Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In connection with their two own cases, the authors deal about the giant cholesterol cysts of the petrous apex. The lesions which are to be differentiated from epidermoid cysts are cholesterol granulomas. Their petrous apex location explains their characteristic large appearance. As each cholesterol granuloma, they occur when a bony cell is obstructed. This chronic obstruction induces mucosal edema then bleedings which lead to the formation and, by the lack of drainage, to the accumulation of cholesterol crystals. These crystals initiate a non specific reaction to foreign bodies, a granuloma, which also can bleed. Thus, a continuous cycle perpetuates the growth of the lesion. This lesion, when it is localized in the petrous apex, can reach a big size before the appearance of some signs. Usually, these are otologic (sensorineural hearing loss, tinnitus, vertigo) and/or cranial nerve palsies (V, VI, VII). C.T. scan (well defined, sharply marginated bony expansible lesion with isodense to the brain central part) and M.R.I. (central region of increased intensity on both T1 and T2 weighted images and peripheral rim of markedly decreased signal intensity in all instances) features are characteristic enough to allow diagnose with other petrous apex lesions (cholesteatoma, mucocele, epithelial cyst, histiocytosis X, ...). Surgical treatment must try to evacuate and to aerate the cavity or perhaps to obliterate it with fatty pieces in order to prevent the recurrence.
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PMID:[Giant cholesterol cysts of the petrous apex]. 129 72

Discharge from the ear can be the result of many disease processes. The ear may discharge blood, pus, cerebrospinal fluid (CSF) or wax. Keratosis obturans, stenosis of the external meatus and benign tumours of the external meatus all lead to wax build-up, which may cause recurrent attacks of otitis externa. Malignant tumours, such as basal cell carcinoma, squamous cell carcinoma and tumours of ceruminous gland origin may also present with discharge. Tumours should be excluded by submitting all material removed from the external canal for histological examination. Single or multiple abscesses (known as furuncles) may occur in the hair follicles in the skin of the external acoustic meatus (EAM). Compulsive scratching, hearing aids and foreign bodies placed in the ear predispose to otitis externa, which is also often associated with infection by Pseudomonas aeruginosa, Staphylococcus aureus and faecal organisms. Management may be with aluminium acetate 14%, topical antibiotic/steroid drops, a gauze wick soaked with icthammol 10% in glycerin or polymyxin B sulphate--neomycin sulphate--hydrocortisone acetate cream placed into the EAM and replaced every 24 to 48 hours, or systemic antibiotics according to severity. Malignant (necrotising) otitis externa causes progressive destruction of the temporal bone, and cranial nerve palsies (usually facial first). Treatment is limited debridement of infected bone, accompanied by intravenous aminoglycosides, and local antibiotic treatment and aural cleanout or oral ciprofloxacin. Middle ear conditions causing discharge include acute otitis media, infected grommets, traumatic perforations and chronic suppurative otitis media, as well as tumours of the ear canal skin and middle ear, radiation-induced otitis externa and osteoradionecrosis of the temporal bone, tuberculosis, Langerhans cell histiocytosis, spontaneous or post-traumatic CSF leaks, Wegeners granulomatosis and immune deficiency states. Topical application of aminoglycoside antibiotics to the middle ear of laboratory animals such as rats, guinea pigs and chinchillas causes sensorineural hearing loss, an effect rarely seen clinically in humans. If the external acoustic meatus and tympanic membrane are obscured by discharge cotton buds, microsuction equipment or syringing are used to remove it. It is often useful to initiate treatment (usually with topical drops, wicks or an oral antibiotic) with a provisional diagnosis. A full examination and adequate visualisation of the tympanic membrane must eventually be performed, if necessary under anaesthesia, or else serious progressive conditions may be neglected. The most useful initial investigation is a swab sent for bacteriological assessment; other investigations are usually indicated by clinical findings and the provisional diagnosis.
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PMID:Optimum management of the discharging ear. 137 20

Langerhans' cell histiocytosis is a rare disorder of unknown aetiology in which pathological Langerhans' cells accumulate and destroy local tissue. We report a 38-year-old female who presented with a sudden onset of left sensorineural hearing loss. Magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion in the left mastoid and a second lesion in the hypothalamus. Following left mastoid exploration and biopsy a definitive diagnosis of Langerhans' cell histiocytosis was made and the patient was treated with external beam radiotherapy. Subsequent right femur and right mastoid involvement were successfully treated with steroids and cytotoxic chemotherapy. At one year follow-up the patient had residual left-sided sensorineural hearing loss with normal hearing in the right ear. To our knowledge, Langerhans' cell histiocytosis has not been previously reported as a cause of unilateral sudden onset sensorineural hearing loss. It should be considered in the differential diagnosis of this condition.
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PMID:Langerhans' cell histiocytosis--a rare cause of sudden onset unilateral sensorineural hearing loss. 1076 25

We report a case of a girl with Langerhans cell histiocytosis (LCH) of multifocal bone disease, who developed recurrent bacterial meningitis and unilateral sensorineural hearing loss during the relapsing course of the disease. Mondini dysplasia, a congenital inner ear anomaly, was suspected by high resolution computed tomographic scan and the dysplasia with cerebrospinal fluid leakage was confirmed by surgery in the ipsilateral ear showing hearing loss. Although rare, congenital inner ear anomalies such as Mondini dysplasia should be kept in mind in pediatric patients with hearing impairment and/or recurrent bacterial meningitis during chemotherapy for various types of neoplasms including LCH.
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PMID:Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis. 1517 Aug 97

A case is presented where the authors carried out a cochlear implantation on a girl whose deafness was induced by cytostatic agents. The normally hearing child was diagnosed with Langerhans cell histiocytosis at the age of 3 years and received polychemotherapy for almost 2 years. By her 5th year, she started to develop a bilateral, progressive sensorineural hearing loss, necessitating a hearing aid in both ears. While her histiocytosis was cured, her hearing ultimately deteriorated to total deafness on the right and profound hearing loss on the left ear. After 3 years, her hearing aids no longer provided adequate hearing for the postlingually deafened girl. At the age of 14, a cochlear implantation was performed on her right ear with excellent results.
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PMID:Cochlear implantation for treatment-induced ototoxic deafness in Langerhans cell histiocytosis. A case report. 1594 3

A 56-year-old woman with Langerhans cell histiocytosis of the petrous bone presented with sudden onset of sensorineural hearing loss and vertigo without other neurological impairment, mimicking idiopathic sudden sensorineural hearing loss. Differential diagnosis was difficult until neuroimaging demonstrated a lesion of the petrous bone. The patient eventually underwent removal of the lesion via the transpetrosal approach, and received postoperative chemotherapy consisting of vinblastine, methotrexate, 6-mercaptopurine, and prednisolone. Although her hearing did not recover, complete remission was achieved, and the patient is currently free from disease. Physicians need to be aware that patients with sudden onset of hearing loss may have an unexpected and hidden disease which requires neuroimaging and histological examinations for definitive diagnosis and appropriate treatment.
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PMID:Langerhans cell histiocytosis of the petrous bone with sudden sensorineural hearing loss. Case report. 2080 58

We report a rare case of progressive hearing loss after acquired CMV infection in a child with Langerhans cell histiocytosis (LCH). A 5-month-old female was diagnosed as having LCH. When she was 14 months old, she received an unrelated donor umbilical cord blood transfusion for the treatment of intractable LCH. CMV infection was confirmed after the blood transfusion. Because her own umbilical cord had no CMV, the CMV infection was not congenital. When she was 7 years old, mixed hearing loss was noted with bilateral otitis media with effusion. After that time, the sensorineural hearing loss progressed to bilateral profound hearing loss over 3 years. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging with gadolinium contrast enhancement revealed a high intensity area in the inner ear that suggested bilateral labyrinthitis. This case demonstrates the possibility that, under the immunodeficiency, the acquired CMV infection causes progressive sensorineural hearing loss.
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PMID:Progressive hearing loss following acquired cytomegalovirus infection in an immunocompromised child. 2308 31

Langerhans cell histiocytosis is a rare disorder, with resultant bilateral sensorineural hearing loss unreported in adults. A 42-year-old man presented with 4 months of right-sided tinnitus and hearing loss treated initially as otitis media with effusion. He re-presented 5 months later with progressive bilateral hearing loss--sensorineural (>100 dB) on pure tone audiogram. CT showed bilateral petrous temporal bone and calvarial lesions. Biopsy confirmed diagnosis of Langerhans cell histiocytosis and chemotherapy was started. Though uncommon, Langerhans cell histiocytosis should be considered among the differentials of persistent otological symptoms, as its progressive nature can cause bilateral irreversible sensorineural hearing loss.
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PMID:A case of bilateral sensorineural hearing loss from Langerhans cell histiocytosis. 2634 Nov 62

: A 14-year-old woman presented with right-sided otologic and vestibular symptoms after presenting with hormonal disturbances earlier that year. Imaging showed a gross destruction of the temporal bone, mastoid air cells, and external acoustic meatus with invasion into the otic capsule. The patient experienced complete sensorineural hearing loss in the right ear. Biopsy diagnosed Langerhans cell histiocytosis (LCH) and the patient was treated with chemotherapy. After 1 year of treatment, the patient's hearing partially recovered and imaging showed reconstitution of the temporal bone including the otic capsule. Our case is the first report of complete sensorineural hearing loss with partial recovery after LCH treatment.
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PMID:Unique Case of Hearing Recovery After Otic Capsule Destruction and Complete Sensorineural Hearing Loss Caused by Langerhans Cell Histiocytosis. 2874 30

An 8-year-old girl presented with a 1-year history of bilateral progressive hearing loss and vertigo for 6 months. High-resolution computed tomography of the temporal bones demonstrated multiple lytic lesions. Histopathology examination confirmed a diagnosis of Langerhans cell histiocytosis (LCH). She underwent chemotherapy for 12 months. Following treatment, she was in remission. However, the bilateral profound sensorineural hearing loss persisted. She underwent right cochlear implantation with very good functional audiological outcomes. This is the first known reported case of successful auditory rehabilitation through cochlear implantation in a patient with bilateral profound hearing loss due to isolated bilateral temporal bone LCH.
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PMID:Successful cochlear implantation in Langerhans cell histiocytosis: A rare case. 2915 65


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