Cutaneous lesions and mitochondrial hearing loss: A case report.

Pathogenic sequence changes in mitochondrial DNA (mtDNA) are one of the most common causes of genetic hearing loss. We report an infant with palmoplantar hyperkeratosis, extrapalmoplantar cutaneous features and mitochondrial sensorineural hearing loss caused by the previously reported pathogenic NC_012920:m.7445A > G sequence change in the mitochondrial gene COX1 (COX1, MT-CO1). Next generation sequencing- based technology was key for the diagnosis and management of this patient.

Promontorial cochleostomy in nonhuman primates. Is it atraumatic?

The purpose of this study is to determine if surgical approach to the inner ear is feasible without generating a hearing loss in an animal model. Five Macaca fascicularis were used as experimental animals and followed up for 27 months. Mastoidectomy, posterior tympanotomy and promontorial cochleostomy were performed on four specimens and one specimen was kept as control animal. Before and after drilling and exposing the endosteal layer and the membranous labyrinth, otoacustic emissions (dPOAE) and auditory brainstem responses (ABR) were used to test hearing. In vivo experimental studies prove it is reliable to expose the membranous labyrinth without causing hearing loss. dPOAE were present after 3, 6, 12, 24 and 26 months of follow-up. Regarding the ABR results from the four M. fascicularis in which a cochleostomy has been carried out, auditory thresholds are within the 20-30 dB interval at 27 months of follow-up. Experimental studies support clinical experiences indicating it is feasible to surgically approach the membranous labyrinth of the cochlea without damaging its hearing function.

[Reconstruction of pharyngeal defects]. / Reconstrucción de defectos faríngeos.

Reconstruction of pharyngeal defects continues to present a clinical challenge for the Head and Neck surgeon. We have different reconstructive options to preserve speech, airway and swallowing functions. Reconstructive surgery implies a balance between oncologic cure, patient morbidity and quality of life. Classical reconstructive techniques include pedicled cervical cutaneous or myocutaneous flaps and distal myocutaneous flaps such as from the pectoralis major. Current microvascular technique options have a differing incidence of complications but always with high success rates. This article reviews the most current options on reconstructive techniques in pharyngeal defects.

[Review of audiometric criteria in treatment of neurosensorial deafness with hearing aids and implantable hearing devices]. / Revisión de los criterios audiométricos en el tratamiento de la hipoacusia neurosensorial mediante audífonos y prótesis auditivas implantables.

Sensorineural hearing loss has a high incidence in our population; as a matter of fact, 50% of people above 75 years of age suffer this impairment. Due to the advances in the devices to alleviate this condition and their verified efficacy, it is now appropriate to review the indications for these devices and provide a detailed description of the audioprosthetic systems used. These systems can be classified as external non-implantable devices (hearing aids) and implantable prostheses. The latter can be sub-divided into active implants in the external ear or middle ear, cochlear implants, and auditory brainstem implants (ABI). Indications for each group are determined by the type and location of the underlying condition as well as by the anatomic, functional, and social characteristics of each patient. It must be stressed that the selection and monitoring of the treatment is up to the specialist. Generally speaking, an attempt is made to facilitate the integration of the hypoacusic patients to their sound setting by enhancing their understanding of the spoken word and restoring binaurality, while at the same time, seeking to retain the plasticity of central auditory routes through the stimulation provided by any of these systems. In the course of this review, we refer to newly-emerging indications in both the field of cochlear implants (bimodal stimulation, implantation in patients with residual hearing, bilateral implants, etc) and in the area of ABI in patients with tumoural disease previously treated with radiosurgery or patients with non-tumour pathologies presenting malformations or bilateral cochlear ossification.

Bimodal Stimulation with Cochlear Implant and Hearing Aid in Cases of Highly Asymmetrical Hearing Loss.

OBJECTIVE: Bimodal stimulation is a possible treatment for asymmetrical hearing loss, wherein 1 ear is stimulated with a cochlear implant and the other is stimulated with a hearing aid. This emerging indication has gained significance over the last few years. However, little research has been conducted regarding the performance in different types of asymmetric Hearing loss. This study seeks to prove the bilateral-binaural advantage in a group of patients treated with bimodal stimulation (cochlear implant and hearing aid), with different degrees of hearing loss in their best ear. MATERIALS AND METHODS: In total, 31 patients were recruited for the study. They were divided into 3 groups on the basis of the ear with the hearing aid: Group A, pure tone average (PTA) between 41 and 70 dB HL; Group B, PTA between 71 and 80 dB HL; and Group C, PTA between 81 and 90 dB HL. The performance in PTA and disyllabic word recognition were analyzed separately in each ear and then bimodally. The minimum follow-up period was 2 years. RESULTS: There were statistically significant differences between bimodal and monaural conditions both in PTA and in disyllabic word recognition. The better the residual hearing in the ear with the hearing aid, the greater were the benefits obtained with bilateralism-binaurality. CONCLUSION: Bimodal stimulation provides better results than any monaural hearing mode, regardless of whether it involves the use of a hearing aid alone or a cochlear implant alone.

A new bone conduction implant: surgical technique and results.

OBJECTIVE: To describe the surgical technique under local or general anesthesia of 5 cases that have undergone this procedure and the audiologic results obtained with this new device. PATIENTS: Four patients with mixed hearing loss and 1 patient with single-sided deafness. INTERVENTION: Therapeutic. MAIN OUTCOME MEASURES: The surgery was planned beforehand with a 3D reconstruction of a CT scan. The procedure was documented and timed in every case. Air and bone conductive pure tone audiometry and disyllabic words discrimination were tested after and before the procedure. Results were statistically analyzed. RESULTS: All patients tolerated well the procedure. Four patients were intervened under local anesthesia and 1 under general anesthesia because of an associated procedure. All patients showed statistically significant difference between the presurgery and postsurgery audiologic tests. CONCLUSION: Implantation of the Bonebridge with local or general anesthesia is a safe and feasible procedure, with audiometric results that can come close with the ones provided by BAHD users.

Atraumaticity study of 2 cochlear implant electrode arrays.

HYPOTHESIS: Evaluate, based on morphologic and histologic parameters, the atraumaticity of 2 electrode arrays implanted in 10 human temporal bones. BACKGROUND: Atraumatic surgery and electrode arrays are current topics in otologic surgery. The preservation of cochlear anatomy and its functions is a priority and morphologic evaluation of the surgical trauma is essential to continue improving in this field. METHODS: Ten preserved human temporal bones (TB) without anatomic alterations were used in this study. They were divided into 2 groups of 5, and atraumatic surgery was performed to insert HiFocus 1J (group A) and HiFocus Helix (group B) electrode arrays. Anatomic comparisons were performed using computed tomography and histologic analysis. RESULTS: Group A: the mean length for the cochlear longitudinal axis was 10.30 mm, and the cochlear transversal axis was 7.2 mm. Scala tympani insertion was achieved in 4/5 TB studied, with a mean depth and angle of insertion of 19.2 mm and 325.5°, respectively. Lateral location of the electrode array was achieved in all specimens. No significant correlation was observed between these dimensions and depth of insertion. Group B: the mean length for the cochlear longitudinal axis was 9.52 mm, and cochlear transversal axis was 6.38 mm. Scala tympani insertion was achieved in 4/5 TB studied, with a mean depth and angle of insertion of 17.5 mm and 352°, respectively. Modiolar location of the electrode array was achieved in all specimens. A positive correlation was established between the linear and angular insertion depths (p = 0.044). CONCLUSION: In summary, it is safe to state that neither electrode array shows significant insertion trauma.

[Attic exposure-antrum exclusion as an on demand surgical technique for cholesteatoma]. / Aticoexposición-antroexclusión como técnica quirúrgica a la demanda para el tratamiento del colesteatoma.

INTRODUCTION AND OBJECTIVE: Attic exposure and antrum exclusion (AE-AE) is an on demand surgical technique for the treatment of cholesteatoma. Several techniques have been described with variable recurrence rates. The aim of the study was to describe this surgical technique and its indications and to analyse long-term outcomes. MATERIALS AND METHODS: Forty-two patients were included. They received AE-AE in a tertiary centre and were followed-up from 6 months to 7 years. The surgical technique exposes the attic by drilling the superior wall of the external auditory canal and excludes the antrum and the mastoid cells by closing the aditus with a cartilage graft. It is indicated if the lesion does not go beyond the aditus or, if it does, it is a delimited cholesteatoma not damaging the labyrinth. The patients were followed-up by physical examination and radiology (CT scan and Non-EPI diffusion-weighted MRI since 2009). RESULTS: The recurrence rate of the cholesteatoma was 4.8%. At recurrence, an open canal-wall-down mastoidectomy technique was performed. The rate of otorrhea (2/42 cases), serous otitis (2/42) and other complications was low, so aquatic activities were not contraindicated. The AE-AE did not modify ossicular chain reconstruction with respect to the rest of surgical techniques. CONCLUSIONS: AE-AE is a canal-wall-down technique. Its purpose is to remove the lesion and to reduce the recurrence of cholesteatoma. In addition, it allows the entrance of water and reduces the need for 2(nd) look surgery. Non-EPI diffusion-weighted MRI is a reliable technique for follow-up, especially for the excluded mastoid.

La importancia de la vacunación frente al rotavirus / The importance of vaccination against rotavirus

A lo largo de los años hay dos medidas que han tenido un extraordinario impacto positivo en la salud de los ciudadanos del mundo: la potabilización del agua y la vacunación. El rotavirus (RV), cuya transmisión es por vía oral, está presente en todos los países y en los distintos continentes. Su afectación produce daño sistémico, aunque es el aparato digestivo de los niños menores de 2 años el más afectado. Los cuadros de diarrea y fiebre alta ocasionan del 14 al 30% de todos los casos de gastroenteritis aguda (GEA), requiriendo hospitalización aproximadamente una cuarta parte de ellos. También conlleva un elevado uso de los recursos sanitarios, pérdida de horas laborales y gran angustia y preocupación para los familiares. Con la utilización de las vacunas se han disminuido en un 60-80% los ingresos por gastroenteritis aguda por rotavirus (GEA-RV) y un 30-40% la mortalidad por diarrea en menores de 5 años. En España se dispone de dos vacunas, financiada para los prematuros nacidos entre las 25 y las 32 semanas. Ambas vacunas solo tienen sentido utilizarlas en las primeras semanas de vida, con ciertas diferencias entre ellas, tanto en el número de dosis como en la consecución de la máxima protección.(AU)

Two measures have had an extraordinarily positive impact on the health of world citizens over the years: water potabilization and vaccination. Rotavirus (RV), which has oral transmission, is present in all countries and different continents. It causes systemic damage, although the digestive system of <2-year-old children is the most affected. Diarrhea and high temperature cause from 14 to 30% of all cases of acute gastroenteritis (AGE), and one fourth of these will require hospitalization. This also entails a high use of health resources, work hours missed, and high anxiety and concern among relatives. The use of vaccines has reduced by 60-80% the hospital admissions due to acute gastroenteritis caused by rotavirus (RV-AGE) and by 30-40% the mortality by diarrhea in <5-year-olds. There are two vaccines available in Spain, only reimbursed for pre-term children born between 25 and 32 weeks of pregnancy. It only makes sense to use these vaccines during the first weeks of life, with certain differences between them in number of doses and in the achievement of maximum protection.(AU)

Aticoexposición-antroexclusión como técnica quirúrgica a la demanda para el tratamiento del colesteatoma / Attic exposure-antrum exclusion as an on demand surgical technique for cholesteatoma

Introducción: La aticoexposición-antroexclusión (AE-AE) simple o ampliada, es una técnica a la demanda para el tratamiento del colesteatoma. Las tasas de recurrencia/recidiva descritas con diferentes técnicas quirúrgicas son variables. El objetivo de este estudio es describir la técnica quirúrgica, sus indicaciones y analizar los resultados a largo plazo. Material y métodos: Se incluyen 42 pacientes intervenidos de AE-AE en un centro terciario con un seguimiento de 6 meses-7 años. Esta técnica expone completamente el ático fresando la pared superior del conducto auditivo externo y excluye el antro y mastoides cerrando el additus con injertos de cartílago. Se indica en pacientes con colesteatoma localizado en la caja timpánica sin sobrepasar el additus o en caso de hacerlo estar encapsulado sin erosionar la cápsula laberíntica. El seguimiento es clínico y radiológico (TC de peñascos y RM con secuencias de difusión desde 2009). Resultados: El 4,8% de los casos presentó una recidiva, los cuales fueron rescatados en un segundo tiempo quirúrgico. En el seguimiento la tasa de otorrea (2/42), otitis serosa (2/42) u otras complicaciones fue baja, no contraindicando las actividades acuáticas. La AE-AE no modificó el tipo de reconstrucción osicular respecto a otras técnicas. Conclusiones: La AE-AE es una técnica abierta. Pretende eliminar totalmente la lesión y reducir la recidiva de colesteatoma, facilitando una normalización en los baños y una disminución de revisiones posquirúrgicas. La RM en secuencias de difusión es una técnica fiable en el despistaje y seguimiento, especialmente para el control de los espacios mastoideos tras la AE-AE (AU)

Introduction and objective: Attic exposure and antrum exclusion (AE-AE) is an on demand surgical technique for the treatment of cholesteatoma. Several techniques have been described with variable recurrence rates. The aim of the study was to describe this surgical technique and its indications and to analyse long-term outcomes. Materials and methods: Forty-two patients were included. They received AE-AE in a tertiary centre and were followed-up from 6 months to 7 years. The surgical technique exposes the attic by drilling the superior wall of the external auditory canal and excludes the antrum and the mastoid cells by closing the aditus with a cartilage graft. It is indicated if the lesion does not go beyond the aditus or, if it does, it is a delimited cholesteatoma not damaging the labyrinth. The patients were followed-up by physical examination and radiology (CT scan and Non-EPI diffusion-weighted MRI since 2009). Results: The recurrence rate of the cholesteatoma was 4.8%. At recurrence, an open canal-wall-down mastoidectomy technique was performed. The rate of otorrhea (2/42 cases), serous otitis (2/42) and other complications was low, so aquatic activities were not contraindicated. The AE-AE did not modify ossicular chain reconstruction with respect to the rest of surgical techniques. Conclusions: AE-AE is a canal-wall-down technique. Its purpose is to remove the lesion and to reduce the recurrence of cholesteatoma. In addition, it allows the entrance of water and reduces the need for 2nd look surgery. Non-EPI diffusion-weighted MRI is a reliable technique for follow-up, especially for the excluded mastoid (AU)

Reconstrucción de defectos faríngeos / Reconstruction of pharyngeal defects

La reconstrucción de defectos faríngeos sigue siendo un reto para el cirujano de cabeza y cuello. Las diferentes opciones reconstructivas van orientadas a mantener la función vocal, la vía aérea y la deglución. La técnica de reconstucción supone un equilibrio entre la curación oncológica, la morbilidad del paciente y la calidad de vida. Las técnicas reconstructivas clásicas incluyen los colgajos pediculados cutáneos y miocutáneos cervicales y los colgajos miocutáneos distales, como el de pectoral mayor. Actualmente, las técnicas de reconstrucción microvascular difieren en la incidencia de complicaciones, pero siempre con un alto nivel de viabilidad. Este trabajo tiene como objetivo revisar las opciones actuales en técnicas reconstructivas de defectos faríngeos (AU)

Reconstruction of pharyngeal defects continues to present a clinical challenge for the Head and Neck surgeon. We have different reconstructive options to preserve speech, airway and swallowing functions. Reconstructive surgery implies a balance between oncologic cure, patient morbidity and quality of life. Classical reconstructive techniques include pedicled cervical cutaneous or myocutaneous flaps and distal myocutaneous flaps such as from the pectoralis major. Current microvascular technique options have a differing incidence of complications but always with high success rates. This article reviews the most current options on reconstructive techniques in pharyngeal defects (AU)

Revisión de los criterios audiométricos en el tratamiento de la hipoacusia neurosensorial mediante audífonos y prótesis auditivas implantables / Review of audiometric criteria in treatment of neurosensorial deafness with hearing aids and implantable hearing devices

La hipoacusia neurosensorial tiene alta incidencia en nuestra población. Sirva de ejemplo que el 50 % de las personas mayores de 75 años tiene este tipo de discapacidad. Los avances en los dispositivos utilizados para su tratamiento paliativo y su eficacia comprobada hacen necesaria la revisión de sus indicaciones y la descripción detallada de los sistemas audioprotésicos empleados. Éstos pueden ser clasificados en prótesis externas no implantables (audífonos) y prótesis implantables. El grupo de las prótesis implantables se subdivide a su vez en implantes activos de oído externo, implantes activos de oído medio, implantes cocleares e implantes auditivos de tronco cerebral (IATC). Las indicaciones establecidas para cada grupo audioprotésico se definen por la tipología y la topología de la enfermedad subyacente y por las características anatomofuncionales y socioculturales de cada paciente. En esta cuestión debe hacerse hincapié en el protagonismo del especialista a la hora de elegir y seguir el tratamiento. Como norma general, se procura favorecer el acceso del paciente hipoacúsico a su entorno sonoro realzando la comprensión de la palabra hablada restableciendo la binauralidad y, a la vez, se busca mantener la plasticidad de las vías auditivas centrales a través de la estimulación proporcionada por cualquiera de estos sistemas. Se expone las indicaciones emergentes, ya sea en el campo de los implantes cocleares (estimulación bimodal, implantación en pacientes con audición residual, implantaciones bilaterales, etc.) o en el campo de los IATC, en pacientes con afección tumoral previamente tratada con radiocirugía y en pacientes con trastornos no tumorales afectos de osificación coclear bilateral o malformaciones

Sensorineural hearing loss has a high incidence in our population; as a matter of fact, 50 % of people above 75 years of age suffer this impairment. Due to the advances in the devices to alleviate this condition and their verified efficacy, it is now appropriate to review the indications for these devices and provide a detailed description of the audioprosthetic systems used. These systems can be classified as external non-implantable devices (hearing aids) and implantable prostheses. The latter can be sub-divided into active implants in the external ear or middle ear, cochlear implants, and auditory brainstem implants (ABI). Indications for each group are determined by the type and location of the underlying condition as well as by the anatomic, functional, and social characteristics of each patient. It must be stressed that the selection and monitoring of the treatment is up to the specialist. Generally speaking, an attempt is made to facilitate the integration of the hypoacusic patients to their sound setting by enhancing their understanding of the spoken word and restoring binaurality, while at the same time, seeking to retain the plasticity of central auditory routes through the stimulation provided by any of these systems. In the course of this review, we refer to newly-emerging indications in both the field of cochlear implants (bimodal stimulation, implantation in patients with residual hearing, bilateral implants, etc) and in the area of ABI in patients with tumoural disease previously treated with radiosurgery or patients with non-tumour pathologies presenting malformations or bilateral cochlear ossification