Production and evaluation of a Spanish version of the LittlEARS(®) Auditory Questionnaire for the assessment of auditory development in children.

BACKGROUND AND AIMS: To adapt the LittlEARS(®) Auditory Questionnaire (LEAQ) into Spanish and evaluate the psychometric properties of the Spanish version of the questionnaire. METHODS: The LEAQ was translated into Spanish by a back-translation design. Following the Guidelines for Adapting Tests of the International Test Commission (ITC), for the first time with the LEAQ adaptations, two qualitative methods were used to evaluate the translated version of the LEAQ: an expert appraisal method followed by cognitive interviewing. Having improved the Spanish version of the LEAQ with these evaluations, a psychometric analysis was conducted. 215 parents of children with normal hearing aged between 1.7 and 24.0 months participated in the study. Corrected item-total correlations were calculated to analyze to what extent items distinguish levels of auditory development of assessed children. Cronbach's alpha coefficient - to evaluate internal consistency across items - was also calculated. To obtain validity evidence, correlations between item-total score and age were calculated. A non-linear regression model was also estimated to obtain normative data for expected and minimum value of total scores from the questionnaire according to age. RESULTS: Expert appraisal and cognitive interviewing pointed out some translation errors and difficulties parents had while responding to the Spanish LEAQ. Such errors and difficulties were fixed in the Spanish LEAQ version applied for psychometric analysis. Corrected item-total correlations ranged from 0.15 to 0.75. Cronbach's alpha coefficient value was 0.92, indicating that the measurements are highly reliable. The value of the correlation between total scores and age was 0.86 (p<001). The regression analysis conducted to obtain normative data shows that 79% of the variation in the total scores can be explained by age. CONCLUSIONS: The results of psychometric analyses provide evidence supporting the use of the Spanish version of the LEAQ as a valid and culturally appropriate tool to assess the development of auditory behaviour in Spanish children who are 24 months old or younger.

Cochlear implantation in patients with inner ear bone malformations with posterior labyrinth involvement: an exploratory study.

Inner ear bone malformations are one cause of profound sensorineural hearing loss. This investigation focused on those affecting the posterior labyrinth, especially enlarged vestibular aqueduct syndrome, which is associated with fluctuating and progressive hearing loss. The objectives of this study were to analyze the behavior of the electrical stimulation, auditory functionality and linguistic development in patients with inner ear malformations involving the posterior labyrinth. The study included ten patients undergoing cochlear implantation (cases: five with enlarged vestibular aqueduct, two with vestibular aqueduct stenosis/aplasia, and three with semicircular canal disorders). Post-implantation, data were gathered on the electrical stimulation threshold and maximum comfort levels and on the number of functioning electrodes. Evaluation of Auditory Responses to Speech (EARS) subtests were used to assess auditory functionality and language acquisition at 6, 12, and 24 months post-implantation. Results were compared with findings in a control group of 28 cochlear implantation patients without these malformations. No significant differences were found between case and control groups in electrical stimulation parameters; auditory functionality subtest scores were lower in cases than controls, although the difference was only statistically significant for some subtests. In conclusion, cochlear implantation patients with posterior labyrinth bone malformations and profound hearing loss, including those with enlarged vestibular aqueduct syndrome, showed no significant difference in electrical stimulation threshold with controls. Although some auditory functionality test results were lower in cases than in controls, cochlear implantation appears to be beneficial for all patients with these malformations.

Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement.

OBJECTIVE: To study electrical stimulation, auditory functionality, and language development in patients with inner ear malformations involving the anterior labyrinth who underwent cochlear implantation. STUDY DESIGN: Retrospective case review. SETTING: Reference hospital for cochlear implantation. PATIENTS: Review of 14 cases of severe hearing loss with major (common cavity deformity and cochlear hypoplasia) or minor (e.g., incomplete partition and basal turn aplasia) malformations. INTERVENTIONS: After cochlear implantation, data were gathered on the threshold (THR) and maximum comfort level (MCL) of the electrical stimulation and the number of functioning electrodes. Auditory responses to speech (EARS protocol) subtests were used to evaluate auditory functionality and language acquisition at 6, 12, and 24 months post-implantation. Tests used were: LIP profile, MTP (3, 6 and 12 words), OLD (open set test) and CLD (close set test). Results were compared with findings in a control group of 28 cochlear implantation patients without these malformations and with congenital hearing loss. RESULTS: The mean THR was 11.02µC in patients with malformations versus 3.5µC in those without, a significant difference. The THR also significantly differed between groups with major and minor malformations. Fewer functioning electrodes were used in patients with malformations. Auditory functionality scores were best in controls than in patients with malformations, who scored ≤50%, finding the lowest scores in those with major malformations. CONCLUSION: Patients with inner ear malformations undergoing cochlear implantation require greater stimuli to obtain an auditory response and have worse auditory functionality outcomes; these differences are greater in those with major versus minor malformations Nevertheless, cochlear implantation appears to be beneficial for all patients with these malformations to a greater or lesser extent.

Absceso secundario a tofo gotoso: una causa rara de tumoración en la región parotídea / Abscess secondary to tophaceous gout: a rare cause of parotid region tumour

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Incidencia, estancia hospitalaria y factores de riesgo asociados a la aparición de fístula faringocutánea poslaringectomía / Incidence, hospital stay and risk factors associated with the occurrence of pharyngo-cutaneous fistula after total laryngectomy

Introducción y objetivos: La fístula faringocutánea (FFC) constituye uno de los principales problemas para los pacientes sometidos a laringectomía total. Son numerosos los factores de riesgo descritos y asociados a la aparición de la FFC, sin embargo algunos de ellos se encuentran en discusión. El objetivo es analizar la incidencia de la FFC, la estancia hospitalaria y los factores de riesgo asociados en los pacientes intervenidos de laringectomía total en un hospital de segundo nivel. Métodos: Estudio analítico observacional en una cohorte retrospectiva. Se incluyeron todos los pacientes intervenidos de laringectomía total entre los años 2006 y 2011. Se tuvieron en cuenta variables sociodemográficas, clínicas y la mayoría de los factores de riesgo estudiados en la literatura por otros autores (traqueostomía, estadificación tumoral, quimio/radioterapia previa, etc.). Los análisis estadísticos se llevaron a cabo mediante el programa SPSS® v.15.0 y el Epidat® v.3.1. Resultados: Se analizaron 70 laringectomías totales. La incidencia de FFC en el período de estudio fue del 48,6%. Hubo diferencias significativas entre la estancia hospitalaria media de los pacientes con y sin FFC. Se encontró como único factor de riesgo para la aparición de la FFC la presencia de adenopatías lo corregionales afectadas [RR= 1,60 (1,006-2,54)]. Conclusión: La aparición de FFC se encuentra asociada a la presencia de adenopatías locorregionales afectadas, y no se asocia con la traqueostomía, la administración de radio/quimioterapia previa a la intervención o la localización tumoral (AU)

Introduction and objectives: Pharyngo-cutaneous fistula (PCF) is one of the main problems for patients undergoing total laryngectomy. There are many risk factors described and associated with the appearance of PCF; however, some of these are currently under discussion. The aim of this work is to analyse the incidence of PCF, hospital stay and associated risk factors in patients undergoing total laryngectomy in a secondary hospital. Methods: This was an observational study in a retrospective cohort. We included all patients who underwent total laryngectomy between 2006 and 2011. We considered sociodemographic and clinical items, and most of the risk factors studied in the literature by other authors (tracheostomy, tumour stage, chemotherapy or radiotherapy before surgery, etc.). Statistical analyses were performed using SPSS® 15.0 and Epidat® 3.1. Results: We analysed 70 total laryngectomies. The incidence of PCF in the time period studied was 48.6%. There were significant differences between the mean hospital stay of patients with and without PCF. Affected locoregional lymph nodes were found to be the only risk factor for the occurrence of PCF [RR=1.60 (1.006-2.54)]. Conclusion: The incidence of PCF is associated with the presence of affected locoregional lymph nodes. It is not associated with tracheostomy, administration of radio/chemotherapy before surgery or tumour location (AU)

[Incidence, hospital stay and risk factors associated with the occurrence of pharyngo-cutaneous fistula after total laryngectomy]. / Incidencia, estancia hospitalaria y factores de riesgo asociados a la aparición de fístula faringocutánea poslaringectomía.

INTRODUCTION AND OBJECTIVES: Pharyngo-cutaneous fistula (PCF) is one of the main problems for patients undergoing total laryngectomy. There are many risk factors described and associated with the appearance of PCF; however, some of these are currently under discussion. The aim of this work is to analyse the incidence of PCF, hospital stay and associated risk factors in patients undergoing total laryngectomy in a secondary hospital. METHODS: This was an observational study in a retrospective cohort. We included all patients who underwent total laryngectomy between 2006 and 2011. We considered sociodemographic and clinical items, and most of the risk factors studied in the literature by other authors (tracheostomy, tumour stage, chemotherapy or radiotherapy before surgery, etc.). Statistical analyses were performed using SPSS(®) 15.0 and Epidat(®) 3.1. RESULTS: We analysed 70 total laryngectomies. The incidence of PCF in the time period studied was 48.6%. There were significant differences between the mean hospital stay of patients with and without PCF. Affected locoregional lymph nodes were found to be the only risk factor for the occurrence of PCF [RR=1.60 (1.006-2.54)]. CONCLUSION: The incidence of PCF is associated with the presence of affected locoregional lymph nodes. It is not associated with tracheostomy, administration of radio/chemotherapy before surgery or tumour location.

Common cavity and custom-made electrodes: speech perception and audiological performance of children with common cavity implanted with a custom-made MED-EL electrode.

OBJECTIVE: Few studies exist on children with common cavity, fewer still on their long-term audiological development after having received a cochlear implant. Our goal was to observe and report the long-term audiological progress of children with common cavity who were implanted with a custom-made electrode. METHODS: In this longitudinal, multi-center study, 19 children were implanted with a MED-EL custom-made electrode via either single slit cochleostomy or double posterior labyrinthotomy. We observed their audiological development with a test battery consisting of Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), and Ling 6-Sounds tests. We tested the children 1 month prior to the surgery; at first fitting; at 1, 3, 6, 12, and 18 months post first-fitting; at 2 years after first-fitting; and, whenever possible, at 3, 4, and 5 years after first-fitting. RESULTS: Children with common cavity tend to steadily and significantly improve their audiological skills over time. This development may, however, be highly individual; probably in part due to relatively high levels of additional needs. Parents should be counseled to establish realistic post-implantation expectations. Surgically, contrary to our expectations, we cannot confirm that double posterior labyrinthotomy reduces intracochlear electrode movement or that the MED-EL custom electrode leads to fewer incidences of intra- or post-implantation complications. CONCLUSIONS: Cochlear implantation is a safe and effective treatment option in children with common cavity. The majority of children with CC derive significant audiological benefit from implantation. Intra- and post-surgical complications, while serious, and be dealt with effectively in most cases.

Manifestaciones otorrinolaringológicas de las vasculitis sistémicas / Otolaryngologic manifestations of systemic vasculitis

Las vasculitis sistémicas son un grupo muy heterogéneo de enfermedades de diversas etiologías y manifestaciones. En general, la clínica se deriva de la isquemia producida por la inflamación vascular de la cual depende el órgano afecto. Se clasifican según el consenso de la conferencia de Chapel Hill. Se pueden presentar con relativa frecuencia como manifestaciones otorrinolaringológicas tanto en su debut como a lo largo de su evolución, por lo tanto deben incluirse en el diagnóstico diferencial del especialista en otorrinolaringología en pacientes de difícil control o presentación atípica de manifestaciones ORL. Es nuestra intención revisar la clínica otorrinolaringológica más frecuente en cada una de estas enfermedades(AU)

Systemic vasculitis is a heterogeneous group of diseases of various aetiologies and manifestations. In general, the clinical results derive from ischemia caused by vascular inflammation, which depends on the organ affected. Such vasculitis cases are classified according to the classification of the Chapel Hill conference. They can present with relative frequency as ENT manifestations in both their debut and throughout their evolution. Consequently, the ENT specialist should include them in the differential diagnosis in patients with ENT manifestations that are difficult to control or of atypical presentation. Our objective was to review the most common ENT clinical signs and symptoms in each of these diseases(AU)

Otolaryngologic manifestations of systemic vasculitis.

Systemic vasculitis is a heterogeneous group of diseases of various aetiologies and manifestations. In general, the clinical results derive from ischemia caused by vascular inflammation, which depends on the organ affected. Such vasculitis cases are classified according to the classification of the Chapel Hill conference. They can present with relative frequency as ENT manifestations in both their debut and throughout their evolution. Consequently, the ENT specialist should include them in the differential diagnosis in patients with ENT manifestations that are difficult to control or of atypical presentation. Our objective was to review the most common ENT clinical signs and symptoms in each of these diseases.

A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy.

Autosomal recessive nonsyndromic hearing impairment (NSHI) is a heterogeneous condition, for which 53 genetic loci have been reported, and 29 genes have been identified to date. One of these, OTOF, encodes otoferlin, a membrane-anchored calcium-binding protein that plays a role in the exocytosis of synaptic vesicles at the auditory inner hair cell ribbon synapse. We have investigated the prevalence and spectrum of deafness-causing mutations in the OTOF gene. Cohorts of 708 Spanish, 83 Colombian, and 30 Argentinean unrelated subjects with autosomal recessive NSHI were screened for the common p.Gln829X mutation. In compound heterozygotes, the second mutant allele was identified by DNA sequencing. In total, 23 Spanish, two Colombian and two Argentinean subjects were shown to carry two mutant alleles of OTOF. Of these, one Colombian and 13 Spanish subjects presented with auditory neuropathy. In addition, a cohort of 20 unrelated subjects with a diagnosis of auditory neuropathy, from several countries, was screened for mutations in OTOF by DNA sequencing. A total of 11 of these subjects were shown to carry two mutant alleles of OTOF. In total, 18 pathogenic and four neutral novel alleles of the OTOF gene were identified. Haplotype analysis for markers close to OTOF suggests a common founder for the novel c.2905_2923delinsCTCCGAGCGCA mutation, frequently found in Argentina. Our results confirm that mutation of the OTOF gene correlates with a phenotype of prelingual, profound NSHI, and indicate that OTOF mutations are a major cause of inherited auditory neuropathy.

Estudio comparativo entre potenciales evocados auditivos de estado estable, potenciales evocados auditivos de tronco cerebral y audiometría tonal liminar / Comparative Study Between Auditory Steady-State Responses, Auditory brain-stem responses and liminar tonal audiometry

Objetivos: Los potenciales evocados auditivos de estado estable (PEAee) con frecuencias de modulación de 70-110 Hz son una nueva técnica para explorar la audición. Nos proponemos valorar su aportación al diagnóstico audiológico. Material y método: Se estudió las diferentes aportaciones al diagnóstico del umbral auditivo de los PEAee y los potenciales evocados auditivos de tronco cerebral (PEATC) en respuesta al clic. Además se estudió las diferencias entre estos umbrales y los de la audiometría tonal liminar (ATL). Se analiza los coeficientes de correlación entre los umbrales obtenidos en PEAee y los de la ATL. Resultados: Los PEAee permitieron detectar restos de audición que no eran detectados mediante PEATC transitorios. Se encontraron diferencias de ­13,750 dB HL (­22,291 a ­5,209) y de ­13,250 dB HL (­19,163 a ­7,337) entre los valores registrados mediante PEAee para las frecuencias de 500 y 1.000 Hz y los umbrales de la ATL para esas mismas frecuencias. Se encontraron diferencias de 1,625 dB HL (­6,967 a 10,217) y de ­2,875 dB HL (­7,446 a 1,696) entre los valores estimados en los PEAee y los umbrales de la ATL para las frecuencias de 500 y 1.000 Hz. Los coeficientes de correlación fueron estadísticamente muy significativos (p = 0,01) entre los umbrales registrados y los estimados en los PEAee para las portadoras de 500 y 1.000 Hz y los de la ATL para las mismas frecuencias. Conclusiones: Los PEAee con frecuencias de modulación entre 70 y 110 Hz constituyen una nueva técnica de exploración de la audición que es más específica en frecuencia que los PEATC en respuesta al clic. La respuesta obtenida no se modificará por el estado de conciencia y además es una técnica doblemente objetiva. Los umbrales obtenidos en los PEAee permiten realizar una estimación del umbral de audición

Objetives: Auditory steady-state responses (ASSR) using frequencies of modulation between 70-110 Hz are a new auditive exploration technique. The aim of the study was to evaluate the contribution of the ASSR to diagnostic of the audition. Material and method: Different aportations of auditory steady-states responses (ASSR) and auditory brain-stem responses (ABR) to diagnostic of threshold of audition were studied Differences between these thresholds and thresholds obtained by liminar tonal audiometry (LTA) were studied too. Correlations between thresholds obtained by ASSR and LTA were studied. Results: ASSR detected rest of audition that transients ABR did not detect. Differences about ­13.750 dB HL (­5.209 to ­22.291) and ­13.250 dB HL (­7.337 to ­19.163) were found between registered values for carriers of 500 and 1000 Hz and the thresholds by LTA for these carriers. Differences about 1.625 dB HL (­6.967 to 10.217) and ­2.875 dB HL (­7.446 to 1.696) were found between estimations for the carries of 500 and 1000 Hz and thresholds by TLA. Stadistically very signifficant (P=.01) coefficients of correlation were found between registered and estimated thresholds by ASSR for carrier of 500 and 1000 Hz and threshold by TLA for these frecuencies. Conclusions: Auditory steady-state responses (ASSR) using frequencies of modulation between 70-110 Hz are a new auditive technique of exploration. This stimulus is more frecuency-specific than clicks for auditory brain-stem responses (ABR). Response is not modificated by steady of consciousness. The technique is doublely objetive. Thresholds obtained by ASSR permits to estimation of the audition threshold

[Comparative study between auditory steady-state responses, auditory brain-stem responses and liminar tonal audiometry]. / Estudio comparativo entre potenciales evocados auditivos de estado estable, potenciales evocados auditivos de tronco cerebral y audiometría tonal liminar.

OBJECTIVES: Auditory steady-state responses (ASSR) using frequencies of modulation between 70-110 Hz are a new auditive exploration technique. The aim of the study was to evaluate the contribution of the ASSR to diagnostic of the audition. MATERIAL AND METHOD: Different aportations of auditory steady-states responses (ASSR) and auditory brain-stem responses (ABR) to diagnostic of threshold of audition were studied Differences between these thresholds and thresholds obtained by liminar tonal audiometry (LTA) were studied too. Correlations between thresholds obtained by ASSR and LTA were studied. RESULTS: ASSR detected rest of audition that transients ABR did not detect. Differences about -13.750 dB HL (-5.209 to -22.291) and -13.250 dB HL (-7.337 to -19.163) were found between registered values for carriers of 500 and 1000 Hz and the thresholds by LTA for these carriers. Differences about 1.625 dB HL (-6.967 to 10.217) and -2.875 dB HL (-7.446 to 1.696) were found between estimations for the carries of 500 and 1000 Hz and thresholds by TLA. Statistically very significant (P=.01) coefficients of correlation were found between registered and estimated thresholds by ASSR for carrier of 500 and 1000 Hz and threshold by TLA for these frequencies. CONCLUSIONS: Auditory steady-state responses (ASSR) using frequencies of modulation between 70-110 Hz are a new auditive technique of exploration. This stimulus is more frequency-specific than clicks for auditory brain-stem responses (ABR). Response is not modificated by steady of consciousness. The technique is doublely objective. Thresholds obtained by ASSR permits to estimation of the audition threshold.

[Dermoid cyst of the floor of the mouth]. / Quiste dermoide de suelo de boca.

Dermoid cysts are congenital lesions composed of tissues with different origins: ectoblastic, mesoblastic, or endoblastic, caused by a defect in the fusion of the embryonic lateral mesenchymatic mass. A true dermoid cyst is a cavity covered with epithelium showing keratinisation and presenting identifiable dermal appendices. We present a case of a male patient aged 53 years presenting tumouration on the floor of the mouth with an evolution of about 30 years. A bibliographic revision of this pathology is also presented.

Quiste dermoide de suelo de boca / Dermoid cyst of the floor of the mouth

Los quistes dermoides son lesiones congénitas constituidas por tejidos de varias procedencias: ectoblástico, mesoblástico o endoblástico. Tienen su origen en un defecto de fusión de las masas mesenquimatosas laterales embrionarias. El quiste dermoide verdadero está recubierto de epitelio que muestra queratinización y tiene apéndices de piel identificables. Presentamos el caso de un varón de 53 años de edad que presenta una tumoración en suelo de boca de unos 30 años de evolución. Se realiza una revisión bibliográfica de dicha patología

Dermoid cysts are congenital lesions composed of tissues with different origins: ectoblastic, mesoblastic, or endoblastic, caused by a defect in the fusion of the embryonic lateral mesenchymatic mass. A true dermoid cyst is a cavity covered with epithelium showing keratinisation and presenting identifiable dermal appendices. We present a case of a male patient aged 53 years presenting tumouration on the floor of the mouth with an evolution of about 30 years. A bibliographic revision of this pathology is also presented