Discussion
Behcet’s disease is a type of vasculitis that affects arteries and veins of any size. Its presentation commonly involves the mucus membranes, skin, and vasculature. It has an autoimmune etiology after exposure to specific environmental or infectious agents in genetically prone people. The otologic manifestations of Behcet’s disease include anything from tinnitus to sensorineural deafness [3].
Our case was a known Behcet’s disease gentleman who presented with recurrent episodes of sensorineural hearing loss, which was attributed to an autoimmune pathology because of his Behcet’s disease.
Literature review using Pubmed search has shown multiple case series and case reports of sensorineural deafness as a manifestation in patients with Behcet’s disease. A retrospective review of 33 patients with Behcet’s disease in tertiary hospital in Spain showed 5 (15%) of total patients had audiovestibular symptoms (including sensorineural hearing loss, vertigo, and bilateral vestibular dysfunction) [3]. Another study which included 63 subjects with Behcet’s disease and 63 sex and age-matched healthy subjects as a control group showed that 35 patients (55%) had hearing loss equal or more than 30 Decibell (DB) and 24 of the 35 patients (68% of the patients with hearing loss) showed hearing loss at higher frequencies of (6-8 kHz) [4]. In another study which recruited 62 Behcet’s disease patients and compared with 62 healthy subjects, around 32% (20 of the 62 Behcet’s patients) had evidence of sensorineural deafness (at 25 DB in at least two frequencies) by pure tone audiogram, all of the 20 patients with hearing loss had cochlear involvement evidenced by the recruitment investigation tests and auditory brain stem response. 15/20 patients with hearing loss had HLA-B51 antigen-positive [5].
In a prospective placebo-controlled study that included 29 patients with Behcet’s disease and 28 normal subjects, pure tone audiometry (PTA) and transient evoked otoacoustic emission (TEOAE) were determined, and it was found that 10 out of the 29 patients with Behcet’s disease had sensorineural hearing loss (34.5%) at 1,2,3,4, and 8 kHz (p< 0.498) and this was statistically significant compared to the control group. The difference in TEOAE levels that revealed sound to noise ratio between the two groups was statistically not significant at 1, 1.5, 2, and 3 but was significant at 4 kHz (p<0.02), and the difference was reproducible (p<0>03) [6]
In a review, 24 Behcet’s disease patients with 24 age and sex-matched healthy subjects as control groups were studied to investigate hearing loss in Behcet’s disease. It showed that 15 of the 24 subjects with Behcet’s disease had sensorineural deafness [7].
In another study of 35 subjects with Behcet’s disease compared with 35 healthy subjects as a control group and were followed up for a year, 28 patients with Behcet’s disease (80%) showed some degree of hearing loss [8].
Some case reports about the association between Behcet’s disease and sensorineural hearing loss were also found in the literature [9] [10] [11].
A possible pathophysiological explanation for the sensorineural hearing loss in Behcet’s disease includes autoimmune inflammation of the common cochlear artery affecting primarily the cochlea’s outer hair cells resulting in decreased sensitivity and impaired sound evoked neural activity. [8]
Anti- TNF is usually used in refractory Behcets disease manifestations, mainly sight-threatening disease with frequent relapse of uveitis in addition to other refractory manifestations [12]. But its use in sensorineural hearing loss is less well established.
Possible treatment options for the sensorineural loss in Behcet’s disease include high dose systemic steroids initially followed by steroid sparing agents, intratympanic steroid injections and hyperbaric O2 therapy [9] [10]. Initial management for our patient was with oral prednisolone 70 mg and intratympanic steroid injection, which successfully controlled his symptoms with partial improvement and decreased the acute attacks rate. However, the symptoms recurred once the steroid dosage was reduced. So, we started him on infliximab and later azathioprine was added, both successfully helped to control his symptoms and discontinue steroid.