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...
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| Format: | Article |
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Korean Society of Otorhinolaryngology-Head and Neck Surgery
2011-09-01
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| Series: | Clinical and Experimental Otorhinolaryngology |
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| Online Access: | http://www.e-ceo.org/upload/pdf/ceo-4-155.pdf |
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| author | Eun-Jung Lim Sung-Hee Kim Su-Hwan Lee Kyu-Yup Lee Jae-Hyuk Choi Eon-Jeong Nam Sang-Heun Lee |
| author_facet | Eun-Jung Lim Sung-Hee Kim Su-Hwan Lee Kyu-Yup Lee Jae-Hyuk Choi Eon-Jeong Nam Sang-Heun Lee |
| author_sort | Eun-Jung Lim |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T04:03:22Z |
| format | Article |
| id | doaj.art-5e3cc4290d61497cb780e2d36c6f492f |
| institution | Directory Open Access Journal |
| issn | 1976-8710 2005-0720 |
| language | English |
| last_indexed | 2024-12-23T04:03:22Z |
| publishDate | 2011-09-01 |
| publisher | Korean Society of Otorhinolaryngology-Head and Neck Surgery |
| record_format | Article |
| series | Clinical and Experimental Otorhinolaryngology |
| spelling | doaj.art-5e3cc4290d61497cb780e2d36c6f492f2022-12-21T18:00:40ZengKorean Society of Otorhinolaryngology-Head and Neck SurgeryClinical and Experimental Otorhinolaryngology1976-87102005-07202011-09-014315515810.3342/ceo.2011.4.3.155137Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCAEun-Jung Lim0Sung-Hee Kim1Su-Hwan Lee2Kyu-Yup Lee3Jae-Hyuk Choi4Eon-Jeong Nam5Sang-Heun Lee6Department of Otorhinolaryngology-Head and Neck Surgery, Daegu Fatima Hospital, Daegu, Korea.Department of Otorhinolaryngology-Head and Neck Surgery, Daegu Fatima Hospital, Daegu, Korea.Department of Otorhinolaryngology-Head and Neck Surgery, Daegu Fatima Hospital, Daegu, Korea.Department of Otorhinolaryngology-Head and Neck Surgery, Kyungpook National University School of Medicine, Daegu, Korea.Department of Otorhinolaryngology-Head and Neck Surgery, Kyungpook National University School of Medicine, Daegu, Korea.Division of Rheumatology, Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea.Department of Otorhinolaryngology-Head and Neck Surgery, Kyungpook National University School of Medicine, Daegu, Korea.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.http://www.e-ceo.org/upload/pdf/ceo-4-155.pdfHypertrophic pachymeningitisMultiple cranial neuropathiesp-ANCAWegener's granulomatosis |
| spellingShingle | Eun-Jung Lim Sung-Hee Kim Su-Hwan Lee Kyu-Yup Lee Jae-Hyuk Choi Eon-Jeong Nam Sang-Heun Lee Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA Clinical and Experimental Otorhinolaryngology Hypertrophic pachymeningitis Multiple cranial neuropathies p-ANCA Wegener's granulomatosis |
| title | Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA |
| title_full | Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA |
| title_fullStr | Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA |
| title_full_unstemmed | Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA |
| title_short | Reversible Sensorineural Hearing Loss due to Pachymeningitis Associated with Elevated Serum MPO-ANCA |
| title_sort | reversible sensorineural hearing loss due to pachymeningitis associated with elevated serum mpo anca |
| topic | Hypertrophic pachymeningitis Multiple cranial neuropathies p-ANCA Wegener's granulomatosis |
| url | http://www.e-ceo.org/upload/pdf/ceo-4-155.pdf |
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