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DISEASE/DISORDER:

Definition

Cranial nerve (CN) injuries are a common complication after  traumatic brain injury (TBI).3 The 12 pairs of cranial nerves provide motor and sensory innervation to the head, neck, glands, vasculature and viscera.1,2 Understanding the anatomy and function of each individual cranial nerve is essential for accurate diagnosis and effective treatment of these injuries.

Etiology

There are various causes of CN injury which may include:

  • Mechanical – e.g. Skull fracture, intracranial mass lesion, brain stem herniation, compression from increased intracranial pressure, traction, or transection
  • Vascular – e.g. Ischemic event from an infarct, vascular occlusion, aneurysm, intracranial hemorrhage4
  • Motor neuron disorders – e.g. Amyotrophic Lateral Sclerosis
  • Demyelinating diseases – e.g. Multiple Sclerosis
  • Other – Acceleration-deceleration injury, shearing injury, or meningitis4,5,6
  • Rheumatological conditions
  • Metabolic (e.g. diabetes mellitus)
  • Infectious (e.g. syphilis, HIV)

Epidemiology including risk factors and primary prevention

The exact incidence of cranial nerve injuries is unknown. The following is the reported order of frequency after cranial nerve injury:5

  1. Olfactory nerve (CN I)
  2. Facial (CN VII)
  3. Vestibulocochlear nerve (CN VIII)
  4. Optic nerve (CN II) and oculomotor nerves (CN III)3,5
  5. Trigeminal (CN V) and lower cranial nerves (CN IX-XII) are rarely injured5

CN I, CN II, CN III, trochlear nerve (CN IV), CN V (first two branches), CN VII and CN VIII are at increased risk for injury from skull fracture secondary to their position next the cranium.1

Patho-anatomy/physiology:4,7

Cranial patho-antomy and clinical features:4,7

Specific secondary or associated conditions and complications

Cranial nerve III, IV and VI lesions may result in secondary vision difficulties. CN VII lesions may result in mobility impairments. A cognitively impaired patient may not describe loss of the ability to smell in a CN I lesion, but may develop anorexia.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Cranial nerve injuries may present immediately or in a delayed fashion, depending on the cause of injury. For example, complete laceration of a nerve via skull fracture may cause immediate paralysis; gradual compression may cause delayed paralysis.

In addition, patients may present with multiple cranial neuropathies, and there is a wide differential for pathologies that manifest as cranial nerve dysfunction.24 The natural history of cranial nerve injury depends on the underlying cause, which may include infectious, inflammatory, neoplastic, vascular, toxic, and trauma. One such example is polycranial neuritis, an oculopharyngeal subtype of GBS, which presents as ocular and pharyngeal weakness without limb weakness or ataxia.25

Incomplete cranial nerve lesions often improve with time, while complete lesions are much less likely to recover. The olfactory nerve, in particular, has a capacity for regeneration and recovery, especially in cases of mild injury.26

ESSENTIALS OF ASSESSMENT

Review of systems

  • Review sense of smell, visual acuity, visual fields, eyelid opening/closing, double vision, dry/painful eyes, facial sensation, chewing/mastication, taste, hearing, vertigo/balance, swallowing, salivation, gag reflex, speaking/vocal quality, and neck/shoulder muscle function.
  • Inquiring about appetite and weight changes may raise suspicion for cranial nerve lesions that a cognitively impaired patient may otherwise have difficulty recognizing.
  • Patient should be queried regarding history of trauma, bleeding/drainage from the ears, fractures, and bruising patterns.8

Physical examination

The CN examination should be performed in conjunction with a thorough neurological examination to determine whether the deficit is isolated or diffuse.

See Pathophysiology section for additional information regarding clinical presentation of cranial nerve dysfunction.

Functional assessment

Cranial neuropathies can affect multiple domains of an individual’s life. The effect on vision, hearing, and balance should be evaluated by checking gait stability and ability to negotiate tight and crowded spaces. A driving assessment allows evaluation of the patients’ vision, reaction time and road safety. When a patient displays signs and symptoms of dysphagia a swallowing assessment not only serves diagnostic purposes but also helps with establishing a treatment program.  The swallowing assessment may include a clinical evaluation, video fluoroscopy and endoscopy. Finally, the impact of the neuropathy on psychological and social functioning and participation needs to be assessed.

Laboratory studies

The following may be considered depending on history and physical examination.

  • Infectious – CBC, cultures, ESR, serologies, CSF, or DNA PCR
  • Autoimmune – circulating antibodies in serum or CSF
  • Endocrine and electrolytic disorders

Imaging

  • Computed Tomography (CT) has great utility in the acute setting after significant trauma, and can assist in assessment for temporal fractures, which are associated with facial and vestibulocochlear nerve injuries.
  • Ethmoid tomography may be warranted when Battle’s Sign (mastoid ecchymosis), raccoon eyes, or other signs of basilar skull fracture are present.
  • MRI often provides visual confirmation of abnormalities in the olfactory bulbs and tracts, as well as in the inferior frontal lobes.9

MRI is the imaging modality of choice for cranial nerve pathologies. It  can diagnose mass-effect-lesions such as vascular lesions or tumors. MRI is also the best imaging modality to detect demyelinating diseases such as multiple sclerosis. Gadolinium administration allows the detection of cranial nerve enhancement which can be seen in malignancies, inflammation, demyelination, ischemia, radiation and axonal degeneration.

Supplemental assessment tools

  • Evoked potentials, such as olfactory event-related potentials, visual evoked responses, and brainstem auditory evoked potentials, provide further diagnostic information in the setting of cranial nerve associated dysfunction in smell, vision, and hearing, as well is in lower neurologically functioning individuals.8,10
  • Electroencephalography should be employed if seizures are implicated.8
  • electrodiagnostic testing with electromyography and nerve conduction studies.11
  • CSF analysis
  • Pain assessment (headaches, trigeminal neuralgia)

Early predictions of outcomes8

A patient may expect moderate recovery initially for 4-6 weeks with resolution of edema and hematoma. Significant impending neurological compromise may be heralded acutely by pupillary fixation, and axonal injury to the optic nerve has a poor prognosis for recovery, given inability of those axons to regenerate.

Environmental

Environmental barriers to function can be vastly amplified by cranial nerve dysfunction.

Social role and social support system

Community integration and employment may be jeopardized by abnormal hearing, vision or communication, and should raise concern regarding the patient’s goals and demands for livelihood and civic involvement. Increased caregiver needs may place strain on the patient’s family.

Professional Issues

Particular care and sensitivity must be undertaken to maximize quality of life following cranial nerve dysfunction, given propensity for significant functional impairment and newfound disability resulting from visual, auditory, vestibular, alimentary, or communicative deficits. Concomitantly, safety must be prioritized concerning impacted functional mobility, self-care, and nutrition, with paramount attention to prevention of further disability.

REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

There are few evidence-based guidelines regarding treatment of cranial nerve injuries in TBI. However, thorough examination of cranial nerves is essential and re-examination as the patient recovers may reveal previously undiagnosed deficits. Proper swallowing evaluation prior to allowing oral intake is essential.4 Avoiding neurotoxic substances, maintaining normal electrolyte and blood glucose levels is encouraged to promote neural recovery. In cases of a delayed CN VII palsy, treatment with high dose steroids with a slow taper minimizes the risk of a rebound paralysis.

Treatments based on deficits:

Coordination of care

The interdisciplinary approach to treat TBI is well established. Specifically, for cranial nerve lesions, vision specialists, audiologists and speech therapists can assist in evaluation and treatment. Occasional surgical referral may be indicated.

Patient & family education for select cranial nerve injuries

  • For olfactory (CN I) injury: Patients may be unable to detect smoke, gas, harmful chemicals or spoiled foods, which may cause harm. Use reminders and fixed schedules to improve hygiene, child or pet care when olfaction is impaired. Dietary changes may be required to optimize nutrition in patients with dysgeusia, including use of spices and textures to improve the appeal of foods.
  • For facial nerve (CN VII) injury: To protect the cornea, use lubricating eye drops regularly, as often as every 30-60 minutes. Additional options to reduce dryness are using an eye bubble or taping the eyelid.29
  • For glossopharyngeal (CN IX) or vagus (CN X) nerve injury: Explain the rationale for specific diets to families of patients with dysphagia, as this is often a source of confusion. Families may feel certain restrictions are preventing patient progress, and families often provide or prepare patient’s food.19
  • For spinal accessory nerve (CN XI) injury: Physical therapy is important for reducing chronic pain from displacement of the humeral head, a consequence of loss of sternocleidomastoid and trapezius muscle function.29

Emerging/unique Interventions

Treatment outcomes of cranial nerve injuries are most commonly assessed on a functional basis. Most cranial nerve deficits have been found to improve over the course of a few months to a year. Persistent or unexpected deficits may be evaluated with electrodiagnostic, vestibular and neuro-ophthalmologic studies described above.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Thorough examination of cranial nerves is essential on initial evaluation and throughout the course of TBI rehabilitation. Visual, hearing, vestibular and swallowing difficulties may be related to cortical or cranial nerve deficits and impede progress in the rehabilitation setting. Recognizing and addressing these deficits may expedite functional gains in a broad range of modalities. Providing reassurance and compensatory strategies may reduce the psychological effects of the deficits.

CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Cutting edge concepts and practice

In 2015, Morries, Cassano, and Henderson published Treatments for traumatic brain injury with emphasis on transcranial near-infrared laser phototherapy, which included a retrospective case series involving ten patients with chronic TBI.22 This study used high-power near-infrared (NIR) light laser phototherapy, and demonstrated improved symptoms of headaches, sleep disturbance, cognition, mood dysregulation, and anxiety. The proposed mechanism underlying light therapy hypothesizes that NIR light reaches cytochrome c oxidase in mitochondria, which activates a signaling cascade that ultimately stimulates the production of inflammatory mediators and growth factors.22

In 2017, Kashkouli et al. published Traumatic optic neuropathy treatment trial (TONTT): open label, phase 3, multicenter, semi-experimental trial.21 This study included 100 patients with traumatic optic neuropathy (TON) and showed that best corrected visual acuity was significantly improved in patients that received either IV erythropoietin, IV steroid, or observation. No significant differences were found between the treatment and control groups. Given the lack of side effects and relative safety of EPO, the authors suggested EPO be recommended as an option in treatment of patients with TON, as well as further research on various EPO doses and dosing schedules.

GAPS IN THE EVIDENCE-BASED KNOWLEDGE

The exact incidence of cranial nerve injuries is unknown. On physical exam of patients with brain injury, patients with altered cognition or consciousness it is difficult to elicit a full and accurate CN exam. Currently there are numerous treatment options for CN injures but no standardized treatment guidelines exist.

REFERENCES

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2. Zollman FS. Manual of Traumatic Brain Injury Management. New York, NY: Demos Medical Pub; 2011. Available at: http://site.ebrary.com/id/10464821. Accessed February 10, 2014.

3. Jin H, Wang S, Hou L, et al. Clinical treatment of traumatic brain injury complicated by cranial nerve injury. Injury. 2010;41(9):918-923. doi:10.1016/j.injury.2010.03.007.

4. Zasler ND, Katz DI, Zafonte RD. Brain Injury Medicine Principles and Practice. New York, NY: Demos Medical; 2013. Available at: http://site.ebrary.com/id/10629311. Accessed February 10, 2014.

5. Coello AF, Canals AG, Gonzalez JM, Martín JJA. Cranial nerve injury after minor head trauma. J Neurosurg. 2010;113(3):547-555.

6. Jannetta PJ. Neurovascular compression in cranial nerve and systemic disease. Ann Surg.1980;192(4):518-525.

7. Henderson CA. The Cranial Nerves. Hattiesburg, Miss.: Medtutor.com; 2010.

8. Horn LJ, Zasler ND. Medical Rehabilitation of Traumatic Brain Injury. Philadelphia, PA; St. Louis, MO: Hanley & Belfus; Mosby [distributor]; 1996.

9. Yousem DM, Geckle RJ, Bilker WB, McKeown DA, Doty RL. Posttraumatic olfactory dysfunction: MR and clinical evaluation. AJNR Am J Neuroradiol.1996;17(6):1171-1179.

10. Geisler MW, Schlotfeldt CR, Middleton CB, Dulay MF, Murphy C. Traumatic brain injury assessed with olfactory event-related brain potentials. J Clin Neurophysiol.1999;16(1):77-86.

11. Electrodiagnostic Medicine. 2nd ed. Philadelphia, PA: Hanley & Belfus; 2002.

12. Ikeda K, Sakurada T, Takasaka T, Okitsu T, Yoshida S. Anosmia following head trauma: preliminary study of steroid treatment. Tohoku J Exp Med.1995;177(4):343-351.

13. Miwa T, Furukawa M, Tsukatani T, Costanzo RM, DiNardo LJ, Reiter ER. Impact of olfactory impairment on quality of life and disability. Arch Otolaryngol Head Neck Surg. 2001;127(5):497-503.

14. Clinical Measurement of Taste and Smell. New York, NY: Macmillan; 1986.

15. Kerkhoff G. Rehabilitation of visuospatial cognition and visual exploration in neglect: a cross-over study. Restor Neurol Neurosci.1998;12(1):27-40.

16. Lagrèze WA. Neuro-ophthalmology of trauma. Curr Opin Ophthalmol. 1998;9(6):33-39.

17. Vestibular Rehabilitation. 3rd ed. Philadelphia, PA: F.A. Davis; 2007.

18. Krebs DE, Gill-Body KM, Parker SW, Ramirez JV, Wernick-Robinson M. Vestibular rehabilitation: useful but not universally so. Otolaryngol–Head Neck Surg.2003;128(2):240-250. doi:10.1067/mhn.2003.72.

19. Logemann JA. Evaluation and Treatment of Swallowing Disorders. 2nd ed. Austin, TX: PRO-ED; 1998.

20. Backonja M-M. Use of anticonvulsants for treatment of neuropathic pain. Neurology. 2002;59(5 Suppl 2):S14-17.

21. Kashkouli, M. B., Yousefi, S., Nojomi, M., Sanjari, M. S., Pakdel, F., Entezari, M., . . . Bagheri, M. (2017). Traumatic optic neuropathy treatment trial (TONTT): Open label, phase 3, multicenter, semi-experimental trial. Graefes Archive for Clinical and Experimental Ophthalmology, 256(1), 209-218. doi:10.1007/s00417-017-3816-5.

22. Henderson, T. A., Morries, L., & Cassano, P. (2015). Treatments for traumatic brain injury with emphasis on transcranial near-infrared laser phototherapy. Neuropsychiatric Disease and Treatment, 2159. doi:10.2147/ndt.s65809.

23. Dubal, P. M., Svider, P. F., Gupta, A., Eloy, J. A., & Liu, J. K. (2015). Chapter 30: Injuries of the Cranial Nerves. In Nerves and Nerve Injuries (Vol. 2, pp. 451–468). Elsevier. doi: https://doi.org/10.1016/C2014-0-03700-8.

24. Carroll, C., & Campbell, W. (2009). Multiple Cranial Neuropathies. Seminars in Neurology, 29(01), 053–065. doi: 10.1055/s-0028-1124023.

25. Wakerley, B. R., & Yuki, N. (2015). Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome. Journal of Neurology262(9), 2001–2012. doi: 10.1007/s00415-015-7678-7.

26. Kobayashi, M., & Costanzo, R. M. (2009). Olfactory Nerve Recovery Following Mild and Severe Injury and the Efficacy of Dexamethasone Treatment. Chemical Senses, 34(7), 573–580. doi: 10.1093/chemse/bjp038.

27. Bajwa, Z. H., Ho, C. C., & Khan, S. A. (2019, March 1). Trigeminal neuralgia. Retrieved November 10, 2019, from https://www.uptodate.com.

28. Singer, R. J., Ogilvy, C. S., & Rordorf, G. (2019, October 10). Brain arteriovenous malformations. Retrieved November 10, 2019, from https://www.uptodate.com.

29. Adunka OF, Buchman CA, eds. Rehabilitation of Cranial Nerve Deficits. Otology, Neurotology, and Lateral Skull Base Surgery. 2011. doi:10.1055/b-0034-83649.

Original Version of the Topic

Blessen C. Eapen, MD, Thomas Shaw, Jeremy Gallant, MD, Carlos A. Jaramillo, MD, PHD. Cranial nerve, visual and hearing dysfunction in disorders of the CNS. 9/20/2014

Author Disclosures

Jeannie Zhang, MD
Nothing to Disclose

Blessen C. Eapen, MD
Nothing to Disclose