An unusual presentation of neurosarcoidosis with progressive hearing loss

Abstract

Sarcoidosis is a granulomatous disease of unknown etiology that can involve several organ system. Neurological manifestations are not common and mostly include cranial neuropathies. However, auricular disorders are rare and exceptionally inaugural. We describe the case of a 46-year-old lady presented with hearing loss as the initial manifestation of sarcoidosis, and aim to raise awareness of this condition, that is often associated with significant morbidity.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

“…A cochlear involvement either isolated or associated with the neuropathy cannot be entirely ruled out as suggested by images of cochlear enhancement on MRI in patient 1 and of labyrinthitis ossificans reported in the literature [Dhanjal et al, 2014;Greene et al, 2017]. Normal morphology and latencies of ABR reported in 1 patient with moderate bilateral hearing loss also suggest possible cochlear involvement [Taous et al, 2017].…”

“…In a review of the literature, Colvin [2006] reported 50 cases. Since then, fewer than 30 adult and pediatric cases have been referenced in PubMed [Colvin, Color version available online DOI: 10.1159/000514479 2006; Agari et al, 2007;Cama et al, 2011;Loor et al, 2012;Szmulewicz, and Waterston, 2012;Dhanjal et al, 2014;Rose et al, 2014;Jardine et al, 2015;Shimizu et al, 2016;Greene et al, 2017;Svrakic et al, 2017;Taous et al, 2017]. In most cases, hearing loss was mild to moderate and reversible after corticosteroid treatment.…”

“…In a review of 305 patients diagnosed with neurosarcoidosis, Carlson et al [2014] reported that an optic neuropathy is the most prevalent cranial nerve lesion (30%) followed by trigeminal, facial, and audiovestibular nerve involvement (19, 18, and 17%, respectively). Audiovestibular manifestations are therefore relatively rare in sarcoidosis and have been described in a number of case reports [Colvin, 2006;Agari et al, 2007;Cama et al, 2011;Loor et al, 2012;Szmulewicz and Waterston, 2012;Dhanjal et al, 2014;Rose et al, 2014;Jardine et al, 2015;Shimizu et al, 2016;Greene et al, 2017;Svrakic et al, 2017;Taous et al, 2017]. The typical symptom is a mild to moderate sensorineural hearing loss usually bilateral (75%) and asymmetric (75%), slowly or rapidly progressive, sudden or fluctuating, and usually associated with signs of vestibular impairment [Colvin, 2006;Carlson et al, 2014].…”