|Year : 2012 | Volume
| Issue : 2 | Page : 95-97
Bilateral facial (VII) and vestibulocochlear (VIII) nerves palsy: What is the cause?
Vanita Sarin, Baldev Singh, Vanika Anand, Jaskaran Singh
Department of Otorhinolaryngology and Head and Neck Surgery, SGRD IMSR, Amritsar, India
|Date of Web Publication||6-Sep-2012|
Department of Otorhinolaryngology and Head and Neck Surgery, SGRD IMSR, Amritsar
Source of Support: None, Conflict of Interest: None
Bilateral facial (VII th) and vestibulocochlear (VIII th) nerves involvement is a rare presentation and often indicates a severe underlying medical condition. The differential diagnosis of its causes are extensive and so it presents as a diagnostic challenge. Both, physicians and ENT surgeons should be aware of these various diagnostic possibilities to avoid life long complications. We present here a case of 37-year-old female with sequential bilateral facial nerve and vestibulocochlear nerve involvement, which was successfully managed.
Keywords: Diagnostic challenge, Differential diagnosis, Facial nerve, Rare, Vestibulocochlear nerve
|How to cite this article:|
Sarin V, Singh B, Anand V, Singh J. Bilateral facial (VII) and vestibulocochlear (VIII) nerves palsy: What is the cause?. Indian J Otol 2012;18:95-7
|How to cite this URL:|
Sarin V, Singh B, Anand V, Singh J. Bilateral facial (VII) and vestibulocochlear (VIII) nerves palsy: What is the cause?. Indian J Otol [serial online] 2012 [cited 2019 Apr 24];18:95-7. Available from: http://www.indianjotol.org/text.asp?2012/18/2/95/100735
| Introduction|| |
Bilateral sequential facial nerve paralysis with vestibulocochlear involvement is a very rare clinical entity. Though in literature there are many articles of bilateral facial nerve palsy but simultaneous involvement of both the VII th and VIII th cranial nerves has rarely been reported. Adam found only three bilateral cases in a consecutive series of 1000 patients with Bell's palsy. Simultaneous onset is defined as the involvement of the opposite side within 30 days of onset of the first side. It is most often a special finding in a symptom complex of a systemic disease occurring in 0.3% to 2% of facial nerve palsy cases. 
We report a case of 37-year-old female who presented to our department with sequential bilateral facial (VII th ) and vestibulocochlear (VIII th ) nerves involvement in which unilateral VII th and VIII th nerve was followed by contralateral VII th and VII th nerve involvement in the next 5 days. We also discuss here complete evaluation of the underlying cause and specific management as relevant.
| Case Report|| |
A 37-year-old female, housewife presented with a 5 days history of high grade fever (unrecorded) followed by right sided facial nerve weakness with hearing loss and vertigo. Seeing her general condition, she was admitted and a day after her admission, she developed facial weakness with hearing loss of the left side also. There was no history of travelling, adventure or exposure to ticks in the last few days. There was no significant past history of any disease or medication. The patient was a non-smoker and non-alcoholic.
A detailed otorhinolaryngological examination revealed bilateral lower motor neuron type of facial nerve palsy (House and Breekman grade VI) with Rinne's negative for all these frequencies (256,512,1024 Hz). There was rotator nystagmus but Hallpike's maneuver was negative and the cerebellar functions were normal. Rest of the cranial nerves were normal.
Blood tests for full counts, electrolytes, serum angiotensin converting enzyme (ACE) were all within normal limits. Vasculitis screening tests, VDRL and blood cultures were negative. Similarly, Monospot test for infectious mononucleosis, antinuclear antibody (ANA), antinuclear cytoplasmic antibody (ANCA) and tests for HIV, HCV and HBsAg were negative.
Chest X-ray and lumbur puncture results were normal. Pure tone audiometry revealed bilateral severe Senseri Neural Hearing Loss (SNHL) in both ears with bilateral absence of stapedial reflexes on tympanometry.
MRI imaging scan and computed tomography (CT) scan of the head and cerebellopontine angle was normal. Thus, having excluded all the possible causes of this disorder after extensive evaluation, we could assure that the most likely cause of VII th and VIII th cranial nerve palsy was viral in origin as the symptoms were preceded with high grade fever, sore throat and malaise.
She was commenced with high doses of steroids (solumedrol 1gm I/V daily) which were tapered off once the symptoms started improving and were completely stopped by the end of sixth week. Antiviral treatment (with valcivir 1 gm tds) was given for 10 days and along with it appropriate eye protection and symptomatic treatment for vertigo was given. Continuous facial massage and facial exercises with alternate day bilateral facial nerve stimulation were also part of the treatment package.
Her signs and symptoms markedly improved in the first week of starting medication beginning with the right side first and on the 16 th day (day of discharge) her right facial nerve functions had completely recovered with partial recovery of left side. She had mild sensorineural hearing loss with near to complete resolution of vertigo.
Advice was given to continue steroid therapy for 6 weeks and eye protection till then. On her consecutive visits, improvement was seen and by the end of 6 weeks her bilateral VII th and VIII th nerves had completely recovered with mild sensorineural hearing loss in both ears.
| Discussion|| |
From the first time that bilateral facial nerve palsy was reported in 1989, till date none of the reports have claimed simultaneous bilateral facial, as well as vestibulocochlear nerves involvement.  Bilateral multiple cranial nerves involvement, especially VII th and VIII th is often due to systemic causes in comparison to unilateral paralysis, and a wide differential diagnosis must be considered. The diseases often associated with bilateral peripheral paralysis are neurosarcoidosis, multiple idiopathic cranial neuropathies, brain stem encephalitis, benign intracranial hypertension, Guillain-Barre syndrome, bacterial meningitis, leukemia, syphilis, lyme disease, HIV infection and orofacial granulomatosis ( Melkersson-Rosenthal syndrome More Details). ,,, Thus, it should be carefully investigated before establishing any diagnosis of the underlying cause. 
The most common cause of bilateral VII th nerve palsy is Lyme's disease, caused by spirochete Borrelia burgdoferi whose carrier is a common tick.  Diagnosis is serological and IgM antibodies increased in second week and tend to decrease with treatment. In our patient there was no recent history of contact with ticks or travel to endemic areas so diagnosis was less likely.
Guillain-Barre syndrome or ascending inflammatory demyelinating polyneuropathy (AIDP) presents as progressive development of palsy of the voluntary muscles including the face with multiple cranial nerves involvement (VII th , IX th and X th ). In 27-50% of the cases the seventh nerve is involved.  Fifty percent of the patients with facial nerve palsy have bilateral involvement. In our patient her voluntary muscle functions including all the tendon reflexes were normal, thus excluding the possibility of this disease.
Sarcoidosis,  systemic lupus erythematosus (SLE),  polyarthritis nodosa (PAN) were unlikely because of normal ESR and negative autoantibodies screening.
The patient's presentation with normal MRI imaging made intracranial leukemia, intracranial lymphoma and benign intracranial hypertension unlikely.
Other causes listed in literature include amyloidosis, syphilis, poliomyelitis, tuberculosis (TB) and porphyria  but in view of their rarity in our patient's circumstances these possibilities were not explored further.
Traumatic skull base fractures and cerebellopontine angle tumors were excluded by CT and MRI of the brain. According to literature, 40% of Ebstein barr virus (EBV) infections associated with facial nerve palsy cases are bilateral.  But, this disease was also excluded by monospot test. Differential diagnosis mandating further investigation is very important because the treatment and diagnosis depends on the cause.
After keeping all the above diseases into consideration, diagnosis of virus as a causative organism was taken into view by criteria of exclusion.
The treatment plan was thus started in the form of heavy doses of steroids, antiviral and symptomatic treatment for vertigo in the form of vestibular sedatives. Proper eye care, facial massage and bilateral facial nerve stimulation were also administered. As mentioned earlier, patient showed marked improvement after first week of administered therapy. The sequential audiogram showed marked improvement in hearing from severe to moderately severe to mild SNHL over a period of 3-4 weeks. The bilateral stapedial reflexes appeared. Vestibular sedatives were given to combat vertigo and it also showed remarkable improvement 3-4 weeks following treatment.
So, in our case of bilateral VII and VIII cranial nerve involvement presented a diagnostic dilemma and definite etiology could not be ascertained. Recovery is similar to that in unilateral palsy, although one side of face may recover earlier than the other.
| Conclusion|| |
Simultaneous presentation of bilateral VII and VIII nerve palsy is very rare and to our knowledge, none have been reported yet in literature. This condition can be life threatening and therefore both the otorhinolaryngologists and the physicians should be aware of the diagnostic possibilities that cause this rare condition. Early diagnosis is critical as early treatment can reduce the morbidity associated with the disease as seen in this case.
| Summary|| |
Bilateral facial (VII) and vestibulocochlear (VIII) nerves palsy is a rare condition. A comprehensive diagnostic work up can provide the clue to the underlying cause.
| References|| |
|1.||Stahl N, Ferit T. Reccurent bilateral peripheral facial palsy. J Laryngol Otol 1989;103:117-9. |
|2.||Serrano P, Hernandez N, Arroyo JA, Llobet JM. Bilateral Bell palsy and acute HIV type 1 infection:report of 2 cases and review. Clin Infect Dis 2007;44:e57-61. |
|3.||Rowlands S, Hooper R, Hughes R, Burney P. The epidemiology and treatment of Bell's palsy in the UK. Eur J Neurol 2002;9:63-7. |
|4.||Keane JR. Bilateral seventh nerve palsy: analysis of 43 cases and review of the literature. Neurology 1994,44:1198-2002. |
|5.||May M, Klein SR. Differential diagnosis of facial nerve palsy. Otolaryngol Clin North Am 1991,24:613-45. |
|6.||Serrano P, Hernandez N, Arroyo JA, de Llobet JM, Domingo P. Bilateral Bell palsy and Acute HIV Type 1 infection: Report of 2 cases and review. Clin Infect Dis 2007:44:e57-61. |
|7.||Price T, File DG. Bilateral simultaneous facial nerve palsy. J Laryngol Otol 2002;116:46-8. |
|8.||Clark JR, Clarson RD, Pachner AR. Facial paralysis in Lyme disease. Laryngoscope 1985;95:1341-5. |
|9.||May M. The Facial Nerve. New York: Thieme Inc; 1986. p. 181. Chapter 9 |
|10.||James DG. Differential diagnosis of facial nerve palsy. Sarcoidosis Vasc Diffuse Lung Dis 1997;14:115-20. |
|11.||Blaustein DA, Blaustein SA. Antinuclear antibody negative systemic lupus erythematosus presenting as bilateral facial nerve paralysis. J Rheumatol 1998;25:798-800. |
|12.||Price T. Bilateral simultaneous facial nerve palsy. J Layngol Otol 2002;116:46-8. |
|13.||Terada K, Niizuma T, Kosaka Y. Bilateral facial nerve palsy associated with Epstein-Barr virus infection with a review of the literature. Scan J Infect Dis 2004;36:75-7. |