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.

STUDY DESIGN

Retrospective case review.

SETTING

A tertiary referral center.

PATIENTS

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

INTERVENTION

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.

CONCLUSION

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.