A carcinoid tumor of the middle ear masquerading as a glomus tympanicum presenting with temporal lobe hemorrhage in a 70-year-old woman: Case report and review of the literature.

BACKGROUND: Paragangliomas in the central nervous system account for 0.6% of all head and neck neoplasms, with glomus tympanicum being the most common middle ear tumor. Carcinoid tumors are neuroendocrine tumors, representing less than 1% of neuroendocrine neoplasms in the middle ear. Misdiagnoses have been reported in the literature regarding glomus and carcinoid tumors, however, none have been in the central nervous system or middle ear. CASE DESCRIPTION: A 70-year-old female with a history of left temporal lobe tumor underwent unsuccessful resection due to intraoperative bleeding at an outside institution. However, biopsy prior to aborting the case led to the diagnosis of paraganglioma. Eight years postoperatively, the patient presented at our institution with acute confusion, aphasia, and altered mental status. Imaging revealed a 4cm left temporal intraparenchymal hematoma at the known tumor site with concern for intracranial tumor extension. Surgical resection was performed and previous symptoms resolved. Final pathology revealed a Grade II atypical carcinoid tumor with an unusually high Ki-67 of 50%. CONCLUSIONS: Carcinoid tumors of the middle ear constitute a differential diagnosis for patients presenting with temporal lobe hemorrhage. A combination of immunohistochemical staining with electron microscopy can assist in differentiating the tumor types. This atypical presentation for a carcinoid tumor in the middle ear suggests the need to consider carcinoid as the diagnosis in patients with a middle ear tumor invading into the temporal lobe and causing hemorrhage. These tumors may demonstrate an unusually high Ki-67 rate, in which case they should be treated aggressively.

The transcochlear approach revisited.

OBJECTIVE: To evaluate the indications for, techniques used in, and patient outcomes after surgery with the transcochlear approach in the treatment of petroclival tumors. METHODS: Retrospective review of 24 cases between 1985 and 1995 at the House Ear Clinic (Los Angeles, CA, U.S.A.). RESULTS: Meningioma was the most common tumor. Complete removal was achieved in 82% of tumors after one-or two-stage surgeries (average follow-up time, 36 months). The second-stage surgery was a middle fossa transpetrous approach. Most patients had some degree of facial nerve dysfunction immediately after surgery, and 12 of 20 patients subsequently improved to House-Brackmann Grade III or better. Fifty-nine percent of patients had permanent neurologic sequelae because of either the surgery or their disease. CONCLUSION: The transcochlear approach is best suited to treating petroclival intradural tumors that extend ventrally to the brainstem in patients without serviceable hearing. Temporary facial weakness is expected as a result of posterior facial nerve transposition.

Estenosis laringotraqueal. Experiencia con la técnica de resección cricotraqueal / Laryngotracheal stenosis. Experience with cricotracheal resection

La estenosis laringotraqueal es una complicación de la entubación endotraqueal. Su manejo requiere diferentes técnicas reconstructivas según la severidad de la estenosis. La laringotraqueoplastia de expansión con injerto costal autólogo es la técnica más popular, aunque los resultados reportados han sido variables. Motivados por los resultados inadecuados con esta técnica en casos de estenosis de grado severo, comenzamos a usar la técnica de resección cricotraqueal con anastomosis tiro-traqueal término-terminal. Ocho pacientes, niños y adultos, han sido operados a lo largo de 2 años. Cinco de ellos habían sido previamente operados con laringotraqueoplastia sin éxito. Siete de los ocho pacientes fueron decanulados, y la morbilidad fue tolerable. Con respecto a la morbilidad postoperatoria, casi todos los pacientes sufren de disfonía leve, uno sufre de disnea a esfuerzos, y otro paciente sufre de aspiración leve. Este último paciente necesitó además aritenoidectomía para corrección de estenosis glótica concomitante. Presentaremos nuestra evaluación preoperatoria, la técnica quirúrgica, los cuidados posoperatorios, las complicaciones y los resultados. Nuestros resultados demuestran que la resección cricotraqueal con anastomosis tiro-traqueal primaria es una técnica efectiva y relativamente segura para la correlación de estenosis laringotraqueal severa tanto en niños como adultos y que puede combinarse con la aritenoidectomía para estenosis glótica asociada

An analysis of a large postlingually deaf family with hereditary non-syndromic profound hearing loss.

We initiated a clinical and genetic linkage study on members of a large Venezuelan family with hereditary hearing loss. A medical history and a physical examination were performed on 30 family members. Audiometry was carried out in 25 subjects, and in 2 additional children auditory brainstem responses were obtained. Additional testing (site-of-lesion, electronystagmography and computed tomography) was also obtained in a few subjects. DNA was extracted from blood samples from 25 family members. The type of deafness in this family is neurosensorial, non-syndromic and postlingual. The average age of onset of deafness is 7 years and there is a rapid progression leading to profound deafness. Deafness is possibly of cochlear origin and there is no associated vestibular pathology. Analysis of the pedigree discloses a maternal pattern of inheritance with a significant female predominance, compatible with a mutation of the mitochondrial DNA. The molecular DNA analysis for the known mitochondrial mutations are discussed.

Primary lymphoma of the internal auditory canal. Case report and review of the literature.

We report a rare case of a primary intracranial B cell lymphoma originating in the internal auditory canal. The clinical manifestations were indistinguishable from those of other, more common tumors of the same region. We achieved total gross tumor removal with preservation of the facial nerve. A detailed histologic examination and a systemic workup confirmed the primary nature of this tumor. To our knowledge, this is the second case reported in the literature of a primary malignant lymphoma originating in the internal auditory canal. This is the first instance that includes immunohistochemical and cytometric studies of fresh tissue. We discuss the management of primary lymphomas of the central nervous system, with special emphasis on their association with acquired immunodeficiency syndrome and other immune system diseases. Awareness of primary central nervous system lymphomas is important, since a greater occurrence of these rare tumors in the cerebellopontine angle is probable in the future.

Surgical treatment of the facial nerve in facial paralysis.

This article discusses the surgery for acute facial paralysis of idiopathic, traumatic, infectious, and neoplastic etiologic agents. Indications, results, and details of surgical techniques of facial nerve exploration, decompression, rerouting, and repair are provided.

Langerhans' cell histiocytosis of the head and neck in children.

This is a retrospective analysis of a 29-year institutional experience with Langerhans' cell histiocytosis (LCH) in children. Cases of LCH were categorized by disease extent into three groups: group 1, with a solitary focus of LCH; group 2, with multiple non-vital organ foci; and group 3, with vital organ disease. Sixteen patients averaging 7.3 years of age and with 11.0 years of follow-up presented in group 1. One child experienced a spontaneous remission; all other children responded to local treatment. The skull was the most common site of involvement. Eight children averaging 4.6 years in age and with 8.0 years of follow-up presented in group 2; 3 children had diabetes insipidus. Treatment included radical surgery, radiotherapy, and chemotherapy; however, disease persisted in all patients. Iatrogenic complications were a significant cause of morbidity and mortality. Only 1 patient, 1 year of age, presented with lung and liver involvement (group 3). He died after a fulminant course. Current recommendations for diagnosis, evaluation, and treatment of LCH are discussed.

Glomus jugulare tumors masquerading as benign intracranial hypertension.

Intracranial hypertension has been reported as a complication of massive glomus jugulare tumors with intracranial extension. We describe a patient with papilledema, failing vision, and a diagnosis of benign intracranial hypertension with bilateral glomus jugulare tumors that went undetected for 2 years. There was no evidence of intracranial invasion by the tumors to explain the elevation of intracranial pressure. Embolization followed by surgical removal of the left tumor resulted in stabilization of the neurologic condition and preservation of the lower cranial nerves, including intact hearing. The likely mechanism of intracranial hypertension in this case is an impairment of cerebrospinal fluid absorption. This unusual presentation should be recognized to avoid delayed diagnosis and treatment. Furthermore, intracranial hypertension is not always associated with massive intracranial tumor involvement, as had been previously proposed.

Indium 111-labeled white blood cell scintigraphy as an unreliable indicator of malignant external otitis resolution.

The field of otolaryngology-head and neck surgery has seen many advances in the treatment and prognosis of malignant external otitis (MEO). However, establishing the resolution of the infection remains problematic. A recent report suggests that indium 111-labeled white blood cell scintigraphy may be a reliable and timely indicator of resolution of infection. We present a case of a false-negative white blood cell scan in a patient with persistent MEO. A discussion of this case and a review of the literature illustrate that there continues to be no "gold standard" for establishing MEO resolution.