Langerhans' cell histiocytosis of the labyrinth in adults.
OBJECTIVE
To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults.


METHODS
Retrospective case review.


METHODS
A tertiary referral center.


METHODS
Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone.


METHODS
Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy.


RESULTS
The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred.


CONCLUSIONS
Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.
Mosnier
Isabelle
I
Department of Otolaryngology, Hôpital Louis Mourier, AP-HP, Faculté Xavier Bichat, Université Parist, Colombes, France. isabelle.mosnier@lmr.ap-hop-paris.fr
Rondini-Gilli
E
E
Crosara
Paulo Tormin Borges
PT
Belmatoug
N
N
Cyna-Gorse
Françoise
F
Cazals-Hatem
D
D
Abbey-Toby
A
A
Bozorg-Grayeli
A
A
Sterkers
Olivier
O
D000328:Adult; D007758:Ear, Inner / Q000473:pathology*; D004712:Endolymphatic Sac / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D007801:Langerhans Cells / Q000276:immunology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D015837:Vestibular Diseases / Q000209:etiology*