1/50. Congenital malformation of the inner ear associated with recurrent meningitis.Congenital deformities of the labyrinth of the inner ear can be associated with meningitis and varying degrees of hearing loss or deafness. A recurrence of meningitis is due to the development of a fistulous communication between the subarachnoid space and the middle ear cavity, and can prove lethal. An illustrative case of a 4-year-old Japanese girl with bilateral severe hearing loss, recurrent meningitis and malformations of the inner ear and stapes footplate is presented. Removal of the stapes during tympanotomy provoked a gush of cerebrospinal fluid. The defect was repaired successfully, and there has been no further episodes of meningitis to date.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/50. facial nerve schwannoma in the cerebellopontine cistern. Findings on high resolution CT and MR cisternography.We report a case of facial nerve schwannoma originating in the cerebellopontine cistern, diagnosed by cochlear sensorineural hearing loss, and involvement in the facial nerve canal and the anterior epitympanic recess. Precise analyses of neuro-otological and neuroradiological studies are crucial to obtain an accurate preoperative diagnosis.- - - - - - - - - - ranking = 350.29196230433keywords = canal (Clic here for more details about this article) |
3/50. Lesions of the internal auditory canal and cerebellopontine angle in an only hearing ear: is surgery ever advisable?OBJECTIVE: To define the indications for surgery in lesions of the internal auditory canal (IAC) and cerebellopontine angle (CPA) in an only hearing ear. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. patients: Seven patients with lesions of the IAC and CPA who were deaf on the side opposite the lesion. Five patients had vestibular schwannoma (VS), and one each had meningioma and progressive osseous stenosis of the IAC, respectively. The opposite ear was deaf from three different causes: VS (neurofibromatosis type 2 [NF2]), sudden sensorineural hearing loss, idiopathic IAC stenosis. INTERVENTION(S): Middle fossa removal of VS in five, retrosigmoid resection of meningioma in one, and middle fossa IAC osseous decompression in one. MAIN OUTCOME MEASURE: Hearing as measured on pure-tone and speech audiometry. RESULTS: Preoperative hearing was class A in four patients, class B in two, and class C in one. Postoperative hearing was class A in three patients, class B in one, class C in two, and class D in one. CONCLUSIONS: Although the vast majority of neurotologic lesions in an only hearing ear are best managed nonoperatively, in highly selected cases surgical intervention is warranted. Surgical intervention should be considered when one or more of the following circumstances is present: (1) predicted natural history of the disease is relatively rapid loss of the remaining hearing, (2) substantial brainstem compression has evolved (e.g., large acoustic neuroma), and/or (3) operative intervention may result in improvement of hearing or carries relatively low risk of hearing loss (e.g., CPA meningioma).- - - - - - - - - - ranking = 1751.4598115217keywords = canal (Clic here for more details about this article) |
4/50. Extended middle fossa surgery for meningiomas within or at the internal auditory canal.OBJECTIVE: To show the clinical outcome in patients with meningioma within or at the internal auditory canal (IAC) operated on by the extended middle cranial fossa approach. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. patients: Twelve patients, 9 women and 3 men, whose ages ranged from 37 to 70 years (mean 57 years). One tumor was entirely intracanalicular, 5 had an intra-extracanalicular growth, 3 were centered at the posterior porus lip, and 3 tumors were localized in the midpetrosal region with spread into the IAC. INTERVENTION: Extended middle cranial fossa approach. MAIN OUTCOME MEASURES: magnetic resonance imaging and computed tomography were used for follow-up and reevaluation of the operative sites. facial nerve function and hearing were examined. RESULTS: Complete resection was achieved in 10 (83%) of 12 patients. The retrosigmoid approach was necessary to complete tumor resection in one patient, and in another, partial resection was done because of the en plaque type of tumor growth. There was one unexpected recurrence. All patients retained normal or near-normal facial nerve function postoperatively. Three patients were deaf before surgery, and preoperative hearing level was preserved in 42%. CONCLUSIONS: Attempted hearing preservation surgery is justified in patients with small tumors and preserved hearing, because tumor exposure and safety of resection are comparable with that of hearing-destructive procedures. Lateral extension of the tumor to the fundus does not prevent complete resection with preservation of function, and elective bone resections beyond the visible tumor margins seem not to substantially influence the tumor recurrence rates.- - - - - - - - - - ranking = 2452.0437361303keywords = canal (Clic here for more details about this article) |
5/50. Proximal symphalangism with "coarse" facial appearance, mixed hearing loss, and chronic renal failure: new malformation syndrome?A 25-year-old man is described with short stature, moderate mental retardation, an abnormal facial appearance, a webbed neck, skeletal abnormalities including proximal symphalangism of bilateral second through fifth fingers, mixed hearing loss, and slowly progressive, sclerosing nephropathy. He was large at birth with generalized edema, more pronounced around the jaw, neck and the upper part of the body, but became short with increasing age, and currently measures 143 cm (-4.9 SD). He had intermittent proteinuria and slowly progressive deterioration of the renal function. A biopsy of the left kidney showed global glomerular sclerosis with interstitial fibrosis. He was placed on maintenance peritoneal dialysis at age 17 years, and now on hemodialysis. His skeletal abnormalities included, in addition to proximal symphalangism, stenosis of the cervical canal, scoliosis, brachydactyly of the hands, hypoplastic hip joints, and pes valgus. Other abnormalities noted were a communicating defects of the diaphragm (surgically corrected), bilateral inguinal hernia and cryptorchidism. These clinical manifestations indicate a hitherto undescribed combination of manifestations and nephropathy.- - - - - - - - - - ranking = 350.29196230433keywords = canal (Clic here for more details about this article) |
6/50. Rasmussen encephalitis associated with segmental vitiligo of the scalp: clinicopathologic report.A 3-year-old boy with segmental vitiligo, poliosis, and alopecia over the right side of the scalp developed epilepsia partialis continua involving the left side of the body and progressive atrophy of the right cerebral hemisphere. There was a right ear dysacusia and a perilimbal vitiligo associated with an area of iris depigmentation in the right eye. Pleocytosis and hypergammaglobulin were detected in cerebrospinal fluid. Because medical treatment that included phenobarbital, phenytoin, carbamazepine, oxcarbazepine, benzodiazepines, corticosteroids, gamma-globulin, and a ketogenic diet was ineffective, he underwent a right hemispherectomy. Neuropathologic examination showed a widespread scattered inflammatory process with numerous microglial nodules and perivascular lymphocytic cuffing associated with degenerative changes with severe neuronal loss, loosening of the neuropil, and microcystic changes with tissue collapse. The coexistence of vitiligo and possibly Vogt-Koyanagi-Harada syndrome in this child reinforces the autoimmune theory as the pathogenesis of Rasmussen syndrome.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
7/50. facial nerve neuromas: report of 10 cases and review of the literature.OBJECTIVE: This study reviewed the management and outcomes of facial neuromas during the past decade at our institution. The goal was to analyze differences in presentation on the basis of location of the facial neuroma, review facial nerve function and hearing preservation postoperatively, and understand the characteristics of patients with tumors limited to the cerebellopontine angle or internal auditory canal. We also report an unusual case of a facial neuroma limited to the nervus intermedius. methods: Nine patients with facial neuromas and one with Jacobson's nerve neuroma underwent surgery, and total resection was accomplished in nine patients. A chart review for pre- and postoperative data was performed, after which all patients were evaluated on an outpatient basis. RESULTS: The mean age of the patients was 47 years; mean follow-up time was 33.1 months. The most common presenting symptoms were hearing loss (six patients) and facial paresis (five patients). A total of five patients had progressive (four patients) or recurrent (one patient) facial paresis. No patient experienced worsened hearing as a result of surgery, and one experienced improvement in a conductive hearing deficit. Five patients required cable graft repair of the facial nerve; four improved to House-Brackmann Grade 3 facial paresis. Four of five patients with preserved anatomic continuity of the facial nerve regained normal facial function. There were no surgical complications. No tumors have recurred during follow-up. We report the second nerve sheath tumor limited to the nervus intermedius. CONCLUSION: This series documents that facial neuromas can be resected safely with preservation of facial nerve and hearing function. Preservation of anatomic continuity of the facial nerve should be attempted, and it does not seem to lead to frequent recurrence. Tumors limited to the cerebellopontine angle/internal auditory canal are a unique subset of facial neuromas with characteristics that vary greatly from facial neuromas in other locations, and they are indistinguishable clinically from acoustic neuromas.- - - - - - - - - - ranking = 700.58392460866keywords = canal (Clic here for more details about this article) |
8/50. Trauma to the temporal bone: diagnosis and management of complications.The temporal bone contains important sensory and neural structures that may be damaged in patients who experience craniofacial trauma. The most serious complications of temporal bone trauma include facial nerve paralysis, cerebrospinal fluid leak, and hearing loss. Injury to the temporal bone often presents with subtle signs and symptoms, such as otorrhea, facial palsy, and hemotympanum. A high index of suspicion and a thorough knowledge of how to diagnose injury to the temporal bone are paramount in treating patients who present to the emergency room with craniofacial trauma. This article provides an overview of temporal bone trauma, outlines a methodical approach to the patient with temporal bone trauma, details four cases, and describes the treatment of complications.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
9/50. Long-term results and experience with the first-generation semi-implantable electromagnetic hearing aid with ossicular replacement device for mixed hearing loss.OBJECTIVE: To document the long-term surgical and audiologic results of the implantation of a first-generation semi-implantable hearing aid for mixed hearing loss, consisting of a magnetic partial or total ossicular replacement prosthesis and an electromagnetic driver fitted in the ear canal. The short-term results have previously been published as excellent in both surgical and audiologic terms. To analyze and discuss reasons of treatment failure, as well as future perspectives. STUDY DESIGN: Patient file review, follow-up otomicroscopic examination and audiometry, as well as patient interview. SETTING Tertiary referral center at a University hospital. patients: Of nine patients operated on, six with a mixed hearing loss after chronic otitis media could be evaluated primarily. Two underwent implantation with a partial and four with a total ossicular replacement prosthesis. MAIN OUTCOME MEASURES: Use of and satisfaction with the hearing aid, apparatus function and integrity, hearing without electromagnetic driver, prostheses containment, drum abnormalities, nonaudiologic ear symptoms, e.g., discharge. RESULTS: All patients attended follow-up for a mean of 9.5 years after implantation (range 8.8-10 years). No patients used the semi-implantable hearing aid at follow-up. The mean semi-implantable hearing aid using time was 24 months (range 3-60 months). The main reasons for discontinuation of use were problems fitting the electromagnetic driver correctly in the ear canal and prosthesis extrusion or dislocation. CONCLUSIONS: Despite excellent short-term surgical and audiologic results, it is concluded that these first long-term results of the first-generation electromagnetic semi-implantable hearing aids with ossicular replacement prosthesis for mixed hearing loss are disappointing. However, improvements in the surgical approach and in the reshaping, refinement, and overall improvement of the semi-implantable hearing aid could lead to satisfactory treatment of this group of patients.- - - - - - - - - - ranking = 700.58392460866keywords = canal (Clic here for more details about this article) |
10/50. pneumocystis carinii infection of the middle ear and external auditory canal. Report of a case and review of the literature.We present a 50-year-old male with pneumocystis carinii infection involving the middle ear and the external auditory canal as the first manifestation of a previously unknown hiv infection. In case of therapy-resistant otitis with a polypoid mass in the external auditory canal histological evaluation should be considered to rule out malignancy or pathogens, like pneumocystis carinii, that cannot be cultured. Oral or intravenous antiprotozoal agents are the treatment in line with current practice. Exploration of the os petrosum is never required.- - - - - - - - - - ranking = 2101.751773826keywords = canal (Clic here for more details about this article) |
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