Cases reported "Ear Diseases"

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1/32. 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.
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2/32. Cervical necrotizing fasciitis.

    Necrotizing fasciitis is a severe soft tissue infection that results in necrosis of the fasciae and subcutaneous tissues; the infection can quickly prove fatal. Although involvement of the head and neck is rare, causes are usually odontogenic or pharyngeal but can also be insect bites, local trauma, burns or surgery. We present a clinical case of a 31-year-old Italian woman with cervical necrotizing fasciitis having an uncommon presentation. While under treatment, the patient's husband was admitted for necrotizing fasciitis of the medial fasciae of his left leg subsequent to an insect sting. The causes, diagnosis and treatment of necrotizing fasciitis are reviewed.
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3/32. A case of giant keratoacanthoma of the auricle.

    Although keratoacanthomas are not rare in the head and neck area, patients with this type of tumor rarely consult an otolaryngologists for treatment. keratoacanthoma should be considered in the differential diagnosis of squamous cell carcinoma. This tumor grows rapidly, usually attaining a size of about 10-20 mm in approximately 6 weeks. This is followed by slow involution over a period of 2-6 months. A keratoacanthoma larger than 20-30 mm is called as 'giant keratoacanthoma' and it is scarce. We encountered a case of giant keratoacanthoma (50 mm in diameter) on the right auricle of 84-year-old Japanese woman with a 3-year history of gradual tumor growth. Several clinical and histopathological factors made the diagnosis difficult. The tumor was completely removed by surgery and diagnosed as a keratoacanthoma by histopathological examination.
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4/32. Management of first branchial cleft anomalies: report of two cases.

    Embryological anomalies of the first branchial apparatus result in rare forms of developmental abnormality of the head and neck. Their presentation may be similar to other conditions and they may easily be overlooked by the unwary when considering the differential diagnosis of a parotid swelling or a neck sinus. Consequently, they may be mismanaged on one or more occasions. We encountered two patients in whom such problems arose and conclude that appropriate investigation and management by a team skill ed in surgery of this region is necessary if a satisfactory outcome is to be achieved.
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5/32. Otolaryngologic manifestations of Rosai-Dorfman disease.

    PURPOSE: To describe an unusual head and neck occurrence of Rosai-Dorfman disease (RDD) and to review the otolaryngologic manifestations of this rare entity. methods: A case presentation with review of the literature describing Rosai-Dorfman disease and its head and neck involvement. Setting: A tertiary care, urban children's hospital. RESULTS: This is the first description, to the best of our knowledge, of RDD [Sinus histiocytosis with Massive Lymphadenopathy (SHML)] involving bilateral external auditory canals and middle ear in a 12 year old patient previously diagnosed with 'asthma' and hearing loss. This patient also had extensive involvement of the tracheobronchial tree. Representative clinical, radiographic and histological findings are presented. Its etiology, diagnosis and management are also reviewed. CONCLUSION: This is the first reported case of middle ear and external auditory canal involvement of RDD in a patient with hearing loss and associated tracheobronchial lesions. RDD, although rare, may be considered in the differential diagnosis of unusual histiocytic lesions of the external auditory canal, especially with findings of similar or comparable lesions involving the respiratory tract. Confirmation is with identification of emperipolesis and appropriate immunohistochemical staining (S-100 positive, CD-68 positive and CD-1a negative). Intervention is recommended in cases where there is increased risk of mortality, as in severe obstruction of the tracheobronchial tree. Otherwise, since these lesions are self-limiting, the patients can be observed closely.
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6/32. Otologic manifestations of amyloidosis.

    OBJECTIVE: To describe otologic manifestations of amyloidosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. patients: Case report of a patient with bilateral external auditory meatal stenosis secondary to amyloidosis. RESULTS: The patient is a 60-year-old man who presented with several months' history of bilateral ear blockage and drainage. physical examination revealed markedly thickened skin in each ear in the area of concha and meatus that was associated with fissuring. The meatus were markedly stenosed. The medial aspect of the external auditory canal and the tympanic membrane were normal. He had partial improvement on medical therapy with topical steroid-containing eardrops and ointment. Past medical history was positive for multiple myeloma. Meatal biopsy was performed. Grossly, the tissue was hypovascular and markedly thickened. Histopathologic examination was consistent with amyloidosis. The patient was continued on topical steroid ointments, frequent ear cleaning, and close follow-up. He was subsequently diagnosed with systemic amyloidosis. A review of the literature was done. head and neck involvement with amyloidosis is reviewed, with emphasis on otologic manifestations. CONCLUSION: Otologic involvement with amyloidosis is extremely rare. The pathophysiology may be related to the local factors in the external auditory canal.
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7/32. A rare case of a sebaceous nevus in the external auditory canal.

    We report the case of a patient who came to us for evaluation of a progressive unilateral hearing loss and who was found to have a sebaceous nevus in an unusual location: the external auditory canal. A sebaceous nevus is a congenital organoid mass that occurs primarily on the face, scalp, and periauricular regions. Despite the predilection of sebaceous nevi for the head and neck, reports of this lesion rarely appear in the otolaryngology literature. Left untreated, the lesion can progress through three stages of gross and histopathologic development; a sebaceous nevus begins as a small benign papule, grows into an enlarging mass with different characteristics, and ultimately becomes a secondary neoplasm. The lesion's potential for malignant transformation and its association with syndromes underscores the importance of prompt recognition and appropriate management. This case report adds the sebaceous nevus to the differential diagnosis of external auditory canal lesions and provides essential information about this rare mass.
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8/32. hamartoma of the middle ear: a case study.

    Hamartomas and teratomas are rare tumors in children. Its location in head and neck is quite exceptional. An unusual case of hamartoma in the middle ear is reported in a 5-month-old child. The tumor was isolated without associated malformation. Definitive diagnosis was made after surgery and based on histological results. At 2 years follow-up, no recurrence was observed. CT-scan is useful in order to reach a final diagnosis. Although rare, this type of tumor should be considered in the differential diagnosis of a middle ear mass during childhood.
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9/32. Otolaryngic myofascial pain syndromes.

    It has been long recognized in the otolaryngic community that despite great effort dedicated to the physiology and pathology of the ear, nose, throat/head and neck, there are a number of symptoms, including pain in various locations about the head and neck, which cannot be explained by traditional otolaryngic principles. The tenets of myofascial dysfunction, however, as elucidated by Dr. Janet Travell, explain most of these previously unexplained symptoms; furthermore, treatment based on Dr. Travell's teachings is effective in relieving these symptoms.
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10/32. Otolaryngologic manifestations of the mucopolysaccharidoses.

    A retrospective review of 45 children with mucopolysaccharidoses was performed to determine the frequency of complications related to the head and neck. In this series, every patient had at least one complication involving the head and neck region, and in over half, operative intervention by the otolaryngologist was required. Upper airway obstruction occurred in 17 (38%) and necessitated a tracheostomy in 7 (16%). Cervical spine instability occurred in 8 (18%), making airway management difficult. Recurrent respiratory infections occurred in 17 (38%), and chronic recurrent middle ear effusions were noted in 33 (73%). This review demonstrates that children afflicted with the mucopolysaccharidoses frequently have otolaryngologic-related complications that are common throughout their life span and often the primary management issue in their continuing care. The otolaryngologic management of these patients is outlined based on the results of this study and review of the relevant literature.
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