Otologic manifestations of histiocytosis X

Hudson, W R; Kenan, Patrick D.

doi:10.1288/00005537-196904000-00010

Cited by 13 publications

(4 citation statements)

References 18 publications

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“…The classic triad of diabetes insipidus, exophthalmos, and lytic lesions occurs in only 10% of patients suffering from the disease. 3 The Letterer-Siwe designation applies to disseminated visceral disease almost always occurring in infants less than 2 years of age and exhibiting a rapid downhill progression. Because of the difference in clinical course between the first two forms, which are relatively benign, and the latter disseminated form, which is often fatal, there is valid skepticism over the inclusion of the Letterer-Siwe form within the unified hypothesis.t" Histologic characteristics are similar in the three forms.…”

Section: Discussionmentioning

confidence: 99%

Histiocytosis X of the Temporal Bone

1984

Otolaryngol.--head neck surg.

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EDITOR'S NOTE: Each of the rare lesions reported in this group of case reports initially presented as a common otologic problem: middle ear effusion. (apparent) Meniere's disease. recurrent acute otitis media. and otitis extema. Today a diagnosis of necrotizing ("malignant") otitis extema would immediately be suspected in an elderly diabetic with granulation tissueon the ear canal floor. However. the other cases would stillfool most of us, at least on the firstor second visit. The solution isnot to order excessive testsand x-ray films on every patient.A 73-year-old woman was admitted to the hospital for evaluation and treatment of paralysis of the left face. She had undergone bilateral stapedectomies several years prior to admission. Three months prior to admission she developed bilateral acute otitis media and was given antibiotic therapy. The otitis media failed to resolve, and bilateral myringotomies with placement of rympanostorny tubes were carried out. There was still no improvement. so the tubes were removed and antibiotics continued. Subsequently, left facial paralysis developed along with mild vertigo and tinnitus.Pertinent physical findings included an amber effusion in the right ear. The myringotomy on the left was still patent and allowed passage of amber fluid. Tuning fork tests were consistent with a bilateral conductive hearing loss. There was complete paralysis of the entire left face.Laboratory studies included a white blood cell count of 1O,7oo/mm", with a shift to the left. Blood glucose was 178 mg/dl. Temporal bone tomograms and computerized tomography of the head revealed opacification of the mastoid air cells without bone destruction. There was mucosal thickening in the left sphenoid sinus. Intravenous cefazolin was begun, and subsequent cultures grew a bacillus species sensitive to clindamycin, which was added to the regimen. Audiologic assessment revealed profound hearing loss bilaterally. HowFrom the Departmentof Otorhinolaryngology and Human Communication, University of Pennsylvania School of Medicine.

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Section: Discussionmentioning

confidence: 99%

Histiocytosis X of the Temporal Bone

1984

Otolaryngol.--head neck surg.

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“…Exclusion criteria included articles that were non‐English or not relevant, reported on nonhuman populations, and did not provide access to full text or extraction of relevant data. All studies were assessed for grade of evidence ( Table 1) 1,3–63 and queried for demographics, otologic manifestations, diagnosis, clinical and radiologic findings, as well as medical and surgical management.…”

Section: Methodsmentioning

confidence: 99%

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review

Chen

Ashman

Bojrab

et al. 2021

Otolaryngol.--head neck surg.

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Objective To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management Data Sources PubMed/MEDLINE, Embase, and Cochrane Library. Review Methods A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. A review of the references of all included articles was also conducted. Results Sixty-two articles encompassing 631 patients met inclusion criteria. Otologic symptoms at presentation were found in 246 (39%) patients in the reported studies with 48% reporting bilaterality. The mean age was 14.8 years with a male predominance (64%). The most common otologic presenting symptom was otorrhea (46%). A majority had the multisystem variant (52%). The most common treatment modalities were chemotherapy (52%), followed by surgery (50%), systemic steroids (45%), and radiotherapy (31%). Surgery was performed in 75.8% with unisystem involvement and in 50.6% with multisystem involvement. The most effective treatments included radiotherapy (56% success rate, 17% of treated patients), systemic steroids (44% success, 20% treated), chemotherapy (41% success, 21% treated), and surgical modalities (36% success, 19% treated). Conclusions Otologic manifestations that occur with the multisystem variant or are at high risk for central nervous system involvement necessitate systemic treatment. For unifocal lesions, surgery is recommended. Lastly, radiotherapy should be reserved for extensive lesions involving vital structures or presenting in older patients.

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“…Because of its similarity to chronic otitis media, frequent mastoid surgery prior to correct diagnosis has been reported in the literature [95,96,102,103]. Bone destructions are found on temporal bone CT [95,96,104]. The diagnosis is confirmed by deep biopsy from the lesion (▶Fig.…”

Section: Letterer-siwe Syndromementioning

confidence: 99%

“…Aufgrund der Ähnlichkeit zur chronischen Otitis media wird in der Literatur über häufige Mastoidoperationen vor der eigentlichen Diagnosestellung berichtet[95,96,102,103]. In der CT des Schläfenbeines finden sich knochendestruktive Läsionen[95,96,104]. Die Diagnose wird durch eine Biopsie aus den tiefen Abschnitten der Läsion gesichert (▶ abb.…”

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Seltene Erkrankungen des Mittelohres und der lateralen Schädelbasis

Weiss

2021

Laryngorhinootologie

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ZusammenfassungOtalgie, Otorrhoe, Tinnitus und Hörminderung zählen zu den häufigsten ohrbezogenen Symptomen, die zum Aufsuchen eines Hals-Nasen-Ohrenarztes führen. Weiterhin spielen Gleichgewichtsstörungen und Affektionen der Hirnnervenfunktion für eine Konsultation eine Rolle. In großen akademischen Zentren, aber auch in der HNO-ärztlichen Grundversorgung kommt der Erkennung von seltenen Erkrankungen des Mittelohres und der lateralen Schädelbasis eine zentrale Rolle zu, da sie häufig eine interdisziplinäre Zusammenarbeit erfordern um die korrekte Diagnose zu stellen und eine adäquate Behandlung zu initiieren. Die folgende Arbeit gibt einerseits eine Übersicht über seltene Formen von osteologischen, neoplastischen, hämatologischen, autoimmunologischen und infektiologischen Erkrankungen sowie Fehlbildungen, die sich im Mittelohr und der lateralen Schädelbasis manifestieren können, andererseits soll ein Überblick über internistische Erkrankungen gegeben werden, deren Manifestation im Mittelohr selten ist. Die Kenntnis von seltenen Erkrankungen des Mittelohrs und der Schädelbasis ist für die Qualitätssicherung in der Patientenversorgung und insbesondere auch bei entsprechenden chirurgischen Maßnahmen wesentlich. Insbesondere bei untypischen, komplizierten und langwierigeren Krankheitsverläufen muss an seltene Differenzialdiagnosen gedacht werden.

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Otologic manifestations of histiocytosis X

Cited by 13 publications

References 18 publications

Histiocytosis X of the Temporal Bone

Histiocytosis X of the Temporal Bone

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review

Seltene Erkrankungen des Mittelohres und der lateralen Schädelbasis

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