An exceptional cause of unilateral sensorineural hearing loss: Progressive multifocal leukoencephalopathy

Abstract

We describe progressive multifocal leukoencephalopathy (PML) as an extremely rare cause of unilateral sensorineural hearing loss. A 46-year-old HIV-infected and immunocompromised patient suffered from hearing loss in the left ear since several days. Because of the discrepancy between the normal tone audiogram and the abnormal speech audiogram, MRI was indicated. The scan showed a hyperintense lesion at the left side of the cerebellum, extending to the cranial portion of the medulla oblongata. The lesion was initially diagnosed as PML. Since the symptoms progressed despite earlier initiated and effective treatment with combination antiretroviral therapy, the disease was eventually interpreted as PML-immune reconstruction inflammatory syndrome (PML-IRIS). This case demonstrates that the diagnosis of PML or PML-IRIS should be considered as cause of hearing loss in immunocompromised patients. Also, the importance of complete audiological evaluation including speech audiometry in analysis of subjective hearing impairment is illustrated.

Keywords

• Sensorineural hearing loss
• Audiometry
• Immunology