected by several factors, including the severity of autism, and this can be effective in providing pre-implant counseling to parents. The application of the alternative communication methods could be taken into account as a potential rehab technique.

Cochlear implantation through retro-facial approach with congenital microtia malformation with facial nerve deformity: a case report

The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performance（CAP） questionnaire was 7, and the score of the speech intelligibility rating（SIR） questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury.

Factors Affecting the Development of Speech in Children After Cochlear Implant

Objective: To determine the odds of different factors for developing poor speech intelligibility in pre-lingually deaf children who have undergone cochlear implantation. Study Design: Case-control study. Place and Duration of Study: Department of ENT, Combined Military Hospital, Rawalpindi Pakistan, Jan 2020 to Aug 2022. Methodology: This study was based on a sample of 60 patients: 30 cases with cochlear implants placed before two years of age, with a total time of using a cochlear implant of ≥1 year, and 30 controls. Both groups consisted of both genders aged between 3 and 12 years. All patients underwent assessment using the Speech Intelligibility Rating system and were documented for var-ious factors influencing speech development. Results: The mean age of the sample at the time of the study was 6.37±3.17 years. A total of 34(56.7%) participants were male. Factors associated with poorer speech outcomes were male gender (OR0.44[CI 95% 0.15–1.30]), no peers in the household (OR 0.53 [CI 95% 0.15–1.84]), higher maternal literacy (OR 0.42[CI 95% 0.13–1.30]). Factors associated with improved speech devel-opment included primarily oral form of communication (OR 2.14[CI 95%0.41–11.17]), less than one-year age at implantation (OR 1.29 [CI 95% 0.24–6.96]), a total duration of speech therapy less than two years (OR 1.70[CI 95% 0.32–8.74]) and the pres-ence of unilateral implants, (OR 6.33[CI 95% 1.00–40.07]). Conclusion: Earlier cochlear implantation with frequent verbal skill exercise appears essential to developing good speech in-telligibility in pre-lingually deaf children. Keywords: Children, Cochlear Implantation, Pre-Lingual Deafness, Speech intelligibility

Cochlear implantation in patients with Streptococcus suis meningitis: clinical characteristics and postoperative evaluation

Background Hearing loss is a common sequala of Streptococcus suis (S. suis) meningitis, but few have addressed cochlear implantation (CI) candidates with S. suis meningitis. Objectives To assess the clinical characteristics and CI postoperative outcomes in S. suis meningitis patients. Material and methods Eight S. suis meningitis patients underwent CI at Sun Yat-sen Memorial Hospital between 2020 and 2023. Control groups included (1) non-Suis meningitis patients (n = 12) and (2) non-meningitis patients (n = 35). Electrode impedances and neural response telemetry (NRT) thresholds were recorded at one month after surgery. The auditory performance-II (CAP) and speech intelligibility rating (SIR) were recorded at the last visit. Results CAP scores of S. suis meningitis patients were significantly lower than those of non-Suis meningitis and non-meningitis patients (p = .019; p<.001). And NRT thresholds of S. suis meningitis patients were higher than those of non-Suis meningitis and non-meningitis patients (p = .006; p = .027). Conclusions and significance It is recommended for S. suis meningitis CI candidates to undergo CI promptly after controlling infection, preferably within four to six weeks. CI users with S. suis meningitis tend to exhibit suboptimal hearing rehabilitation outcomes, possibly associated with the more severe damage on spiral ganglion cells after S. suis meningitis.

Cochlear implantation outcomes in children with multiple disabilities: a topic that’s worth revisiting

ObjectivesTo determine the benefits of cochlear implantation in hearing loss children with multiple disabilities (MD) in terms of auditory outcomes, speech performance, and their quality of life. MethodsThis was a cross sectional study from January 2019 to December 2020 in which thirty-one children with hearing loss and multiple disabilities were evaluated. Their improvement in auditory and speech performances were assessed using Categories of Auditory Performance version II (CAP-II) and the Speech Intelligibility Rating (SIR) scales. The assessment was done at 6-month intervals, with the baseline evaluation done at least six months after activation of the implant. Parents were asked to fill the Parents Evaluation of Aural/Oral Performance of Children (PEACH) diary and Perceived Benefit Questionnaire (PBQ) to evaluate the child’s quality of life. ResultsAll 31 children have Global Developmental Delay (GDD), with 11 having an additional disability. Both mean CAP-II and SIR scores showed significant improvement with increased hearing age (p < 0.05) after 6-month intervals. In addition, 20 out of 31 children (64.5%) have achieved verbal communication after implantation. The mean PEACH score in quiet was significantly better than in noise (p = 0.007) and improved with the increased of hearing age. The majority of parents (96%‒100%) perceived a cochlear implant as beneficial to their child in terms of auditory response, awareness, interaction, communication, and speech development. ConclusionsCochlear implantation had shown benefits in children with multiple disabilities. Outcome measures should not only focus on auditory and speech performances but the improvement in quality of life. Hence, individualized each case with realistic expectation from families must be emphasized in this group of children. Level of evidenceLevel 3.

Machine learning-based prediction of the outcomes of cochlear implantation in patients with inner ear malformation.

The objectives of this study are twofold: first, to visualize the structure of malformed cochleae through image reconstruction; and second, to develop a predictive model for postoperative outcomes of cochlear implantation (CI) in patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation. The clinical data from patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation who underwent cochlear implantation (CI) at Beijing Tongren Hospital between January 2016 and August 2020 were collected. Radiological features were analyzed through 3D segmentation of the cochlea. Postoperative auditory speech rehabilitation outcomes were evaluated using the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR). This study aimed to investigate the relationship between cochlear parameters and postoperative outcomes. Additionally, a predictive model for postoperative outcomes was developed using the K-nearest neighbors (KNN) algorithm. In our study, we conducted feature selection by using patients' imaging and audiological attributes. This process involved methods such as the removal of missing values, correlation analysis, and chi-square tests. The findings indicated that two specific features, cochlear volume (V) and cochlear canal length (CDL), significantly contributed to predicting the outcomes of hearing and speech rehabilitation for patients with inner ear malformations. In terms of hearing rehabilitation, the KNN classification achieved an accuracy of 93.3%. Likewise, for speech rehabilitation, the KNN classification demonstrated an accuracy of 86.7%. The measurements obtained from the 3D reconstruction model hold significant clinical relevance. Despite the considerable variability in cochlear morphology across individuals, radiological features remain effective in predicting cochlear implantation (CI) prognosis for patients with inner ear malformations. The utilization of 3D segmentation techniques and the developed predictive model can assist surgeons in conducting preoperative cochlear structural measurements for patients with inner ear malformations. This, in turn, can offer a more informed perspective on the anticipated outcomes of cochlear implantation.

A Long-Term Follow-Up Study on the Auditory Performance and Speech Intelligibility of Mandarin-Speaking Prelingually Deaf Children With Isolated Large Vestibular Aqueduct Syndrome After Cochlear Implantation.

The purpose of this study was to evaluate the auditory performance and speech perception of 104 children with isolated large vestibular aqueduct syndrome (LVAS) and 523 children with no inner ear malformation (IEM) for 5 years after cochlear implantation, in order to explore whether isolated LVAS can affect the long-term hearing and speech rehabilitation of deaf children after cochlear implantation. A cohort study was established consisting of 627 children who underwent cochlear implantation at Beijing Tongren Hospital from 1999 to 2016. The children were examined at 0, 6, 12, 24, 36, 48, and 60 months after cochlear implantation to assess their auditory performance and speech perception using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) questionnaires. The CAP scores of the isolated LVAS group increased significantly during the baseline to the 24th month, after which they gradually rose until reaching the plateau during the 24th to the 60th month. The CAP scores of the non-IEM group increased significantly during the baseline to the 36th month and then increased steadily. The SIR scores went up significantly during the baseline to the 48th month, and increased in a gradual manner in other stages of isolated LVAS evaluation. In comparison, The SIR scores of non-IEM group grew remarkably from the baseline to the 60th month. There were no significant differences in the CAP or SIR scores between isolated LVAS and non-IEM groups in each stage of evaluation, with the only exception being the CAP score at baseline month after cochlear implantation. The CAP and SIR questionnaires are helpful tools for quantifying the early hearing and speech skills of younger prelingually deafened cochlear implant recipients. This long-term follow-up study shows that the speech and hearing development of children in isolated LVAS and non-IEM groups follow similar patterns, and isolated LVAS does not affect the long-term rehabilitation of deaf children after cochlear implantation.

Electrically evoked auditory brainstem responses in deaf children with cochlear nerve canal stenosis

Background Deaf children with cochlear nerve canal stenosis (CNCs) are always considered poor candidates for cochlear implantation. Objectives To investigate the function of the peripheral auditory pathway in deaf children with CNCs, as revealed by the electrically evoked auditory brainstem response (EABR), and postoperative cochlear implants (CIs) outcomes. Materials and Methods Thirteen children with CNCs and 13 children with no inner ear malformations (IEMs) who received CIs were recruited. The EABR evoked by electrical stimulation from the CI electrode was recorded. Postoperative CI outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Rate (SIR). Results Compared with children with no IEMs, children with CNCs showed lower EABR extraction rates, higher thresholds, a longer wave V (eV) latency and lower CAP and SIR scores. The auditory and speech performance was positively correlated with the diameter of the cochlear nerve canal and the number of channels showing wave III (eIII) and eV in children with CNCs. Conclusions and Significance The physiological function of the peripheral auditory pathway in children with CNCs is poorer than that in children with no IEMs. Postoperative auditory and speech abilities may depend on the severity of cochlear nerve malformation and auditory conduction function.

Should left- and right-sided unilateral cleft lip and palate patients be grouped together when reporting the outcomes?

Investigate associations between cleft laterality in patients with non-syndromic unilateral cleft lip and palate (UCLP) and oral-health, dental-arch, speech, audiological, psychological and nasolabial-aesthetic outcomes. Secondary data analysis of the outcomes of 5-year-old children with non-syndromic complete UCLP identified from three studies: Cleft Collective (n = 155), Cleft Care UK (CCUK) (n = 266) and Clinical Standards Advisory Group (CSAG) study (n = 238). Outcome measures included occlusal assessment using the 5-year-old's index score, speech intelligibility rating using the CAPS-A Audit tool, audiological assessment using pure tone audiometry, nasolabial aesthetic assessment using the Asher-McDade tool, oral-health assessment using decayed, missing, filled teeth scores and parent-reported outcomes. Logistic regression with adjustment for age, sex and index-of-multiple-deprivation scores were performed. No differences were found in patient-reported outcomes between the left and right clefts in the Cleft Collective study. From the CCUK study, right clefts had poorer speech (n = 236; 95% CI 1.09, 3.42; and P = .03) and hearing outcomes (n = 211; 95% CI 1.03, 3.43; P = .04). In the CSAG study, patients with left clefts were more likely to be teased (n = 213; 95% CI 0.26, 0.85; and P = .01). Weak associations between cleft laterality, speech, hearing and psychological outcomes were found, however the findings were inconsistent across the studies. This study contributes to evidence of associations between laterality and outcomes in children born with UCLP.

Can Mandarin-speaking prelingual deaf adults benefit from cochlear implant?

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.

Genetic and phenotypic analysis of MYO15A rare variants associated with autosomal recessive hearing loss

Objective:To analyze the phenotype and genotype characteristics of autosomal recessive hearing loss caused by MYO15A gene variants, and to provide genetic diagnosis and genetic counseling for patients and their families. Methods:Identification of MYO15A gene variants by next generation sequencing in two sporadic cases of hearing loss at Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. The sequence variants were verified by Sanger sequencing.The pathogenicity of these variants was determined according to the American College of Medical Genetics and Genomics（ACMG） variant classification guidelines, in conjuction with clinical data. Results:The probands of the two families have bilateral,severe or complete hearing loss.Four variants of MYO15A were identified, including one pathogenic variant that has been reported, two likely pathogenic variants,and one splicing variant of uncertain significance. Patient I carries c. 3524dupA（p. Ser1176Valfs*14）, a reported pathogenic variant, and a splicing variant c. 10082+3G>A of uncertain significance according to the ACMG guidelines. Patient I was treated with bilateral hearing aids with satisfactory effect, demonstrated average hearing thresholds of 37.5 dB in the right ear and 33.75 dB in the left ear. Patient Ⅱ carries c. 7441_7442del（p. Leu2481Glufs*86） and c. 10250_10252del（p. Ser3417del）,a pair of as likely pathogenic variants according to the ACMG guidelines. Patient Ⅱ, who underwent right cochlear implantation eight years ago, achieved scores of 9 on the Categorical Auditory Performance-Ⅱ（CAP-Ⅱ） and 5 on the Speech Intelligibility Rating（SIR）. Conclusion:This study's discovery of the rare c. 7441_7442del variant and the splicing variant c. 10082+3G>A in the MYO15A gene is closely associated with autosomal recessive hearing loss, expanding the MYO15A variant spectrum. Additionally, the pathogenicity assessment of the splicing variant facilitates classification of splicing variations.

Auditory and speech outcomes of cochlear implantation in patients with Waardenburg syndrome: a meta-analysis.

This study aims to assess the potential efficacy of cochlear implantation as a treatment for patients with Waardenburg syndrome (WS) and to guide clinical work by comparing the effect of auditory and speech recovery after cochlear implantation in patients with WS and non-WS. PubMed, the Cochrane Library, CNKI, and Wanfang Data were sources for retrieving literature on cochlear implantation in WS, and clinical data meeting the inclusion criteria were meta-analyzed using RevMan5.41. A total of nine articles were included in this study, including 132 patients with WS and 815 patients in the control group. Meta-analysis showed that there are no significant differences in the scores for categories of audit performance (CAP), speech intelligibility rating (SIR), and parents' evaluation of aural/oral performance of children (PEACH) between the WS group and the control group. Cochlear implantation demonstrates comparable auditory and speech recovery outcomes for WS patients and non-WS patients.