Rafael Ramírez-Camacho

Cogan's syndrome: an oculo-audiovestibular disease

Typical Cogans syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Ménière-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). Life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogans syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogans syndrome.

Familial Isolated Unilateral Large Vestibular Aqueduct Syndrome

Large vestibular aqueduct syndrome (LVAS) is one of the most frequent inner ear malformations. It is associated with varying degrees of hearing loss, that may be progressive, sudden or fluctuant; nevertheless normal hearing in cases with LVAS has been described. Although unilateral LVAS has been described, all patients show clinical and radiological findings in both ears, in contrast to the family presented here, where 2 siblings are affected by unilateral sensorineural hearing loss associated with ipsilateral LVAS, with an autosomal recessive pattern. In order to search for the origin of this inner ear malformation, mutations in genes GJB2 (connexin-26 gene), PDS (Pendred’s syndrome gene) and mitochondrial 12sRNA were studied, without positive results. In this paper we hypothesize the probable embryological origin for this malformation.

Chondrosarcoma of the Temporal Bone and Otosclerosis

Chondrosarcoma constitutes 6% of all primary bone tumors and 11% of malignant primary bone tumors. Nevertheless, in a review of the tumor registry of the University of Michigan covering a period of 50 years, there were only 3 cases involving the temporal bone. A case of a woman with a chondrosarcoma of the temporal bone that was partially resected by means of an infratemporal approach at the skull base is presented. This patient had previously undergone surgical treatment for otosclerosis of the other ear. Several considerations regarding survival factors in this type of tumor are dealt with in terms of the histological features, therapeutic options and anatomic location. The possibility that this lesion may originate from the persistence of the cartilaginous inclusions that some authors consider to be involved in the origin of otosclerosis is discussed.

Microscopia electrónica de barrido ambiental para la detección de biopelículas en las amígdalas

Introduccion y objetivo Describir un metodo ambiental con microscopio electronico de barrido para el estudio de biopeliculas en muestras clinicas. Se realiza una comparacion con la microscopia electronica de barrido convencional. Pacientes y metodo Se intervino de amigdalectomia a 9 pacientes con antecedentes de amigdalitis de repeticion. Se obtuvieron muestras de cada uno para microscopia electronica de barrido tanto convencional como ambiental. Como controles se empleo las amigdalas extirpadas de 2 pacientes intervenidos por sindrome de apnea del sueno. Resultados De las 9 amigdalas, 8 mostraron biopeliculas en su superficie. La microscopia electronica de barrido convencional mostro acumulaciones de bacterias cubiertas de estructuras fibrilares, originadas en el procedimiento de deshidratacion de las muestras. La tecnica ambiental proporciono una imagen de las bacterias sumergidas en una sustancia homogenea y amorfa, que se pudo conservar durante el examen microscopico. Conclusiones La microscopia electronica de barrido en su modalidad ambiental permite la observacion de muestras humedas en diferentes grados de deshidratacion. Por lo tanto, permite al investigador la observacion de las biopeliculas en su estado natural de hidratacion.

Environmental Scanning Electron Microscopy for Biofilm Detection in Tonsils

INTRODUCTION AND OBJECTIVE To describe an environmental scanning electron microscopic method for the study of biofilms in clinical samples. A comparison with standard scanning electron microscopy is performed. PATIENTS AND METHOD Nine patients with a past history of recurrent tonsillitis underwent tonsillectomy. Samples from each patient were obtained for both conventional and environmental scanning electron microscopy. The tonsils removed from 2 patients with sleep apnoea syndrome were used as controls. RESULTS Eight of nine tonsils had biofilms on their surface. Scanning electron microscopy showed accumulations of bacteria covered by fibrillar structures resulting from the sample dehydration process. Environmental scanning electron microscopy provided a view of bacteria embedded in a homogeneous, amorphous substance that was preserved during the examination. CONCLUSIONS Environmental scanning electron microscopy permits the imaging of wet systems at different degrees of dehydration. It therefore allows researchers to observe biofilms in their natural hydrated state.

Radiological diagnostic of the non-pathological conditions of the petrous apex

Abstract Many patients with otological symptoms are remitted to the otolaryngology outpatient clinics every day. These patients commonly undergo imaging studies, generally magnetic resonance imaging (MRI). In some cases, a positive unilateral result is found in the form of a potentially pathological signal that can be observed in the petrous apex region. We present the cases of 6 patients (aged between 26 and 62 years) with asymmetric bone marrow distribution or trapped mucous fluid secretions in the petrous apex, collected over a 6-year period. Diagnosis was made with the use of CT scans and MRI. All of the patients were referred for skull base surgery. In all cases a non-pathologic asymmetry was diagnosed in the petrous apex. Certain non-pathologic conditions of the petrous apex must be treated expectantly without any surgery.