line skin defects, neurocutaneous lesions, or unusual pigmented lesions. Ear, nose, Headache as a manifestation and throat structures, including dentition and lymphatics, should be assessed. Lim- of otolaryngologic disease ited or asymmetric jaw movement or tem- poromandibular joint (TMJ) tenderness, as well as enlarged or tender temporal MATHEW J. COSENZA, RPh, DO scalp arteries should be noted. Trapezius or posterior cervical trigger points or small vesicular eruptions in the distribution of CN V, including the external ear or por- tions of the hair-bearing scalp, must be identified. Significant deviations in stature can indicate hypothalamic or pituitary disor- ders. Head enlargement can be familial or raise the possibility of hydrocephalus or Headache can be caused by a multitude of factors, but experienced accus- occult tumor. Vascular anomalies can tomed to treating patients with headache are adept at making an accurate diag- produce asymmetric bruits. Localized ten- nosis. Occasionally, however, a patient has an unusual presentation of headache derness about the scalp, face, or neck can or facial pain. In these cases, it can be difficult to classify the etiology of the indicate trauma or local inflammation. headache despite the performance of a thorough physical examination and the Papilledema, retinal hemorrhage, or pal- acquisition of appropriate diagnostic tests. Awareness of some of the otolaryngologic lor of the optic disks can be clues to seri- diseases that can manifest as facial pain or headache may help the bet- ous central nervous system (CNS) dis- ter diagnose and treat this complex problem. ease. Neck stiffness suggests meningeal (Key words: headache, atypical facial pain, otolaryngologic disease, sinusi- inflammation and sometimes early cere- tis, neuralgia, disorders, arteritis) bellar tonsillar herniation. Focal neuro- logic signs can signify a localized intra- cranial pathologic lesion. Posterior fossa disease or hydrocephalus can cause cere- he otolaryngologist is not excluded rate and mode of onset; bellar ataxia. Examination of the skin can Tfrom seeing patients with complaints presence of trigger points or provoca- reveal hyperpigmented or hypopigmented of chronic headaches or facial pain. tion by facial or jaw movement; areas, skin cancer, trauma, or midline Headache is a constituent of many oto- frequency; lesions. laryngologic ailments, generally resulting site; from one of four basic mechanisms: trac- status of the overall condition, whether Laboratory tests tion, distension, inflammation, or direct it is getting better, worse, or the same; The physician must be selective and order pressure. Headaches resulting from dis- precipitating factors; laboratory tests with high diagnostic yield. eases directly affecting structures of the family history; and Computed tomography of the head is the head and neck are described in this article. measures that provide relief. test of choice for excluding most struc- tural or space-occupying lesions. Mag- Patient evaluation Examination netic resonance imaging (MRI) is used History Initial assessment should include evalua- when images of deeper soft tissue struc- The physician must bear in mind that tion of the patient’s mood, affect, and tures of the brain or spinal cord are some headaches signal serious and urgent speech, and measurement of the patient’s desired. conditions and that a properly obtained height, weight, and blood pressure. The Simple laboratory tests such as those history will usually lead to the correct scalp and skull should be examined, and, listed in Figure 1 are helpful in some cases. diagnosis. It is recommended that infor- in younger patients, the head circumfer- Lumbar puncture with analysis of spinal mation be obtained about the following: ence should be measured and plotted. Pal- fluid and recording of pressure is the test the characteristics of the pain; pation and percussion of the head and of choice in determining the presence of duration of headache, associated signs auscultation for bruits over the head, eyes, CNS infection. Cerebrospinal fluid should and symptoms; and neck should be done. Passive and be sent for the analyses that are listed in active flexion, extension, and rotation of Figure 2. the neck must be assessed. A full cranial Dr Cosenza is a Rhinology and Sinus nerve and ophthalmologic examination Clinical syndromes Fellow, Massachusetts Eye and Ear Infirmary, is done. The motor, reflexes, sensory, and Clinical syndromes of otolaryngologic dis- Harvard , Boston, Mass. Correspondence to Mathew J. Cosenza, cerebellar systems should be examined eases that can manifest as facial pain or RPh, DO, Massachusetts Eye and Ear Infir- and attention paid to inspection and pal- headache are outlined in Figure 3 and mary, Harvard Medical School, Zero Emerson pation of the spine. The skin should also described in the following text. Treatment Pl, Suite 2D, Boston, MA 02114. be examined, particularly for any mid- options are suggested, as well.

S22 • JAOA • Vol 100 • No 9 • Supplement to September 2000 Cosenza • Headache as a manifestation of otolaryngologic disease Atypical facial pain the styloid process proves curative in the Atypical facial pain is described as a majority of cases. ✔ chronic burning or aching pain without ✔Checklist focal findings or any discernible etiology Glossopharyngeal neuralgia and is considered a diagnosis of exclu- Glossopharyngeal neuralgia is a neuritic- sion. There appears to be no trigger point type pain initiated by nonnoxious stimu- Complete blood cell count that initiates the pain cycle. Rather, the lus of a trigger point and similar in all Antibody tests pain is characteristically bilateral and aspects to that of trigeminal neuralgia. Erythrocyte sedimentation rate changes locations frequently over weeks to The trigger point is in the oropharynx, Electrolytes months. The intensity fluctuates slowly and the pharyngeal stimulus can be swal- Blood urea nitrogen level over time and is rarely entirely absent. lowing or coughing. Sharp and lancinat- Serum glucose level Typically located in the face, the pain can ing pain tends to be unilaterally located in Urinalysis spread to the cranium. Women are more the posterior pharynx, soft , base commonly affected than men in an age of the tongue, ear, mastoid, or side of the Figure 1. Laboratory tests that may be group from 30 to 50 years.1 Significant head. There may be associated hiccup- helpful in diagnosing cause of headache. psychiatric findings, most commonly ing, nausea, vertigo, , aural fullness, depression, somatization, or adjustment hearing loss, or dysgeusia. The pattern of disorders, are found in 60% to 70% of the attacks is unpredictable. Treatment the patients.1 Psychiatric evaluation is rec- involves carbamazepine or intracranial ommended. Treatment consists of antide- nerve section. ✔✔ pressants. Many of these patients become Checklist the victim of iatrogenic problems resulting Gradenigo’s syndrome from multiple invasive evaluations and The classic tetrad of retro-orbital pain, Cryptococcal antigen excessive medication. Patients with atyp- suppurative otitis, abducens palsy, and Venereal Disease Research ical facial pain respond less frequently to diplopia defines this syndrome. The Laboratory (VDRL) test carbamazepine and neurosurgical decom- intense orbital pain and persistent aural Cytology pression of vascular structures than do discharge are caused by extradural abscess Routine blood cell counts patients in whom trigeminal neuralgia is involving the petrous apex of the tempo- Analysis for protein diagnosed. ral bone. Lateral rectus palsy results from Analysis for glucose irritation of cranial nerve VI as it passes Tuberculosis Cluster headache under the petrosphenoid ligament in Bacterial cultures Cluster headache is a headache of vascu- Dorello’s canal. Treatment involves antibi- lar etiology, characterized by an explosive otics and surgery in refractory cases. Figure 2. Recommended analyses of cere- onset of severe, unilateral, periorbital or brospinal fluid for determining presence retro-orbital pain without nausea or pho- Herpetic facial pain of central nervous system infection. tophobia. Ipsilateral nasal stuffiness and Herpetic papulovesicular skin eruption lacrimation are prominent, and individu- can occur in a dermatomal distribution al headaches last less than 2 hours, occur- of the trigeminal nerve presenting with ring over weeks or months. Treatment burning, itching, and lancinating pain. ✔✔ options include nonsteroidal anti-inflam- This disorder is caused by the varicella- Checklist matory drugs (NSAIDs), ergotamine, oxy- zoster virus, which infects the trigeminal gen inhalation, calcium channel blockers, nerve in childhood as chickenpox. The Atypical facial pain and histamine-2 blockers. trigeminal nerve is involved in 18% of Cluster headache patients with herpes zoster, but other cra- Eagle’s syndrome Eagle’s syndrome nial nerves and facial dermatomes might Glossopharyngeal neuralgia Also known as stylohyoid syndrome, also be involved. The virus remains dor- Gradenigo’s syndrome Eagle’s syndrome results from elongation mant in the ganglion for decades and can Herpetic facial pain of the styloid process or from calcifica- be reactivated by trauma or stress or if Posttraumatic neuralgia tion of the stylohyoid ligament. Elongation the patient is immunocompromised. The Rhinopathic headaches of the styloid process is estimated to occur reactivated virus is transported distally in Superior laryngeal neuralgia in 4% to 28% of the general population, the axon and produces small, crusting Temporal arteritis although only a small percentage of these pustules on the skin. Treatment is a 10- Temporomandibular disorders 2 patients are symptomatic. Patients with day course of oral prednisone. The addi- Tolosa-Hunt syndrome Eagle’s syndrome describe a moderate tion of antiviral agents improves outcome Trigeminal neuralgia pain deep in the and upper and relieves acute pain; however, opioid Trotter’s syndrome neck that can radiate to the ear and that pain medications are used in severe cases. can be aggravated by swallowing. Repre- Advanced age portends a worse response sentative pain produced by transoral pal- to the medication and decreased chance of Figure 3. Clinical syndromes of oto- pation of the styloid process through the spontaneous recovery, presumably because laryngologic diseases that can manifest tonsillar fossa is diagnostic. Excision of of decreased cell-mediated immunity. as facial pain or headache.

Cosenza • Headache as a manifestation of otolaryngologic disease JAOA • Vol 100 • No 9 • Supplement to September 2000 • S23 A diagnosis of postherpetic neuralgia is nerve as a result of extraction of third purulent rhinorrhea, and nasal conges- made when the pain persists more than 2 molars. The reported incidence of trigem- tion are the most common. The associat- months. Tricyclic , anti- inal nerve injuries is 3% to 5% for third ed headache is usually poorly localized, convulsants, or baclofen is used for pain molar removal and 90% to 100% for but patients complain most often of retro- relief as NSAIDs and opioids are ineffec- mandibular osteotomies.3 Most injuries orbital or vertex pain. tive in this stage. produce anesthesia; pain is a rare conse- Acute sinusitis is treated medically with The most common infectious cause of quence. The mandibular nerve is most antibiotics and, in some cases, mucolytics, facial nerve paralysis is herpes zoster. commonly injured by compression of the decongestants, and antihistamines. Max- Papulovesicular lesions around the auricle nerve in the mandibular canal, either by imal medical for chronic sinusitis in conjunction with ipsilateral facial nerve direct pressure on the root tip or local includes topical nasal steroids, mucolytics, paralysis are the hallmarks of the Ramsay swelling and edema after tooth removal. decongestants, antihistamines, and antibi- Hunt syndrome (herpes zoster oticus). Most cases present as persistent anesthe- otics. Patients with refractory acute sinusi- Associated findings include sensorineural sia or paresthesia after the local anesthet- tis are treated surgically. hearing loss, vertigo, tinnitus, intense otal- ic has worn off. Complete recovery occurs Some patients have chronic headaches gia, and vesicular eruptions that can in more than 90% of the cases in 3 to 4 caused by sinus abnormalities other than extend to the tympanic membrane. Rising months. Fewer than 10% of patients have acute purulent sinusitis. These patients titers of varicella zoster virus antibodies permanent anesthesia. Anesthesia per- have sinonasal anatomic variations or and enhancement of the facial nerve can sisting after this period portends a poor abnormalities or occult inflammatory dis- confirm diagnosis by MRI using gadolin- prognosis. Antiepileptic drugs such as car- ease that either causes or exacerabates ium as the contrast medium. Systemic bamazepine, phenytoin, or baclofen are their headaches. In these patients, the pres- may help to reduce pain, most useful for shooting, shocklike pain, ence or absence of nasal symptoms is minimize the incidence of postherpetic and tricyclic antidepressants such as often unreliable as an indication of an neuralgia, and decrease vertigo. amitriptyline hydrochloride are used for underlying pathologic condition. Treat- Additionally, herpes zoster can involve burning, aching dysesthetic pain. When ment includes steam inhalation and saline the auriculotemporal nerve, producing medication fails, surgical repair can be irrigations, topical sprays, or papulovesicular skin lesions along the attempted. Microsurgical repair produces topical decongestants for mild or inter- nerve’s distribution. These lesions can be 50% to 60% pain reduction in patients mittent headaches. Systemic deconges- obscured by scalp hair, and the pain can with hyperalgesia and hyperpathia and tants are also effective, although tachy- mimic that of a TMJ disorder. 15% to 20% pain reduction in patients phylaxis generally develops. Experimental with anesthesia dolorosa and sympathet- therapy consists of capsaicin, substance-P Posttraumatic neuralgia ically maintained pain.4 antagonists, or neutral endopeptidase ago- Neuroma or neuritic pain in the parietal nists. Surgical therapy is indicated if the or occipital regions is occasionally asso- Rhinopathic headaches diagnosis of rhinopathic headache is well ciated with head trauma. Predisposing Headache is a common feature of acute established and if more conservative mea- factors include poor wound closure, local sinusitis. The diagnosis is usually obvi- sures fail. infection, retained foreign materials, skull ous because of the associated nasal symp- fracture, extensive subcutaneous swelling toms of congestion, obstruction, and puru- Superior laryngeal neuralgia or hematoma, diabetes mellitus, and coex- lent discharge. The pain of acute sinusitis Superior laryngeal neuralgia is analogous isting peripheral neuropathy elsewhere in is typically more severe than that associ- to trigeminal neuralgia or glossopharyn- the body. A variable interval exists ated with chronic sinus disease, and chron- geal neuralgia. It is confined to severe lan- between the onset of pain and the initial ic sinusitis often presents without other cinating pain in the small region near the injury, although a time span of 2 to 6 obvious nasal symptoms. Patients with thyrohyoid membrane innervated by the months is most common. At first, pain acute ethmoiditis have symptoms of nasal superior laryngeal nerve. There may be may be mild and nonneuritic, evolving obstruction, purulent rhinorrhea, and a trigger area in the throat stimulated by into a more typical pattern over several headache that can be bilateral or unilateral talking, coughing, or swallowing. Treat- months. Although trigger areas may be with associated pain located in the medi- ment is carbamazepine or peripheral nerve evident at examination, the pain is often al canthal region. Acute maxillary sinusi- section. spontaneous and poorly correlated with tis typically presents as pain and pressure the patient’s activities. Treatment includes over the involved sinus along with nasal Temporal arteritis the same medications as for trigeminal congestion and purulent nasal discharge. Also known as giant cell arteritis or Hor- neuralgia. Recurrent infiltration of trig- The pain often radiates to the teeth, zygo- ton-Magath syndrome, temporal arteri- ger areas with a local anesthetic such as ma, or periorbital or temporal regions. tis results from inflammation of the super- bupivacaine hydrochloride can be sur- The pain of acute frontal sinusitis is often ficial temporal artery. This finding is prisingly effective, providing extended severe, located over the forehead and asso- actually a harbinger of more widespread relief for many months after a series of ciated with tenderness on percussion of the involvement of the arteries of the head three to four injections. involved sinus. The pain is exacerbated and neck. More than 95% of these Injuries to the trigeminal nerve occur as with bending over, a Valsalva’s maneuver, patients are older than 60 years.5 These the result of facial trauma and surgery. or changes in barometric pressure. Acute patients display a constellation of signs Perhaps the most common injury is to sphenoid sinusitis has a variable presen- and symptoms, including fever, sweating, the mandibular branch of the trigeminal tation of symptoms, but postnasal drip, weakness, generalized fatigue, anorexia

S24 • JAOA • Vol 100 • No 9 • Supplement to September 2000 Cosenza • Headache as a manifestation of otolaryngologic disease with weight loss, and jaw claudication. derness to palpation over the condyle and Tolosa-Hunt syndrome Many patients complain of moderate to by pain on jaw movement. Acutely A deep retro-orbital pain associated with severe persistent throbbing or a dull aching inflamed TMJs also produce a steady ophthalmoplegia characterizes Tolosa- pain in the region of the superficial tem- aching when the jaw is not moving. The Hunt syndrome. The etiology is associ- poral artery either unilaterally or bilater- characteristic pain and that ated with an inflammatory lesion of the ally. Occasionally, patients report brief patients with TMJ disorders have is due to cavernous sinus with involvement of cra- instances of sharp, shooting head pain or displacement of the articular disk. nial nerves III, IV, and VI. Corticosteroids pain that is located exclusively in the A history of prior facial injury, jaw are the treatment of choice; however, occipital region. Palpation reveals a tender fracture, or stressful life situations can be recurrences can occur months to years nodular pulseless superficial temporal significant contributing factors. Other later. artery with adjacent sensitivity of the scalp. important findings include unilateral or Biopsy shows intimal thickening and bilateral distribution of pain, duration of Trigeminal neuralgia necrosis with a foreign body giant cell clicking or popping, and jaw position Trigeminal neuralgia is also known as tic reaction. Blindness is the most feared con- when the click or pop occurs. Treatment douloureux or trifacial neuralgia. This sequence of this disease and occurs in includes interarticular corticosteroid injec- syndrome is characterized by progressive 30% to 50% of untreated cases, with tions, dental splints, or orthodontic ther- or fluctuating repetitive brief paroxysms of bilateral blindness occurring in 25% of apy, either singly or in combination. severe, sharp, jabbing or lancinating pain these patients.3 Forty percent of patients with a TMJ lasting seconds to 30 minutes and occur- The erythrocyte sedimentation rate disorder have been found to have histo- ring in the distribution of one or more (ESR) is the “gold standard” diagnostic logic evidence of degenerative joint dis- divisions of CN V. However, facial numb- test and is consistently elevated in all but ease (DJD).4 The incidence of DJD is ness or weakness, loss of corneal reflex, the rarest cases. Diagnosis is confirmed known to increase with age.5 Crepitus, changes in taste or smell, or impairment of by arterial biopsy although skip lesions pain on jaw movement, and the presence other cranial nerve function is inconsistent in the vessel necessitate a specimen of of grating or grinding sounds characterize with the diagnosis of trigeminal neuralgia. greater than 1 cm in diameter and occa- DJD. Usually self-limiting, the painful The maxillary division is the most com- sional biopsy of the contralateral tempo- stage of DJD typically lasts less than 1 mon branch of the trigeminal nerve to be ral artery. year. There exists a characteristic radio- affected, followed by the mandibular and Treatment is high-dose prednisone graphic appearance resulting from remod- the ophthalmic branches. Some external started promptly before biopsy confir- eling of the entire joint by osteophytes stimulus such as touching, rubbing, or mation of the diagnosis and resulting in that form on the anterior surface of the even cold air blowing across a specific dramatic reduction of pain within days. condyle, causing flattening of the condyle. trigger point precipitates these attacks. Lack of a definite clinical response to Degenerataive joint disease results from The most frequent location of the trigger prednisone within several weeks makes either gross trauma or infection of the point is the lateral aspect of the nose or at the diagnosis much less secure without a joint or from long-term abuse in the form the corner of the mouth. Attacks may positive biopsy finding. The ESR usually of , malrelationships of articular occur many times in rapid succession and falls within weeks of starting therapy with disks, or from other chronic repetitive many times a day with spontaneous remis- steroids. The prednisone is tapered to a manipulations of the jaw made by many sions lasting many months or even years. maintenance schedule of every other day patients as a behavioral response to stress. The nasal sinuses, , peri- over a period of weeks after the headache Treatment consists of NSAIDs, physical odontal ligaments, and muscles of facial has resolved and the ESR has returned to therapy, intra-articular corticosteroid injec- expression or mastication can also serve as normal. The disease lasts 1 to 2 years, tions, or joint reconstruction surgery. triggers. Diagnosis includes MRI of the during which maintenance steroid thera- Myofascial pain dysfunction syndrome head in patients of any age with symp- py is continued to prevent loss of vision. is considered the most common painful toms of trigeminal neuralgia. In patients disorder of the TMJ. Eighty percent of with signs and symptoms in addition to Temporomandibular disorders patients with this syndrome have painful the classic presentation and when MRI Temporomandibular disorders encom- muscle spasm of the TMJ.6,7 The cause of is inconclusive, a lumbar puncture with pass multiple disorders associated with this disorder is thought to be muscle analyses of CSF as listed in Figure 2 is the TMJ presenting as headache, earache, fatigue produced by chronic oral habits warranted. or facial pain (singly or in combination) that are often an involuntary tension- Medical treatment includes carba- with limited opening of the jaw and joint relieving mechanism. Positive findings mazepine, baclofen, phenytoin, sodium noise. The TMJ undergoes both gliding include pain of unilateral origin, muscle valproate, or chlorphenesin carbamate. and hinge motions and is thus termed a tenderness, clicking or popping noise in the Each drug should be tried for at least 2 ginglymoarthrodial joint. The etiology TMJ, and limitation of jaw movement. weeks; combinations of the two drugs and natural history of these disorders are There should be no clinical, radiographic, may also be used. Adjunctive therapy poorly understood and the source of con- or biochemical evidence of organic TMJ includes tricyclic antidepressants and non- siderable debate. The current classifica- changes. steroidal anti-inflammatory drugs. Surgi- tion of temporomanidibular disorders Treatment includes NSAIDs, muscle cal treatment is considered when medi- includes internal derangements, degener- relaxants, application of moist heat, mas- cations cannot control the pain. Surgical ative joint disease, and myofascial pain. sage, soft diet, and acrylic splints, thereby management options include microvas- True TMJ pain is characterized by ten- decreasing muscle spasm and pain. cular decompression of the nerve root via

Cosenza • Headache as a manifestation of otolaryngologic disease JAOA • Vol 100 • No 9 • Supplement to September 2000 • S25 a posterior fossa surgical approach, alco- correlated with good clinical judgment to 6. Behrens MM. Headaches associated with disorders hol block of the involved division of the make the appropriate diagnosis. Aware- of the eye. Med Clin North Am 1978;62:507-521. trigeminal nerve, and percutaneous ness of some of the otolaryngologic dis- 7. Blackwood HJJ. Arthritis of the mandibular joint. Br radiofrequency thermocoagulation of the eases that can manifest as facial pain or Dent J 1963;115:317-326. trigeminal sensory root as it exits the headache may help the physician in the 8. Schwartz LL, Marbach JL. Changes in the tem- gasserian ganglion. diagnosis and treatment of this complex poromandibular joint with age. J Am Soc Periodont problem. 1965;3:184-189.

Trotter’s syndrome 9. Laskin DM. Etiology of the pain-dysfunction syn- Nasopharyngeal tumors can produce drome. J Am Dent Assoc 1969;79:147-153. facial pain and ipsilateral conductive hear- ing loss. Facial pain results from pressure References 10. Griffin CJ, Sharpe CJ. The structure of the adult tem- poromandibular joint. Aust Dent J 1960;5:190-195. on cranial nerves V, VII, IX, or X (singly 1. Mann J D, Lundeen TF. Headaches and facial pain. or in combination). The conductive hear- In: Bailey BJ, ed. Head & Neck Surgery—Otolaryngol- ing loss is due to obstruction of the ogy. Philadelphia, Pa: JB Lippincott Company; eustachian tube and resultant serous oti- 1993:761-773. Suggested reading tis media. All patients with these symp- 2. Kaufman SM, Elzay RP, Irish, EF. Styloid process Lee KJ, ed. Essential Otolaryngology—Head and Neck toms warrant nasophayrngoscopy to rule variation. Radiologic and clinical studyArch Otolaryngol Surgery, 7th ed. Stamford, Conn: Appleton & Lange; out a nasopharyngeal mass. 1970;91:460-463. 1999.

3. Olesen J. Classification and diagnostic criteria for Paparella MM, Shumrick DA, Gluckman JL, Meyer- Comment headache disorders, cranial neuralgias and facial pain. hoff WL, eds. Otolaryngology. Philadelphia, Pa: WB As in many other medical fields, the oto- Cephalalgia 1988;8(suppl 7):28. Saunders Co; 1991. laryngologist is often confronted with the 4. Clough C. Non-migrainous headaches. Br Med J daunting task of delineating the etiology 1989;299:70-72. of a patient’s complaint of chronic cephal- gia. Laboratory and radiographic data 5. Saper JR. Chronic headache syndromes. Neurol Clin 1989;7:387-412. are helpful; however, it requires a thor- ough history and physical examination

S26 • JAOA • Vol 100 • No 9 • Supplement to September 2000 Cosenza • Headache as a manifestation of otolaryngologic disease