Tuberculous otitis media: report of 2 cases on Long Island, N.Y., and a review of all cases reported in the United States from 1990 through 2003

We report 2 cases of tuberculous otitis media that were diagnosed at Stony Brook University Hospital in New York since 1999. Both patients were women, aged 30 and 31 years. One patient had grown up in Russia, the other was a native-born American who had never left the East Coast region of the United States. Both patients had been symptomaticfor many months; one complainedof chronic otorrhea, and the other reported otorrhea, hearing loss, and discomfort. Neither patient responded to medical management, and both ultimately underwent surgery. One was diagnosed after surgical pathology revealed acid-fast bacilli on frozen-section analysis. In the other, pathology revealed chronic inflammation and granulomata, butstains were negative and her diagnosis was delayed for almost 2 years. We also review 9 other cases of tuberculous otitis media in the United States that have been reported in the literature since 1990. Our review suggests that the number of cases is rising in areas where tuberculosis is most common--that is, in major U.S. cities. Although 3 of these 9 cases occurred as reactivation disease in immigrants, most might have occurred as a result of local transmission. Clinicians should maintain a high degree of suspicion for tuberculosis in patients with chronic otitis symptoms, particularly those who are at higher risk of exposure to tuberculosis.     

Nasopharyngeal neuroglial heterotopy - Choristoma: Case report and review of the literature

We describe the case of a nine-month-old patient with a nasopharyngeal choristoma. The case presented includes the retrospective review of the historical, radiological, surgical and histological assessment of this pathology as well as a literature review of this entity. This case was presented in an infant with difficulty feeding, nasal obstruction and failure to thrive, evaluated with flexible nasal endoscopy, CT and MRI. The lesion was then surgically removed without complications. Nasopharyngeal choristoma is a rare congenital non-malignant mass, which may present within a range of symptoms and severity according to its size, growth and location.     

Diagnostic difficulties of chondrosarcoma of the jaw. Apropos of a case and review of the literature

Chondrosarcoma is an uncommon malignant mesenchymal tumor characterized by the production of cartilaginous tissue and the absence of production of bone tissue. Maxillary and mandibular localizations are extremely rare and have a poor prognosis. The clinical and radiographic findings are similar to those seen in other tumors of the jaw, often delaying diagnosis and treatment and subsequently jeopardizing prognosis. In the literature, the mean delay from first clinical signs to diagnosis is about 8 months. As in all tumoral diseases, pathology confirms the diagnosis. We report a case of chondrosarcoma of the maxillary bone and review the literature, focusing on the difficulty in establishing the diagnosis, even at the pathology examination. We propose wide surgical excision, which, in agreement with other reports in the literature, is the only therapeutic possibility. We observed no sign of recurrence at 5-year follow-up. Radiotherapy and chemotherapy may be useful in certain cases.     

Schwannoma of the chorda tympani nerve

We report a case of schwannoma of the chorda tympani. This is a very rare benign tumour and only five other cases have been found in the literature. This is the first case to mimic a cholesteatoma presenting as a pearly tumour in the postero-superior segment of the drum with aural discharge and conductive deafness. Diagnosis is usually by biopsy and treatment is surgical with preservation of facial and auditory function. A summary of the other presentations of this tumour together with a review of the histopathology of the disease is presented.     

Importance of the stapedial reflex in the diagnosis of several pathologies

The stapedius muscle plays an important role in auditory physiology. Its function may be assessed using a test named impedancemetry, where its reflex is measured. For the stapedial reflex to function, it is necessary that the middle ear and the afferent and efferent pathways are intact. Several pathologies of the middle and internal ear, as well as other otoneurological disorders and systemic illnesses, may change the physiology of the stapedius muscle. This review has incorporated various bibliographic sources with the objective to show the modifications of the stapedial reflex in several types of pathology. It reveals that the research of the stapedial reflex as well as its interpretation constitute a very important diagnostic supplement in the clinical audiology.     

Ameloblastoma: a rare nasal polyp

Ameloblastoma is an odontogenic neoplasm of enamel organ type tissue which does not undergo transformation to the point of enamel formation. We present the second case in the English literature of maxillary ameloblastoma that presented with nasal obstruction and rhinorrhoea, and the first to be excised using a combined maxillotomy and endoscopic ethmoidectomy. The patient had no previous dental history. The unusual presenting symptoms, as well as the highly destructive nature of these lesions when arising in the maxilla, make them worthy of consideration in the differential diagnosis of nasal and maxillary masses. We discuss the clinical features, pathology and management of these lesions and review the literature.     

A role for epigenetics in hearing: Establishment and maintenance of auditory specific gene expression patterns

Epigenetics is a large and diverse field encompassing a number of different mechanisms essential to development, DNA stability and gene expression. DNA methylation and histone modifications work individually and in conjunction with each other leading to phenotypic changes. An overwhelming amount of evidence exists demonstrating the essential nature of epigenetics to human biology and pathology. This field has spawned a vast array of knowledge, techniques and pharmaceuticals designed to investigate and manipulate epigenetic phenomena. Despite its centricity to molecular biology, little work has been conducted examining how epigenetics affects hearing. In this review, we discuss both the basic tenets of epigenetics and highlight the most recent advances in this field. We discuss its importance to human development, genomic stability, gene expression, epigenetic modifying agents as well as briefly introduce the expansive field of cancer epigenetics. We then examine the evidence of a role for epigenetics in hearing related processes and hearing loss. The article concludes with a discussion of areas of epigenetic research that could be applied to hearing research.     

Laryngo-tracheal estenosis in adults. A restrospective study of 21 cases and literature review

We are reporting a retrospective study of 21 adults diagnosed of laryngo-tracheal stenosis and admitted to our Department between 1990 and 2001. In 9 cases (42.85%) it was due to prolonged orotracheal intubation; other 10 were secondary to tracheostomy (47.62%) and the rest, to coniotomy. We have studied and analysed these variables: Age, gender, personal history, level of stenosis, its grade according to Cotton's clasification, treatment and clinical evolution with an average follow-up of 12 months. Our main goal was to get people to know on experience with this pathology and to carry out a literature review in order to compare our data and results with other published series.     

Post-traumatic carotid cavernous fistula as a cause of objective tinnitus

Reporting the case of a patient, seen in our Hospital Consulting Department, enduring heavy pulsatile tinnitus, following a craniocerebral traumatism. ENT examination was negative, but having the feeling of a carotid-cavernous fistula, angiographies confirmed this pathology. Embolisation of the fistula as treatment stopped the patient's symptomatology. We take the advantage of this case in order to review the etiology, the pathology, diagnostic measure and treatment of this condition.     

Cervical osteophytosis as cause of dysphagia. Forestier-Rotes Querol's disease

The so called Forestier-Rotes Querol's disease or diffuse idiopathic skeletal hyperostosis can affect the neck caused by osteophytos and ossification-calcification of the vertebral ligaments. The location in that region can produce dysphagia or hoarseness, among other symptoms, so that the patient can assist or be sent to ENT valoration. We are reporting a case of this pathology which was diagnosed in our hospital. Finally a bibliographic review is performed.     

Current update on established and novel biomarkers in salivary gland carcinoma pathology and the molecular pathways involved

This review aims to take stock of the new information that has accumulated over the past decade on the molecular pathology of salivary gland cancer. Emphasis will be placed on established and novel immunohistochemical markers, the pathways involved, and on findings of prognostic importance as well as new therapeutic concepts. Whenever reasonable, analogies to tumors of better explored, histologically related glandular organs such as pancreas and breast are established.     

Infiltrating lipoma of the head and neck: a report of one pediatric case

Infiltrating lipomas are rare benign tumors. Several cases have previously been reported in the oral cavity but only three cases have been reported to date in children. We report a case of a 7-year-old child with an infiltrating lipoma of the neck and a posterior extension to the fourth and fifth cervical roots and the vertebral artery. The absence of any neurological signs, negative clinical and radiological examination results, as well as, the surgical risk of total removal and high rate of recurrence suggested a period of watchful waiting. After 5 years, the patient's clinical and radiological characteristics remain stable. A review of the literature regarding this pathology in the head and neck area, in both children and adults is also presented.     

Kikuchi's disease: a case report and review of the literature

Kikuchi's disease is a necrotizing lymphadenitis that is prevalent in Asia and is being increasingly recognized in other areas of the world. It usually occurs in women in their late 20s or early 30s and manifests as a posterior cervical adenopathy. It resolves spontaneously, usually over a period of several weeks to 6 months. Its initial clinical appearance is commonly similar to that of a lymphoma, and it can be pathologically misdiagnosed as such. Kikuchi's disease might be associated with systemic lupus erythematosus. We report a case of Kikuchi's disease that occurred in a 36-year-old Asian woman. We discuss the clinical features, differential diagnosis, radiographic evaluation, and pathology of this case, and we review the literature in an effort to assist otolaryngologists in diagnosing this benign and uncommon entity.     

Chiari-I malformation associated with asymmetric sensorineural hearing loss.

Chiari-I malformation is a deformity of the structures of the posterior fossa in which there is inferior herniation of the cerebellar tonsils through the foramen magnum without significant caudal dislocation of the brainstem. Patients are usually asymptomatic until adulthood, when they commonly present with recurrent headaches, weakness, vertigo and/or imbalance, nystagmus and hearing loss. A review of 226 consecutive patients evaluated for asymmetric sensorineural hearing loss revealed 32 patients with retrocochlear pathology. Three of these patients were discovered to have a Chiari-I malformation by magnetic resonance imaging as their only pathology. We suggest a possible association between a Chiari-I malformation and isolated asymmetric sensorineural hearing loss secondary to long-standing traction on the eighth cranial nerve.     

The adenoid cystic carcinoma

The adenoid cystic carcinoma arising in the external auditory canal is a rare tumor, as other malignant neoplasias of the EAC and middle ear. The early diagnosis of these malignant tumors of the ear is very important to improve the prognosis. In this sense, the clinic experience is frequently the most useful diagnosis instrument to suspect this pathology when we have a confrontation with chronic otorrea and otalgia, common presentation form of this disease. In this report we show a new case of adenoid cystic carcinoma of external auditory canal and review the most significant medical literature existing in relationship with this pathology.     

Brown bone tumor as the first manifestation of primary hyperparathyroidism

We report a clinical case of a 26 years old female who had a 2 years evolution chin tumour with hypercalcemia (11.8 mg/dl) and PTH (paratohormone) of 761 pg/ml. She underwent a CT scan and MRI of the mandible, as well as a biopsy followed by excision of the tumour by the maxilofacial surgeons. Our ENT Department asked for a Scintigraphy (Tc99s-mibi) and thoracic-cervical CT, which showed a lesion that turned out to be an adenoma of the lower right parathyroid gland after surgery and pathological examination. The patient suffered a Primary hyperparathyroidism that was the main stimulus for the Brown Tumour made up by macrophagos and multinuclear giant cells, being this the first manifestation of the metabolic disorder. This form of hyperparathyroidism is very rare in the clinic. We do a literature review to establish the differential diagnosis for such pathology.     

Metastatic testicular seminoma--a case report

We present the case of a 42-year-old male who presented with a hot, tender swelling in the left supraclavicular fossa. He was pyrexial on presentation with a mildly elevated leucocyte count of 12.4x10(9)/l. Clinical examination, including full ear, nose and throat assessment, proved unremarkable. The medical history revealed that 2 years earlier the patient had been diagnosed with a testicular seminoma for which he underwent a right inguinal orchidectomy and abdominal radiotherapy. CT scan highlighted a 6 cm para-laryngeal mass, of mixed attenuation, with an adjacent region of inflammation. Overall appearance was suggestive of an infective mass. Ultrasound-guided fine needle aspiration cytology revealed a metastatic seminomatous deposit. Imaging of the chest and abdomen revealed this as the only site of metastasis. He is currently undergoing chemotherapy, and is responding well. We review the pathology of testicular seminoma. This case highlights the myriad of pathologies that may present as a lump in the neck.     

Tinnitus and brain activation: insights from transcranial magnetic stimulation

The mechanisms underlying tinnitus are still not completely elucidated, but advances in neuroimaging and brain stimulation have provided us with new insights. Evidence suggests that tinnitus might actually be generated by central rather than peripheral structures. To illustrate the importance of brain activity changes in the pathology of tinnitus, we report the cases of 2 patients who experienced a recurrence/worsening of their tinnitus after they had undergone treatment for major depression with repetitive transcranial magnetic stimulation. We suggest that the tinnitus in these 2 patients was induced by changes in brain activity resulting from transcranial magnetic stimulation of the prefrontal cortex. We also review the pathophysiology and other aspects of tinnitus, focusing on associated brain reorganization.     

Thyroidectomy and neck dissection for the carcinoma of the thyroid gland in children

A peculiarity in thyroid cancer in children is discussed in terms of clinical course and pathology. A case report of well differentiated papillary carcinoma of the thyroid gland in a 6-year-old boy is presented, with emphasis on the clinical course and pathology. The clinical course indicated a slowly growing, firm tumor lateral to the superior cornu of the thyroid cartilage, up to the hyoid bone. As treatment of the tumor and its lymph node metastases, a hemithyroidectomy with simple neck dissection was performed. Thyroid hormone was administered for suppression of endogenous thyroid-stimulating hormone postoperatively, and the patient tolerated this therapy well. An review of the literature dealing with the question of whether or not a radical neck dissection is appropriate in thyroid cancer in children was carried out.