OBJECTIVES/HYPOTHESIS: Evaluate presentation, management, and clinical outcomes of @entity1 with temporal bone @entity1222 ( @entity1222 ). STUDY DESIGN: Retrospective chart review. METHODS: Reviewed all @entity1 with temporal bone @entity1222 at a tertiary academic referral center between 1978 and 2014. Presentation, disease course, intervention, and clinical outcomes were analyzed. RESULTS: Between 1978 and 2014, 29 temporal bones in 20 @entity1 (12 males; median age 32 years, range 1.3-88 years) were diagnosed with temporal bone @entity1222 , representing 4% of all @entity1 diagnosed with @entity1222 at our institution during that time. Twelve (60%) @entity1 presented purely with @entity135 , nine (45%) with bilateral temporal bone involvement, seven (35%) with intracranial spread, and eight (40%) with @entity66 . The most common presenting symptoms were @entity2972 (n = 11; 55%) and subjective @entity393 (n = 10; 50%). Treatment included primary chemotherapy or radiation alone(n = 7; 35%), local resection alone (n = 2; 10%), and chemotherapy or radiation with surgery(n = 11; 55%). Ten @entity1 suffered local or systemic relapse (50%; median 12 months). Median follow-up for @entity1 was 31 months. One @entity1 died secondary to @entity358 of multisystem @entity1222 . CONCLUSIONS: @entity1222 is an uncommon @entity27056 with a range of clinical manifestations and disease severity. Otologic involvement is rare, and frequently manifests with symptoms similar to otomastoiditis. Evaluation and close follow-up of both ears is important. Bilateral simultaneous or sequential @entity1222 can be seen in up to 45% of cases. @entity1 with temporal bone @entity1222 should be managed by a multidisciplinary team with treatment tailored to the @entity1 . Relapse is more common in @entity1 with multisystem involvement. Even in subjects with isolated otologic involvement, long-term follow-up is critical, with relapses occurring years beyond treatment. LEVEL OF EVIDENCE: 4. Laryngoscope, 2015.

XXXX of the temporal bone: A review of 29 cases at a single center.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'patient'] @entity135 :: ('MESH:D006258', 'Disease') :: ['head and neck disease'] @entity27056 :: ('MESH:D015620', 'Disease') :: ['histiocytic disorder'] @entity66 :: ('MESH:D004194', 'Disease') :: ['multisystem disease'] @entity1222 :: ('MESH:D006646', 'Disease') :: ['Langerhans cell histiocytosis', 'LCH'] @entity2972 :: ('MESH:D002558', 'Disease') :: ['otorrhea'] @entity358 :: ('MESH:D008171', 'Disease') :: ['pulmonary complications'] @entity393 :: ('MESH:D034381', 'Disease') :: ['hearing loss']
@entity1222 :: (MESH:D006646,Disease) :: ['Langerhans cell histiocytosis']
