Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus

Abstract

Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus. Objective: To present a clinical case of an adult affected by Langerhans cell histiocytosis with bilateral, nonsimultaneous, involvement of the temporal bone, associated with diabetes insipidus and to review the literature.

Methodology: A rare case of bilateral temporal bone involvement of Langerhans cell histiocytosis in a 42-year-old woman affected by diabetes insipidus is reported. We present patient’s clinical history supported by radiologic, histopathologic and audiologic findings.

Results: The patient was submitted to a series of otologic surgical procedures due to the progression of the disease. Ossicular chain was always preserved, so that conservative surgery (canal wall-up technique) was performed, permitting the achievement of good hearing results, bilaterally.

Conclusions: Temporal bone involvement of Langerhans cell histiocytosis may lead to a progressive chronic disease. However, the ossicular chain can remain uninvolved, making a conservative surgical treatment possible. Careful followup is essential for detecting new lesions and serial CT scans are mandatory.