Successful cochlear implantation in Langerhans cell histiocytosis: A rare case

Abstract

An 8-year-old girl presented with a 1-year history of bilateral progressive hearing loss and vertigo for 6 months. High-resolution computed tomography of the temporal bones demonstrated multiple lytic lesions. Histopathology examination confirmed a diagnosis of Langerhans cell histiocytosis (LCH). She underwent chemotherapy for 12 months. Following treatment, she was in remission. However, the bilateral profound sensorineural hearing loss persisted. She underwent right cochlear implantation with very good functional audiological outcomes. This is the first known reported case of successful auditory rehabilitation through cochlear implantation in a patient with bilateral profound hearing loss due to isolated bilateral temporal bone LCH.

Langerhans cell histiocytosis mimicking acute otitis media in childhood—a case presentation

“…Otological findings are similar to otitis media, otitis externa, and cholesteatoma [1]. This diversity in clinical appearance may mislead the physician.…”

“…While surgery is preferred in a singlesystem disease, chemotherapy is used in multisystem diseases. Radiotherapy is no longer recommended for the treatment of temporal bone LCH [1]. In this case, the pediatric hematology and oncology department used chemotherapy and steroids after diagnosis due to multisystem presentation.…”

“…Despite LCH being considered a non-hereditary disorder, a subset of LCH is familial [5]. Currently, neoplastic or inflammatory disease is primarily accused with predisposing factors [1,6]. Although it can be seen in any age group, it mostly occurs in childhood.…”

“…It is slightly more common in boys than girls. The clinical picture can range from solitary or multifocal bone lesions to common diseases with multiorgan involvement [1]. Temporal bone involvement is seen in 15-60% of cases, and bilateral involvement is seen in 25-30% of cases.…”

“…Langerhans cell histiocytosis (LCH) are tumors of unknown etiology that affect children and young adults, characterized by clonal proliferation of Langerhans cells [1]. LCH can occur in different clinical presentations, ranging from unifocal or multifocal bone lesions or other tissues to multi-organ involvement involving the lung, liver, pituitary gland, skin, mucous membranes, lymphatics, or other organs [1,2]. Temporal bone involvement is not uncommon.…”