Parvicella tangerina gen. nov., sp. nov. (Parvicellaceae fam. nov., Flavobacteriales), first cultured representative of the marine clade UBA10066, and Lysobacter luteus sp. nov., from activated sludge of a seawater-processing wastewater treatment plant.

Two strains isolated from a sample of activated sludge that was obtained from a seawater-based wastewater treatment plant on the southeastern Mediterranean coast of Spain have been characterized to achieve their taxonomic classification, since preliminary data suggested they could represent novel taxa. Given the uniqueness of this habitat, as this sort of plants are rare in the world and this one used seawater to process an influent containing intermediate products from amoxicillin synthesis, we also explored their ecology and the annotations of their genomic sequences. Analysis of their 16S rRNA gene sequences revealed that one of them, which was orange-pigmented, was distantly related to Vicingus serpentipes (family Vicingaceae) and to other representatives of neighbouring families in the order Flavobacteriales (class Flavobacteriia) by 88-89 % similarities; while the other strain, which was yellow-pigmented, was a putative new species of Lysobacter (family Xanthomonadaceae, order Xanthomonadales, class Gammaproteobacteria) with Lysobacter arseniciresistens as closest relative (97.3 % 16S rRNA sequence similarity to its type strain). Following a polyphasic taxonomic approach, including a genome-based phylogenetic analysis and a thorough phenotypic characterization, we propose the following novel taxa: Parvicella tangerina gen. nov., sp. nov. (whose type strain is AS29M-1T=CECT 30217T=LMG 32344T), Parvicellaceae fam. nov. (whose type genus is Parvicella), and Lysobacter luteus sp. nov. (whose type strain is AS29MT=CECT 30171T=LMG 32343T).

Multi-headed (bifid and trifid) mandibular condyles in archaeological contexts: Two posttraumatic cases.

OBJECTIVE: Bifid and trifid mandibular condyles are infrequent morphological alterations of the mandibular condyle. With the aim of better identifying its possible causing factors in the past and provide clues on the potential types and severities of joint dysfunction that may occur if clinical intervention is not undertaken, two archaeological cases of multi-headed mandibular condyles are presented, and their possible aetiology and pathogenesis are discussed. DESIGN: In this study, 143 adult mandibles recovered in the northwest of Spain were examined: 91 exhumed from San Juan Bautista church (Guardo, Palencia; 16th-19th centuries), and 52 from San Salvador de Palat de Rey church (León, 13th-19th centuries). All mandibles were observed macroscopically for the presence of any morphological and/or pathological alterations. When justified, the mandibles were also scrutinized through computed tomography. RESULTS: Two isolated mandibles (n = 1, Palencia; n = 1, León) with multi-headed mandibular condyles (bifid and trifid) were identified. The computed tomography scan of the affected mandibles revealed alterations compatible with traumatic events, namely line fractures and deformations at the angle of the mandible. CONCLUSIONS: Both the bifid and trifid mandibular condyles here described probably result from traumatic events during childhood leading to a posttraumatic deformation. Although no severe impairment of mandibular use was identified, some asymmetries probably resulted in some sort of malfunction, with TMJ-OA and gonial angle eversion as supporting evidence. Nevertheless, the degree of bone remodelling observed indicates that both individuals lived long after the traumatic occurrence.

Mycobacterium leprae diversity and population dynamics in medieval Europe from novel ancient genomes.

BACKGROUND: Hansen's disease (leprosy), widespread in medieval Europe, is today mainly prevalent in tropical and subtropical regions with around 200,000 new cases reported annually. Despite its long history and appearance in historical records, its origins and past dissemination patterns are still widely unknown. Applying ancient DNA approaches to its major causative agent, Mycobacterium leprae, can significantly improve our understanding of the disease's complex history. Previous studies have identified a high genetic continuity of the pathogen over the last 1500 years and the existence of at least four M. leprae lineages in some parts of Europe since the Early Medieval period. RESULTS: Here, we reconstructed 19 ancient M. leprae genomes to further investigate M. leprae's genetic variation in Europe, with a dedicated focus on bacterial genomes from previously unstudied regions (Belarus, Iberia, Russia, Scotland), from multiple sites in a single region (Cambridgeshire, England), and from two Iberian leprosaria. Overall, our data confirm the existence of similar phylogeographic patterns across Europe, including high diversity in leprosaria. Further, we identified a new genotype in Belarus. By doubling the number of complete ancient M. leprae genomes, our results improve our knowledge of the past phylogeography of M. leprae and reveal a particularly high M. leprae diversity in European medieval leprosaria. CONCLUSIONS: Our findings allow us to detect similar patterns of strain diversity across Europe with branch 3 as the most common branch and the leprosaria as centers for high diversity. The higher resolution of our phylogeny tree also refined our understanding of the interspecies transfer between red squirrels and humans pointing to a late antique/early medieval transmission. Furthermore, with our new estimates on the past population diversity of M. leprae, we gained first insights into the disease's global history in relation to major historic events such as the Roman expansion or the beginning of the regular transatlantic long distance trade. In summary, our findings highlight how studying ancient M. leprae genomes worldwide improves our understanding of leprosy's global history and can contribute to current models of M. leprae's worldwide dissemination, including interspecies transmissions.

Intralabyrinthine neurinoma: Management, exeresis and auditory restoration with cochlear implant.

BACKGROUND AND OBJECTIVE: Vestibular schwannoma is a benign tumour that originates in the eighth cranial nerve. It is termed intralabyrinthine schwannoma (ILS) when it develops in the inner ear, this being a rare origin. We present our experience in the management of three patients with ILS. MATERIALS AND METHODS: The results of tumour excision and cochlear implantation were evaluated in three patients with ILS: two intracochlear schwannomas (ICS) and one intravestibular schwannoma (IVS). RESULTS: Prior to surgery, all patients presented progressive sensorineural hearing loss and tinnitus. Complete tumour resection and cochlear implantation was possible in all patients, with favourable hearing rehabilitation. CONCLUSIONS: The therapeutic approach will depend on tumour size, growth rate, degree of hearing loss and presence of vestibular symptoms. Cochlear implantation (CI) in patients with ILS is possible when the cochlear nerve is present and functional. CI in patients, whether or not preceded by tumour excision, is an option with good hearing results in selected patients.

Neurinoma intralaberíntico: manejo, exéresis y restauración auditiva con implante coclear / Intralabyrinthine neurinoma: Management, exeresis and auditory restoration with cochlear implant

Introducción y objetivo: El schwannoma vestibular es un tumor benigno que se origina en el octavo par craneal. Se denomina schwannoma intralaberíntico (SIL) cuando se origina en el oído interno, siendo este origen poco frecuente. Presentamos nuestra experiencia en el manejo de 3 pacientes con SIL.Material y métodosLos resultados de la exéresis tumoral e implantación coclear fueron evaluados en 3 pacientes con SIL: 2 casos de schwannoma intracoclear y un caso de schwannoma intravestibular.ResultadosPrevio a la cirugía, todos los pacientes presentaban hipoacusia neurosensorial progresiva y acúfeno ipsilateral. La resección tumoral completa y la colocación de un implante coclear fue posible en todos los casos, con una rehabilitación auditiva favorable.ConclusionesLa actitud terapéutica dependerá del tamaño del tumor, el crecimiento, el grado de pérdida auditiva y los síntomas vestibulares. La implantación coclear en pacientes con SIL es posible cuando el nervio coclear está presente y es funcionante. El IC en el SIL, ya sea con exéresis previa o no del tumor, es una opción con buenos resultados auditivos en pacientes seleccionados. (AU)

Introduction and objective: Vestibular schwannoma is a benign tumour that originates in the eighth cranial nerve. It is termed intralabyrinthine schwannoma (ILS) when it develops in the inner ear, this being a rare origin. We present our experience in the management of three patients with ILS.Material and methodsThe results of tumour excision and cochlear implantation were evaluated in three patients with ILS: two intracochlear schwannomas (ICS) and one intravestibular schwannoma (IVS).ResultsPrior to surgery, all patients presented progressive sensorineural hearing loss and tinnitus. Complete tumour resection and cochlear implantation was possible in all patients, with favourable hearing rehabilitation.ConclusionsThe therapeutic approach will depend on tumour size, growth rate, degree of hearing loss and presence of vestibular symptoms. Cochlear implantation (CI) in patients with ILS is possible when the cochlear nerve is present and functional. CI in patients, whether or not preceded by tumour excision, is an option with good hearing results in selected patients. (AU)

A possible case of ameloblastoma in an adult male from the early 20th century Coimbra, Portugal.

OBJECTIVE: This article discusses the differential diagnosis of an unusual lesion found in the mandible of an adult male individual (late 19th to early 20th century). MATERIALS: A cranium from the Identified Skulls Collection Escolas Médicas of the University of Coimbra, Portugal. METHODS: Macroscopic analysis, conventional radiography. RESULTS: Macroscopically, a conspicuous osteolytic lesion was identified in the middle vestibular area of the mandible. The conventional radiography examination revealed a multilocular and non-expansive lesion, with undefined contours and irregular walls. CONCLUSIONS: These characteristics are compatible with a benign lesion, possibly an ameloblastoma or an odontogenic keratocyst. SIGNIFICANCE: The present work contributes substantially to the knowledge of the development of cystic and tumour lesions in the past and generates a broader body of knowledge about these lesions. LIMITATIONS: Destructive methods were not authorized. SUGGESTIONS FOR FURTHER RESEARCH: In the future, computed tomography scans and 3D reconstruction analysis, not performed in the current study, may add new and valuable information.

Improving the Management of Patients with Hearing Loss by the Implementation of an NGS Panel in Clinical Practice.

A cohort of 128 patients from 118 families diagnosed with non-syndromic or syndromic hearing loss (HL) underwent an exhaustive clinical evaluation. Molecular analysis was performed using targeted next-generation sequencing (NGS) with a custom panel that included 59 genes associated with non-syndromic HL or syndromic HL. Variants were prioritized according to the minimum allele frequency and classified according to the American College of Medical Genetics and Genomics guidelines. Variant(s) responsible for the disease were detected in a 40% of families including autosomal recessive (AR), autosomal dominant (AD) and X-linked patterns of inheritance. We identified pathogenic or likely pathogenic variants in 26 different genes, 15 with AR inheritance pattern, 9 with AD and 2 that are X-linked. Fourteen of the found variants are novel. This study highlights the clinical utility of targeted NGS for sensorineural hearing loss. The optimal panel for HL must be designed according to the spectrum of the most represented genes in a given population and the laboratory capabilities considering the pressure on healthcare.

Osseous mass in a maxillary sinus of an adult male from the 16th-17th-century Spain: Differential diagnosis.

OBJECTIVE: To undertake a differential diagnosis of a large mass found in the left maxillary sinus of a cranium dated to the 16th-17th-century, and to expand knowledge of the diagnosis of osseous tissue formation in osteoarchaeological studies. MATERIAL: A cranium recovered from the cemetery of San Salvador de Palat de Rey church, León (Spain). METHODS: Macroscopic analysis, CT scanning. RESULTS: Macroscopic analysis indicated that the individual was probably a male over 30 years old with an ossified mass in the left maxillary sinus, measuring 24 × 19 × 24 mm, occupying approximately 27 % of the maxillary antrum. Computed tomography revealed a well-demarcated radiolucent unilocular mass with some radiopaque areas, with no communication with the alveoli of the premolars or molars. No erosive lesions or signs of inflammation were found. CONCLUSIONS: Neither the macroscopic, nor the radiological characteristics are compatible with inflammatory or malignant pathology, favoring a diagnosis of ossifying fibroma. SIGNIFICANCE: This case adds to the few reported cases in the osteoarchaeological literature, especially since there is limited relevant reference data to assist diagnosis. The CT scans and 3D reconstruction presented here facilitate differential diagnosis in future paleopathological studies. LIMITATIONS: Destructive methods were not authorized. SUGGESTIONS FOR FURTHER RESEARCH: In the future, micro-CT analysis, which was not performed in the current study, may add new and valuable information.

Stafne's bone defects from Spain: report of four cases and brief archaeological literature review.

OBJECTIVE: To present for the first time in the north-western Spanish osteological record prevalence data on Stafne's bone defect, to compare the results with those reported by other studies, and to increase the dataset for future inter-population comparisons. MATERIAL: In all, 143 complete adult mandibles recovered from two necropolises were analyzed (n = 118, San Juan Bautista of Guardo, Palencia province, 16th-19th centuries; n = 25, Plaza del Grano, city of León, 12th-15th centuries). METHODS: Differential diagnosis of the lesions was made through macroscopic and Computed Tomography (CT) analyses. RESULTS: Four mandibles (n = 3, Guardo, Palencia; n = 1, León) presented bone cavities on the lingual aspect of the mandible below the mylohyoid channel, between the first molar and the angle of the mandible. CT scan showed unilateral well-defined unilocular oval/round concavities in the lingual mandibular cortex below the inferior alveolar canal. Neither the macroscopic nor the radiological characteristics are compatible with inflammatory or malignant pathology, favoring instead a diagnosis of Stafne's bone defect. CONCLUSIONS: Four cases of Stafne's defects are added to the bioarchaeological inventory. The calculated prevalence is 2.54% for San Juan Bautista and 4% for Plaza del Grano, values in close agreement to those presented in other osteoarchaeological studies. SIGNIFICANCE: The identification of all examples of Stafne's bone defects in past populations will contribute to elucidate which factors may be responsible for this trait's cultural, ecological, temporal, and geographical patterning. LIMITATIONS: The skeletal samples are relatively small. SUGGESTIONS FOR FURTHER RESEARCH: In future investigations of Stafne's bone defects, CT analysis of dry bone specimens is recommended, whenever possible.

Outbreak of hepatitis A in a post-vaccination era: High rate of co-infection with sexually transmitted diseases.

INTRODUCTION AND OBJECTIVES: After hepatitis A (HAV) mandatory immunization in 2005 in Argentina, the incidence of HAV declined drastically. However, several new autochthonous cases of HAV have been reported since 2017. We aimed to evaluate the clinical and epidemiological characteristics and possible transmission routes of affected patients. PATIENTS OR MATERIALS AND METHODS: We performed a cross-sectional study of patients residing in Argentina with acute hepatitis A between 30.06.2017 and 31.12.2018. RESULTS: 66 cases of HAV were registered. Fifty-six patients (86%) were males, with a mean age of 34 ±â€¯12 years old. The most likely routes of transmission were sexual intercourse of men with men, reported by 31 patients. Additionally, 23% and 26% of patients tested positive for HIV and syphilis, respectively. In total, 35% of patients required hospitalization. When assessing outcomes, 79% had a mild presentation and 21% had a severe/fulminant presentation: one patient underwent liver transplantation, and one patient died. CONCLUSIONS: Our study describes that during the study period, HAV infection affected predominantly young adults, particularly men who have sex with men. An elevated proportion of them was diagnosed with a concomitant sexually transmitted disease, and several patients had a severe presentation of the disease.

Congenital syphilis or mercury treatment: dental alterations in a twelfth- or thirteenth-century child from Medinaceli, Soria, Spain.

Syphilis, together with its variant congenital syphilis, is a disease caused by Treponema pallidum subsp. pallidum. This paper documents possible new skeletal evidence for congenital syphilis from the Medieval Era (twelfth and thirteenth centuries CE) burial site of Medinaceli in the Province of Soria in North-Central Spain. What is involved is dental alteration due to congenital syphilis, mercury treatment, or a combination of both. This study focuses on the hypoplastic dental changes observed in a child approximately eight years of age. Only a fragmented skull with left maxilla and the left side of the mandible were preserved. Macroscopic analysis, X-rays, computerized tomography (CT) and mercury detection analysis by inductively coupled plasma mass spectrometry (ICP-MS) techniques were used to observe dental abnormalities. In addition to extensive caries in the upper second deciduous molar, pulpo-alveolar lesions and facial alterations were observed. The absence of the rest of the skeleton tends to make a diagnosis of congenital syphilis difficult. However, the dental stigmata observed do permit a reasonable diagnosis.

The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey.

The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.

Guía clínica sobre implantes de conducción de vía ósea / Clinical guideline on bone conduction implants

Introducción y objetivos: En la última década se han producido numerosos y relevantes avances en el tratamiento de la hipoacusia transmisiva y mixta que han desembocado en una ampliación de las indicaciones de los implantes de conducción de vía ósea y la aparición de nuevos dispositivos. La Comisión Científica de Audiología de la Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello (SEORL-CCC), junto con las comisiones de Otología y Otoneurología, ha llevado a cabo una revisión del estado actual de los implantes de vía ósea con la finalidad de ofrecer a los especialistas de Otorrinolaringología, a los profesionales de la sanidad, a las autoridades sanitarias y a la sociedad en general una guía clínica sobre implantes de conducción de vía ósea. Métodos: Esta guía clínica sobre implantes de conducción ósea contiene información sobre los siguientes temas: 1) definición y descripción de los implantes auditivos de vía ósea; 2) indicaciones actuales y emergentes de los implantes de vía ósea; compatibilidad y resonancia magnética, y 3) requisitos organizativos para un programa de implantes de vía ósea. Resultado y conclusiones: La finalidad de esta guía es describir los diferentes sistemas de conducción ósea, sus particularidades e indicaciones, con el objeto de aportar unas coordenadas que ayuden a todos estos agentes en las tomas de decisiones que deban asumir en los diferentes ámbitos de responsabilidad en los que están enmarcados en sus áreas de trabajo

Introduction and goals: During the last decade there have been multiple and relevant advances in conduction and mixed hearing loss treatment. These advances and the appearance of new devices have extended the indications for bone-conduction implants. The Scientific Committee of Audiology of the Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello SEORL-CCC (Spanish Society of Otolaryngology and Head and Neck Surgery), together with the Otology and Otoneurology Committees, have undertaken a review of the current state of bone-conduction devices with updated information, to provide a clinical guideline on bone-conduction implants for otorhinolaryngology specialists, health professionals, health authorities and society in general. Methods: This clinical guideline on bone-conduction implants contains information on the following: 1) Definition and description of bone-conduction devices; 2) Current and upcoming indications for bone conduction devices: Magnetic resonance compatibility; 3) Organization requirements for a bone-conduction implant programme. Results and conclusions: The purpose of this guideline is to describe the different bone-conduction implants, their characteristics and their indications, and to provide coordinated instructions for all the above-mentioned agents for decision making within their specific work areas

Clinical guideline on bone conduction implants. / Guía clínica sobre implantes de conducción de vía ósea.

INTRODUCTION AND GOALS: During the last decade there have been multiple and relevant advances in conduction and mixed hearing loss treatment. These advances and the appearance of new devices have extended the indications for bone-conduction implants. The Scientific Committee of Audiology of the Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello SEORL-CCC (Spanish Society of Otolaryngology and Head and Neck Surgery), together with the Otology and Otoneurology Committees, have undertaken a review of the current state of bone-conduction devices with updated information, to provide a clinical guideline on bone-conduction implants for otorhinolaryngology specialists, health professionals, health authorities and society in general. METHODS: This clinical guideline on bone-conduction implants contains information on the following: 1) Definition and description of bone-conduction devices; 2) Current and upcoming indications for bone conduction devices: Magnetic resonance compatibility; 3) Organization requirements for a bone-conduction implant programme. RESULTS AND CONCLUSIONS: The purpose of this guideline is to describe the different bone-conduction implants, their characteristics and their indications, and to provide coordinated instructions for all the above-mentioned agents for decision making within their specific work areas.

Comparison of a Traditional and Novel Evoked Compound Action Potentials Recording Approach and Evoked Auditory Brainstem Responses in Pediatric Cochlear Implants Users.

OBJECTIVES: Electrically evoked compound action potentials (eCAP) recordings are widely used in functional evaluation and fitting of cochlear implants (CI) in clinics. We compared the results from two eCAP recording approaches (StandardART and FineGrain, MED-EL, Austria). The FineGrain method is more advanced than the Auditory Nerve Response Telemetry (StandardART) method in terms of the stimulation and algorithm for the eCAP threshold detection. To understand the benefits of these alterations, we compared the two methods on a larger scale in pediatric CI users alongside evoked auditory brainstem responses (eABR). MATERIALS AND METHODS: We collected the eCAP recordings obtained with both methods from a population of pediatric subjects with CI, either intra- or post-operatively. The eABR recordings were only collected post-operatively. For comparability reasons, we used the same stimulation rate and similar amplitude levels for all three approaches. RESULTS: Our results demonstrate that, although the success rates are similar, the FineGrain method outperforms traditional StandardART in terms of robustness and measurement duration. The eCAP recordings in general outperform the eABR in terms of speed. CONCLUSION: We conclude that the eCAP recordings are the method of choice for measuring the auditory neural activity, and FineGrain outperforms StandardART. From the three investigated approaches, we conclude that FineGrain performed best and should be the first-choice method in pediatric patients.

Electrically Evoked Auditory Brainstem Response over Round Window by Bipolar Stimulation.

OBJECTIVES: Auditory brainstem potentials can be elicited by electrical stimulation of the round window (RW). In this technique, extracochlear stimulation is objectively used in the selection of cochlear implant (CI) candidates to avoid cochlear damage. However, until now, its use is limited due to the large artifacts generated by electrical stimulation. Our objective was to obtain reliable and reproducible electrically evoked auditory brainstem responses (eEABRs) using a new method of stimulation. MATERIALS AND METHODS: This was a prospective study including subjects who underwent electrical stimulation on RW during CI surgery between 2013 and 2016. A "Stimulator Box," which produces electric stimuli identical to those provided by a CI, and an evoked potential recording equipment were used. The results obtained with monopolar and bipolar electrodes were compared. RESULTS: RW eEABR recordings of 49 subjects (mean age, 34 years) were characterized by their stability and by having wave V between 3 and 5.5 ms. A higher percentage of responses were obtained on increasing the phase duration instead of the pulse amplitude. A significantly greater percentage of positive responses were obtained using bipolar stimulation than using monopolar stimulation (p<0.001). CONCLUSION: Using extracochlear electrical stimulation technique, described herein, and bipolar electrical stimulation probe allows for reliable and reproducible eEABR recordings in CI candidates.

Rápida resolución de la dactilitis en un paciente con artritis psoriásica tras tratamiento con ustekinumab / Rapid dactylitis resolution in a patient with psoriatic arthritis after treatment with ustekinumab

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Hallazgos audiológicos en niños con mucopolisacaridosis tipos i-iv / Audiological findings in children with mucopolysaccharidoses type i-iv

Objetivo: Revisión y descripción de la afectación otoaudiológica en el seguimiento de 23 niños con diagnóstico de mucopolisacaridosis (MPS) tipo I, II, III y IV. Métodos: Estudio retrospectivo de los hallazgos clínicos, audiológicos y tratamiento (médico y/o quirúrgico) de 23 niños con diagnóstico de MPS tipo I, II, III o IV en seguimiento en un hospital terciario entre 1997 y 2015. Resultados: Seis casos de MPSI, 8 de MPSII, 4 de MPSIII y 5 de MPSIV fueron revisados. Al inicio del seguimiento el 71,2% de los pacientes presentaban otitis media serosa (OMS) y el 54% de los casos presentaban algún tipo de hipoacusia. El comportamiento de la hipoacusia fue fluctuante en cada uno de los subgrupos de MPS, encontrando mayor afectación y variabilidad en los tipos I y II. Conclusiones: Los niños afectos de MPS tienen un alto riesgo de hipoacusia, siendo MPS tipo I y II los casos con mayor porcentaje de afectación audiológica y con un comportamiento menos homogéneo, mostrando un importante porcentaje de hipoacusias transmisivas que progresan a componentes mixtos o neurosensoriales. Se requiere un seguimiento periódico dada la importante repercusión de esta patología en la calidad de vida y en el desarrollo de estos pacientes (AU)

Objective: The aim of our study is to reflect hearing impairment of 23 children diagnosed with mucopolysaccharidosis (MPS) type I, II, III and IV. Methods: Retrospective study of the clinical, audiological and treatment (medical vs surgical) findings of 23 children diagnosed with MPS type I, II, III or IV followed at a Tertiary Referral Hospital between 1997 and 2015. Results: Six cases of MPSI, 8 of MPSII, 4 of MPSIII and 5 of MPSIV were reviewed. 71.2% of patients had secretory otitis media (SOM) and 54% of patients had some type of hearing loss (HL). The behaviour of hearing loss was variable in each of the subgroups of MPS, finding greater involvement and variability in types I and II. Conclusions: Children with MPS have a high risk of hearing loss. A significant percentage of transmissive HL progressing to mixed or sensorineural HL was observed. This was more common in types I and II. Periodic follow up of these patients is mandatory because of hearing impairment and consequences for their development and quality of life (AU)

Implante coclear en paciente con schwannoma intracoclear / Cochlear implant in patient with intracochlear schwannoma

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