Outcomes of cochlear implantation in children with Usher syndrome: a long-term observation.

PURPOSE: To evaluate auditory performance and speech intelligibility of children with Usher syndrome up to 10 years after cochlear implantation. METHODS: Thirty-five children with USH were compared to 46 non-syndromic patients regarding age at implantation. Auditory performance and speech intelligibility was assessed with standard tools. Genetic counseling, vestibular tests, imaging studies, and ophthalmological findings were evaluated, depending on the availability. RESULTS: The mean age of implantation in USH children was 6.3 years (SD 4.6, range 0.3-17.6 years). Post-implantation values of the studied parameters were compared between USH and NS children and presented as follows: PTA = 25.0 dB HL vs. 28.4, CAP = 5.3 vs. 5.1, SIR = 4.1 vs. 3.9, MAIS = 82.3% vs. 80.5%, MUSS = 81.8% vs. 76.6%. There were no statistically significant differences between the USH and NS groups (p > 0.005). USH patients reached a higher score ceiling earlier compared to NS patients. Children implanted before 3 years of age achieved significantly higher results than older children in USH and NS groups (p < 0.005). In all patients with USH, the electroretinogram was abnormal. Vestibular examination was abnormal in 29 of 31 patients with USH1. Imaging studies revealed no inner ear or auditory nerve anomalies in patients with USH. CONCLUSION: Cochlear implantation successfully improves auditory performance and speech intelligibility in patients with USH, especially those implanted under 3 years of age. The electroretinogram is the only reliable test to establish a diagnosis of USH. Logopedic outcomes are associated with early implantation, and early diagnosis of USH contributes to optimizing speech therapy.

Hyperbaric oxygen therapy as a therapeutic option in cochlear implants extrusion treatment in infected wounds.

Background: Cochlear implant extrusion as a result of infection is an uncommon, but serious complication, which can lead to implant removal as the ultimate solution.Objectives: (1) to identify the incidence of cochlear implant extrusion and its causes, (2) to report our management of patients presenting skin complications after cochlear implant surgery (3) to propose new therapeutical options with hyperbaric oxygen therapy (HBOT).Materials and methods: A retrospective analysis of medical documentation of 1250 patients who were operated on with cochlear implants in our department between 1993 and 2015. The medical charts of 25 patients were selected due to reported skin flap complications resulting in CI extrusion. Five of those patients were subsequently removed from the study because of no infection signs.Results: Non-traumatic cochlear implant extrusion occurred in 1.6% of implanted patients, and secondary treatment was effective in 90% of all cases (18 of 20 patients). HBOT as additional treatment was applied in 9 patients.Conclusions: Hyperbaric oxygen therapy can be considered as safe adjuvant treatment option in individual cases of proceeding with cochlear implant extrusion with signs of wound infection.Significance: HBOT may contribute to reducing the need for cochlear implant explantation due to infectious skin flap complication.

An Opera Singer's Voice Mutation over Time Due to Acromegaly.

Acromegaly is a rare disease with vocal changes being a common clinical finding. The authors present a very rare case of an opera singer with undetected acromegaly for years, whose tessiture progressively changed from tenor, to baritone, to bass. We analyze the evolution of vocal parameters over the years and the outcome after surgical treatment.

Long-term Outcomes in Down Syndrome Children After Cochlear Implantation: Particular Issues and Considerations.

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.

Aspects of ageing on professional opera singer's voice - preliminary findings.

OBJECTIVES: The main goals of this study were to assess the most significant morphological changes and acoustic measures for the ageing process of a professional opera singer's voice. DESIGN: This investigation included 58 healthy professional opera singers, who were compared with 42 young opera singers from the control group. METHODS: All participants underwent a voice assessment protocol: ENT specialist examination and speech therapist evaluation. Acoustic parameters and subjective observations were obtained and analysed. R esults: Fundamental frequency (F0) level was distinctly decreased in the case of older female singers, but F0 in older male singers had stable levels in comparison to that in younger singers. Older singers were found not to have substantially different values of jitter than younger ones. Maximal phonation time (MPT) was longer in the older women's group when compared to the younger singers, but not relatively different in the men's group. Shimmer value presented no age-related change. Morphological changes seem to correlate with the age of subjects. C onclusions: The main characteristic of voice change with age was a decreased F0 level among older female professional singers and rather stable F0 levels in male singers. This study gives preliminary results on the ageing of voice in the population of professional opera singers.

Association Between the Development of Pediatric Voice Disorders and Singing in Children's Choir.

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.

CT-based Morphometric Analysis of Professional Opera Singers' Vocal Folds.

PURPOSE AND STUDY DESIGN: Our work aimed to study the relationship between the length of vocal folds and classical voice type among professional opera singers. Also, the present paper attempts to assess if individual features such as height and body mass index are associated with the vocal folds length (VFL) and the vocal range. Our research compared VFL and voice type by evaluation of neck computed tomography imaging of professional opera singers obtained between 2009 and 2015. Vocal range was measured after scanning procedure by a speech-language pathologist. The results were used to compare VFL with vocal range, voice type, and individual features of every singer. MATERIAL AND METHODS: The group included 93 opera singers (female: 31 sopranos, 15 mezzos, and 5 contraltos; male: 17 tenors, 16 baritones, 6 basses, and 3 countertenors). Different values such as VFL, vocal range, and individuals' features (height, weight, body mass index) were analyzed statistically. RESULTS: Some significant differences and correlation were found. The VFL was diversified between classical voice types. VFL for soprano, mezzo-soprano, and bass was significantly different from every other type of voice. The vocal range has been shown to have a negative correlation with VFL value, which reflects the narrowest vocal range among bass voices. The analysis confirmed a linear correlation between VFL and individual features of the body such as height and body mass index. CONCLUSION: Presented data support the hypothesis that there are significant differences of VFL, vocal range, and body morphology between professional singers of different voice classification.