Noncaseating epithelioid granulomas are found in the central nervous system in 5%
to 26%
of patients diagnosed with systemic sarcoidosis. The most common presentation of central
nervous system sarcoidosis (neurosarcoidosis) is cranial neuropathy, followed by
meningeal disease, including aseptic meningitis and mass lesions.
1
Neurosarcoidosis has a predilection for basal meninges that surround the cranial nerves
with infiltrative, perivascular granulomas. In the case of cranial nerve involvement,
eighth nerve symptoms occur in up to 20% of patients and are associated with other
cranial nerve neuropathies or overt systemic disease.
1,2
The incidence of sensorineural
hearing loss (SNHL) is only 5% to 9% among those diagnosed with neurosarcoidosis.
2
The likely mechanism of injury is vasculitis that leads to transient ischemia and
neural damage.
3
Approximately 70% of patients will recover at least some hearing, either
spontaneously or with corticosteroid therapy.
2
Progression to profound hearing loss is exceedingly rare. Consequently, little is
known about cochlear implantation as a rehabilitation option in this group of
patients.
Case Report
A 54-year old man presented to the otology clinic with sudden left moderate SNHL
(speech reception threshold [SRT], 50 dB; speech discrimination score [SDS], 60%)
and intermittent dizziness. He was treated with oral and intratympanic
corticosteroids without improvement. Four months later, the hearing on the left
declined to severe and unaidable (SRT, 80 dB; SDS, 18%); that on the right
progressed from normal (SRT, 5 dB; SDS, 100%) to mild (SRT, 25 dB; SDS, 86%).
Magnetic resonance imaging (MRI) demonstrated bilateral enhancing lesions of the
internal auditory canal (
Figure 1
, left). Eighteen months after presentation, the hearing on the left
fluctuated but did not recover, and the hearing on the right deteriorated to severe
and unaidable (SRT, 70 dB; SDS, 8%). Subsequent MRI demonstrated the internal
auditory canal lesions fluctuating in size and not correlating to the degree of
SNHL. During this time, the patient was seen by multiple specialists, and the workup
yielded negative results, including serum angiotensin-converting enzyme (ACE)
levels, Lyme titers, and antinuclear cytoplasmic antibodies. His chest radiograph
finding was negative for sarcoidosis. In addition to corticosteroid therapy, he was
treated with etanercept and valacyclovir. Despite medical treatment, his hearing did
not recover, and cochlear implantation was performed on the left, followed by the
right 2 months later. Postoperative consonant-nucleus-consonant word scores were
>88%.
Figure 1.
Magnetic resonance imaging: left, coronal T1 gadolinium sequence shows
enhancing lesions in the distal internal auditory canals (arrows); right,
axial T1 gadolinium sequence shows an enhancing lesion of the left
hippocampus (open arrow).
Six months after cochlear implantation, the patient developed neurologic symptoms
(jaw chatter, oscillopsia, cognitive dysfunction) discovered to be complex partial
seizures, culminating in a grand mal seizure. MRI revealed a new enhancing left
hippocampal lesion. Lumbar puncture was negative for inflammatory or infectious
processes, including antineuronal antibodies. Cerebrospinal fluid ACE level was
normal. Despite treatment with acyclovir for presumed viral encephalitis, the
hippocampal lesion continued to grow (
Figure 1
, right). An image-guided biopsy was obtained through the temporal gyrus
approach. The abnormal tissue appeared friable and slightly hemorrhagic.
Pathologic examination revealed nonnecrotizing granulomas with a perivascular
lymphocytic infiltrate within the brain parenchyma, most consistent with
neurosarcoidosis (
Figure 2
). Nationally recognized advanced diagnostic laboratories analyzed the
specimens, excluded a lymphoproliferative process, and did not identify a causative
infectious agent. Two years later, in addition to seizures, the patient continues
to
suffer further neurologic manifestations of sarcoid, including dysphonia, dysphagia,
dysarthria, worsening ambulation, and some cognitive decline despite
immunosuppressive therapy.
Figure 2.
Top left: CD3+ stain demonstrating a T-cell lymphocytic infiltrate.
Hematoxylin and eosin stains show a noncaseating granuloma (lower right,
arrow) and perivascular infiltrates (upper right, arrowheads). Bottom left:
reticulin stain highlighting reticulin surrounding and permeating the
histiocytic cluster.
Discussion
The typical clinical course of neurosarcoid-associated SNHL is an asymmetric sudden
or rapidly progressive mild to moderate loss, fluctuating and involving both ears.
2
In rare cases of profound loss, patients may demonstrate radiologic
abnormalities, such as inflammatory lesions along the cochlear nerve, labyrinthine
enhancements, and even cochlear ossification.
4
While definitive diagnosis of neurosarcoid depends on a central nervous
system biopsy, for most patients the diagnosis of probable neurosarcoidosis is
obtained by a combination of radiologic findings, abnormal cerebrospinal fluid
profile, biopsy of extraneural sites, chest radiography, and serum ACE levels.
5
With SNHL, most patients experience concurrent neurologic symptoms, and half
the patients will have associated cranial nerve neuropathies.
1
-3
Successful cochlear
implantations have been reported in only a handful of cases with probable
neurosarcoidosis. Our case report is the first to describe implantation in
definitive neurosarcoid confirming that cochlear implants remain a viable option and
should be considered for patients with unaidable, medically unresponsive disease
despite retrocochlear or intralabyrinthine lesions.
1,4
Author Contributions
Maja Svrakic, concept and design, interpretation of data, drafting,
final approval, accountability for all aspects of the work; John G.
Golfinos, acquisition of data, critical revision, final approval,
accountability for all aspects of the work; David Zagzag, acquisition
of data, interpretation of data, critical revision, final approval, accountability
for all aspects of the work; J. Thomas Roland Jr, concept and design,
acquisition of data, critical revision, final approval, accountability for all
aspects of the work.
Disclosures
Competing interests: J. Thomas Roland Jr, on the advisory board for
Cochlear Americas.
Sponsorships: None.
Funding source: None.