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1.
Am J Otolaryngol ; 44(1): 103669, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36283164

RESUMEN

PURPOSE: The present study assessed the prevalence of audio-vestibular symptoms following SARS-COV-2 infection or COVID-19 vaccination among children, comparing the two groups. A further aim was to evaluate whether children with pre-existing unilateral hearing loss were more prone to adverse events. MATERIALS AND METHODS: This retrospective study included children aged 5-11 years with normal hearing or a proven history of unilateral hearing loss who contracted SARS-CoV-2 or received two doses of COVID-19 vaccine. Tinnitus, hyperacusis, aural fullness, otalgia, otorrhea, new-onset hearing loss, vertigo and dizziness were investigated as possible complications of SARS-CoV-2 infection or the COVID-19 vaccine. RESULTS: This study included 272 children (143 boys, 129 girls), with a mean age of 7.8 ± 2.3 years. Among these, 120 were affected by pre-existing unilateral hearing loss. The most common audio-vestibular symptoms reported by children following SARS-CoV-2 infection and COVID-19 vaccination were aural fullness (33/132, 25 %) and dizziness (5/140, 3.6 %), respectively. All symptoms following COVID-19 vaccination resolved within 24 h. Compared to children who received the COVID-19 vaccine, those infected with SARS-CoV-2 had a higher prevalence of tinnitus (p = 0.009), hyperacusis (p = 0.003), aural fullness (p < 0.001), otalgia (p < 0.001), otorrhea (p < 0.001), and vertigo (p = 0.006). Two girls also experienced new-onset unilateral sensorineural hearing loss following SARS-CoV-2 infection. Children with a known history of unilateral hearing loss did not have a higher prevalence of audio-vestibular symptoms than children with normal hearing. CONCLUSIONS: Our results suggest that the COVID-19 vaccine is safe and can be recommended for children with unilateral hearing loss without fear of possible audio-vestibular sequelae.


Asunto(s)
COVID-19 , Pérdida Auditiva Unilateral , Acúfeno , Masculino , Niño , Femenino , Humanos , Preescolar , Vacunas contra la COVID-19/efectos adversos , COVID-19/complicaciones , COVID-19/epidemiología , COVID-19/prevención & control , SARS-CoV-2 , Mareo/epidemiología , Mareo/etiología , Hiperacusia , Dolor de Oído , Estudios Retrospectivos , Vacunación/efectos adversos , Vértigo
2.
Hum Genet ; 141(3-4): 951-963, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34410490

RESUMEN

In this study, we investigated the association of ACAN variants with otosclerosis, a frequent cause of hearing loss among young adults. We sequenced the coding, 5'-UTR and 3'-UTR regions of ACAN in 1497 unrelated otosclerosis cases and 1437 matched controls from six different subpopulations. The association between variants in ACAN and the disease risk was tested through single variant and gene-based association tests. After correction for multiple testing, 14 variants were significantly associated with otosclerosis, ten of which represented independent association signals. Eight variants showed a consistent association across all subpopulations. Allelic odds ratios of the variants identified four predisposing and ten protective variants. Gene-based tests showed an association of very rare variants in the 3'-UTR with the phenotype. The associated exonic variants are all located in the CS domain of ACAN and include both protective and predisposing variants with a broad spectrum of effect sizes and population frequencies. This includes variants with strong effect size and low frequency, typical for monogenic diseases, to low effect size variants with high frequency, characteristic for common complex traits. This single-gene allelic spectrum with both protective and predisposing alleles is unique in the field of complex diseases. In conclusion, these findings are a significant advancement to the understanding of the etiology of otosclerosis.


Asunto(s)
Otosclerosis , Regiones no Traducidas 3' , Regiones no Traducidas 5' , Agrecanos/genética , Susceptibilidad a Enfermedades , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Humanos , Otosclerosis/genética , Fenotipo , Polimorfismo de Nucleótido Simple
3.
Audiol Neurootol ; 27(1): 64-74, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-33895732

RESUMEN

INTRODUCTION: Sudden sensorineural hearing loss (SSHL) is a relatively frequent disease, but a sensitive marker or a reliable test to identify the underlying cause is still unavailable. Neuroradiology appears to offer the most promising tools, especially magnetic resonance imaging (MRI). In a recent study from our group, we explored the ability of MRI to detect subtle changes in the inner ear compartments by means of a 3D-fluid-attenuated inversion recovery sequence, aiming at identifying 3 distinct MRI patterns (haemorrhagic, inflammatory, brain-labyrinth barrier breakdown). In the present study, we contrasted the MRI patterns at onset with relevant prognostic factors, with the audiological features of each patient's SSHL and with treatment outcomes. METHODS: In this retrospective study, we enrolled 50 adult subjects (54.61 ± 18.26 years) with SSHL. They underwent an MRI within 72 h from admission, and 5 audiological evaluations: at admission, on the 5th day after the start of medical therapy, at the end of the first cycle of hyperbaric oxygen therapy, then 1 and 6 months later. RESULTS: Abnormalities of the MRI signal and/or post-contrast enhancement asymmetry of the cochlea ("pattern+ MRI") correlated with worse audiological outcomes at 1 month, but the different MRI patterns were not correlated with any specific prognostic model, despite rigid protocol settings. However, a significant difference was found for low-tone SSHL, which were always "pattern" negative at MRI (p = 0.01), and for profound SSHL which demonstrated a pattern+ MRI in 80% (p = 0.04). At the onset of SSHL, a pattern+ MRI was found in 29/50 cases (58.0%) and was related with lesser degree of recovery of pure-tone average at 1 month and lesser chance to retain the hearing threshold benefit in the long term. Given the limited numbers of patients enrolled so far, the relative impact of comorbidities on each MRI pattern remains uncertain. At 6 months, we observed a trend of greater and more stable recovery (p = 0.023) and less frequent recurrence of SSHL in patients with a normal MRI. CONCLUSIONS: The 3 observed MRI patterns did not correlate consistently with specific audio-vestibular features or any peculiar aspect of the patient's clinical history. Larger series of patients with SSHL are needed, possibly from multicentric studies.


Asunto(s)
Pérdida Auditiva Sensorineural , Pérdida Auditiva Súbita , Vestíbulo del Laberinto , Adulto , Pérdida Auditiva Sensorineural/complicaciones , Pérdida Auditiva Súbita/diagnóstico por imagen , Pérdida Auditiva Súbita/etiología , Pérdida Auditiva Súbita/terapia , Humanos , Imagen por Resonancia Magnética/métodos , Pronóstico , Estudios Retrospectivos
4.
Int J Audiol ; 59(6): 406-415, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32027195

RESUMEN

Objective: The aim of this study was to evaluate the effect of cochlear implantation (CI) in advanced Ménière's disease (MD).Design: The initial search on PubMed, EMBASE, and Cochrane databases yielded 171 articles; no language restriction was applied.Study sample: A total of 11 articles met the inclusion criteria and were included in this systematic review. Six articles provided patient-level data on improvement in speech recognition testing after CI.Results: The methodological quality of included studies was assessed by examining the study design, level of evidence, method of measurement and adequacy of outcome reporting. A random-effect model was fitted for calculating weighted means. Post-operative improvement in word recognition score (WCS) was 50.8% (95% confidence interval: 34.6-67.1%); general improvement of vestibular symptoms after CI was found in 67% of the pooled patients; when reported in the studies, quality of life (QoL) and tinnitus were also generally improved after CI.Conclusions: CI in advanced MD is a valid option providing good outcomes in terms of speech performances, regardless of the disease duration, uni- or bilaterality, age at implantation, previous therapeutic procedures and stage of activity of MD.


Asunto(s)
Implantación Coclear , Enfermedad de Meniere/cirugía , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Periodo Posoperatorio , Calidad de Vida , Estudios Retrospectivos , Percepción del Habla , Resultado del Tratamiento
5.
ORL J Otorhinolaryngol Relat Spec ; 82(5): 285-294, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32791498

RESUMEN

INTRODUCTION: Atopy and ear, nose and throat (ENT) diseases are frequently associated; however, no clinical tool has been proposed so far to discriminate which patients could be atopic and therefore deserving of a further immunoallergological evaluation. OBJECTIVE: The aim of this study was to assess and validate a set of dichotomous responses suitable for predicting the presence of atopy in adult patients. METHODS: An 11-item questionnaire, i.e., the Atopy Index Inventory (AII), comprised of 4 questions regarding the clinical history for allergic disease and 7 questions evaluating the presence of the most frequent clinical signs affecting allergic patients, was developed and administered to 226 adult subjects (124 atopic subjects and 102 healthy, not atopic subjects). The atopic condition was proven by an immunoallergological evaluation according to the diagnostic criteria of the EAACI guidelines. Internal consistency and clinical validity were tested. RESULTS: In healthy subjects, the first 4 variables of the AII returned a 100% correct response (all answered "no") and were defined as "decisive" responses. In the logistic regression analysis, when decisive items were negative, the atopic condition was confirmed when answering "yes" to at least 3 "probability" items (cut-off = 2.69). The difference in AII scores between allergic and healthy group was significant using the Mann-Whitney U test (p < 0.0001). The sensitivity and specificity of the AII were 0.97 and 0.91, respectively, with a true predictive value of 0.92 and a false predictive value of 0.97. The ROC curve showed an area of 0.94, with an OR of 0.88 (95% CI 0.87-0.97, p = 0.0001). The internal consistency as determined by the Cronbach α coefficient was 0.88. CONCLUSION: The AII has been proven to be a brief, simple and sufficiently accurate tool for screening ENT patients in search of atopic individuals and to allow their clinical management.


Asunto(s)
Hipersensibilidad , Inmunoglobulina E , Adulto , Humanos , Curva ROC , Sensibilidad y Especificidad , Encuestas y Cuestionarios
6.
Mol Genet Genomics ; 294(4): 1001-1006, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30968248

RESUMEN

Otosclerosis is a common form of hearing loss (HL) due to abnormal remodeling of the otic capsule. The genetic causes of otosclerosis remain largely unidentified. Only mutations in a single gene, SERPINF1, were previously published in patients with familial otosclerosis. To unravel the contribution of genetic variation in this gene to otosclerosis, this gene was re-sequenced in a large population of otosclerosis patients and controls. Resequencing of the 5' and 3' UTRs, coding regions, and exon-intron boundaries of SERPINF1 was performed in 1604 unrelated otosclerosis patients and 1538 unscreened controls, and in 62 large otosclerosis families. Our study showed no enrichment of rare variants, stratified by type, in SERPINF1 in patients versus controls. Furthermore, the c.392C > A (p.Ala131Asp) variant, previously reported as pathogenic, was identified in three patients and four controls, not replicating its pathogenic nature. We could also not find evidence for a pathogenic role in otosclerosis for 5' UTR variants in the SERPINF1-012 transcript (ENST00000573763), described as the major transcript in human stapes. Furthermore, no rare variants were identified in the otosclerosis families. This study does not support a pathogenic role for variants in SERPINF1 as a cause of otosclerosis. Therefore, the etiology of the disease remains largely unknown and will undoubtedly be the focus of future studies.


Asunto(s)
Proteínas del Ojo/genética , Factores de Crecimiento Nervioso/genética , Otosclerosis/genética , Análisis de Secuencia de ADN/métodos , Serpinas/genética , Regiones no Traducidas 3' , Regiones no Traducidas 5' , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Linaje
7.
Genet Med ; 21(5): 1199-1208, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-30287925

RESUMEN

PURPOSE: To characterize new molecular factors implicated in a hereditary congenital facial paresis (HCFP) family and otosclerosis. METHODS: We performed exome sequencing in a four-generation family presenting nonprogressive HCFP and mixed hearing loss (HL). MEPE was analyzed using either Sanger sequencing or molecular inversion probes combined with massive parallel sequencing in 89 otosclerosis families, 1604 unrelated affected subjects, and 1538 unscreened controls. RESULTS: Exome sequencing in the HCFP family led to the identification of a rare segregating heterozygous frameshift variant p.(Gln425Lysfs*38) in MEPE. As the HL phenotype in this family resembled otosclerosis, we performed variant burden and variance components analyses in a large otosclerosis cohort and demonstrated that nonsense and frameshift MEPE variants were significantly enriched in affected subjects (p = 0.0006-0.0060). CONCLUSION: MEPE exerts its function in bone homeostasis by two domains, an RGD and an acidic serine aspartate-rich MEPE-associated (ASARM) motif inhibiting respectively bone resorption and mineralization. All variants associated with otosclerosis are predicted to result in nonsense mediated decay or an ASARM-and-RGD-truncated MEPE. The HCFP variant is predicted to produce an ASARM-truncated MEPE with an intact RGD motif. This difference in effect on the protein corresponds with the presumed pathophysiology of both diseases, and provides a plausible molecular explanation for the distinct phenotypic outcome.


Asunto(s)
Proteínas de la Matriz Extracelular/genética , Parálisis Facial/congénito , Glicoproteínas/genética , Otosclerosis/genética , Fosfoproteínas/genética , Adulto , Huesos/metabolismo , Proteínas de la Matriz Extracelular/metabolismo , Parálisis Facial/etiología , Parálisis Facial/genética , Parálisis Facial/metabolismo , Familia , Femenino , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Variación Genética/genética , Glicoproteínas/metabolismo , Pérdida Auditiva/genética , Heterocigoto , Humanos , Masculino , Linaje , Fenotipo , Fosfoproteínas/metabolismo , Secuenciación del Exoma/métodos
8.
BMC Infect Dis ; 19(1): 652, 2019 Jul 22.
Artículo en Inglés | MEDLINE | ID: mdl-31331274

RESUMEN

BACKGROUND: Congenital Cytomegalovirus (cCMV) is the most common cause of non-genetic hearing loss in childhood. A newborn hearing screening program (NHSP) is currently running in Italy, but no universal cCMV nor statewide hearing-targeted CMV screening programs have been implemented yet. This observational monocentric study was aimed at estimating the rate of cCMV infections identified by CMV-DNA analysis on Dried Blood Spots (DBS) samples in deaf children identified via NHSP in Northern Italy in the period spanning from 2014 to 2018. METHODS: Children with a confirmed diagnosis of deafness and investigated for CMV-DNA by nucleic acid extraction and in-house polymerase-chain reaction (PCR) on stored newborns screening cards (DBS-test) were included in this study. Deafness was defined by a hearing threshold ≥20 decibel (dB HL) by Auditory Brainstem Responses (ABR); all investigated DBS samples were collected within 3 days of life. RESULTS: Overall, 82 children were included (median age: 3.4 months; lower-upper quartiles: 2-5.3 months; males: 60.9%). Most of them (70.7%) presented bilateral hearing loss with a symmetrical pattern in 79.3% of the cases. ABR thresholds were ≥ 70 dB HL (severe/profound deafness) in 46.5% of children. Among all tested children, 6.1% resulted positive for cCMV. The rate of severe/profound deafness was statistically higher in children with cCMV infection. CONCLUSIONS: The addition of DBS-test to the NHSP allowed the identification, in their first months of life, of a cCMV infection in 6.1% of children who had failed NHS. The introduction of a targeted CMV screening strategy could help clinicians in the differential diagnosis and in the babies' management. DBS samples can be considered a "universal newborns biobank": their storage site and duration should be the subject of political decision-making.


Asunto(s)
Infecciones por Citomegalovirus/diagnóstico , Pruebas con Sangre Seca/métodos , Pérdida Auditiva/diagnóstico , Tamizaje Neonatal/métodos , Citomegalovirus/genética , Infecciones por Citomegalovirus/sangre , Femenino , Pérdida Auditiva/virología , Pruebas Auditivas , Humanos , Lactante , Recién Nacido , Italia , Masculino , Reacción en Cadena de la Polimerasa
9.
Audiol Neurootol ; 24(4): 166-173, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31390618

RESUMEN

OBJECTIVE: To describe clinical and imaging findings in a group of patients affected by nonsyndromic deafness A9 (DFNA9), using advanced magnetic resonance imaging (MRI) with 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) sequence. METHOD: A retrospective case review was conducted in a tertiary referral center in Italy. Four sequential adult DFNA9-affected patients, who had undergone MRI at our Department between January 2017 and June 2018, were enrolled (male = 2, female = 2; median age: 65.6 years; 8 diseased ears analyzed). Three patients were relatives; the fourth was unrelated. The main outcome measures - age, sex, records of audiological and vestibular testing, genetic assessment, MRI findings - were analyzed. RESULTS: All subjects suffered from bilateral progressive sensorineural hearing loss, more severely at the high frequencies and with a typical clinical pattern of bilateral chronic degenerative cochleovestibular deficit. Aural fullness was reported at the onset of the disease. All patients revealed a pathogenic heterozygous mutation in the Limulus factor C, Coch-5b2 and Lgl1 domain of cochlin. None of the patients showed a significant vestibular and cochlear endolymphatic hydrops at MRI, while high bilateral contrast enhancement on 4-h delayed postcontrast 3D FLAIR sequence was observed in all ears. CONCLUSIONS: Increased perilymph enhancement on 4-h delayed postcontrast 3D FLAIR sequence is the common imaging feature of DFNA9 ears, suggesting that blood-labyrinthine barrier breakdown may play the main role in the pathophysiology of this disease. Significant hydrops has been excluded by MRI. This finding might be clinically useful in differentiating DFNA9 disease from other pathologies with similar clinical findings like Ménière's disease.


Asunto(s)
Sordera/diagnóstico por imagen , Proteínas de la Matriz Extracelular/genética , Pérdida Auditiva Sensorineural/diagnóstico por imagen , Mutación , Fenotipo , Adulto , Anciano , Sordera/genética , Femenino , Pérdida Auditiva Sensorineural/genética , Heterocigoto , Humanos , Italia , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Perilinfa/diagnóstico por imagen , Estudios Retrospectivos , Vestíbulo del Laberinto/diagnóstico por imagen
10.
J Am Coll Nutr ; 37(2): 149-153, 2018 02.
Artículo en Inglés | MEDLINE | ID: mdl-29087236

RESUMEN

OBJECTIVE: Since 1930, dietary modification has been proposed as adjunct treatment in Ménière's disease (MD) with different and controversial results. We report the case of a 42-year-old female suffering from definite MD and intermittent seasonal allergic rhino-conjunctivitis because it highlights the importance of evaluating the different combinations of defined causative elements in an atopic patient with MD. METHODS: An immunological and audiological evaluation was performed, including pure-tone, speech, and immittance audiometry; glycerol dehydration test; bithermal caloric testing; video head impulse test; cervical vestibular evoked myogenic potentials; static posturography; and Dizziness Handicap Inventory questionnaire. RESULTS: A milk-free diet was crucial to relief from MD symptoms and a cow's milk challenge test was able to evoke them but vestibukar symptoms persist. CONCLUSIONS: The effect of dietary modification was evident only after specific immunotherapies against other allergens. This highlights the importance of evaluating different combinations of defined causative elements in the allergic treatment of MD.


Asunto(s)
Enfermedad de Meniere/dietoterapia , Enfermedad de Meniere/inmunología , Hipersensibilidad a la Leche , Proteínas de la Leche/inmunología , Adulto , Animales , Bovinos , Femenino , Humanos , Inmunoterapia , Sustitutos de la Leche/administración & dosificación
13.
Eur Arch Otorhinolaryngol ; 275(8): 1987-1993, 2018 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-29971494

RESUMEN

OBJECTIVE: To report neuroradiologic findings, surgical strategies and clinical and audiological results in a series of children with CHARGE syndrome (CS) who had been evaluated for cochlear implantation (CI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral university hospital. PATIENTS: Eight profoundly deafened children with CS were included. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre- and post-operatively. Neuroradiological and intraoperative findings, surgical planning, and post-operative complications were analyzed. RESULTS: Six children were profoundly deaf from birth and 2 had progressive hearing loss to profound levels. Cochlear nerve deficiency (CND) was noted in 5 out of the 6 patients with congenital sensorineural hearing loss (SNHL). Seven children underwent CI. Surgery was performed using standard transmastoid facial recess approach in 3 ears, subtotal petrosectomy in 3, and transmastoid single-slit labyrinthotomy in one. Temporary facial palsy occurred in one patient. In the group of patients with congenital SNHL, 2 children benefitted from CI and developed spoken language; the remaining 3 children obtained improved access to environmental sounds and used signs and gestures as their main mode of communication. The two patients with progressive SNHL had preoperative verbal language and continued to use verbal language after CI. CONCLUSIONS: The constant presence of temporal bone anomalies in children with CS requires surgical expertise in performing non-standard approaches for safe and effective CI. Patients with progressive SNHL and normal cochlear nerves had satisfactory results with CI. Limited benefits have been observed in presence of CND.


Asunto(s)
Síndrome CHARGE/cirugía , Implantación Coclear , Sordera/cirugía , Adolescente , Síndrome CHARGE/complicaciones , Niño , Preescolar , Nervio Coclear/anomalías , Femenino , Pérdida Auditiva Sensorineural/congénito , Pérdida Auditiva Sensorineural/cirugía , Humanos , Lactante , Desarrollo del Lenguaje , Masculino , Estudios Retrospectivos , Lengua de Signos , Hueso Temporal/anomalías
14.
Audiol Neurootol ; 22(4-5): 226-235, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-29232662

RESUMEN

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed.


Asunto(s)
Percepción Auditiva/fisiología , Implantación Coclear , Implantes Cocleares , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Ruido , Sonido , Percepción del Habla/fisiología , Adulto Joven
15.
Trop Anim Health Prod ; 47(6): 1095-100, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25912973

RESUMEN

The objective of this study was to evaluate the effect of five feeding strategies on calf weaning weight, and cow milk production and composition in Brazilian Holstein × Zebu cows. A total of 60 cows and their calves were allocated to each of five treatments. Cows in treatments 1, 2 and 3 were milked for 270 days and cows in treatments 4 and 5 were milked for 180 days. Calves in treatment 1 (CON) were not supplemented with concentrate whereas calves from treatment 2 (CLPN) received 1 kg of concentrate daily from 90 to 270 days of age and calves from treatment 3 received 1 kg of concentrated from 180 to 270 days of age. Calves in treatment 4 (CCPS) were supplemented with 1 kg of concentrate from 90 to 180 days of age and calves in treatment 5 (CLPS) were supplemented with 1 kg of concentrate from 90 to 270 days of age. Calves from the CLPS treatment had greater milk and protein intakes (P < 0.05) and greater growth rate than calves from the other treatments. Our results indicate that the traditional system of feeding calves with no concentrate results in a weight gain of 600 g/day. The CLPS treatment produced calves with the highest live weight and growth rate. The nutritional strategy with restricted supply of milk for the calves with concomitantly short-term concentrate supplementation does not improve performance of calves but did increase feed costs.


Asunto(s)
Alimentación Animal , Crianza de Animales Domésticos , Bovinos/fisiología , Lactancia , Leche/metabolismo , Fenómenos Fisiológicos Nutricionales de los Animales , Animales , Animales Lactantes , Peso Corporal , Brasil , Cruzamientos Genéticos , Suplementos Dietéticos , Femenino , Masculino , Aumento de Peso
16.
Eur Arch Otorhinolaryngol ; 271(1): 3-13, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23404468

RESUMEN

The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.


Asunto(s)
Implantes Auditivos de Tronco Encefálico , Sordera/cirugía , Pérdida Auditiva/cirugía , Implantes Cocleares , Contraindicaciones , Pérdida Auditiva Central/cirugía , Humanos , Neuroma Acústico/cirugía , Osificación Heterotópica , Otosclerosis/cirugía , Resultado del Tratamiento , Enfermedades del Nervio Vestibulococlear/patología
17.
Children (Basel) ; 11(3)2024 Mar 09.
Artículo en Inglés | MEDLINE | ID: mdl-38539359

RESUMEN

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL.

18.
J Clin Med ; 13(11)2024 Jun 04.
Artículo en Inglés | MEDLINE | ID: mdl-38893014

RESUMEN

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment.

19.
Artículo en Inglés | MEDLINE | ID: mdl-38613183

RESUMEN

OBJECTIVE: To assess the prevalence of otitis media with effusion (OME) among children who attended a tertiary level audiologic center 2 and 3 years after the COVID-19 lockdown, and to determine the impact of temporary interruption of day care center attendance on chronic OME. STUDY DESIGN: Retrospective study. SETTING: Tertiary level referral audiologic center. METHODS: We assessed the prevalence of OME among children aged 6 months to 12 years in 3 different periods (May-June 2022, January-February 2023, and May-June 2023) and compared the results with those of the corresponding periods before the COVID-19 lockdown. We also compared the disease resolution rates between a subgroup of children with chronic OME who interrupted day care center attendance for a 2-month period (Subgroup A) and a similar subgroup who continued attending day care centers (Subgroup B). RESULTS: The prevalence of OME was 38.5% (138/358) in May-June 2022, 51.9% (193/372) in January-February 2023, and 40.9% (149/364) in May-June 2023. No significant prevalence differences were observed between the periods May-June 2019, May-June 2022, and May-June 2023 (P = .78), and between the periods January-February 2020 and January-February 2023 (P = .93). At the May-June 2023 assessment, the children belonging to Subgroup A presented a greater rate of disease resolution (85.7%, 18/21) than the children belonging to Subgroup B (32%, 8/25, P < .001). CONCLUSION: This study suggests that the prevalence of OME has returned to prelockdown levels, and that interrupting day care center attendance for a 2-month period could be effective in resolving most cases of chronic OME.

20.
Audiol Res ; 14(3): 401-411, 2024 Apr 26.
Artículo en Inglés | MEDLINE | ID: mdl-38804458

RESUMEN

Despite the spread of novel-generation cochlear-implant (CI) magnetic systems, access to magnetic resonance imaging (MRI) for CI recipients is still limited due to safety concerns. The aim of this study is to assess and record the experiences of Hires Ultra 3D (Advanced Bionics) recipients who underwent an MRI examination. A multicentric European survey about this topic was conducted focusing on safety issues, and the results were compared with the current literature. We collected a total of 65 MRI scans performed in 9 otologic referral centers for a total of 47 Hires Ultra 3D recipients, including, for the first time, 2 children and 3 teenagers. Preventive measures were represented by scanning time and sedation for children. Head wrapping was used in eight cases, and six of the eight cases received local anesthesia, even if both measures were not needed. Only three patients complained of pain (3/65 examinations, 4.6%) due to the tight head bandage, and one of the three cases required MRI scan interruption. No other adverse events were reported. We believe that these results should encourage MRI execution in accordance with manufacturer recommendations for Ultra 3D recipients.

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