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.