Expression of brain-derived neurotrophic factor and its receptor mRNA in the vestibuloauditory system of the bullfrog.

Brain-derived neurotrophic factor (BDNF) is a neurotrophin which has been suggested to play a crucial role in the development and maintenance of the inner ear. In the present study, we investigated the expression of mRNAs of BDNF and its high-affinity receptor trkB in the vestibuloauditory system of the adult bullfrog. In situ hybridization was performed using riboprobes transcribed from Xenopus BDNF and trkB cDNA clones. BDNF mRNA was expressed in the sensory epithelia of the ampullary cristae, utricular and saccular maculae, lagena, and amphibian and basilar papillae. Strong hybridization for BDNF mRNA was also found in neuron somata of the vestibuloauditory nuclear complex. trkB mRNA was detected in the sensory epithelia of all vestibular and auditory endorgans. High levels of both BDNF and trkB mRNAs were found in vestibuloauditory ganglion cells. These results support the hypothesis that BDNF participates in the maintenance of vestibuloauditory neurons and may be important for the trophic regulation of vestibular and auditory sensory epithelia in this animal model.

Orbital lesions in the blue rubber bleb nevus syndrome.

PURPOSE: To identify ophthalmologic manifestations of the blue rubber bleb nevus syndrome, a rare cutaneovisceral hemangiomatosis. METHODS: The authors report two patients with a diagnosis of blue rubber bleb nevus syndrome with orbital hemangiomas. RESULTS: In one patient, the orbital lesion presented with signs and symptoms similar to an orbital varix and in the other with lid ecchymosis from an eyelid lesion. CONCLUSION: Patients with the blue rubber bleb nevus syndrome may have vascular orbital lesions associated with intermittent proptosis. Ophthalmologists should be familiar with the syndrome and its life-threatening complication of gastrointestinal hemorrhage.

Cholesteatoma of the maxillary sinus.

Cholesteatoma of the maxillary sinus, also known as keratoma, primary epidermoid tumor, epidermoid cyst, and keratocyst, is a rare entity, with a clinical presentation and radiologic findings that are difficult to distinguish from those of malignancy. We report a case of cholesteatoma of the maxillary antrum that occurred in a 12-year-old boy without any history of predisposing factors. Multiple theories of pathogenesis have been proposed throughout history; currently there are two accepted ones. We discuss these theories and present, along with the differential diagnosis, a review of the literature and the characteristic radiologic and pathologic findings. While it is a rare entity, the diagnosis of cholesteatoma should be considered for any slowly expanding lesion of the maxillary antrum.

Selective identification of the paternal mitochondrion in living sea urchin eggs and embryos by chlorotetracycline.

When sea urchin eggs are pretreated with fluorescent chelate probe chlorotetracycline (CTC) and then fertilized with unlabeled sperm, a small, brightly fluorescent particle resembling the mitochondrion of free-swimming sperm both in size and fluorescent staining characteristics appears in the egg cytoplasm. This particle first appears near the base of the insemination cone and, like the paternal mitochondrion identified in previous ultrastructural studies, remains closely associated with the male pronucleus during its microtubule-dependent migration toward the egg center. These similarities strongly suggest that the fluorescent particle observed in the cytoplasm of living, CTC-pretreated sea urchin eggs is, in fact, the mitochondrion of the fertilizing sperm.

Rhabdomyosarcoma of the head and neck.

A retrospective review of all cases of rhabdomyosarcoma seen at UCLA was done. Survival prior to the advent of cyclic multidrug chemotherapy was compared to survival with this therapy. In the latter group, unlike other published series, survival was 100% better with gross total removal of disease as compared to biopsy only. All received at least 5000 rads. Fifteen percent presented with regional nodal metastases. However 75% of these also had intracranial extension and all died. An additional 19% developed late regional nodal metastases; 80% of these also developed systemic metastases and all died. Survival in the absence of nodal disease was 41%.

Osteosarcoma and chondrosarcoma of the head and neck.

Osteosarcomas and chondrosarcomas are rare malignant neoplasms in the head and neck. Eighteen cases seen at the UCLA Center for the Health Sciences are reviewed. Osteosarcomas of the maxilla tended to occur a decade later than those of the mandible or cranial bones. Several followed prior irradiation for retinoblastoma. Complete excision offers the only realistic chance for cure. Adjuvant therapy has little to offer these patients at this time.

Verrucous carcinoma of the frontal sinus: a case report and review of the literature.

Carcinoma of the frontal sinus is rare. Treatment is controversial, and prognosis is poor, The etiology is unknown, although some risk factors for other paranasal sinus carcinomas have been identified. A 70-year-old male presented with proptosis and x-ray findings consistent with a mucocele of the frontal sinus. At operation the patient was felt to have a cholesteatoma. Pathologic diagnosis was verrucous carcinoma of the frontal sinus. The case is discussed and the literature is reviewed.

Carcinoma of the tongue in persons younger than 30 years of age.

Squamous cell carcinoma of the tongue in young people is a rare disease. The usual causal agents associated with this disease in older patients may not be operative in this younger age group or may involve a greatly reduced latency. Failure to perform a biopsy of tongue lesions in young patients often leads to late diagnosis. The prognosis is similar, stage for stage, as for older patients. Thirteen cases of squamous cell carcinoma of the tongue occurred in patients younger than 30 years of age. Their clinical appearance, stage and histologic state, treatment, and survival are discussed. A high index of suspicion leading to early diagnosis and systematic adequate treatment are needed to alter the unfavorable prognosis of this lesion.

Rhabdomyosarcoma of the head and neck.

A retrospective review of all cases of rhabdomyosarcoma seen at UCLA was done. Survival prior to the advent of cyclic multidrug chemotherapy was compared to survival with this therapy. In the latter group, unlike other published series, survival was 150% better with gross total removal of disease as compared to biopsy only. All received at least 5000 rads. Fifteen percent presented with regional nodal metastases. However 75% of these also had intracranial extension and all died. An additional 19% developed late regional nodal metastases. Eighty percent of these also developed systemic metastases and all died. Survival in the absence of nodal disease was 41%. This is the first study to address the influence of regional nodal metastases, early and late on the outcome of treatment. In rhabdomyosarcoma of the head and neck, regional nodal metastases at any time implies a grave prognosis and more seriously adversely affects the progress than it does in squamous cell carcinoma in adults.

Familial carcinoma of the submandibular gland. A case report and an epidemiologic review.

Cases of submandibular carcinomas in two siblings are reported. A familial occurrence of submandibular gland neoplasms has not been previously reported. Postulated causes and predisposing factors in salivary neoplasms are reviewed. None of these theories explains the cause of the unusual familial occurrence of the neoplasms in these cases.

Superior laryngeal nerve paralysis and benign thyroid disease.

A case of an isolated superior laryngeal nerve paralysis from a thyroid adenoma is presented. Superior laryngeal nerve paralyses should be sought, particularly in the preoperative and postoperative examination of thyroidectomy patients. Symptoms of a change in vocal strength or pitch and aspiration along with the laryngoscopic findings of a glottis posteriorly rotated toward a bowed vocal cord are diagnostic. Surgical trauma to the superior laryngeal nerve, though a risk of any thyroidectomy, usually can be avoided if one knows its possible anatomic variations and meticulously dissects the superior thyroid pole and its vessels.

Histopathology of congenital subglottic stenosis.

Three patients with congenital subglottic stenosis are presented and whole organ serial-section studies of their larynges are discussed. A superiorly displaced first tracheal ring is observed to form a cartilaginous subglottic stenosis in one. This "trapped first ring" is demonstrated in horizontal, sagittal and coronal planes. Subglottic stenosis is a clinical diagnosis which describes multifarious histopathological forms of narrowing within the subglottic larynx.