1/45. E.N.T. manifestations of Von Recklinghausen's disease.Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/45. Salivary gland choristoma of the middle ear: case treated with KTP laser.Salivary gland choristoma of the middle ear is rare. It consists of non-malignant, non-growing, normal salivary gland tissue in the middle ear. It is a developmental abnormality that occurs around the proximal part of the second branchial arch before the fourth month of intrauterine life. The authors found the 25th recorded case in our centre and another 24 reported cases from a review of the literature between 1961 and 1999. Intratympanic salivary gland choristoma frequently occurs during the first and second decades of life and with a female preponderance (56 per cent). Nearly all the patients (96 per cent) in our review presented with a hearing loss, that had begun since birth, in infancy, or during childhood. tinnitus (28 per cent), and serous otitis media (24 per cent) were also commonly present. One case complained of otorrhoea. Intratympanic and extratympanic anomalies were found in 96.2 per cent and 34.6 per cent of cases respectively. Of these anomalies, ossicular chain (88.5 per cent), facial nerve (65.4 per cent), middle-ear muscles (30.8 per cent) and labyrinthine windows (23 per cent) were the four most common sites. Therefore, salivary gland choristoma may represent a manifestation of a congenital ear anomaly. Diagnosis of salivary gland choristoma is generally not documented pre-operatively, but is based on surgical biopsy and histopathological investigations. Treatment of this rare lesion depends on the size, location and extent of the mass, degree of anatomical abnormality and expertise of the surgeon. In difficult cases where the mass is attached to the dehiscent or inferiorly placed facial nerve, only biopsy is recommended. However, complete surgical removal is advocated for a mass that is easy to remove. KTP laser use via a 200 micron fibre-optic light carrier can facilitate removal especially in cases with ossicular chain involvement.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
3/45. Complications of venous insufficiency after neurotologic-skull base surgery.OBJECTIVE: To characterize the incidence and complications resulting from venous insufficiency after neurotologic-skull base surgery. STUDY DESIGN: Retrospective case review of >3,500 cases. SETTING: Tertiary referral center, inpatient surgery. patients: Six patients: four with complications related to chronic venous insufficiency and two with complications related to acute venous insufficiency. INTERVENTION(S): Medical (steroids, acetazolamide, hyperventilation, mannitol) and surgical (lumboperitoneal shunt, optic nerve decompression, embolectomy) interventions were undertaken. MAIN OUTCOME MEASURE(S): Chronic venous insufficiency: nonobstructive hydrocephalus manifested by headache, disequilibrium, and papilledema with resultant visual loss. Acute venous insufficiency: acute nonobstructive hydrocephalus resulting in mental status abnormalities in the postoperative period. CONCLUSIONS: (1) incidence of 1.5 per 1,000 cases. (2) Acute and chronic forms with different pathogenesis. (3) Acute form presents postoperatively with change in consciousness and herniation, and may proceed to death. (4) Chronic form presents months or years postoperatively with headache, disequilibrium, and visual changes from papilledema. (5) Occurs almost solely in patients with preoperative abnormalities of the venous collecting system. (6) Causes mental status changes postoperatively.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/45. choristoma of the middle ear: a component of a new syndrome?CASE REPORT: Salivary choristoma of the middle ear is a rare entity. The authors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the otic capsule and facial nerve in ways not yet reported. Our patient presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the literature revealed salivary choristomas of the middle ear to be frequently associated with branchial arch abnormalities, most commonly the second, as well as abnormalities of the facial nerve. review OF THE literature: All 25 cases were reviewed and the results reported with respect to clinical presentation, associated abnormalities, operative findings, and hearing results. It has been proposed that choristoma of the middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of the facial nerve. CONCLUSION: Eighty-six percent of the reported patients with choristoma have three or four of the four criteria listed to designate middle ear salivary choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
5/45. Wegener's granulomatosis presenting with bilateral facial nerve palsy.Primary manifestation of Wegener's granulomatosis in the mucosa of the middle ear is rather rare, and has been reported as presenting with serous otitis media, chronic otitis media, sensorineural hearing loss, and, in rare instances, unilateral facial palsy. Bilateral facial palsy has never been reported. This last fact constitutes the interest in our report of a 23-year-old female patient who presented with symptoms of recurrent bilateral otitis media, eventually developing sensorineural hearing loss and bilateral facial palsy. Soon thereafter neurological symptoms appeared and lung extension was noted. Histological examination of repeated biopsies taken from the nasal and middle ear mucosa was not conclusive for the suspected disease, and c-ANCA titers were also initially repeatedly negative. Eventually, positive lung biopsy and elevated c-ANCA titers when the patient had developed pulmonary granulomas confirmed the diagnosis of Wegener's granulomatosis. mastoid surgery with facial nerve decompression of the most severely afflicted side did not result in the recovery of facial nerve function. Medical therapy with corticosteroids and cyclophosphamide improved the clinical picture but were ineffective in improving the bilateral sensorineural hearing loss and the facial paralysis on the operated side. We would contribute to the literature a unique case of bilateral facial nerve palsy due to Wegener's granulomatosis.- - - - - - - - - - ranking = 7keywords = nerve (Clic here for more details about this article) |
6/45. Eighth nerve aplasia and hypoplasia in cochlear implant candidates: the clinical perspective.OBJECTIVE: To identify the clinical and radiologic characteristics of aplasia and hypoplasia of the eighth nerve. STUDY DESIGN: Retrospective case-note review. SETTING: Cochlear implant program. patients: All children at the authors' institution in whom the cochlear implant assessment failed because of absence or hypoplasia of the eighth nerve. INTERVENTION: Computed tomography of petrous bones and magnetic resonance imaging of the brain. MAIN OUTCOME MEASURES: Presence or absence of eighth nerve and other radiologic factors contraindicating implantation. RESULTS: Of 143 cochlear implant candidates, 237 were judged ineligible for cochlear implantation. The preimplant assessment failed in 10 candidates of 143 because of bilateral aplasia or hypoplasia of the eighth nerve (7 cases) or unilateral aplasia or hypoplasia of the eighth nerve and a contraindication to operation on the other side (3 cases). The aplasia or hypoplasia of the eighth nerve was confirmed by magnetic resonance imaging in seven cases (5%): six were syndromic (3 CHARGE, 1 VATER-RAPADILLINO, 1 Mobius, 1 Okihiro), and one was nonsyndromic autosomal-recessive. All seven children had delayed motor milestones and absence of auditory brainstem responses. CONCLUSION: Aplasia and hypoplasia of the eighth nerve are not uncommon in pediatric cochlear implant candidates, particularly in the presence of a syndrome such as CHARGE. magnetic resonance imaging of the brain is mandatory before implantation because it can identify the presence or absence of the eighth nerve. parents of children with profound hearing loss, delayed motor milestones, absence of auditory brainstem responses, and a syndromic diagnosis, should be made aware of this possible abnormality.- - - - - - - - - - ranking = 12keywords = nerve (Clic here for more details about this article) |
7/45. False-positive magnetic resonance image in the diagnosis of small acoustic neuroma.A patient presented with sudden hearing loss on her first visit to our department. gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) of the posterior cranial fossa portrayed an intracanalicular tumour image (2-3 mm), and the pure tone average (PTA) and speech discrimination score (SDS) values were 65 dB and 60 per cent, respectively. Surgical intervention to remove the suspected tumour was scheduled by the translabyrinthine approach. Intracanalicular observations by the retrolabyrinthine approach revealed limited oedema on the inferior vestibular nerve with vascular dilation. The tumour image disappeared two years after the operation. Surgical findings and the post-operative course advocate that gadolinium-DTPA-enriched MRI image of an intracanalicular lesion such as arachnoiditis might produce a false-positive result.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/45. 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 = nerve (Clic here for more details about this article) |
9/45. hamartoma in the internal auditory canal.An 11 year old girl presented with hearing loss in her left ear and left trigeminal and facial nerve palsy. Radiological examinations revealed an enlargement of the left internal acoustic canal and the existence of a mass protruding from the canal into the cerebellopontine angle. The partial resection of the mass by a suboccipital craniectomy resulted in ceasing the progression of her symptoms. Histological diagnosis was hamartoma with cerebellar heterotopia. The concurrence of heterotopic cerebellar tissue could help to understand the pathogenesis of hamartomas.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/45. otitis media and CNS complications.Intracranial complications from otitis media can be quite devastating to the patient if an early diagnosis is not made. patients may develop meningitis, venous sinus thrombosis or cranial nerve palsies, as well as intracranial abscess. The presenting features in such cases may be subtle and include headache, nausea, vomiting, personality changes and signs of increased intracranial pressure as well as focal neurological deficits. A case of intracranial brain abscess is presented in a patient with a history of chronic otitis media with cholesteatoma. Delay in the diagnosis of intracranial complications of otitis media can lead to improper treatment with increased morbidity and mortality. The etiology and treatment of complications affecting the CNS is discussed.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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