Objective: To assess the long-term outcomes after cochlear implantation in children under 3 years of age with Waardenburg syndrome (WS), concerning preoperative radiological imaging, intraoperative surgical findings and postoperative auditory and speech performance. Material and methods: A retrospective analytical study conducted on the young WS children with bilateral profound sensorineural hearing loss (SNHL) who underwent the cochlear implantation. Postoperative outcomes were confronted with results obtained by aged-matched peers without additional disabilities. The auditory performance was assessed with the MAIS and CAP. The speech and language development were evaluated with the MUSS and SIR. Radiological evaluation was performed with the MRI and HRCT scans. Results: There were 22 children with WS, who received a multichannel implant at a mean age of 23.5 months of age (SD 7.5, range: 12–36 months). WS group outperformed non-syndromic (NS) children in SIR (4.7, 95%, SD 0.8 vs. 4.3, 95%, SD 0.8) and MUSS (35.6, 95%, SD 3.4 vs. 30.3, 95%, SD 7.6). Auditory performance assessed in MAIS/IT-MAIS improved similarly in both groups (34.8, 95%, SD 1.8 in WS vs. 35, 95%, SD 2.5 in NS children). The reference group gained significantly higher scores in CAP (6.2, 95%, SD 0.7 vs. 5.8, 95%, SD 1.8). Additional anomalies of the inner ear were observed including the enlarged vestibular aqueduct (EVA), common cavity deformity (CCD), incomplete partition type II (IP-II). Two cases presented various forms of mental retardation. Conclusion: The study demonstrated that cochlear implantation in young children with WS is a beneficial method treating profound SNHL. The most beneficial outcomes may be achieved thanks to the early implantation, parental involvement and comprehensive care from speech therapists, preferably implemented altogether. The detailed radiological examination and psychological assessment should be considered before the implantation. The results support the concept of early cochlear implantation in children with genetic disorders.

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Almost one out of two singers was likely to present arytenoid asymmetry in adduction. The presence of this asymmetry does not appear to be correlated with any vocal symptoms.

IMPORTANCE Pediatric vocal fold pathology is important because having a healthy voice free from disorders is crucial in a child's emotional and educational development. OBJECTIVE To determine whether there is an association between singing in a children's choir and the development of voice disorders. DESIGN, SETTING, AND PARTICIPANTS Prospective cohort study of children (aged 8 to 14 years) singers selected from local children's choirs and nonsingers selected from local schools evaluated at Clarós Otorhinolaryngology Clinic in Barcelona, Spain, from October 2016 through April 2018. EXPOSURES Singing for a mean time of 7.5 hours per week for 2.5 years. MAIN OUTCOMES AND MEASURES The primary outcome of the study was the prevalence of voice disorders measured using videostroboscopy. The obtained values were analyzed statistically and used to compare the characteristics of the children and the frequency of voice disorders between the groups. RESULTS Of 1495 enrolled children (745 male [49.8%]; median age, 9.3 years [range, 8-14 years]), 752 were singers and 743 were nonsingers. No differences in baseline characteristics were observed between the groups. Voice disorders were more frequent in the nonsinging group than in the singing group (32.4% vs 15.6%; difference, 16.8%; 95% CI, 12.3%-21.4%). Of 12 voice disorders considered in this study, all 12 were more frequent in the nonsinging group. Functional voice disorders were more frequent in the nonsinging group than in the singing group (20.2% vs 9.4%; difference, 10.8%; 95% CI, 7.2%-14.3%), as were organic voice disorders (12.2% vs 6.1%; difference, 6.1%; 95% CI, 2.6%-9.6%). CONCLUSIONS AND RELEVANCE Voice disorders were less common among children in the cohort who sing in choirs, possibly because of voice training and the commonly observed habit of attending regular ear, nose, and throat examination. Voice disorders may be prevented in nonsinging children if the same solicitude for voice is observed.

Objective: The aim of the study was to analyze the long-term outcomes after cochlear implantation in deaf children with Down syndrome (DS) regarding age at the first implantation and refer the results to preoperative radiological findings as well as postoperative auditory and speech performance. Additionally, the influence of the age at implantation and duration of CI use on postoperative hearing and language skills were closely analyzed in children with DS. Study Design: Retrospective analysis. Setting: Referral center (Cochlear Implant Center). Materials and Methods: Nine children with Down syndrome were compared with 220 pediatric patients without additional mental disorders or genetic mutations. Patients were divided into four categories depending on the age of the first implantation: CAT1 (0–3 yr), CAT2 (4–5 yr), CAT3 (6–7 yr), and CAT4 (8–17 yr). The auditory performance was assessed with the meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) scales. The speech and language development were further evaluated with meaningful use of speech scale (MUSS) and speech intelligibility rating (SIR). The postoperative speech skills were analyzed and compared between the study group and the reference group by using nonparametric statistical tests. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging (MRI) and high-resolution computed tomography of the temporal bones (HRCT). Results: The mean follow-up time was 14.9 years (range, 13.1–18.3 yr). Patients with DS received a multichannel implant at a mean age of 75.3 months (SD 27.9; ranging from 21 to 127 mo) and 220 non-syndromic children from reference group at a mean age of 51.4 months (SD 34.2; ranging from 9 to 167 mo). The intraoperative neural response was present in all cases. The auditory and speech performance improved in each DS child. The postoperative mean CAP and SIR scores were 4.4 (SD 0.8) and 3.2 (SD 0.6), respectively. The average of scores in MUSS and MAIS/IT-MAIS scales was 59.8% (SD 0.1) and 76.9% (SD 0.1), respectively. Gathered data indicates that children with DS implanted with CI at a younger age (<6 years of age) benefited from the CI more than children implanted later in life, similarly in a control group. There were additional anomalies of the temporal bone, external, middle, or inner ear observed in 90% of DS children, basing on MRI or HRCT. Conclusions: The early cochlear implantation in children with DS is a similarly useful method in treating severe to profound sensorineural hearing loss (SNHL) as in non-syndromic patients, although the development of speech skills present differently. Due to a higher prevalence of ear and temporal bone malformations, detailed diagnostic imaging should be taken into account before the CI qualification. Better postoperative outcomes may be achieved through comprehensive care from parents/guardians and speech therapists thanks to intensive and systematic rehabilitation.