Clin Exp Otorhinolaryngol.  2011 Sep;4(3):155-158.

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

• Lim EJ
• Kim SH
• Lee SH
• Lee KY
• Choi JH
• Nam EJ
• Lee SH

Abstract

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.

Keyword

Hypertrophic pachymeningitis; Multiple cranial neuropathies; p-ANCA; Wegener's granulomatosis

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Autoimmune Diseases
Brain
Cranial Nerve Diseases
Cyclophosphamide
Cytoplasm
Dietary Sucrose
Dura Mater
Female
Follow-Up Studies
Gadolinium
Headache
Hearing Loss
Hearing Loss, Sensorineural
Humans
Inflammation
Meningitis
Middle Aged
Reference Values
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Dietary Sucrose
Gadolinium

Figure

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  Fig. 1 Pure tone audiograms and word recognition scores before and after treatment. Initial pure tone audiogram (A). Pure tone audiogram after a 4-month period of steroid and cyclophosphamide treatment. Hearing improvement was detected, especially in the high frequency region (arrow) (B). Serially checked word recognition scores in the left ear after steroid and cyclophosphamide treatment (C). Note that the word recognition score went up daily after steroid administration and in month 4 it reached 100%. On the other hand, the word recognition score in the right ear was 100% in every test.

• 

  Fig. 2 Initial brain MRI. Axial T1-weighted image shows hypo-intense thickened dura mater (A). Axial T2-weighted image shows isointense dura mater with hyperintense border (B).

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  Fig. 3 The gadolinium enhanced T1-weighted axial and coronal images. Initial images. They demonstrate diffusely enhanced thick dura mater around cerebellopontine angle (arrow) (A). After 4-month steroid and cyclophosphamide treatment (B). After 9-month steroid and cyclophosphamide maintenance (C). Note that diseased dura mater (arrow) became less thickened compared with initial MR image as treatment was maintained.

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  Fig. 4 Changes in myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer during steroid and cyclophosphamide treatment. Note that the initial high titer of MPO-ANCA (388AAU) decreased to within the normal range as treatment was maintained. Brain MRI scans were done at 4 and 9 months after treatment.

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