Optometry - Journal of the American Optometric Association
Volume 81, Issue 3 , Pages 137-141, March 2010

Neuro-ophthalmic manifestations of a complicated cholesteatoma

  • Melanie A. Crandall, O.D.

      Affiliations

    • Nova Southeastern University College of Optometry, Fort Lauderdale, Florida
    • ,
  • Maryke N. Neiberg, O.D.

      Affiliations

    • Southern College of Optometry, Memphis, Tennessee
    • Corresponding Author InformationCorresponding author: Maryke N. Neiberg, O.D., Southern College of Optometry, 1245 Madison Ave., Memphis, Tennessee 38104.
    • ,
  • Kenneth R. Seger, O.D.

      Affiliations

    • Nova Southeastern University College of Optometry, Fort Lauderdale, Florida

Article Outline

Abstract 

Background

A cholesteatoma, also known as an epithelioma, is a mass of soft tissue that results from the accumulation of keratin protein debris enclosed by a layer of stratified squamous epithelium.

Case Report

A patient with cholesteatoma surrounding both internal carotid arteries presented with cerebral ischemic symptoms expressed as visual and ocular symptoms. Because of its location, mass effect, and potential stenosis of the internal carotids, this slow-growing tumor is potentially sight threatening. Asymmetric cupping of the optic nerves associated with symptoms such as dizziness, diplopia, or facial pain should be worked up carefully.

Conclusion

The primary care optometrist can play an important role in the diagnosis and continued management of a patient with a cholesteatoma, carefully monitoring early signs of progression or change. This case illustrates how an intracranial lesion, away from the eye, can impact a number of ocular and visual functions.

Keywords: Cholesteatoma, Internal carotid artery, Cerebellopontine angle, Diplopia, Ptosis, Blurred vision, Dry eye, Foville's syndrome

 

A cholesteatoma, also known as an epithelioma, is a mass of soft tissue that results from the accumulation of keratin protein debris enclosed by a layer of stratified squamous epithelium.1, 2 An instance in which squamous epithelium is trapped within the temporal bone during embryogenesis is considered primary or congenital cholesteatoma.2 Primary cholesteatomas may occur in the temporal bone within the middle ear, the mastoid, the petrous bone, and within the tympanic membrane.3 Intradural congenital cholesteatomas involve the cerebellopontine angle and affect hearing and balance.3 Secondary or acquired cholesteatomas are more common and are often a complication of chronic osteomastoiditis.3 Inflammation and infection are almost always present; there are no cellular or molecular differences between primary and secondary cholesteatoma.4 In most cases, they are minimally proliferative, nonmigratory, and noninvasive.3, 4

We present a patient with bilateral cholesteatomas surrounding both internal carotid arteries presenting with an array of visual and ocular symptoms associated with the erosion of the petrous bone at the cerebellopontine angle, invasion of the cavernous sinus, and ischemia of the pons. In addition, this slow-growing nonmetastatic tumor has the potential to be sight threatening because of the ocular ischemia caused by stenosis of the internal carotids.

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Case 

A 60-year-old woman presented to our clinic complaining of intermittently blurred vision. She reported that she suffered debilitating episodes of diplopia, hearing loss, dizziness, and extreme vertigo. She also had bouts of numbness on the left side of her face.

The patient had been identified elsewhere as a “glaucoma suspect” because of her asymmetric cup-to-disc ratios of 0.55/0.55 in the right eye (O.D.) and 0.70/0.70 in the left eye (O.S.). The presenting asymmetry can be seen in Figure 1, and this asymmetry has remained unchanged since her first visit.

As a result of her debilitating episodes of diplopia, hearing loss, dizziness, and extreme vertigo, magnetic resonance imaging (MRI) was ordered and resulted in a diagnosis of benign, bilateral and inoperable cholesteatoma. The mass was larger on the left side and wrapped around the left internal carotid. The irregular mass had eroded and remodeled the apex of the petrous bone that lies at the anterior and superior part of the temporal bone. (Anatomically, the base of the petrous bone is bound by the labyrinth and the carotid lies anterior to it.) The mass extended into the carotid canal and partially surrounded the left internal carotid artery just below the cavernous portion. The lesion on the right side was similar in presentation, but smaller. Figure 2 shows an MRI of the brain with the lesions clearly visible around the carotids.

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  • Figure 2 

    The irregular mass is visible in the left petrous apex where it has eroded and remodeled the petrous apex. Its mass extends into the carotid canal and appears to partially surround the left internal carotid artery just below the cavernous portion. A similar, smaller lesion is present on the right side.

Her current medications were lisinopril, meclizine and Lipitor (Pfizer, New York, New York) for hypertension, dizziness, and hypercholesterolemia, respectively. Blood pressure at this visit was 115/70 mmHg right arm sitting (RAS). Both pupils were round and reactive to light and accommodation without afferent pupillary defect. Her extra-ocular motilities were full without pain, restriction, or diplopia. Entering cover test showed orthophoria at distance and 2 exophoria at near. Although our patient complained of episodic double vision, we could not elicit or measure it in the clinic. The exophthalmometer readings were 24 mm O.D. and 26 mm O.S. at a base of 121. Confrontation visual fields were full to finger counting O.D. and O.S. Color vision was normal using Ishihara plates. Manifest refraction was O.D. -0.75-1.00x 75, 20/20 and O.S. -0.75-0.50 x105, 20/20.

Consistent with the systemic hyperlipidemia, prominent arcus senilis was noted in both corneas. The slit lamp evaluation found a reduced tear breakup time of 5 seconds in each eye, as well as inferior punctate staining on the left cornea evident of dry eye. Intraocular pressures were 18 mmHg O.D. and O.S. Fundus evaluation confirmed the previously known cup-to-disc asymmetry: O.D., 50/0.50, and O.S., 70/0.70. The optic disc margins were distinct. A posterior vitreous detachment was noted in the left eye. Both maculae were healthy, and foveal reflexes were present in each eye. No hypertensive retinopathy was noted. The retinal periphery was flat in both eyes (OU), intact, and otherwise unremarkable.

The patient was scheduled to return for a visual field evaluation, at which time she complained of episodic drooping of the left lid. She had also experienced some swelling and pain around her left eyebrow. The Humphrey 24-2 threshold visual fields showed some isolated missed points in both eyes. When the visual fields were repeated a year later, a superior arcuate defect in the left eye and a nasal defect in the right were present. During this time, the mean deviation measurement in both eyes reduced by approximately 2 dB. The changes in the nerve fiber layer, as measured by scanning laser polarimetry with corneal compensation (GDXVcc), showed thinning of the inferior nerve fiber layer, more marked on the left than the right (see Figure 3). It was performed again at a 1-year interval, and slight progression was evident in our results (not shown here). The optic nerve appearance has since remained unchanged.

Our patient is being monitored carefully for any visual or ocular changes. Artificial tears were prescribed for her dry eyes. The MRI was repeated approximately 1 year later with very little increase in the size of the lesion noted.

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Discussion 

A cholesteatoma or epithelioma is composed of a core of keratin debris surrounded by a layer of squamous epithelium that spreads along the path of least resistance.1 Congenital cholesteatoma is the most common embryonal lesion found in the cerebellopontine angle (CPA) and third most common tumor.5 The majority (85%) of CPA lesions are acoustic neuromas.5 Congenital cholesteatomas develop where separated dorsal epidermal cells are misplaced by an aberrant closure of the neural tube.1, 6 Rarer tumors of the CPA include meningiomas, primary cholesteatomas, cranial nerve schwannomas, and arachnoid cysts.1, 2 Cohen's classification estimates the incidence of primary tympanic cavity cholesteatoma to be 71% and temporal bone primary cholesteatoma to be 29%.7

The earliest symptoms of congenital cholesteatoma frequently include headache, hemifacial spasm, hearing loss, and tinnitus.5, 8 Compression at the foramen ovale can cause trigeminal (CNV3) paresthesia or numbness, whereas stretching of the dura may cause headaches. The diagnosis of cholesteatoma is made based on the location of the tumor and its features visible with MRI.1, 3 An MRI is considered superior to computed tomography (CT) when the tumor is in the petrous apex, cerebellopontine angle and when the facial nerve, labyrinth, and other intracranial structures are involved.3 A cholesteatoma shows up on an MRI as a large homogenous, nonenhancing lesion.9

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Erosion of the petrous bone 

Cholesteatomas are known to cause symptoms and signs of a slowly expanding mass lesion and can occasionally narrow intracranial vessels, destroying the bone, cochlea, labyrinth, and facial nerve.6 Destruction of the facial nerve by petrous apex erosion can lead to facial palsies and ptosis, and, when accompanied by vertigo, surgery may be indicated.6 The facial nerve (CN VII) is affected in 20% to 50% of cases.6 The facial motor nucleus is located in the lower third of the pons and in close proximity to the abducens nucleus. Involvement of CN VI (abducens), and CN VII (facial) presenting as diplopia and numbness, respectively, indicates proximity of the lesion close to the fourth ventricle.10 An ischemic lesion on the lateral pons, causing ipsilateral facial paresis, ipsilateral facial analgesia, ipsilateral Horner's syndrome, and ipsilateral deafness or tinnitus, is known as Foville's syndrome.10

The CN VII (facial) and the CN VIII (vestibulocochlear) course along the cerebellopontine angle together, whereas the nervus intermedius lies in between these 2 cranial nerves. The function of the nervus intermedius is to carry taste fibers, but more importantly, as illustrated by our case, the preganglionic parasympathetic innervations to the submandibular, sublingual, and lacrimal glands. In addition to the facial paralysis and CN VI abducens paresis, interference with the parasympathetic innervation of the lacrimal gland results in reduced tearing and therefore symptoms of dry eye and blurred vision.10

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Ocular ischemic syndrome 

Our patient is at risk for the development of ocular ischemic syndrome because of the progressive nature of the cholesteatoma. Congenital cholesteatomas occur on the temporal bone and the petrous apex, proximal to the internal carotid, from which the ophthalmic artery arises. The artery branches off just as the internal carotid emerges from the cavernous sinus. This is the vascular source for both the vessels surrounding the eye and the central retinal artery as well as the short and long posterior ciliary vessels. The cavernous portion of the internal carotid branches into the ganglionic, anterior meningeal, ophthalmic, anterior cerebral, and middle cerebral arteries. Branches of the ophthalmic artery supply the globe, lid, and muscles of the eye.11

Stenosis (greater than 70%) or compression of the internal carotid can lead to ocular ischemic syndrome, characterized by a progressive loss of visual acuity with or without pain around the eye.5, 12 The signs and symptoms of anterior ischemic syndrome are dilated episcleral vessels, corneal edema, anterior chamber inflammation, and iris neovascularization.13 Low pressure ischemic retinopathy presents with dilated veins, microaneurysms, and blot hemorrhages in the periphery.14 Hemodynamic transient monocular blindness is most commonly described by patients as a “white-out” of vision.14 Reduced retinal artery pressure can be measured with ophthalmodynamometry.12

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Vascular compression of the optic nerves 

Cholesteatomas rarely cause sudden ischemic symptoms, but ischemic symptoms have been reported as presenting symptoms.15 Asymmetric cupping seen in conjunction with normal intraocular pressure and stable visual field defects, atypical of glaucoma, must raise suspicion of a nonglaucomatous etiology.16, 17 The mass effect of the cholesteatoma alone is a significant cause of compression of the optic nerves with optic atrophy and attendant visual field defects. Direct compression of the lesion of the optic nerve by the lesion may lead to symptoms of progressive blurring of vision or dull pain in the retro-orbital area.9 The vascular rigidity induced by the cholesteatoma to the internal carotids may be a further contributor to the optic atrophy as the carotids compress the optic nerve.16, 18

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Management 

Complete surgical excision is usually performed on cholesteatomas. The location, extent of the lesion, and the hearing status of the patient, determine the surgical approach.19 Facial palsy in conjunction with cholesteatoma should be treated as soon as possible, so that residual function is maintained and further deterioration prevented.20 The petrous bone is fairly inaccessible, and complete removal of the tumor while preserving the normal structures is as a surgery, technically challenging.6 Complications of untreated cholesteatoma include life-threatening conditions, such as meningitis, sinus thrombosis, and brain abscess.7, 21

The neurosurgeons involved in our case weighed the risk-benefit ratio of surgery and decided against surgery at this time. Meanwhile, management of cardiovascular risk factors to the carotids, such as hypertension, hyperlipidemia, diabetes, smoking, and alcohol use should be addressed to reduce additional chance of stenosis of the carotids.22, 23 Because of the variable nature of the double vision and dizziness, prescription of prism was not useful for this patient. Episodic diplopia is best managed with temporary patching. Safety eyewear must be considered in cases in which there is a risk of injury from dizziness or vertigo.

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Conclusion 

Asymmetric cupping of the optic nerves associated with symptoms such as dizziness, diplopia, or facial pain, should be worked up carefully. The primary care optometrist plays an important role in the diagnosis and continued management of a patient with a cholesteatoma, carefully monitoring early signs of progression or change. This case illustrates how an intracranial lesion, away from the eye, can impact a number of ocular and visual functions.

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References 

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PII: S1529-1839(09)00595-8

doi:10.1016/j.optm.2009.11.001

Optometry - Journal of the American Optometric Association
Volume 81, Issue 3 , Pages 137-141, March 2010

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