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

Abstract

A 56-year-old woman with Langerhans cell histiocytosis of the petrous bone presented with sudden onset of sensorineural hearing loss and vertigo without other neurological impairment, mimicking idiopathic sudden sensorineural hearing loss. Differential diagnosis was difficult until neuroimaging demonstrated a lesion of the petrous bone. The patient eventually underwent removal of the lesion via the transpetrosal approach, and received postoperative chemotherapy consisting of vinblastine, methotrexate, 6-mercaptopurine, and prednisolone. Although her hearing did not recover, complete remission was achieved, and the patient is currently free from disease. Physicians need to be aware that patients with sudden onset of hearing loss may have an unexpected and hidden disease which requires neuroimaging and histological examinations for definitive diagnosis and appropriate treatment.

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

“…Smoking cessation and pulmonary LCH disease resolution as reported in our case have been previously described . Treatment options for temporal bone LCH include intralesional or systemic steroids, systemic chemotherapy and surgical excision . Surgical debridement has been reported to carry a high risk of complications such as postauricular fistula and facial nerve palsy .…”

“…Temporal bone involvement occurs in 15–61% of all LCH cases and lesions are usually isolated to the mastoid or squama, or rarely as part of multifocal disease . The petrous portion is rarely involved and, in this event, the otic capsule is usually spared . As a result, conductive hearing loss is common, whereas SNHL has been reported in only 17 cases to date .…”

Langerhans Cell Histiocytosis of the Temporal Bone with Otic Capsule Involvement

“…The mastoid and the squamous portion of the temporal bone are most often affected. The petrous portion is rarely involved and if so, the otic capsule is often left unscathed [6]. As a result, conductive hearing loss is common but a sensorineural component has only been reported in approximately 15 cases [4,[7][8][9][10][11][12][13][14][15][16][17][18][19].…”

Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis

“…Typically presentation of the disease symptoms is similar to that of otitis media. Petrous temporal lesions, although rare, may present with facial nerve palsy and sensorineural hearing loss, as seen in this case (Suzuki et al, 2010). Only 10% of patients with temporal bone LCH hearing loss are of sensorineural hearing loss type with common anatomical findings of a bone erosion of the otic capsule involving the semicircular canal, the internal auditory canal, or the cochlea (Saliba and Sidani, 2009).…”