TABLE 2.

Topical Diagnoses of Lesions of the Facial Nerve at Various Levels

Level Supranuclear

Signs Tone and upper face intact, loss of volitional movement with intact spontaneous expression, slurred speech (tongue weakness), hemiparesis on side of facial involvement. Paresis of upper extremity begins with involvement of thumb, finger, and hand movement. Increased salivary flow, spontaneous facial movement impaired, volitional facial movement intact, masked face of parkinsonism or dystonia, progressive hemifacial spasm Oculomotor paresis on opposite side of lower facial paresis and hemiparesis Involvement of cranial nerves VII and VI on side of facial paresis; associated ataxia, cerebellovestibular signs, and contralateral hemiparesis Cranial nerves VII and VI palsy noted from time of birth with or without other congenital anomalies. Facial motor involvement usually incomplete with sparing of corner of mouth or lower lip common. Another type of presentation is involvement of the lower lip with complete or partial sparing of upper face. Anomalies of the pinna, canal, or mandible associated with facial palsy indicate developmental defect of facial nerve.

Probable Diagnosis

Cortex and internal capsule

Lesion of motor cortex or internal capsule on opposite side of facial involvement

Extrapyramidal

Tumor or vascular lesion of basal ganglia: parkinsonism, Meige's syndrome (cervical facial dystonia) Syndrome of Weber Involvement of pons at level of VII and VI nuclei by pontine glioma, multiple sclerosis, infection, infarction

Midbrain

Pontine nucleus

Developmental facial palsy (noted at birth), oculofacial syndrome, Mbius' syndrome, thalidomide toxicity. Nondevelopmental facial or abducens nerve anomalies are most often due to infranuclear lesions.

Infranuclear Intracranial cerebellopontine angle Impairment of hearing, (especially discrimination out of proportion to pure tone scores), possible ataxia, Acoustic schwannoma abnormalities of tearing or taste, stapes reflex decay, decreased corneal sensation, facial motor deficit (late sign)

Level

Signs Abnormalities in trigeminal, acoustic vestibular, and facial nerve function, starting with facial pain or numbness. Lesion noted on CT (enhancement with contrast) may show evidence of calcification.

Probable Diagnosis

Meningioma

Abnormalities of facial and acoustic Cholesteatoma or facial vestibular nerve function, may start with schwannoma arising in facial twitching. Erosion or lytic area temporal bone evident on CT of temporal bone. Abnormalities of cranial nerves VII, VIII, IX, X, XI, and XII; pulsatile Glomus jugulare tumor tinnitus and purplered pulsating mass bulging through the tympanic membrane Abnormalities of abducens nerve in addition to above Glomus jugulare tumor extending to petrous apex to involve middle fossa

Skull base

Conductive or sensorineural hearing loss, acute or recurrent facial palsy, Osteopetrosis positive family history, abnormalities of bone density on CT Carcinomatous meningitis, leukemia, Landry-GuillainBarr, mononucleosis, diphtheria, tuberculosis, sarcoidosis, malignant external otitis

Multiple cranial nerve involvement in rapid succession

Transtemporal Bone Ecchymosis around pinna and mastoid prominence (Battle's sign), hematotympanum with sensorineural hearing loss (tuning fork lateralizes to Internal auditory normal side), vertigo, nystagmus (fast Temporal bone fracture canal and component away from involved side), (transverse, longitudinal, or labyrinthine segment sudden complete facial paralysis after combination) of facial nerve head trauma (usually associated with basilar skull fracture), loss of consciousness, and cerebrospinal fluid leak. Transection of facial nerve is more likely with this injury compared with

Level

Signs longitudinal fracture.

Probable Diagnosis

Dry eye, decreased taste and salivation. Erosion of geniculate ganglion area or Geniculate ganglion middle fossa is demonstrated by pluridirectional tomography and CT of temporal bone.

Schwannoma, meningioma, cholesteatoma, hemangioma, arteriovenous malformation

Ear pain, vesicles on pinna, dry eye, decreased taste and salivary flow, sensorineural hearing loss, nystagmus, Herpes zoster cephalicus vertigo, red chorda tympani nerve. (Ramsey-Hunt syndrome) Facial palsy may be complete or incomplete, or may progress to complete over 14 days. Same as above with vesicles; no other cause evident. Facial palsy may be Idiopathic (Bell's) palsy (viralcomplete or incomplete, or may progress inflammatory immune disorder) to complete over 10 days. Same as above but no recovery in 6 months Ecchymosis around pinna and mastoid (Battle's sign), hematotympanum, conductive hearing loss (tuning fork lateralizes to involved ear), no vestibular involvement unless stapes subluxed into vestibule (causes fluctuating sensorineural hearing loss and vertigo with nystagmus) Decreased taste and salivation, loss of stapes reflex and symmetrical tearing, sudden-onset facial palsy, which may be complete or incomplete or may progress to complete Pain, vesicles, red chorda tympani Pain without vesicles, red chorda tympani Red, bulging tympanic membrane, conductive hearing loss, usually history of upper respiratory infection. Lower face may be involved more than upper. Pulsatile tinnitus, purple-red pulsatile Herpes zoster cephalicus Bell's palsy Tumor Longitudinal fracture of temporal bone; may be proximal or at geniculate ganglion (tearing symmetrical; tear test valid only in acute injury)

Tympanomastoid

Acute suppurative otitis media Glomus tympanicum or

Level

Signs Recurrent facial paralysis, positive family history, facial edema, fissured tongue; may present with simultaneous bilateral facial paralysis Incomplete facial nerve paresis; hearing balance, tearing, stapes reflex, taste, salivary flow spared Uveitis, salivary gland enlargement, fever

Probable Diagnosis

mass noted through tympanic membrane jugulare Melkersson-Rosenthal syndrome Penetrating wound of face; sequelae of parotid surgery; malignancy of parotid, tonsil, or oronaso-pharynx, rarely with benign lesion of parotid gland compressing facial nerve Sarcoidosis (Heerfordt's syndrome), lymphoma Mbius' syndrome Landry-Guillain-Barr syndrome, sarcoidosis, mononucleosis, leukemia, idiopathic (Bell's) palsy

Extracranial

Sites Variable

Bilateral facial paralysis from birth Bilateral facial paralysis acquired

Facial paralysis, especially simultaneous bilateral facial paralysis with ascending areflexic quadriparesis; sensory changes Landry-Guillain-Barr usually mild, with spinal fluid typically syndrome showing albuminocytologic dissociation (elevated protein without cells) Deficits of cranial nerves VI and VII or VII, VI, and III, possibly in association with marked increase in jaw jerk or gag reflex Carcinoma of nasopharynx, metastatic carcinoma from breast, ovary, prostate; meningitis, leukemia, diabetes mellitus

Inappropriate or exaggerated laughing or Demyelinating, degenerative, or Pseudobulbar Palsy crying; may be associated with marked vascular process involving increase in jaw jerk or gag reflex bilateral corticobulbar pathways CT, computed tomography

CORTEX AND SUPRANUCLEAR PATHWAY The voluntary responses of facial muscles, such as smiling or grimacing on command, are dependent on discharges from the facial motor area situated in the precentral gyrus of the frontal

cerebral cortex. The facial motor areas are represented with the forehead uppermost and the eyelids, midface, nose, and lips located sequentially below (Fig. 3). Supranuclear motor neurons from the cortical face area are carried as fascicles of the corticobulbar tract to the genu of the internal capsule, then via the cerebral peduncles to the lower pons or upper medulla. The portion of the facial nucleus that supplies the muscles of the upper face (frontalis, orbicularis oculi, and corrugator muscles) receives corticobulbar fibers from both right and left precentral motor cortices, but the supranuclear tracts innervating the lower face are crossed only. For this reason, the muscles that raise and wrinkle the forehead and close both eyes are bilaterally innervated. Thus, a unilateral lesion in the cortex or supranuclear pathways spares eyelid closure and forehead movement but results in paralysis of the contralateral lower face. This dissociation is characteristic of supranuclear lesions. Because the supranuclear pathways may descend to the ventromedial medulla before decussating to innervate the facial nucleus, low brainstem lesions may produce contralateral lower facial weakness.6 However, it is also possible to show upper facial sparing with lesions of the pontine facial nucleus, with selective defects within the temporal bone, or even with an injury to nerve rootlets within the parotid gland or facial musculature. This phenomenon is related to the general tendency for facial nerve function to be spatially dispersed, not only in its cortical distribution but also within the pontine nucleus and in the peripheral nerve. Fig. 3 Organization of facial motor function. Note cortical distribution, pontine nuclear connections, and relationships to other cranial nerves on ventral view of brain stem.

Because preservation of forehead function is insufficient evidence of a cortical lesion, other neurologic signs should be sought. A cortical lesion that produces a contralateral lower facial palsy is usually associated with a motor deficit of the tongue and with weakness of the thumb, fingers, and hand on the same side as the hemifacial weakness, with lesser involvement of the leg. The cortical motor areas of the face, tongue, thumb, fingers, hand, and upper extremities lie near each other in the precentral territory nourished by the rolandic branch of the middle cerebral artery. Patients with such cortical lesions are unable to voluntarily smile, but facial expression is appropriate in response to an amusing story. Similar clinical findings may occur with lesions that involve the descending corticobulbar and corticospinal tracts.7 Although facial muscle tone is not significantly impaired with a supranuclear lesion, slight flattening of the nasolabial fold and drooping in the corner of the mouth may be detected contralateral to the cortical lesion.

EXTRAPYRAMIDAL SYSTEM The extrapyramidal system (Fig. 4) consists of the basal ganglia and the descending motor projections other than the fibers of the corticospinal (pyramidal) tract. Anatomically, this system appears to be a diffuse, multisynaptic network that interconnects extensively with centrencephalic and brain stem structures. These pathways are not as well clarified as those of the primary (pyramidal) motor tracts. The extrapyramidal system is concerned with automatic and emotional facial language. The dull, expressionless face of parkinsonism is a well-known result of extrapyramidal pathway disease, whereas the spontaneous facial dystonia of Meige's syndrome is characterized by unilateral or, more often, bilateral blepharospasm associated with dystonic movements of the mouth and other lower facial muscles. Progression of this latter disorder may lead to chaotic contractions of the tongue and cervical muscles. Fig. 4 Extrapyramidal connections for supranuclear facial pathways.

PONS The facial motor nucleus contains about 7,000 motor nuclei 8 and is located in the ventrolateral angle of the lower pontine tegmentum (see Fig. 1). The facial nucleus can be divided into four separate cell groups that supply specific muscle groups: (1) dorsomedial (auricular and occipital muscles), (2) intermediate (frontalis, corrugator, and orbicularis oculi muscles), (3) ventromedial (platysma), and (4) lateral (buccinator and buccolabial).9 The motor axons exit the nucleus dorsally, loop around the abducens (VI) nucleus, and form the facial genu before emerging from the lateral aspect of the pons. The superior salivatory nucleus, which is located just rostral to the facial motor nucleus, is the origin of the parasympathetic fibers that supply the sublingual, submandibular, and lacrimal glands. These salivary and lacrimal fibers join the facial nerve as the nervus intermedius in the cerebellopontine angle. Because the facial nucleus is located ventromedial to the cochlear nuclei and the spinal tract and nucleus of the trigeminal nerve, a lesion of the lateral pons may result in ipsilateral facial paresis, ipsilateral facial analgesia, ipsilateral Horner's syndrome, and ipsilateral deafness (Foville's syndrome). If the lesion extends further dorsally, an ipsilateral gaze paresis would result from involvement of the sixth nerve nucleus. The combination of a unilateral sixth nerve palsy, an ipsilateral seventh nerve palsy, and a contralateral hemiparesis is known as the MillardGubler

syndrome. Intrinsic lesions of the brain stem are usually the result of infarction, hemorrhage, tumor, or demyelination. CEREBELLOPONTINE ANGLE The facial nerve, the nervus intermedius, and the eighth (vestibuloacoustic) cranial nerve exit together from the ventrolateral aspect of the pons, surrounded by a leptomeningeal covering. In the lateral pontine cistern, the anteroinferior cerebellar artery may loop between the seventh and eighth cranial nerves as the artery courses posteriorly to supply the dorsolateral pons and cerebellum.10 As the nervus intermedius approaches the internal auditory meatus, it joins the facial nerve. Because of the association of the facial nerve with the nervus intermedius and the vestibuloacoustic nerves at the level of the cerebellopontine angle and in the internal auditory canal, tearing, taste, submandibular saliva flow, hearing, and balance are disturbed with mass lesions at this level (Fig. 5). Fig. 5 Anatomic relationships of facial nerve trunk and brain stem structure. Anterior view of ventral aspect of brain stem and clivus, especially cerebellopontine angle. The cerebral hemispheres were removed and the oculomotor and trigeminal nerves were divided to provide this view of the anterior surface of the brain stem. A. The superior cerebellar arteries (SCA) and posterior cerebral arteries (PCA) arise from the superior end of the basilar artery (BA). The oculomotor (III) and trochlear (IV) nerves are above and the trigeminal (V) nerves are below the superior cerebellar arteries. The right anterior inferior cerebellar artery (AICA) arises from the basilar artery, courses below the abducens nerve (VI), and passes between the nervus intermedius (VII NI) and the facial motor root (VII) anteriorly and the vestibulocochlear nerve (VIII) posteriorly. The left AICA is a duplicate artery. The rostral duplicate AICA (Dup. Ro. AICA) is not nerve related, but the caudal AICA (Dup. Ca. AICA) is nerve related and passes between the facial and vestibulocochlear nerves. The left posteroinferior cerebellar artery (PICA) arises from the left vertebral artery (VA). The hypoglossal (XII), glossopharyngeal (IX), vagus (X), and spinal accessory (XI) nerves are lateral to the vertebral arteries. The carotid arteries (CA) and the pituitary stalk (Stalk) have been divided. The premeatal segments (Pre. Mea. Seg.) approach the nerves, the meatal segments (Mea. Seg.) pass between the nerves, and the postmeatal segments (Post. Mea. Seg.) pass above the flocculus. A recurrent perforating artery (RPA) and an internal auditory artery (IAA) arise from the right AICA. B. Enlarged anterosuperior view of the right cerebellopontine angle. The premeatal segment passes below the abducens nerve, the meatal segment passes between the eighth cranial nerve and the nervus intermedius, and the postmeatal segment passes above the flocculus. A recurrent perforating artery and an internal auditory artery arise from the meatal segment. Pontine arteries arise from the right side of the basilar artery. (Martin RG, Grant JL, Peace D: Microsurgical relationship of the anterior inferior cerebellar artery and the facial-vestibulocochlear nerve complex. Neurosurgery 6:483, 1980)

From the brain stem to the internal auditory canal, the facial nerve is covered only by a thin layer of glia, which makes it quite vulnerable to any type of surgical manipulation but quite resistant to a slow process of stretching or compression, as might occur with an acoustic schwannoma. Large tumors that fill the cerebellopontine angle compress neighboring cranial nerves and cause defects of the 5th and, later, the 9th, 10th, and 11th cranial nerves. Lesions that occur in this area include temporal bone fractures, acoustic neuromas (schwannomas), meningiomas, and primary cholesteatomas. Hyperkinetic disorders are attributed to vascular compression of the root of the facial nerve. TEMPORAL BONE The motor portion of the facial nerve and the nervus intermedius are loosely joined together as they enter the internal auditory meatus with the acoustic nerve. In this region, the facial nerve and nervus intermedius course superiorly to the vestibuloacoustic nerve. As the facial nerve emerges from the internal auditory meatus, it departs from the vestibuloacoustic nerve to enter the fallopian (facial) canal. The course of the facial nerve through the fallopian (facial) canal is unique; no other nerve traverses so long a distance through a canal (28 to 30 mm). The nerve follows a remarkable Zshaped course in its intratemporal portion (Fig. 6).11 Furthermore, it incorporates a sensory ganglion, the geniculate. In the fallopian canal, the nerve trunk can be divided into labyrinthine, tympanic, and mastoid segments. The labyrinthine segment includes the geniculate ganglion, and it is at this level that the first branch of the facial nerve arises, the greater superficial petrosal nerve. This nerve traverses the dura of the floor of the middle cranial fossa, synapsing in the sphenopalatine ganglion; postganglionic secretory nerve fibers travel with the zygomaticotemporal nerve of the fifth nerve (V-2) and eventually join the lacrimal nerve of V-1 to innervate the lacrimal gland. Involvement of the greater superficial nerve in the middle fossa (e.g., from neoplastic invasion, inflammatory processes, and trauma) impairs reflex tear secretion. When defective tearing accompanies abducens or trigeminal nerve palsy, a lesion in the middle cranial fossa is indicated. Although lacrimal fibers are classically carried by the nervus intermedius, parasympathetic neurons variably reach their destination by way of branches of the fifth or ninth cranial nerves, because there are ample opportunities for intermingling among these nerves (Fig. 7). Fig. 6 Z-shaped course of right facial nerve, anterolateral view. CPA, cerebellopontine angle segment; IAC, internal auditory canal segment; Tymp., tympanic segment in middle ear; Mast., mastoid segment; SMF, stylomastoid foramen. Segment lengths as indicated. (Adapted from Guerrier Y: Surgical anatomy, particularly vascular supply of the facial nerve. In Fisch U (ed): Facial Nerve Surgery. Birmingham, AL: Aesculapius, 1977:1223)

Fig. 7 Peripheral communications of the facial nerve. Note interconnections with cranial nerves V, IX, and X and with the cervical plexus. (Modified from Gray's Anatomy, 36th British Edition. Philadelphia: WB Saunders, 1980:1068)

At the geniculate ganglion, the facial nerve makes a sharply angled turn posteriorly, forming a knee (genu) to enter the tympanic, or horizontal, portion of the fallopian canal. The distal tympanic segment emerges from the middle ear between the posterior wall of the auditory canal and the horizontal semicircular canal, just beneath the short process of the incus. At this point, the fallopian aqueduct makes another turn downward, forming the second genu. This marks the beginning of the mastoid segment. The nerve continues vertically downward on the anterior wall of the mastoid process to the stylomastoid foramen. The chorda tympani is the terminal branch of the nervus intermedius and usually arises from the distal third of the mastoid segment of the facial nerve. The chorda tympani nerve contains secretory motor fibers to the submaxillary and sublingual salivary glands. It also carries special sensory afferents from the anterior two-thirds of the tongue (taste) and somatic sensory fibers from the posterior wall of the external auditory meatus (pain and temperature). The afferent fibers for taste synapse in the rostral nucleus solitarius of the medulla, whereas those somatic sensory fibers from the periauricular region terminate in the nucleus of the spinal tract of the fifth nerve. EXTRACRANIAL SEGMENT As the nerve exits the stylomastoid foramen behind the mandibular angle, and before it bifurcates, motor branches are given off to the posterior belly of the digastric, stylohyoid, and posterior auricular muscles. The main trunk of the facial nerve enters the substance of the parotid gland and then bifurcates into an upper and lower division (Fig. 8). These divisions can be further subdivided into the temporal, zygomatic, buccal, mandibular, and cervical branches. After emerging from the parotid gland, the facial nerve passes over the fascia of the masseter muscle. Although the course in this region is variable, there are some relationships that are relatively constant. There are communications between the upper and lower divisions that form a variety of patterns.12 This rich plexus of nerve filaments in the peripheral zone, just before entering the undersurface of the facial muscles, permits extensive intermingling between peripheral branches of the upper and lower divisions. These anastomoses provide the substrate for misdirected peripheral regeneration that may follow a facial nerve palsy. Such inappropriate axonal sprouting accounts for spontaneous lower facial movement upon blinking or an eye closure provoked by smiling (facial synkinesis). Considering the number of possible routes available to each interrupted neuron, it is truly remarkable that any patients are able to voluntarily control appropriate individual muscle movement. Similarly, crocodile tears (see later discussion) are the result of faulty regeneration of parasympathetic fibers that mistakenly innervate the lacrimal gland instead of the salivary glands. Thus, increased ipsilateral lacrimation associated with eating may occur after a denervating lesion of the facial nerve appears at or

above the site of the geniculate ganglion or along the course of the greater superficial petrosal nerve. Fig. 8 Patterns of peripheral distribution of facial nerve. (Modified from Davis RA, Anson BJ, Puddinger JM, Kurth RE: Surgical anatomy of the facial nerve and parotid gland based upon a study of 350 cervical facial halves. Surg Gynecol Obstet 102:385, 1956)

BLOOD SUPPLY The cortical motor area of the face is nourished by the rolandic branch of the middle cerebral artery. Within the pons, the facial nucleus and its motor axons receive their blood supply primarily from a combination of the anteroinferior cerebellar artery and the short and long circumferential arteries. The extramedullary blood supply to the facial nerve as described by Nager and Nager13 is derived from three sources: the anteroinferior cerebellar artery, which enters the internal auditory meatus in close association with the seventh and eighth cranial nerves; the petrosal branch of the middle meningeal artery, which accompanies the greater petrosal nerve; and the stylomastoid branch of the posterior auricular artery, which enters the facial canal at the stylomastoid foramen. The territories supplied by the three arteries tend to overlap at any given level. Despite the richness of the blood supply to most segments of the facial nerve, vascular compromise is likely a factor in the pathogenesis of facial palsies. The area proximal to the geniculate ganglion is especially vulnerable to ischemic compression, not only because this is the narrowest part of the fallopian canal but also because there are no anastomoses between the arterial systems immediately proximal to the geniculate ganglion.14,15