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Objectives: The objective of this study was to present a review article about internal auditory canal hemangioma.
Data Sources: Published English-language literatures.
Review Methods: We included studies about internal auditory canal hemangioma.
Results: 42 studies were included in this study. The results showed that most patients were male, the age of patients varied between 5 to 69 years old. Two cases were multiple familial hemangioma, while the most other cases were sporadic. Hearing loss was the most common symptoms, 37% patients had facial palsy, and 38% patients had vestibular symptoms. Since most patients had non- serviceable hearing loss so, translabrynithine approach was the most common surgical approach. Most serviceable hearing patients lost their serviceable hearing after surgery. Only one third of patients with facial palsy had improvement after surgery. Most cases had attachment to facial nerve and can be removed totally.
Conclusion: we should suspect IAC hemangioma in any patient with considerable hearing loss and facial palsy with small lesion in internal auditory canal.
Cavernous hemangioma of the internal auditory canal (IAC) is a rare disease. It comprises 10 to 20% of all central nervous vascular malformations. They are composed of large, sinusoidal, thin-walled capillary spaces that may invade the surrounding neural tissue. They can mimic the symptoms of
vestibular shwannomas. We review the clinical features and the management of the IAC hemangioma .
78 patients had hearing loss (96%). 11 patients of them
had sudden hearing loss (14%), while the other 67 patients had
progressive hearing loss (86%). 16 patients had serviceable
hearing loss (20%), while the other 52 patients had nonserviceable
hearing a loss (80%).
46 patients had tinnitus (56%), 31 patients had vestibular
symptoms (38%) (16 patients had vertigo and 15 patients had
unsteadiness). 30 patients had facial palsy (37%), 6 patients
of them had sudden facial palsy (20%), 3 patients of them had
recurrent facial palsy (10%), while the other 21 had progressive
facial palsy (70%). 13 patients had facial spasm (16%). 2
patients had headache (3%), and 2 patients had familial multiple
hemangioma (3%) (Table 2).
Two patients were managed by observation. 79 patients had
a surgery, 3 of them had normal hearing, and 15 patients of them
had serviceable hearing loss, while the other 51 patients had
non-serviceable hearing loss (Table 3). Only 11 articles reported
the postoperative clinical progress of vestibular symptoms,
and all reported patients with vestibular symptoms improved.
13 patients had preoperative facial spasm, and it disappeared
in all of them postoperatively. Two patients were managed
conservatively, 8 patients had MFA, and 29 patients had RS
approach, while the other 42 patients had TR approach (Table
4). 30 patients with facial palsy had a surgery, 11 patients of
them had facial weakness improvement (Table 5). 16 patients
had facial reconstruction (20%) (Table 6). 68 patients had a
limited disease to IAC, while 13 patients had disease extension
into CPA (Figure 3). 72 articles reported the surgical removal
type, 52 patients of them had a total surgical resection, and 17
patients of them had a near total resection while the other 3
patients had only partial resection (Figure 4). Hemangioma
attachments were reported in 63 patients, 27 patients of them
had CN VII attachment, and 21 patients of them had CN VII &
CN VIII attachment, while the other 15 patients had CN VIII
attachment (Figure 5).
Most patient were male with age ranging between 20
to 50 years old. Most patients had non-serviceable hearing
loss.38% patients had vestibular symptoms, 37% patients had
facial palsy, 16% patients had facial spasm. Only third patient
with serviceable-hearing loss preserved their hearing abilities
after surgery, third patient with facial palsy had significant
improvement after surgery (acute facial palsy has a better
prognosis than progressive facial palsy), and all patients with
facial spasm and vestibular symptoms had a good improvement.
Most cases were located primary to IAC, most cases had
attachment to facial nerve and can be removed totally, and
about20% of patients had facial reconstruction surgery due to
advanced facial nerve involvement.
IAC hemangioma is a rare disease with poor outcomes
prognosis, we should suspect this disease in patients with
progressive hearing loss and facial palsy with small lesion in
internal auditory canal vertigo and Tinnitus.
Vestibular schwannoma is the most common tumor in IAC
and the cerebellopontine angle (CPA). Other tumor lesions of
CPA area include meningioma, primary cholesteatoma, facial nerve neuroma, various vascular tumors, metastatic tumors,
and others. Cavernous angioma was reported rarely in the
IAC. Sundares et al. reported the first case of IAC cavernous
angioma in 1976. These tumors were considered to originate
from the capillary bed of the epineurium nerve. Vascular steal
mechanism in which the blood is taken by tumor instead of
the nerve causing nerve function loss even with a small size
of tumor. Histopathologically, they consist of large thin-walled
blood vessels that lined by flattened endothelium, which stain
positive by endothelial marker CD 31, the stroma is composed of
fibrous component and has mainly myofibroblast and fibroblast
that stain positive for smooth muscle actin.
Depending on nerve origin and location, these tumors can
cause severe progressive or sudden sensorineural hearing loss,
tinnitus, sudden or progressive facial nerve palsy, facial spasm,
vertigo and disequilibrium even when they are in small size. The
tumor size is usually less than 10 mm. On CT scan, IAC hemangioma
appear as iso-or hyper- dense lesion with slight enhancement
after intravenous administration of contrast, usually stippled
calcifications could also be seen with enlargement of the IAC. On
MRI, It appears as lobular and isointense in T1 and hyperintense
on T2 with heterogeneous T1 post - gadolinium enhancement.
The main differential diagnosis is IAC Vestibular shawanoma
(Table 7). Other differential diagnosis may include meningioma,
lipoma, melanoma, hamartoma, and lymphoma.
Complete surgical resection with avoiding complications
such bleeding is the goal of treatment. Radiotherapy may
promote growth and hemorrhage, so it is not recommended. No
symptomatic cases can be managed conservatively. Symptomatic
case should be resected surgically. Surgical approach is
depended on hearing deficit and tumor size. Translabyrinthine
approach is recommended for patient with non-serviceable
hearing loss. Middle fossa approach and retrosigmoid approach
are recommended for patients with serviceable hearing loss.
This lesion could be attached firmly with facial nerve. Since the
tumor is benign and slow growing, it is advocated to perform a
near total resection in case where the tumor is difficult to dissect
from facial nerve [18,26,27].