Acute onset of facial nerve palsy associated with Lyme disease in a 6 year-old child. New York, NY: McGraw-Hill, Companies 1997:1376 (For more information in this disorder, choose вЂњRamsay-Hunt syndromeвЂќ as your search term on the Rare Disease Database.)Д«ennett JC, Plum F., eds. Affected individuals may experience hearing loss or intense pain by the ear. In cases of Ramsay-Hunt syndrome, previously dormant varicella-zoster virus is reactivated and spreads to affect the facial nerves. Ramsay-Hunt syndrome is caused by the varicella zoster virus, the same virus that causes chickenpox in children and shingles (herpes zoster) in adults. Ramsay-Hunt syndrome (RHS), also known as herpes zoster oticus, is a rare neurological disorder characterized by paralysis of certain facial nerves (facial palsy), a rash affecting the ear or mouth, and ear abnormalities such as ringing in the ears (tinnitus). (For more information on this disorder, choose вЂњMyasthenia GravisвЂќ as your search term in the Rare Disease Database.) Eventually muscle weakness extends into the arms and legs resulting in generalized physical weakness. When these symptoms occur on one side (unilateral), the disorder may resemble BellвЂ™s Palsy. The early symptoms of this disorder may include difficulties in speaking, chewing, and/or swallowing the eyelids may droop and double vision may occur.

Initially the muscles of the mouth, lips, tongue, and voice box are the most affected. Myasthenia gravis is a chronic neuromuscular disease characterized by muscle weakness. (For more information in this disorder, choose вЂњAcoustic NeuromaвЂќ as your search term on the Rare Disease Database.) The expansion of the tumor into different areas may result in impaired ability to coordinate movement of the legs and arms (ataxia), numbness in the mouth, slurred speech (dysarthria), and/or hoarseness. An associated compression of the facial nerve (7th cranial nerve) may produce muscle weakness, pressure on the trigeminal nerve (5th cranial nerve) may lead to facial numbness. Pressure on this nerve results in the early symptoms of acoustic neuroma, a ringing sound in the ear (tinnitus), and/or hearing loss may occur. This nerve lies within the internal ear canal. Comparisons may be useful for a differential diagnosis:ДЄcoustic neuroma is a benign tumor of the 8th cranial nerve. Symptoms of the following disorders can be similar to those of BellвЂ™s palsy. Crocodile tears (tears not brought on by emotion) associated with facial muscular contractions occasionally develop in the aftermath of BellвЂ™s palsy. If, as recovery proceeds, the nerve fibers regrow to muscles other than the ones they originally innervated, there may be voluntary muscle movements of the face accompanied by involuntary contractions of other facial muscles (synkinesia). The affected muscles usually regain their original function within one to two months. If facial paralysis is only partial, complete recovery can be expected. Recovery from BellвЂ™s palsy depends on the extent and severity of damage to the seventh cranial nerve. In some cases, an affected individualвЂ™s response to a pinprick behind the ear also is decreased. Some people with BellвЂ™s palsy experience a loss of the sense of taste on one side of the mouth, drooling, and an increased sensitivity to sound (hyperacusis) on the affected side of the head. If the compressed region of the facial nerve is next to the branching of other nerves, there may be a decrease in saliva and/or tear production. People with BellвЂ™s palsy may not have a corneal reflex the eye on the affected side does not close when the cornea is touched. This may result in the inability to close the eye on the affected side. Often the opening between the upper and lower eyelids (palpebral fissure) is enlarged and remains open during sleep. In severe cases of BellвЂ™s palsy, the facial muscles on the affected side are completely paralyzed, causing that side of the face to become smooth, expressionless, and immobile. Occasionally, only the upper or lower half the face is affected. Approximately 80 percent of cases are resolved within three months. Most cases resolve with two to three weeks. In most cases of BellвЂ™s palsy, only facial muscle weakness occurs and the facial paralysis is temporary. The symptoms may begin suddenly and progress rapidly over several hours, and sometimes follow exposure to cold or a draft. The early symptoms of BellвЂ™s palsy may include a slight fever, pain behind the ear, a stiff neck, and weakness and/or stiffness on one side of the face.

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