Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA

Hypertrophic pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. It is seldom reported to be related to sensorineural hearing loss, but it can cause sensorineural hearing loss which can be potentially reversed through treatment. Here, we report the case of a 54-year-old woman who had progressive, bilateral, worse in the left, sensorineural hearing loss and visual disturbance with an accompanying headache over several months. Brain MRI showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titers. After empirical steroid and cyclophosphamide therapy, auditory impairment improved, especially in the high frequency region of the pure tone audiogram, and significant improvement in the word recognition test. Moreover, a follow-up MRI revealed much decreased enhancement of the dura mater, and the MPO-ANCA titer decreased to within the normal range. In the case of rapidly progressive sensorineural hearing loss or hearing impairment accompanying other cranial neuropathy, pachymeningitis should be taken into consideration, and brain MRI with gadolinium enhancement is the best method of detecting it. Also, to ensure proper treatment, a cautious evaluation including an ANCA work-up should be performed.

A Case of Sequential Multiple Cranial Neuropathies in Diabetes Mellitus

Cranial mononeuropathies, manifesting particulary as opthalmoplegia or facial palsy, are common entities in the dia-betic population. However, sequential multiple cranial neuropathies due to diabetes are much less common. It is often associated with other conditions such as a brain tumor or head trauma. A 61-year-old diabetic man presented with ptosis, opthalmoplegia, and facial palsy which were manifestations of multiple cranial neuropathies involving the left 3rd, 4th, 6th, and 7th cranial nerves throughout five weeks. The pupils were not involved. The neurologic evaluation included a CSF study and a brain MRI with MRA. None of them produced any significant results. Blink reflexes revealed evidence of a left facial nerve lesion. The blood glucose was strictly controlled and steroid therapy was administered. The ptosis of the patientanjx left eyelid improved during treatment and he was discharged after 13 days. In a follow-up examination 3 months after onset, focal neurological deficits including opthalmoplegia and facial palsy on the left side were greatly improved and barely noticeable.