1/468. 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. ( info) |
2/468. Multifocal cholesteatoma of the external auditory canal following blast injury. Posttraumatic cholesteatoma of the external auditory canal is a rare condition that may present years after the original injury. A unique case of multifocal cholesteatoma of the external auditory canal following blast injury is presented and discussed. ( info) |
3/468. An aural myiasis case in a 54-year-old male farmer in korea. A 54-year-old male farmer residing in Chunchon, korea, complaining of blood tinged discharge and tinnitus in the left ear for two days, was examined in August 16, 1996. Otoscopic examination revealed live maggots from the ear canal. The patient did not complain of any symptoms after removal of maggots. Five maggots recovered were identified as the third stage larvae of Lucilia sericata (diptera: Calliphoridae). This is the first record of aural myiasis in korea. ( info) |
4/468. Auricular endochondral pseudocysts: diagnosis and management. The auricle can be the site of a variety of cystic lesions, many of which involve either potential spaces between the auricular cartilage and the perichondrium or spaces within the skin and subcutaneous tissues. An auricular endochondral pseudocyst is a fluid collection located within the cartilaginous structure of the auricle. The auricular hematoma and the auricular pseudocyst may represent opposing ends of a continuum of damage and repair of traumatic insults. Whereas the hematoma represents a significant acute traumatic event resulting in cleavage between the perichondrium and the cartilage, the pseudocyst could be the outcome of chronic lower grade trauma. In this instance, the perichondrium not only separates from the cartilage but may be induced to regenerate an outer cartilaginous wall exacting the contour of the separated perichondrium, thus completing a cartilaginous auricular pseudocyst. This firm, cartilaginous outer cyst wall accounts for the increased difficulty in treating pseudocysts and clearly requires a different surgical treatment than for the hematoma. Having reviewed a substantial number of case reports, we recommend incision and drainage of the cavity followed by obliteration of the cavity by curettage, sclerosing agent, and pressure dressing. More invasive techniques should be reserved for an uncommon recurrence after adequate initial treatment. ( info) |
| Autophonia, the hyperperception of one's own voice and breathing, is one of the consequences of rapid weight loss and is explained within the syndrome of the patulous eustachian tube. We report on a female adolescent, who presented to an otologist for autophonia and was finally diagnosed with anorexia nervosa. The occurrence and relevance of this symptom in eating-disorder patients is discussed. ( info) |
6/468. A simple reconstructive procedure for radiation-induced necrosis of the external auditory canal. Localized necrosis of the bone, cartilage, and soft tissue of the external auditory canal is an uncommon side effect of radiotherapy to the parotid region. Five patients developed late onset skin necrosis of a quadrant of the ear canal secondary to an underlying osteoradionecrosis of the tympanic ring. We report a one-stage procedure to excise the necrotic tissue and replace it with a local rotational flap derived from the post-auricular skin. Otological side effects of radiotherapy are discussed. ( info) |
7/468. An unusual case of otogenic pneumocephalus. Otogenic pneumocephalus is a rare entity usually caused by temporal bone trauma. This paper describes a case of otogenic pneumocephalus of traumatic origin, in which the type of the fracture (a bony spicula was detached from the mastoid) and the location (Trautmann's triangle) were uncommon. ( info) |
8/468. 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. ( info) |
9/468. An aneurysm of the petrous internal carotid artery. Internal carotid artery aneurysms are a rare cause of pulsatile tinnitus and conductive hearing loss but should be borne in mind when there is a suspected diagnosis of glomus jugulare or high-riding jugular bulb. Most cases are congenital. We present a case of otorrhagia which was initially thought to be a glomus jugulare, the diagnosis of internal carotid artery aneurysm was made at angiography and treated by balloon embolization. ( info) |
10/468. purpura of the ears: a distinctive vasculopathy with circulating autoantibodies complicating long-term treatment with levamisole in children. The cutaneous side-effects of levamisole include non-specific and lichenoid eruptions, fixed drug eruption and, very rarely, cutaneous vasculitis. We describe a distinctive clinical and histological vasculopathy with immunological abnormalities in children with paediatric nephrotic syndrome receiving long-term levamisole treatment. Four boys and one girl were identified. Their average age was 10 years. levamisole had been used for an average of 24 months. purpura of the ears was the most common finding corresponding histologically to a vasculopathic reaction pattern ranging from a leucocytoclastic and thrombotic vasculitis to a vascular occlusive disease without true vasculitis but with associated antinuclear, antiphospholipid and anticytoplasmic antibodies. The eruption resolved in all patients 2-3 weeks after the discontinuation of levamisole, but serum autoantibodies persisted for 2-14 months. ( info) |