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.

Why the olfactory acuity to Exaltolide test is different in women?

BACKGROUND: Sporadic studies have revealed that sensitivity to Exaltolide depends on the estrogen blood levels in women. AIM: comparing the sensitivity to Exaltolide to levels of estrogen in women. METHOD: A total of 60 women were prospectively tested for both sensitivity to Exaltolide and blood estradiol during the menstrual cycle, pregnancy and menopause. RESULTS: The average score of sensitivity to Exaltolide was 1.2 during menstruation, 4.8 during proliferative phase, 5.8 at ovulation and 2.4 during the luteal phase. In pregnant women, it was 3.6 during the first trimester, 4.7 during the second and 6 during the third trimester. In post-menopausal women, the average score was 0.3. The average estradiol blood level in pg/ml was 42 during menstruation, 207 during proliferative phase, 368 at ovulation and 147 during the luteal phase. In pregnant women, it was 2506 during the first trimester, 5913 during the second and 9968 during the third trimester. The average estradiol blood level during menopause was 14 pg/ml. The higher the levels of estrogen, the higher sensitivity to Exaltolide. Our results were similar to the findings of previous authors. CONCLUSION: In addition to its common use in perfumery, the odor of Exaltolide could be used as a clinical test of estrogen.

Voice After Rhinoplasty. An Important Question for the Opera Singers.

OBJECTIVE: The primary goal of the study was to evaluate the influence of cosmetic and functional rhinoplasty on quality of life and voice performance preservation in opera singers. MATERIALS AND METHODS: This study was conducted in the ENT Department of Clarós Clinic and included 18 opera singers: 16 women (8 sopranos, 5 mezzos, 2 contralto, and 1 unclassified) and 2 men (1 tenor and 1 baritone). Patients underwent either cosmetic or functional rhinoplasty. We excluded patients with previous nasal surgery, allergic or vasomotor rhinitis, laryngeal pathology, nasal pathology except septal deviation, hormonal or psychiatric disorders, psychic lability, or younger than 18 years of age. We evaluated the fundamental frequency, jitter, shimmer, maximal phonation time, Voice Handicap Index-10, and subjective perception of the patient before and 6 months after surgery. RESULTS: Rhinoplasty was indicated for aesthetic reasons in 12 cases. Six patients also associated nasal obstruction due to septal deviation. The fundamental frequency presented no variation and jitter, shimmer and the maximal time phonation improved slightly. Voice Handicap Index-10 scores decreased after the intervention (5.3 vs 5) and the overall perception of the surgical intervention was considered positive both aesthetically and vocally. CONCLUSIONS: Singers evaluated long-term influence of rhinoplasty as positive. Over 88.8% admitted a beneficial effect on vocal emission and function while 11.1% perceived no change regarding their previous voice.

Impact of the ethmoid volume on endoscopic medial wall decompression outcomes in Graves' orbitopathy.

BACKGROUND: Endoscopic orbital decompression is a well-established technique for Graves orbitopathy treatment. AIMS: Measurement of the ethmoid volume and its correlation with proptosis reduction in patients undergoing endoscopic orbital decompression for Graves' orbitopathy. METHODS: We retrospectively reviewed clinical charts of 209 patients (319 orbits) operated by a surgeon at our institution between 1998 and 2019. RESULTS: Average age was 46.9 years. About 83.3% of the patients were female, 54.6% were smokers, 93.8% were euthyroid at the time of decompression, 3.8% were hypothyroid and 2.4% were hyperthyroid. About 77.5% of the patients underwent bilateral surgery, the remainder and unilateral. Subsequent stages included strabismus surgery in 22.5% patients, Müllerectomy 37.8%, with or without canthoplasty (14.8 vs. 23%). Mean proptosis reduction was 4.61 mm. Mean ethmoid volume was 5.57 mm3. Ethmoid volume had a moderate positive correlation with proptosis reduction (r s = 0.49, p < .001). Further investigation, statistically significant moderate correlation was found only in the small (SE) and big (BE) ethmoid groups. CONCLUSION: We advise evaluating ethmoid sinus pneumatization on computed tomography before decompression to estimate possible anatomical limitations. Additional wall decompression might be advocated in most severe cases.

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.

Facial human bites. Vision on STMMs in Africa.

<b>Objectives:</b> Human bites of the face are a frequent and serious health issue as they often compromise patients function and aesthetics as well as lead to further complications. The aim of the study is to review human bites of the face referred to our team during 112 short-term medical missions (STMMs) in Sub-Saharan Africa over the past 20 years and to discuss the epidemiology, appearance, management and outcome, including the most common complications. <br><b>Methods:</b> A retrospective medical documentation review was carried out examining all human bites of the face operated by our team during 112 STMMs from 2000 to 2019 in different countries of Sub-Saharan Africa. <br><b>Results:</b> Out of about 5500 patients medical charts 51 patients were selected due to history of human bite. Patients' age range was 15-65 years, female to male ratio was 1, 55:1, the most often involved parts were: lips, ear and nose. Various surgical procedures were carried out including local flaps and free grafts. Infection and graft necrosis were the most common reported complication (n = 4; 9.3%). <br><b>Conclusion:</b> Human bites injuries are a serious health problem in some African populations because of their frequency and possible severity. The treatment is particularly challenging due to their potential to cause local infections, the risk they pose for transmission of systemic diseases as well as their demanding surgical management.

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.

Orbital cavernous haemangioma; profile and outcome of 76 patients managed surgically.

Background: Orbital cavernous haemangioma (OCH) is one of the most common primary orbital tumours in adults. They can encroach on intraorbital or adjacent structures and be considered 'anatomically' malignant. Aims: To report a series of patients that were all managed surgically, with possible specificities and propose predictors of treatment outcome. Methods: We realised a hospital-based retrospective case review including data from 76 patient files. Results: The mean age was 37.8 years. Unilateral proptosis was observed in all patients. Visual loss was found in 32 patients (42.1%). Findings on eye examination included lagophthalmos (76.3%), blepharoptosis (21.1%), complications of corneal exposure (19.6%), strabismus (13.2%) and fundoscopic abnormalities (60.5%). Abnormal fundoscopy was significantly associated with decreased visual acuity (VA) (p < .001). Small tumours were predominant (65.8%) and size was associated with VA (correlation coefficient r = -0.5, p < .001). Surgery was mainly by lateral orbitotomy (94.7%), with early post-operative complications in 18 patients (23.7%). Multivariable analysis showed that tumour size, preoperative VA and abnormal fundoscopy were significantly associated with postoperative VA. Conclusion: Clinical and radiological profiles are consistent. Tumour size, preoperative VA, and abnormal fundoscopy seem to be important factors that could influence outcome expectations. Surgical management is still predominantly by open approaches. Severe complications remain rare.

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.

Silent sinus syndrome: combined sinus surgery and orbital reconstruction - report of 15 cases.

BACKGROUND: Silent sinus syndrome (SSS) is defined as spontaneous, painless enophthalmos, hypoglobus with orbital floor resorption and maxillary sinus collapse on the ipsilateral side. Different methods of orbital floor reconstruction have been proposed. AIMS/OBJECTIVES: The purpose was to analyse the results of combined endoscopic sinus surgery (ESS) and reconstruction using orbital floor implant of 15 patients with SSS and to present recent histological findings. MATERIALS AND METHODS: Retrospective case review of 15 patients with SSS treated in clinic between 2007 and 2017. RESULTS: Eleven women and four men presented with unilateral, spontaneous enophthalmos. Averaged duration of enophthalmos was 10.7 months. On affected side, mean enophthalmos was 2.6 mm and hypoglobus 2.7 mm. Computed tomography imaging (CT) imaging showed maxillary sinus opacification on the affected side in every case, and the orbital floor was displaced downwards in all cases. In total, 13 patients underwent simultaneous ESS and rebuilding of orbital floor with a titanium implant. Statistical analysis confirmed significant differences for pre- and postoperative measure of enophthalmos and hypoglobus. CONCLUSION AND SIGNIFICANCE: Implementation of titanium implants is the reliable method of reconstruction that allows good aesthetic result, shorter time of procedure with an excellent long-term outcome and satisfactory patient's tolerance.

Lacrimal gland pleomorphic adenoma: a review of 52 cases, 15-year experience.

BACKGROUND: Lacrimal gland (LG) tumours are rare neoplasms. Pleomorphic adenoma (PA) is the most common histologic variant, representing ∼20% of all LG tumours. PA tends to recur leading to great morbidity. AIMS: We carried out this study to share our experience and provide recent data on the clinical aspects, radiologic findings, management and outcome after treatment. METHODS: We realised a hospital-based retrospective case review including data collected from 52 patients, managed over 15 years. RESULTS: The mean age was 39.8 ± 2 years. Unilateral painless proptosis was the most constant sign (51.9%). CT-scan showed predominantly isodense lesions (96.2%), with regular borders (94.2%), measuring on average 2 cm. MRI showed isointense lesions on T1-weighted images in 96.2% of cases. No pre-operative biopsy was done. Surgical management was mainly external lateral orbitotomy (94.2%). There was no recurrence, considering a mean follow-up period of 12.6 years. Size of tumour at the time of diagnosis increased with age (r = +0.36, p = .01). CONCLUSIONS: Clinical and radiologic characteristics are consistent with literature. Older patients seem to present larger tumours. We believe that biopsy is not necessary if appropriate imaging is done. Complete, intact resection is generally sufficient to minimise the risk of recurrence.

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.

Cochlear implantation in patients with enlarged vestibular aqueduct. A case series with literature review.

OBJECTIVES: To report our institutional experience of the management of patients with enlarged vestibular aqueduct (EVA) and compare it to the literature. METHODS: We carried out a retrospective review of patients' records from 1993 to 2015. The age, sex, associated malformations, relevant past medical history, genetic screening results, possible surgical incident, implant model and duration of follow- up, outcome in terms of Categories of Auditory Performance (CAP scores), and integration or resuming mainstream school or work were recorded. RESULTS: We had 11 patients (six boys and five girls) with EVA who underwent cochlear implant surgery in our center during the 22-year study period, out of a total of 827 implanted (1.3%). The mean age at surgery was 8.9 years ranging from 0.6 to 35 years. EVA was bilateral in 10 cases, isolated anatomical finding in seven cases, and associated with other malformations in four. Cochlear implantation was bilateral in five cases and unilateral in six. The mean follow- up duration was 48.3 months (range: 3-120). No postoperative complication was observed and all the patients could regain a serviceable hearing, attending normal school and working normally. CONCLUSION: EVA is frequently observed in the deaf population without an identifiable cause. The hearing loss is usually progressive and may result in cochlear implantation which has proved its efficiency in rehabilitating EVA patients.

Management of subglottic hemangiomas with carbon dioxide laser: Our 25-year experience and comparison with the literature.

INTRODUCTION: Subglottic hemangiomas (SGH) are rare tumors of infancy arising between the perichondrium and the mucosa of the subglottic space. It is a potential life-threatening condition. Many therapeutic options exist for this disease, including lasers. OBJECTIVE: To report our 25-year experience of laser treatment of SGH. MATERIAL AND METHODS: Retrospective review of charts. SETTING: Clarós' Otolaryngology and Head and Neck Surgery Clinic in Barcelona, Spain. RESULTS: We recruited a total of 97 patients of whom 89 were treated with CO2 laser. Mean age at first contact was 2.1 months (range: 1.5-6.5). Sex ratio was 10 girls for 1 boy. Eighty percent of patients presented with stridor and 30.3% with recurrent acute laryngitis. Forty percent had associated cutaneous hemangiomas. They received an average of 1.85 laser session (range: 1-4) and 78.5% were healed after a maximum of 2. We had a 100% success rate and 1.1% complication rate (subglottic stenosis). CONCLUSION: CO2 laser is a very effective and recommendable tool against subglottic hemangiomas. To achieve good results traditional laser safety measures should be respected besides some useful surgical tips.

Parotid gland mucoepidermoid carcinoma in a 4-year-old child.

Salivary gland tumors account for less than 5% of the head and neck neoplasms. Among them, mucoepidermoid carcinoma is the most common malignant salivary gland tumor. About 45% of mucoepidermoid carcinomas occur in the parotid gland, and appear around the fifth decade of life, being unusual in children under 10 years. We report a case of a parotid mass arising in a 4-year-old female, who had no lateral adenopathy. After histological examination, the diagnosis was of a low-grade mucoepidermoid carcinoma of the parotid gland. Three years after surgery no recurrence was observed. As a conclusion, although rare, the presence of a parotid mass with progressive growth in children could correspond to a neoplasm.

Tratamiento médicos en la papilomatosis laringotraqueal juvenil recidivante / Medical treatment the juvenile laringeal papilomatoses

En los últimos 150 años se han ensayado multitud de tratamientos para la PLJR, pero hasta el momento presente no existe una opción válida y definitiva para todos los casos. Con el conocimiento progresivo de la enfermedad llegaremos a encontrar el sistema de afrontar y vencer esta terrible enfermedad. Cada vez más, las imbricaciones de la infección de los virus del Papiloma con los procesos malignos de las vías respiratorias, digestivas y genitales son más evidentes, por lo que es lógico creer que el tratamiento válido para la Papilomatosis será de tipo oncológico. La evolución totalmente caprichosa de la enfermedad con curaciones espontáneas o tras pocas sesiones quirúrgicas nos debe hacer recapacitar en cuáles de los casos deben ser incluidos en los tratamientos en estudio. Efectivamente, la mayoría de las medicaciones actualmente en uso suponen una agresión importante en pacientes habitualmente en fase de crecimiento. A nuestro juicio el tratamiento de la PLJR será en un futuro ya muy próximo de tipo médico y vendra de la mano de la inmunología y los inmunofármacos. El rol a desarrollar por la cirugía será el de la desobstrucción de las vías aerodigestivas con un respeto absoluto a las posibles mutilaciones y lesiones residuales iatrógenas