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Idiopathic pachymeningitis presenting with progressive sensorineural hearing loss, tinnitus and confusion.

The patient was a 74-year-old female who presented with progressive left hearing loss, nonpulsatile tinnitus, and confusion. She denied headache, visual symptoms, and vertigo. She had a normal neurotologic examination. Pure tone audiometry showed left low frequency sensorineural hearing loss (SNHL) and a bilateral symmetric high frequency SNHL, with word recognition of 100% right, 46% left (Fig. 1). Serologic studies revealed a nonspecific monoclonal gammopathy. Other serologic tests were negative, including CBC, ANA, Lyme titer, syphilis, angiotensin converting enzyme, anti-double stranded DNA and ANCA antibodies. Cerebrospinal fluid testing revealed normal cytology with a mild increase in protein. Computed tomography of the abdomen and chest was normal, as was bone marrow biopsy. Magnetic resonance imaging (MRI) demonstrated diffuse smooth dural thickening and enhancement consistent with idiopathic hypertrophic pachymeningitis (IHP) (Fig. 2). 
 
 
 
Figure 1 
 
Pure tone audiograms from 2009 through 2012, demonstrating progressive hearing loss in the left (A) and right (B) ears. At baseline (solid black line), the patient had a left 30 dB SRT and 100% WR, and a right SRT of 20 dB and WR 100%. After onset of ... 
 
 
 
 
 
Figure 2 
 
Axial T1-weighted contrast-enhanced MRI of brain from prior to treatment (A) demonstrating initial enhancement of dura of the internal auditory canals left > right (arrowheads) prior to treatment. Following pulse steroid treatment (B), a significant ... 
 
 
 
The patient was initially treated with oral prednisone with minimal improvement. She then received IV methylprednisolone infusions every two weeks for two months with improvement in left hearing, tinnitus, mental status, and dural enhancement on MRI. Six months following initial presentation, she showed no further progression of her symptoms while off steroid therapy.