Symmetric sensorineural progressive hearing loss from chronic idiopathic pachymeningitis

We present the case of a 68 year-old man with a diffused hypertrophic pachymeningitis (HP) involving both internal auditory canals. The clinical symptoms were headache, decreased vision in one eye, progressive bilateral and symmetrical sensory-neural hearing loss (PSNHL) responsive to steroid treatment. Although hearing loss is a frequent manifestation of HP, only few studies reported an adequate audiological assessment and follow-up. Mechanisms related to the auditory involvement are discussed on the basis of audiological data. Gadolinium enhanced MRI is the most adequate technique for HP detection and for the differential diagnosis. A delay in the diagnosis of HP seems to be quite common and the consequences may be severe, especially in cases of optic nerve involvement. For these reasons, a cerebral MRI should probably be included in the assessment of PSNHL, especially when neurological signs coexist or are reported in the medical history.

[1]  Y. Ota,et al.  A case of idiopathic hypertrophic cranial pachymeningitis manifested only by positive rheumatoid factor and abnormal findings of the anterior falx , 2005, Rheumatology International.

[2]  J. Lirng,et al.  Headache Profile in Patients With Idiopathic Hypertrophic Cranial Pachymeningitis , 2004, Headache.

[3]  R. Jackler,et al.  Chronic Pachymeningitis Presenting as Asymmetric Sensorineural Hearing Loss , 2004, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[4]  M. Kupersmith,et al.  Idiopathic hypertrophic pachymeningitis , 2004, Neurology.

[5]  P. Celli,et al.  Idiopathic intracranial hypertrophic pachymeningitis: two case reports and review of the literature , 2004, Neurosurgical Review.

[6]  T. Morioka,et al.  Spontaneous resolution of hypertrophic cranial pachymeningitis , 2004, Neurosurgical Review.

[7]  S. Iwasaki,et al.  Hypertrophic Cranial Pachymeningitis Presenting as Steroid-Responsive Hearing Loss , 2003, The Annals of otology, rhinology, and laryngology.

[8]  C. Lui,et al.  Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up. , 2003, AJNR. American journal of neuroradiology.

[9]  P. Cherian,et al.  Idiopathic hypertrophic cranial pachymeningitis. , 2002, Neurology India.

[10]  J. Yoshida,et al.  Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options. , 1999, Neurosurgery.

[11]  J. Vanneste,et al.  Aspecific headache during 13 years as the only symptom of idiopathic hypertrophic pachymeningitis , 1998, Journal of neurology, neurosurgery, and psychiatry.

[12]  S. Gaikwad,et al.  Idiopathic hypertrophic pachymeningitis: spectrum of the disease , 1997, Neuroradiology.

[13]  J. Hauw,et al.  Cranial pachymeningitis of unknown origin , 1993, Neurology.