Langerhans' cell histiocytosis of the temporal bone: successful treatment of sensorineural hearing loss with low-dose radiotherapy

Abstract

We suggest that timely radiotherapy for treatment of sensorineural hearing loss secondary to Langerhans' cell histiocytosis is an appropriate treatment option.

Langerhans Cell Histiocytosis of the Petrous Bone With Sudden Sensorineural Hearing Loss -Case Report-

“…Anonsen et al reported that only 4 of 24 LCH cases of the head and neck involved the mastoid, and only one case involved the petrous bone. 1) Patients with petrous bone involvement may present with sensorineural hearing loss, 2,5,8) vertigo, 2,5,21) and facial nerve palsy. 2,4,8,20) Sensorineural hearing losses in previous cases have been chronic, 21) fluctuating, 5) or progressive, 8) whereas the present case showed sudden onset of hearing loss.…”

“…1) Patients with petrous bone involvement may present with sensorineural hearing loss, 2,5,8) vertigo, 2,5,21) and facial nerve palsy. 2,4,8,20) Sensorineural hearing losses in previous cases have been chronic, 21) fluctuating, 5) or progressive, 8) whereas the present case showed sudden onset of hearing loss. Extension of the lesion to the skull base may cause palsy of other cranial nerves such as the abducens, [2][3][4]8,11,13) trigeminal, 4) optic, 11) and vagus 2) nerves.…”

“…10) However, petrous bone lesions are not readily accessible, and most reported cases underwent only diagnostic biopsy or curettage. 3,8,13,[19][20][21] Low-dose radiotherapy is effective for LCH, and is generally used at a dose of 5-25 Gy. 10,13) Chemotherapy is indicated for multifocal or multisystem LCH.…”

Hearing recovery following repair of otic capsule defect secondary to semicircular canal erosion from Langerhans cell histiocytosis

“…LCH is a rare neoplastic proliferation of Langerhans cells of unknown aetiology but smoking is recognized as the main risk factor for pulmonary disease . In our reported case, the patient had asymptomatic pulmonary LCH with extrapulmonary otic capsule involvement .…”

Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis

“…Chemotherapy or radiation, which may have been typically reserved for surgical treatment failure or persistent disease, has provided an alternative therapeutic option since extensive surgery, such as mastoidectomies, has high complication rates. The literature has reported three cases of improvement sensorineural hearing loss with radiotherapy alone after failed treatment with steroids use (Hore et al, 1999;Zlodre et al, 2009). Despite surgical resection and immunosuppression with prednisone, vinblastine, and 6-mercaptopurine, this patient demonstrated persistent LCH with sensorineural hearing loss.…”