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, non-simultaneous, involvement of the temporal bone, associated with diabetes insipidus and to review the literature.


METHODS
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 follow-up is essential for detecting new lesions and serial CT scans are mandatory.
Modugno
G C
GC
Department of Specialistic Surgical and Anaesthesiological Sciences, ENT Section, Policlinico S.Orsola-Malpighi, University of Bologna, Italy.
Brandolini
C
C
Magnani
G
G
Ferri
G G
GG
Sabattini
E
E
Pirodda
A
A
D000328:Adult; D001301:Audiometry, Pure-Tone; D015897:Comorbidity; D003919:Diabetes Insipidus / Q000453:epidemiology*; D005260:Female; D006314:Hearing Loss, Conductive / Q000209:etiology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D013517:Otorhinolaryngologic Surgical Procedures; D013701:Temporal Bone