Audiological outcomes in patients with congenital aural atresia implanted with transcutaneous active bone conduction hearing implant.

OBJECTIVES: The objective of this study is to evaluate the safety and efficacy of the transcutaneous Bone Conduction Implant, the Bonebridge, in patients with congenital aural atresia. METHODS: Audiometry, speech recognition test and free field audiometry were performed. Word recognition scores and speech perception was evaluated using Spanish phonetically-balanced disyllables word list. RESULTS: Fourteen subjects were implanted with the Bonebridge (seven bilateral placements). The study cohort comprised seven males and seven females aged from 3 to 17 years (mean age 9.76yrs). All patients accepted and benefited from the implanted Bonebridge system. The pre-operative PTA4 was 66.4 dB (64.2-68.6, 95-%CI) and improved after activation to 19.2 dB (16.9-21.5, 95%CI), resulting in a mean functional gain of 47,2 dB. Regarding speech discrimination, the pre-operative outcomes of the disyllabic measurements were 34.3% and for monosyllables 27.4%. Following activation the speech discrimination improved to 98.6% and 97.9%, respectively. No infections or adverse device related effects occurred in patient group. CONCLUSION: We have concluded that the Bonebridge implant is an innovative solution for patients with conductive or mixed hearing loss and unilateral loss suffering from congenital atresia. Different surgical techniques may be used for implant placement, based on the patient's anatomy. Studies show improved functional gain, better speech perception, and lower rates of percutaneous complications associated with this implant.

Manifesto on small airway involvement and management in asthma and chronic obstructive pulmonary disease: an Interasma (Global Asthma Association - GAA) and World Allergy Organization (WAO) document endorsed by Allergic Rhinitis and its Impact on Asthma (ARIA) and Global Allergy and Asthma European Network (GA2LEN).

Evidence that enables us to identify, assess, and access the small airways in asthma and chronic obstructive pulmonary disease (COPD) has led INTERASMA (Global Asthma Association) and WAO to take a position on the role of the small airways in these diseases. Starting from an extensive literature review, both organizations developed, discussed, and approved the manifesto, which was subsequently approved and endorsed by the chairs of ARIA and GA2LEN. The manifesto describes the evidence gathered to date and defines and proposes issues on small airway involvement and management in asthma and COPD with the aim of challenging assumptions, fostering commitment, and bringing about change. The small airways (defined as those with an internal diameter <2 mm) are involved in the pathogenesis of asthma and COPD and are the major determinant of airflow obstruction in these diseases. Various tests are available for the assessment of the small airways, and their results must be integrated to confirm a diagnosis of small airway dysfunction. In asthma and COPD, the small airways play a key role in attempts to achieve disease control and better outcomes. Small-particle inhaled formulations (defined as those that, owing to their size [usually <2 µm], ensure more extensive deposition in the lung periphery than large molecules) have proved beneficial in patients with asthma and COPD, especially those in whom small airway involvement is predominant. Functional and biological tools capable of accurately assessing the lung periphery and more intensive use of currently available tools are necessary. In patients with suspected COPD or asthma, small airway involvement must be assessed using currently available tools. In patients with subotpimal disease control and/or functional or biological signs of disease activity, the role of small airway involvement should be assessed and treatment tailored. Therefore, the choice between large- and small-particle inhaled formulations must reflect the physician's considerations of disease features, phenotype, and response to previous therapy. This article is being co-published in Asthma Research and Practice and the World Allergy Organization Journal.

Vibrant soundbridge in aural atresia: does severity matter?

Congenital aural atresia (CAA) poses significant challenges to surgical remediation. Both bone anchored hearing aids (BAHA) and the Vibrant Soundbridge (VSB) have been considered as alternatives or adjuncts to conventional atresiaplasty. A consensus statement on VSB implantation in children and adolescents recommended against implantation when the Jahrsdoerfer score was less than 8. More recent publications suggest that patients with Jahrsdoerfer scores between three and seven may benefit from VSB implantation. The purpose of this study was to further investigate the outcomes of VSB implantation in CAA. The study was a multi-center, retrospective review. A retrospective review of data (patient's demographic, clinical, implant and audiological information) from four collaborating centers that have performed VSB implantation in CAA was performed. Outcomes based on severity of the atresia using the Jahrsdoerfer and Yellon-Branstetter scoring systems were also evaluated. Data from 28 patients from the four centers revealed no iatrogenic facial nerve injuries or change in bone thresholds. Post-operative speech threshold and speech recognition was, respectively, 39 dB and 94%. Jahrsdoerfer and Yellon scores ranged from 4 to 9 and 4 to 12, respectively. The scores did not correlate to or predict outcomes. Three individual elements of the scores did correlate to initial, but not long-term outcomes. Atresiaplasty and BAHA in the management of CAA are not complete solutions. VSB may offer an alternative in these surgically complex patients for achieving amplification, though better metrics for patient selection need to be developed. LEVEL OF EVIDENCE : IV.

Towards a consensus on a hearing preservation classification system.

CONCLUSION: The comprehensive Hearing Preservation classification system presented in this paper is suitable for use for all cochlear implant users with measurable pre-operative residual hearing. If adopted as a universal reporting standard, as it was designed to be, it should prove highly beneficial by enabling future studies to quickly and easily compare the results of previous studies and meta-analyze their data. OBJECTIVES: To develop a comprehensive Hearing Preservation classification system suitable for use for all cochlear implant users with measurable pre-operative residual hearing. METHODS: The HEARRING group discussed and reviewed a number of different propositions of a HP classification systems and reviewed critical appraisals to develop a qualitative system in accordance with the prerequisites. RESULTS: The Hearing Preservation Classification System proposed herein fulfills the following necessary criteria: 1) classification is independent from users' initial hearing, 2) it is appropriate for all cochlear implant users with measurable pre-operative residual hearing, 3) it covers the whole range of pure tone average from 0 to 120 dB; 4) it is easy to use and easy to understand.

Evidence of bacterial biofilms in nasal polyposis.

INTRODUCTION: The pathogeny of chronic rhinosinusitis with nasal polyposis (CRS/NP) has not been elucidated. Bacterial exotoxins have been implicated in many inflammatory chronic diseases, such as chronic otitis, chronic tonsillitis, cholesteatomas, and more recently CRS/NP. We propose that the bacteria in CRS/NP are not only present in a planktonic state, but also occur in microbial communities as biofilms. OBJECTIVE: To determine and characterize the presence of biofilms in CRS/NP. METHODS: We performed a prospective study in 12 patients undergoing endoscopic sinus surgery for nasal polyposis. Ten patients without CRS/NP who underwent septoplasty were included as a control group. Tissue samples were obtained from the inferior turbinate mucosae. The bacteria were isolated and typified and the material was examined in vitro using a spectrophotometer, and in vivo using optical microscopy and confocal scanning laser microscopy. RESULTS: Moderate to high in vitro biofilm-forming capacity was detected in 9 out of 12 patients with CRS/NP (mean [SD] optical density values of between 0.284 [0.017] and 3.337 [0.029]). The microorganisms isolated were Staphylococcus (5 patients), Streptococcus viridans, Pseudomonas aeruginosa, Enterococcus faecalis and Streptococcus viridans/Corynebacterium. Biofilms were demonstrated in vivo in 2 patients and no biofilm structures were evident in any of the controls. CONCLUSION: This study demonstrates the presence of bacterial biofilms in patients with CRS/NP. This chronic inflammatory factor might contribute to nasal mucosa damage, increased inflammatory cells in tissue, and the subsequent hyperplasic process.

A mutation in mitochondrial 12S rRNA, A827G, in Argentinean family with hearing loss after aminoglycoside treatment.

Mutations in mitochondrial DNA (mtDNA) have been found to be associated with sensorineural hearing loss. We report the clinical, genetic, and molecular characterization of one Argentinean family with aminoglycoside-induced impairment in two of their members. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects. Mutational analysis of the mtDNA in these pedigrees showed the presence of homoplasmic 12S rRNA A827G mutation, which has been associated with hearing impairment. The A827G mutation is located at the A-site of the mitochondrial 12S rRNA gene which is highly conserved in mammals. It is possible that the alteration of the tertiary or quaternary structure of this rRNA by the A827G mutation may lead to mitochondrial dysfunction, thereby playing a role in the pathogenesis of hearing loss and aminoglycoside hypersensitivity. However, incomplete penetrance of hearing impairment indicates that the A827G mutation itself is not sufficient to produce clinical phenotype.

[Intrapretosal schwannomas of facial nerve]. / Schwannomas intrapetrosos del nervio facial.

The aim of this study was to determine the factors that are important to the diagnosis and treatment of facial neuroma. The most common tumors of the peripheral nerves are the neurinomas; about 50% of them are found in the head and neck region. Among them, the most frequent is the VIII nerve tumor, while the facial neuroma is extremely rare. Neuromas can be intracraneal, intratemporal and extratemporal. The last one has a higher frequency. We present our experience in the intrapetrosal facial neuromas; usually showing different presentation symptoms wich is a characteristic of them. Retrospective study that was carried out at a private tertiary referral center. 3 patients were referred from other centers for otological surgery. One of them had symptoms of chronic otitis media, the second one was diagnosed of a glomus tumor and the last one showed a facial palsy. All the patients were operated on and then the facial nerve was repaired. Two patients had an end to end anastomosis, and in the other one we managed to keep the integrity of the facial nerve. The Hous -Brackmann facial grading scale was used. Two patients had grade II and the other grade III in this scale in the long-term follow up.

Schwannomas intrapetrosos del nervio facial / Intrapretosal schwannomas of facial nerve

El objetivo de este trabajo es determinar los factores importantes en el diagnóstico y tratamiento de los neurinomas del nervio facial. Los tumores de nervios periféricos más comunes son los neurinomas y casi el 50% de los mismos pertenecen al territorio de cabeza y cuello. Dentro de estos el más frecuente es el del VIII par, siendo el neurinoma del facial raro de encontrar. A su vez podemos tener neurinomas intracraneales, intratemporales y extratemporales, siendo estos últimos los más frecuentes. Aquí se presenta la experiencia de este centro en neurinomas del nervio facial intrapetrosos, que presentaron distinta sintomatología de aparición, característica común en ellos. Se presenta un trabajo retrospectivo, realizado en una institución privada multidisciplinaria de alta complejidad, que recoge tres casos derivados, desde otros centros, para cirugía otológica, con sintomatología muy diversa, en el primero de los casos, más o menos típica de otitis media crónica, el segundo con diagnóstico de posible tumor glómico y el tercero con parálisis facial. Se realizó a todos los pacientes exéresis quirúrgica y tratamiento de la continuidad anatómica del facial. En dos de ellos se realizó anastomosis termino-terminal del facial luego de la exéresis total, en el restante se extirpó solamente el tumor respetando la continuidad anatómica del nervio (perineuro). La función del nervio facial fue evaluada mediante la escala de House-Brackmann. La respuesta a largo plazo de 2 pacientes con función facial grado II y uno con grado III en la escala House-Brackmann (AU)

The aim of this study was to determine the factors that are important to the diagnosis and treatment of facial neuroma. The most common tumors of the peripheral nerves are the neurinomas; about 50% of them are found in the head and neck region. Among them, the most frequent is the VIII nerve tumor, while the facial neuroma is extremely rare. Neuromas can be intracraneal, intratemporal and extratemporal. The last one has a higher frequency. We present our experience in the intrapetrosal facial neuromas; usually showing different presentation symptoms wich is a characteristic of them. Retrospective study that was carried out at a private tertiary referral center. 3 patients were referred from other centers for otological surgery. One of them had symptoms of chronic otitis media, the second one was diagnosed of a glomus tumor and the last one showed a facial palsy. All the patients were operated on and then the facial nerve was repaired. Two patients had an end to endanastomosis, and in the other one we managed to keep the integrity of the facial nerve. The Hous –Brackmann facial grading scale was used. Two patients had grade II and the other grade III in this scale in the long-term follow up (AU)

[Incidence of complications of otitis media]. / Incidencia de complicaciones de otitis media.

OBJECTIVE: Determine the incidence of otological and endocranial complications of acute otitis media, taking into account previous treatments and the development of complications. MATERIAL AND METHODS: Retrospective follow-up study between March 1996-2003 including 16 patients: 9 men, 7 women. RESULTS: Nine patients (56.25%) had intracranial complications and seven otological ones. In the first group, 6 developed meningitis by Streptococcus pneumoniae, one sigmoid sinus thromboflebitis, one multiple abscesses, and one subdural empiema and encephalitis. Otological complications were six acute mastoiditis and the other one a facial paralysis. The child with thromboflebitis underwent mastoidectomy and closed meningeal comunication; 5 of the acute mastoiditis were operated on and the other received traditional treatment. The meningitis received medication. Two died, one because of a subdural empiema and the other due to meningitis. CONCLUSION: Despite the advances made by antibiotherapy and diagnosis, the complications of otitis media are still frequent. The pediatrician and otorhinolaryngologist should be on the alert for these as well as for Pneumococcus and its high resistance.

Incidencia de complicaciones de otitis media / Incidence of complications of otitis media

Objetivos: Determinar la incidencia de complicaciones otológicas e intracraneales por otitis media, evaluando la repercusión del tratamiento instaurado previamente y la evolución de las mismas. Material y métodos: Trabajo retrospectivo realizado durante el período de marzo de 1996 a marzo de 2003. Se incluyeron 16 pacientes (9 hombres y 7 mujeres). Resultados: Del total de pacientes, 9 (56,25%) tuvieron complicaciones intracraneales y 7 complicaciones otológicas. Entre las primeras, hubo 6 casos de meningitis por Streptococcus pneumoniae, un caso de tromboflebitis del seno sigmoideo con absceso cerebral, un caso con múltiples abscesos cerebrales y uno con empiema subdural y encefalitis. Entre las complicaciones otológicas, 6 pacientes sufrieron mastoiditis aguda y un caso parálisis facial recurrente. El niño con abscesos cerebrales fue tratado quirúrgicamente; al paciente con tromboflebitis se le realizó mastoidectomía con ligadura del seno sigmoideo. Cinco de las mastoiditis fueron operadas y la restante fue tratada conservadoramente. Las meningitis se trataron médicamente. Hubo dos fallecimientos, uno por empiema subdural y otro por meningitis. Conclusión: A pesar de los progresos en la antibioticoterapia y de los métodos de diagnóstico, las complicaciones de otitis medias son aún frecuentes, por lo cual deben estar siempre en el pensamiento del médico pediatra y otorrinolaringólogo, además de tener presente el neumococo y su alta resistencia

Objetive: Determine the incidence of othologycal and endocraneal complications of acute otitis media, taking into account previous treatments and the development of complications. Material and Methods: Retrospective follow-up study between March 1996-2003 including 16 patients: 9 men, 7 women. Results: Nine patients (56.25%) had intracraneal complications and seven othologycal ones. In the first group, 6 developed meningitis by Streptococcus pneumoniae, one sigmoid sinus tromboflebitis, one multiples abscesses, and one subdural empiema and encephalitis. Othologycal complications were six acute mastoiditis and the other one a facial paralysis. The child with tromboflebitis underwent mastoidectomy and closed meningeal comunication; 5 of the acute mastoiditis were operated on and the other received traditional treatment. The meningitis received medication. Two died, one because of a subdural empiema and the other due to meningitis. Conclusion: Despite the advances made by antibiotherapy and diagnosis, the complications of otitis media are still frequent. The pediatrician and otorhinolaryngologist should be on the alert for these as well as for Pneumococcus and its high resistance