Lymphoblastic B-Cell Lymphoma in the Internal Auditory Canals Presenting as Bilateral Sudden Sensorineural Hearing Loss

Background: Sudden sensorineural hearing loss has rarely been described as an initial presentation of hematologic disorders. When it does occur, it usually manifests as leukemic infiltration of the cochlear nerve or hemorrhage in the inner ear rather than distinct lesions.

Case Study: We describe a case of a 10-year-old girl who presented with bilateral sudden sensorineural hearing loss as the initial symptom of lymphoblastic B cell lymphoma. Auditory testing demonstrated present otoacoustic emissions (OAEs) but absent acoustic reflexes bilaterally. Auditory brainstem responses (ABR) demonstrated poor waveform morphology with absence of identifiable waves at high frequencies. Magnetic Resonance Imaging (MRI) revealed enhancing T2 hypointense lesions in both internal auditory canals and thickening of the vestibulocochlear nerves. Emergent cranial radiation therapy decreased internal auditory canal enhancement radiographically and improved the patient's hearing.

Conclusion: Though rare, lymphoma should be considered in the differential for sudden sensorineural hearing loss and may present atypically with internal auditory canal lesions. In these rare cases, emergent radiation therapy may be considered as a treatment option.