return to: Vocal Fold Paralysis (Vocal Cord Paralysis) Etiologies

Vocal Cord Paralysis Evaluation and Etiology

see also: Collett-Sicard Syndrome

Jugular Foramen Syndrome

Affected Cranial Nerves

Collet-Sicard syndrome IX,X,XI,XII
Villaret syndrome IX,X,XI,XII sympathetic chain (Horner's syndrome)
Vernet syndrome IX,X,XI
Jackson syndrome X,XI,XII
Schmidt syndrome X,XI
Tapia syndrome X,XII

GENERAL

Vernet's Syndrome is characterized by a constellation of unilateral cranial nerve palsies due the compression or narrowing of the jugular foramen involving the 9th, 10th, and 11th cranial nerves (nerves that travel within the jugular foramen). A diversity of lesions have been shown to be involve in the jugular foramen, such as tumors, vascular lesions, infections, and trauma

SYMPTOMS

Symptoms of this syndrome are consequences of paresis of the above mentioned cranial nerves (9, 10, 11).

- dysphonia/hoarseness

- soft palate dropping

- deviation of the uvula towards the normal side

- dysphagia

- loss of sensory function from the posterior 1/3 of the tongue

- decrease in the parotid gland secretion

- loss of gag reflex

- sternocleidomastoid and trapezius muscles paresis.

CAUSES

There have been case reports of a variety of causes of Vernet's syndrome. Some of the causes of Vernet's syndrome include:

1. Primary tumors

       - Glomus jugulare tumors (most frequently) 

      - Meningioma 

      - Vestibular schwannoma

      - Cerebellopontine angle metastases

2. Inflammation

       - meningitis

       - malignant otitis externa

       - sarcoidosis

       - Guillain-Barre syndrome

3. Trauma

4. Cholesteatoma (very rare)

5. Narrowing of jugular foramen due to bone disease

6. Varizella-zoster virus

7. Giant cell arteritis

8. Internal jugular vein thrombosis

9. Post carotid endarterctomy

10. Systemic erythematous Lupus

11. Large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media

12. Metastatic cancer

DIAGNOSIS

Diagnosis is usually made on clinical exam and imaging with evidence of jugular foramen compression/narrowing

MANAGEMENT

Treat the cause

REFERENCES

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Ha SW, Kim JK, Kang SJ, Kim MJ, Yoo BG, Kim KS, et al. A case of Vernet’s syndrome caused by non-specific focal inflammation of the neck. J Korean Soc Clin Neurophysiol. 2007;9:81–84.

Kim HS, Ko K. Penetrating trauma of the posterior fossa resulting in Vernet’s syndrome and internuclear ophthalmoplegia. J Trauma. 1996 Apr;40(4):647-9. PubMed PMID: 8614050.

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Cherin P, De Gennes C, Bletry O, Lamas A, Launay M, Dubs A, Godeau P. Ischemic Vernet’s syndrome in giant cell arteritis: first two cases. Am J Med. 1992 Sep;93(3):349-52. PubMed PMID: 1524092.

Shima K, Iwasa K, Yoshita M, Yamada M. Vernet’s syndrome induced by internal jugular vein thrombosis. J Neurol Neurosurg Psychiatry. 2016 Nov;87(11):1252-1253. doi: 10.1136/jnnp-2015-311665. PubMed PMID: 26354943.

Tamaki T, Node Y, Saitoum N, Saigusa H, Yamazaki M, Morita A. Vernet’s syndrome after carotid endarterectomy. Perspect Vasc Surg Endovasc Ther. 2013 Dec;25(3-4):65-8. doi: 10.1177/1531003514525476. PubMed PMID: 24625858.

Amano M, Ishikawa E, Kujiraoka Y, Watanabe S, Ashizawa K, Oguni E, Saito A, Nakai Y, Ikeda H, Abe T, Uekusa Y, Matsumura A. Vernet’s syndrome caused by large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media–case report. Neurol Med Chir (Tokyo). 2010 Jan;50(1):45-8. PubMed PMID: 20098025.

Leache Pueyo JJ, Campos del Alamo MA, Gil Paraíso P, Ortiz García A. [Vernet’s syndrome as an early manifestation of systemic erythematous lupus]. An Otorrinolaringol Ibero Am. 1997;24(2):135-41. Spanish. PubMed PMID: 9199109.