Matthew L. Kashima
Epistaxis
Epistaxis is a very common occurrence, with most individuals having at least one “nosebleed” during their lifetime. Most episodes of epistaxis do not require medical attention and are usually self-limited with inconsequential loss of blood. Rarely, severe uncontrolled epistaxis may cause aspiration and substantial blood loss and may be life threatening. Estimates are that only 10% of nose bleeds are brought to the attention of a health care provider. Less than half of these require consultation with an otolaryngologist. Most patients with epistaxis are successfully managed by their primary care provider. The overall goal in the management of epistaxis is to stop the bleeding and ultimately to correct any underlying pathology that precipitated the event.
Pathophysiology
Epistaxis is defined as bleeding emanating from the nose or nasopharynx. The blood may flow anteriorly through the nares or posteriorly and may be expectorated and or swallowed. The nasal mucosa has a rich anastomosis of vessels, arising from multiple sources. The blood supply to the lateral nasal wall is derived from the internal maxillary and facial arteries from the external carotid system. The nasal septum is supplied by the anterior and posterior ethmoid arteries from the internal carotid system.
The nasal mucosa is composed of a thin stratified columnar epithelium with goblet cells supplying the mucous blanket. The nasal secretions help to protect the underlying epithelium. On the lateral nasal wall (i.e., on the turbinates), the submucosa is thickened with the presence of venous sinusoids and mucous glands. The submucosa of the nasal epithelium lining the septum can be quite thin with blood vessels in close proximity to the mucosal surface. Dryness or irritation causes mucosal disruption and bleeding from underlying vessels. These vessels will not be fully protected from rebleeding until the overlying mucosa has healed. A cycle of rebleeding may occur as local infection and inflammation initiate the formation of granulation tissue, which remains quite friable and will cause persistent bleeding. The rebleeding cycle ceases only when the underlying epithelium is regenerated and the submucosal vessels are protected.
The anterior septum is particularly prone to environmental irritation (dry air, smoke and other irritants, allergens, toxic inhalants); it is the site of approximately 90% of all episodes of epistaxis. The most frequent site of bleeding is Kiesselbach plexus or Little area, an area on the anterior portion of the nasal septum rich in capillaries. Anterior epistaxis occurs more frequently when the weather is cold and humidity low. More posteriorly, on the septum and in the lateral nasal wall, the vessels are of a larger caliber, and epistaxis from these posterior vessels is typically quite brisk. With increasing age and concomitant arteriovascular disease, posterior epistaxis becomes more prevalent. The presence of arterial disease in these vessels also compromises vascular contraction during acute epistaxis, which prolongs uncontrolled bleeding (1). The posterior nasal cavity is very difficult to access and evaluate, so posterior epistaxis can be difficult to control.
Etiology
The etiology of epistaxis usually can be readily determined (2). It is more important to ascertain the cause of the nasal
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bleeding in refractory and recurrent cases, as opposed to isolated episodes that are self-limited and not severe. Recurrent and refractory epistaxis may point to a significant underlying medical condition. Epistaxis often is caused by a combination of factors, so it is helpful when evaluating a patient with problematic epistaxis to consider both local factors and systemic factors (Table 111.1).
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TABLE 111.1 Causes of Epistaxis |
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Local Factors
Conditions that alter the physiology of the nasal mucosa and environmental factors, such as decreased temperature and humidity, lead to disruption of the nasal epithelium and subsequent vascular injury and hemorrhage. For example, hospital admissions for epistaxis increase during the winter months, with up to two-thirds occurring during January and February in the northern hemisphere (3). This may be related to low humidity in households with dry-air heating systems. Patients requiring oxygen supplementation either as inpatients or at home are at increased risk of having epistaxis. This is due to the drying effect of the oxygen, which is delivered via nasal cannula. The dry membranes and cannula can be sites that develop crusts and be subject to digital trauma as described below.
Local trauma is also commonly implicated in epistaxis. Any blunt force directed to the mid-face or adjacent regions can cause shearing of the nasal mucosa, which will be accompanied by brisk nasal bleeding. This can be seen in severe facial trauma or in isolated nasal fractures. More common is trauma from persistent nose picking. Patients who use their fingers to remove crusts from their nose are likely to disrupt the mucosa. Chronic digital trauma can lead to persistent ulcerations with concomitant granulation tissue formation, which can increase the frequency of bleeding. Patients with recent nosebleeds may perceive nasal obstruction due to crusting and promote rebleeding while trying to remove the crust. Local inflammation due to upper respiratory infections, chronic sinusitis, allergic rhinitis, or environmental irritants can also alter normal nasal physiology, thinning the nasal mucosa leading to dryness and crusting with subsequent vascular exposure and bleeding.
Anatomical derangements such as nasal septal deviation are also common in patients with epistaxis (4). It is not clear how septal deformities promote epistaxis. Septal deflections can cause disruption of laminar airflow. Eddies occur in these areas, which dry out, making them prone to bleeding. Intranasal foreign bodies are rare in adults but are an important cause of epistaxis in children. Foreign bodies will often present as unilateral, foul smelling rhinorrhea which is refractory to medical therapy. In adults, a nasal foreign body may be found in a victim of a recent motor vehicle accident who unknowingly had a piece of glass impacted in the nasal cavity that was not readily apparent on initial evaluation. Industrial chemical exposures, (e.g., to the fumes of chromates, ammonia, and sulfuric acid) have also been implicated in causing recurrent epistaxis (5). Cigarette smoke, including second-hand sources, is also an irritant that can promote epistaxis.
Epistaxis occasionally occurs subsequent to iatrogenic trauma (nasal and sinus surgery). Benign and malignant intranasal tumors are uncommon but can present with persistent epistaxis.
Systemic Factors
A variety of systemic factors related to either vascular fragi-lity or clotting abnormalities may contribute to epistaxis. Long-standing hypertension may be a factor in promoting epistaxis, but it remains controversial whether there is a higher rate of arterial hypertension in epistaxis patients versus normal control subjects (1). It appears that hypertensive patients on diuretics are more susceptible to epistaxis than those taking β-blockers (6). Many patients have elevated blood pressure at the time of their treatment for epistaxis, but this may simply be a result of pain and anxiety. It is important to ascertain whether or not hypertension returns to normal after treatment, so that patients found to have underlying hypertension can receive appropriate treatment (7). Generalized vascular disease associated with hypertension may also be a factor, because underlying atherosclerotic vessels are known to have decreased elasticity and capacity to contract and assist in clot formation.
Patients with end-stage renal disease on hemodialysis are prone to recurrent epistaxis. This may be because of a decrease in platelet activity or frequent exposure to heparin (8,9). Septal perforation is noted in up to 8% of patients with chronic renal failure, and this perforation can precipitate turbulent airflow, local irritation and crusting, and recurrent hemorrhage similar to septal deviation. Alcohol abuse with associated poor nutrition and vitamin C and K deficiencies may lead to poor wound healing and
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poor clot formation. Additionally, liver disease secondary to alcohol abuse can cause alterations in the normal clotting mechanism, thus setting the stage for persistent and recurrent epistaxis.
Patients with hereditary coagulation abnormalities, such as hemophilia or von Willebrand disease, can have problematic and persistent epistaxis. Acquired conditions, such as thrombocytopenia from a hematologic malignancy or chemotherapy, may also be significant. Osler-Rendu-Weber disease (hereditary hemorrhagic telangiectasia) very commonly presents with recurrent epistaxis. This inherited condition results in a lack of the contractile elements in the vessel walls with formation of telangiectasias on the nasal, oral, intraoral, and gastric mucosa. These patients often have recurrent gastrointestinal (GI) bleeding as well as epistaxis, and treatment can be problematic requiring multiple treatments over the lifetime of the patient. It is not unusual to need multiple transfusions and multiple surgical interventions (e.g., cauterization, embolization).
Numerous medications are also implicated in epistaxis. Nonsteroidal anti-inflammatory drugs (NSAIDs) are commonly a factor, with as many as 75% of epistaxis patients having previously been on one of these drugs (10). It is important to determine by a careful history if these agents, including aspirin, have been used, for what length of time they were used, and the time of their last use (11). These medications interfere with normal platelet function by inhibiting the cyclooxygenase pathway and arachidonic acid metabolism. Other agents that dry the nasal mucosa, such as tricyclic antidepressants, antipsychotic agents, and antihistamines, may promote mucosal disruption and bleeding. Nasal steroid sprays may also cause epistaxis, either because of the vehicle, the medication itself, or trauma from the nasal spray applicator. With prolonged use, nasal steroids can thin the nasal mucosa, leading to an increased risk of epistaxis.
Approach to Management
Management of a patient with epistaxis should proceed at a pace congruent with the severity of bleeding and acuity of the situation. The ABCs (airway, breathing, and circulation) of resuscitation need to be followed. Patients should be assessed for hemodynamic stability and the severity of their blood loss. Intravenous access should be established in all patients who are actively bleeding.
Fortunately, most epistaxis is not accompanied by massive blood loss, and the focus is on local control of the bleeding. It is important, though, not to take a casual approach to epistaxis because fatalities, although rare, are known to occur, especially in patients with existing comorbidities such as coronary artery disease or chronic pulmonary disease. The demeanor of the health care provider can do much to ease the anxiety of the patient with epistaxis. A calm, matter of fact demeanor will help to reassure the patient that they are in good hands and the episode will be resolved. An anxious or harried interaction with the patient can serve to increase their anxiety and stress levels leading to increased pulse and blood pressure, which will make controlling the epistaxis more difficult.
The initial history should ascertain on which side the bleeding began and whether or not it was mostly isolated nasal bleeding or associated with excessive spitting up of blood, which would suggest a posterior bleeding site. The duration of the nosebleed and an estimation of blood loss are also pertinent. A history of previous epistaxis and how those episodes were treated may suggest the initial treatment in a given situation. A past medical history should ascertain whether or not hypertension, liver disease, alcoholism, cardiac disease, or renal insufficiency is present. A review of the patient's medications will determine if nonsteroidal anti-inflammatory agents, warfarin, or aspirin-containing products are contributing to clotting ab-normalities.
At initial presentation, it is reasonable to obtain laboratory studies, including a complete blood count (CBC) (to determine if anemia is present), prothrombin time (PT)/partial thromboplastin time (PTT) and platelets (to assess for significant coagulopathy), and a type-and-screen to make blood available if the episode of epistaxis is intractable. Hematocrit taken at the time of presentation may not accurately reflect the severity of blood loss because of a lack of hemodilution that will occur as intravascular volume is restored.
The next step is to examine the nasal cavity. Universal precautions for all health care personnel are important, because epistaxis management can be quite bloody. Appropriate protective gear should be worn, including eyewear, gowns, gloves, and caps. The patient should be supplied with a handful of gauze or tissues and should be draped appropriately to allow comfortable examination. Strong suction is a must, preferably with a Frazier tip suction. A headlight and nasal speculum are helpful in performing intranasal examination, although an otoscope or other hand held light can be used. Intranasal examination may be aided by decongesting the nose. Topical decongestants (e.g., oxymetazoline 0.05%, two to three sprays in each nostril) will cause significant vasoconstriction and either decrease or stop the flow of blood (12). All clots should be removed from the nose, preferably through intranasal suctioning, and patients also may be asked to blow their noses to remove any clots prior to decongesting the nose. This allows for the decongestant to contact the mucosa directly to maximize its effect.
Treatment
Treatment of epistaxis should be performed in a step-like fashion to provide the least invasive means to control the episode. Because most nasal bleeding is anterior, local
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compression of the nose should be performed initially using the thumb and index finger to compress the cartilaginous portion of the nose. This can be initiated by instructing the patient to perform this maneuver while the other materials are being collected. This allows for a clot to form and set at the bleeding site. Fifteen minutes of direct pressure should be applied. The patient or health care providers should hold pressure while watching a clock in order to complete the entire time. If any break in holding pressure occurs, the time should be reset and 15 minutes of holding pressure begun again. During this time the patient should be instructed to sit calmly with their head held forward and their mouth open. Any draining blood will drip off the tip of the nose or be expectorated out of the mouth into an appropriate receptacle. Holding the head back as is commonly advised will encourage blood to be swallowed, which can lead to GI upset and emesis. The Valsalva will raise the venous pressure in the head and can inhibit successful clotting of a bleeding site in the nose. If compression is unsuccessful, the nose should be cleaned of clot by suctioning and vigorous nose blowing followed by the application of decongestant spray to both sides of the nose. Pressure should then be applied as described above for 15 minutes. The algorithm as described so far can be performed anywhere and does not require special equipment, medications, or specialty training and should be given as instructions to patients following an episode of epistaxis should they have a rebleed.
If bleeding persists despite the above intervention, it is imperative to visualize the bleeding site. Lighting, suction, and patience are invaluable tools to this end. If the bleeding site is seen to be anterior, a cotton ball impregnated with a decongestant and lidocaine may be used to anesthetize and further decongest the area of the bleeding vessel. Silver nitrate cautery can be used to lightly cauterize the area around the vessel. The silver nitrate stick should be used from a peripheral to central fashion around the bleeding vessel, with the stick rolled over the mucosa lightly until a gray residue appears. This technique is difficult to perform during active bleeding and often will be unsuccessful. It is important to avoid deep chemical cautery with silver nitrate, as well as bilateral septal cautery, to avoid injury to the underlying cartilage, which can lead to septal perforation. If the bleeding is controlled with this method, a cotton swab saturated with normal saline should be used to neutralize any residual silver nitrate and curtail the chemical cautery. After silver nitrate cautery, an antibiotic ointment should be applied to prevent crusting and subsequent local bacterial infection.
If the bleeding persists, the next level of intervention is anterior packing. Traditional anterior packing dictates placement of a layer of half-inch lubricated gauze coated in antibiotic ointment. This is a difficult technique to master and is now seldom performed. There are several commercially available expandable nasal tampons (Merocel, Xomed Surgical Products, Jacksonville, FL; Rapid Rhino, Applied Therapeutics, Inc Newbury, Berkshire UK) that can be as effective and are much easier to place. When using a nasal tampon, it is important to have a good idea where the site of bleeding is to determine the length of the nasal tampon to apply. The common length is up to 10 cm, which will pack the entire nose, including the posterior choana. It is also important to understand that the floor of the nose slants caudally from an anterior to posterior direction and that the tampon should be directed somewhat inferiorly as opposed to cranially. The nasal tampon can be coated with an antibacterial ointment to prevent premature expansion of the tampon by blood during the insertion and to provide an impediment to bacterial overgrowth of the packing. The packing should be left in place from 3 to 5 days to allow clotting and healing of the bleeding area. If necessary, bilateral tampons or synthetic sponge packs can be placed. The success rate is high, and minimal experience is needed for placement. Additionally, patient tolerance of this form of packing is superior to the classic layered gauze. After placing the pack, the oropharynx should be examined to determine if there is any residual bleeding. If there is bleeding noted posteriorly, the anterior tampons may be inadequate and posterior packing may be needed. After packing, an oral antibiotic such as a first-or second-generation cephalosporin (e.g., cephalexin 250 mg orally four times daily) or amoxicillin/clavulanate (Augmentin, 500 mg twice daily) with staphylococcal coverage should be used for 10 days to avoid bacterial overgrowth and to prevent toxic shock syndrome (TSS).
A downside to conventional packing is that it needs to be removed. This is usually done 3 to 5 days after the packing was placed. This is usually not an adequate length of time to allow for the site of bleeding to mucosalize. Additionally, the placement and removal of the packing material can abrade the nasal mucosa and cause bleeding. Any time packing is removed preparations should be made and materials available to repack the nose should bleeding recur. To avoid this possibility, many practitioners will use absorbable materials to pack the nose first. Several options are available (Surgicel, Johnson and Johnson, Somerville, NJ; Gelfoam, Pharmacia & Upjohn, Kalamazoo, MI; Avi-tene, Davol Inc., Cranston RI; Floseal, Baxter, Deerfield, IL; Thrombin, JONES PHARMA Inc., St. Louis, MO). These materials are not designed specifically for intranasal use and need to be formed to the desired size and shape. They can be applied to an identified bleeding site, active or quiescent, to stop bleeding and facilitate healing (13).
The posterior vessels are difficult to visualize and are not affected by pressure from the usual anterior nasal packing. Posterior packing is necessary in cases of a posterior site of bleeding and in situations where nasal septal deflection prohibits adequate insertion of a tampon. Although only about 5% of epistaxis originates from a posterior nasal source, it is these patients who are at most risk of complications and adverse sequelae (14). Posterior nasal packing is difficult to perform and requires a complex procedure to
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allow insertion of a nasal pack from the oropharynx into the nasopharynx. Commercially available balloon intrana-sal catheters (Epistat, Xomed Surgical Products, Jacksonville, FL) may be placed by otolaryngologists or other clinicians experienced in their use. A Foley catheter can also be used as a posterior pack. Because insertion of the posterior packing is painful, the clinician should provide mild sedation before placement. Additionally, any patient who undergoes posterior nasal packing should be admitted to the hospital for observation. Posterior packing will cause swallowing difficulty, so maintenance of hydration by administration of intravenous fluids is important. Posterior nasal packing is usually removed after about 48 to 72 hours. It can be difficult to determine whether posterior packing is necessary, and it is reasonable to proceed with anterior packing first and if this fails to control the bleeding, proceed with posterior packing.
Consideration should be given as well to hospitalization of elderly and debilitated patients who require anterior nasal packing. An otolaryngologist should be consulted if there is doubt about the management of such a patient. Bilateral, as well as posterior, nasal packing will obstruct the nasal airway and may promote hypoxia or hypercapnia (15). Also, posterior packing and the mild sedation it requires can promote decreased arterial oxygenation and hypercapnia. Continuous pulse oximetry is indicated and admission to an appropriate level unit to ensure the necessary surveillance based on the patient's age and general health. These patients are at risk for a nasal vagal response, bradycardia, hypertension, apnea, dislodged packing, aspiration, persistent bleeding, and significant hypoxia.
Surgical Interventions
If these packing procedures are ineffective, more invasive procedures may be performed by a consulting otolaryngologist, including extracranial arterial ligations, if necessary (16,17). Embolization of active nasal bleeding can be done in centers with interventional neuroradiology (18).
Special Cases
In patients with severe coagulopathy or thrombocytopenia, placement of intranasal packing can be problematic, because at the time of removal a raw nasal mucosa may continue to bleed. Transfusion of clotting factors and platelets can be helpful in stopping the bleeding temporarily, but, in some circumstances packing may still be needed. In these cases, the use of a porcine pack may be appropriate. This entails using a strip of ordinary salt pork to pack the nose. Homogenates of salt pork contain an activation factor that promotes platelet aggregation. Additionally, the porcine packing is less irritating to the nose, and upon removal there is less nasal mucosal irritation to promote bleeding (19).
Nasal bleeding associated with significant facial or head trauma can be complicated by disruption of the cribriform plate in the anterior cranial base permitting displacement of packing material into the intracranial space. Therefore, in these situations, packing and nasal instrumentation should be deferred and prompt otolaryngology consultation arranged. Arterial embolization is also an effective method for intractable nosebleeds, especially in patients who are not considered surgical candidates (19).
Followup and Patient Education
Most patients can be treated in an outpatient setting and return for packing removal. If the patient's epistaxis is recurrent and persistent, prompt otolaryngologic evaluation is appropriate, even if packing was unnecessary. Persistent and refractory epistaxis can be evaluated by testing for coagulopathy or any hematopoietic malignancies. Sinus radiographs or computed tomography (CT) can rule out an intranasal mass. Nasal endoscopy has revolutionized sinonasal evaluation; it allows a well-illuminated, magnified view of the entire nasal cavity to assess for potential bleeding sites, anatomic abnormalities and tumors. This procedure is easy to perform in the otolaryngology office setting using topical anesthesia.
Many patients with persistent epistaxis, even after determining there is no sinister underlying pathology, will have etiologic factors that cannot be modified. For example, patients may require warfarin or may have an underlying chronic medical condition that is unlikely to change. It is important to educate these individuals about conservative measures that can alleviate the cycle of epistaxis. Patients should be informed about the physiology of epistaxis, with a cycle of desiccation, crusting, mucosal disruption, and hemorrhage followed by crusting and rebleeding. It may be helpful to explain this phenomenon by analogy to a puddle of water that dries and one can then see the ground on the bottom caking and cracking. This exemplifies the nasal mucosa and its fragility and need for humidification. Patients should be instructed to avoid aspirin therapy within 2 weeks of their nosebleed to allow for a reasonable period of time for healing. Additionally, humidification of the nose should be stressed with liberal use of nasal saline spray every hour while awake and application of a nasal emollient (Vaseline, Bacitracin) twice a day to the nasal vestibule. Room humidifiers should also be used to elevate the humidification of the household air and are particularly useful while sleeping.
Patients should be instructed what to do if subsequent nosebleeds occur. This includes instructing the patient in digital compression of the anterior nose. Application of pressure to the entire soft pliable portion of the nose with the thumb and finger should be demonstrated. It is important to stress that this maneuver should be performed for at least 15 minutes by the clock without peeking to see if bleeding is continuing. Patients prone to nosebleeds should keep oxymetazoline spray (0.05%) on hand, remove any intranasal clots by blowing, and spray
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each nostril generously two to three times. These self-administered treatments control simple epistaxis in about two thirds of patients (12). If these methods fail, patients should be instructed to seek care from their provider or an emergency room for more definitive management.
Some patients who are prone to persistent and severe nosebleeds (e.g., those with hereditary hemorrhagic telangiectasias) have learned to self-apply anterior packing. In the situation of recurrent one-sided nosebleeds, certain anatomic abnormalities may respond to “nasal rest.” This is achieved by having a patient take a small cotton ball impregnated with petroleum jelly and place it into the anterior nares to obstruct all airflow in the bleeding nasal cavity. This can be continued for several days at a time to prevent desiccation and allow for healing.
Snoring
Snoring is a significant behavioral and social problem with potentially important medical implications. Approximately 25% to 50% of men and 15% to 30% of women become chronic snorers (20). Chronic snoring may be a symptom of obstructive sleep apnea (OSA) syndrome (see Chapter 7). Although it is the cardinal symptom, snoring is not a specific marker for OSA (21). Snoring without OSA is often designated benign snoring, but a better term may be nonapneic snoring, because the medical implications of benign snoring are still being elucidated. There is no clear evidence that nonapneic snoring is a significant risk factor for the more severe medical consequences of OSA. However, nonapneic snoring may fragment sleep and result in daytime hypersomnolence and dysfunction. The personal and social impact of snoring can be substantial, such as when bed partners require alternative sleeping arrangements (22). Primary care clinicians and otolaryngologists are often called on to evaluate and treat individuals with this condition and differentiate it from other sleep dis-orders.
Pathophysiology
Snoring is a consequence of the elasticity of the soft tissues of the oropharynx. The snoring sound is thought to be generated by vibrations of the collapsing soft tissues of the pharynx, soft palate, uvula and, infrequently, the nasal passages. It can be difficult to determine the exact site responsible for snoring. Compared with nonsnorers, nonapneic snorers are noted to have smaller airways with higher airway compliances, but these changes are not as severe as in patients with sleep apnea (21). Airway collapsibility is deterred by sustained pharyngeal muscle tone, which is absent during rapid eye movement (REM) sleep. Thus, snoring is significantly related to the stage of sleep (23).
One of the difficulties in studying snoring and assessing appropriate management interventions is that there are few objective measures of this phenomenon. Often the clinician has to rely on snoring assessment by the bed partner, because approximately 75% of snoring patients are unaware that they snore and if they are aware of snoring it is usually because of the complaints of others (22). Common risk factors for snoring are male gender, obesity, alcohol consumption, ingestion of sedatives or muscle relaxants, and smoking. Even small amounts of alcohol can exacerbate snoring. The use of sedatives or muscle relaxants will also cause a relaxation of the pharyngeal musculature and promote snoring. Smoking may also contribute by increasing pharyngeal inflammation and edema due to the irritant effects of tobacco smoke, leading to pharyngeal narrowing and subsequent snoring. Nasal obstruction from chronic rhinitis or sinusitis may also be a contributing factor. There may also be a familial predisposition to snoring (24).
Evaluation
The office evaluation of patients with snoring should focus on their medical and sleep histories and include a general examination with emphasis on the head and neck. The main task of the evaluation is to determine how severe and disruptive the snoring is and whether further evaluation for OSA syndrome (as outlined in Chapter 7 and below) is indicated.
Sleep and Medical Histories
Patients should be queried about their awareness of snoring and whether they awake at night with a gasping or choking sensation. They should also be asked if they awake in the morning feeling rested or not and if they awake with a headache. Symptoms of daytime somnolence should be ascertained, especially interference with work, driving, or other tasks.
Because most snorers are unaware of their problem, it is helpful to talk to a bed partner or other close observer. This person should be questioned about the frequency, persistence, and severity of snoring and especially whether there are apneic periods, gasping, or choking. Initial assessment of the degree of personal and social disruption is also pertinent; for example, is the patient able to maintain a normal sleeping arrangement or must the patient or bed partner leave the room for sleep? For the reasons indicated above, it is important to inquire about recent change in weight and its effects on the snoring pattern, as well as the use of alcohol, tobacco, and prescription and over-the-counter drug use. Finally, the patient should be questioned about nasal and sinus symptoms that may be associated with snoring.
Examination
Physical examination is directed at the head and neck. Careful nasal examination should be performed to look for
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nasal polyps, nasal septal deviation, turbinate hypertrophy, or any evidence of anatomic nasal airway obstruction (see Chapter 33). The oral examination should focus on identifying macroglossia and tonsillar hypertrophy. Sometimes the uvula may be elongated, swollen, or inflamed from vibrations produced during snoring.
If snoring is severe or disruptive, referral to an otolary-ngologist is indicated for airway assessment, usually with transnasal flexible fiberoptic laryngoscopy to rule out any anatomic obstruction. Flexible fiberoptic laryngoscopy can be performed easily with topical anesthesia, and it provides a useful evaluation of the entire upper airway.
Because OSA syndrome can have significant and preventable morbidity, it is important to consider whether it is present in patients who snore. Nevertheless, most snorers do not have OSA syndrome. There are as yet no validated clinical criteria for determining whether a patient who snores has sleep apnea; diagnosis is dependent on polysomnography. Important features associated with OSA syndrome include apneas reported by an observer, nocturnal gasping or choking reported by the patient or an observer, very loud or disruptive snoring, daytime somnolence, hypertension, nocturnal or early morning angina or palpitations, and headaches on awakening, which are characteristically brief (25). Additionally, an Epworth Sleepiness Scale can be applied to help determine if further testing is needed. If some of these features are present, and especially if one or more are severe, further testing, such as overnight pulse oximetry or formal polysomnography (sleep study), is indicated (20) (see Chapter 7).
Treatment of Nonapneic Snoring
There are two therapeutic approaches to nonapneic snoring: low-risk conservative measures and surgery, commonly directed at the soft palate. If conservative measures do not produce satisfactory results, and surgical options are considered, the patient should undergo polysomnography to rule out OSA syndrome before referral to an otolaryngologist. This will prevent future masking of OSA symptoms by the surgical intervention and unanticipated perioperative complications of existing OSA.
Nonsurgical Treatment
Conservative measures aimed at decreasing upper airway resistance include improved overall muscle tone and weight loss. This can be achieved by instituting an exercise program. For some patients, weight loss is the only nonsurgical therapy that will relieve benign snoring. Although it is unclear how much weight loss is needed to provide symptomatic improvement, achievement of ideal body weight is not necessary (21). Most patients will note a marked improvement in snoring symptoms with a 10% weight loss. Addressing sleep posture is also helpful in some patients. Bed partners will often relate that snoring is worse in certain positions and absent in others. Many patients achieve marked reduction in snoring when sleeping in the lateral decubitus or prone positions. The classic treatment of sewing a pocket in the back of the pajamas to place one or more tennis balls to force the patient to sleep on the side has anecdotal support.
The use of over-the-counter nasal dilation devices, such as an external adhesive strip (e.g., Breathe Right), may provide relief in some patients, but studies are inconclusive (26). Internal nasal dilators are much less tolerated by patients. In general, nasal devices are not effective, becasue most snoring is not caused by the nasal airway. Nasal devices will help patients with nasal valve collapse as the cause of their snoring. The use of various oromandibular splinting devices that advance the lower jaw (sleep splints) has been advocated by many dental professionals, and some efficacy has been reported (21,27). These devices, unfortunately, are often poorly tolerated and require customized fitting by a dentist or oral surgeon who is familiar with their application to achieve satisfactory results.
The use of phosphocholinamine, a tissue lubricant placed intranasally, has been found to reduce snoring frequency by about 25% in a small study (28). However, this is an oil-based agent, and there is the theoretical risk of aspiration and lipoid pneumonia. Protriptyline, a tricyclic antidepressant, has also been found to reduce snoring by as much as 30% (29) in most patients. The long-term efficacy, however, is unknown, and the anticholinergic side effects may be bothersome. If significant nasal obstruction is present, it is reasonable to treat with systemic decongestants (see Chapter 33) and intranasal steroids (see Chapter 30) to maximize the cross-sectional area of the nasal airway. Improving the nasal airway may be enough in some patients to obviate or reduce snoring. Continuous positive airway pressure (CPAP) administered via a nasal mask is commonly used to treat OSA. This device applies a pneumatic “splint” to the upper airway structures and almost invariably eliminates snoring. Nonapneic snorers are often hesitant to use the CPAP system and usually tolerate it poorly. It is not clear why nonapneic snorers are less tolerant of CPAP, but it may be because of the lack of significant symptomatic benefits compared with patients with OSA (30).
Surgical Treatments
When the snoring patient has an obvious anatomic abnormality that leads to upper airway obstruction (such as enlarged tonsils and adenoids, severely deviated nasal septum, or nasal polyps), surgical therapy may be recommended by an otolaryngologist.
Uvulopalatopharyngoplasty (UPPP) was first described in the early 1960s as a treatment for snoring (31). This technique was subsequently applied as treatment for OSA with some success. The technique entails removal of any
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existing tonsil tissue and partial resection of the soft palate, uvula, and anterior tonsillar pillars. The airway is thus opened, allowing for increased airflow and reducing the extent of vibratory tissue to generate snoring. Although UPPP is initially quite effective in snoring resolution, long-term success rates range only from 46% to 73% (32). This may be due to transient weight loss after surgery. Most patients will lose 5 to 15 pounds after surgery because of poor oral intake after surgery. This weight loss is often not sustained and may explain why the long term success of UPPP. UPPP is an aggressive procedure that requires an approximate 3-week postoperative convalescence. Patients often complain of severe odynophagia postoperatively, and because of this only 60% of patients indicate they would undergo the same treatment again (33). Another disadvantage of UPPP is nasopharyngeal incompetence; almost one fourth of patients complain of intermittent nasopharyngeal regurgitation for up to 1 year after surgery (34).
Because of the limitations of UPPP, there has been an ongoing search for more effective and safer palatal procedures for benign snoring. Laser or cautery-assisted uvuloplasty, in which the uvula alone is truncated in the office setting under local anesthesia, has gained popularity. Uvuloplasty is associated with fewer complications than UPPP, but the technique still entails significant post procedure pain. Uvuloplasty appears to be equally effective as UPPP for snoring treatment, but multiple office excisions are often required and up to 77% of patients abandon their course of uvuloplasty therapy because of pain (35). Uvuloplasty may rarely cause postoperative bleeding and temporary nasopharyngeal regurgitation.
Other forms of surgical therapies for snoring use laser or other modalities to produce palatal stiffening (20). A newer technique involves the delivery of radiofrequency energy (Somnoplasty, Somnus Medical Technologies) with a needle electrode into various sites of the soft palate, resulting in fibrosis and stiffening (36). Radiofrequency ablation is generally safe, minimally invasive, and has few complications (37). Postoperative pain is minimal, because there is no mucosal disruption, and acetaminophen alone is often sufficient for analgesia. Radiofrequency ablation can require three to four treatments to achieve an appropriate level of snoring cessation. Large-scale trials or systematic reviews of surgical therapies for snoring are not yet available (20). This technique can also be used to address the base of tongue to reduce tissue bulk and address snoring at this anatomic site too.
Anosmia
Chemosensory disorders affect up to 2 million adults in the United States (39). Unfortunately, the sense of smell is often ignored by clinicians and it is often thought of as an inconvenience rather than a disease spectrum. Anosmia can result in significant debility, especially in individuals who rely on the sense of smell for their occupation (e.g., chefs, police officers, florists). Additionally, anosmia can be dangerous because affected individuals are unable to discern gas leaks, spoiled food, or smoke. Anosmia and its often accompanying decreased sense of taste can lead to eating displeasure and poor nutritional intake.
Pathophysiology
The sense of smell depends on olfactory chemoreceptors located high in the nasal cavity along the upper septum and cribriform plate, which underlies the anterior cranial fossa. These receptors transmit impulse back to cranial nerve I, the olfactory nerve. The odorant molecules are dissolved in the nasal mucous and presented to the neurepithelium of the olfactory nerve for processing. It is not completely understood how the normally functioning olfactory system is able to process and differentiate the multitude of odors presented to it (40). The sense of taste is a more gross perception, which is mediated by taste buds located on the tongue, soft palate, and oropharynx. The sensation of taste generated by these receptors is limited to the basic qualities of salty, sour, sweet, and bitter. Most individual perceptions of flavor and taste are in fact olfactory sensations, classically evidenced by the fact that children are often convinced to take distasteful medicine by holding their nose and preventing any olfactory input. Thus, a patient who complains of a diminished taste may in fact have hyposmia or anosmia.
There is also a chemosensory sense mediated by the trigeminal nerve, cranial nerve V, which is triggered by pungent and irritant compounds, such as hot peppers, horseradish, or mustard, and perceived as burning or irritating. This pathway is independent from the other modalities of taste and smell.
Dysfunction of smell is classified as complete or partial and is referred to, respectively, as anosmia or hyposmia. Another interesting variation of smell disturbance is phantosmia, in which spontaneous and distorted olfactory hallucinations are manifest. These phantosmias are often described in association in patients with seizure activity, psychiatric illness, and Alzheimer disease (41). Dysosmia is defined as a distorted sense of smell and is often used to describe when person senses nonexistent unpleasant odors instead of the expected smell.
Etiology
As people age, the sense of smell declines naturally, and the decline may not be perceptible because it is gradual. Patients are very sensitive to an acute decrease in their sense of smell. When evaluating a patient with diminished smell or taste, it is important to determine the duration of the complaint, whether the loss was gradual or acute, and whether the sensory loss acutely followed head trauma or an upper respiratory tract infection (a more gradual progression would accompany sinusitis, nasal polyposis,
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or an intracranial tumor). The patient's medication list should be reviewed because many medications alter the senses of taste and smell (42). Table 111.2 shows common medications that can impair the sense of smell.
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TABLE 111.2 Some Common Medications That Can Impair the Sense of Smell |
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Anosmia can be considered in two broad categories: sensorineural, in which the olfactory loss is constant, and conductive, whereby the patient may intermittently have olfactory function. Sensorineural loss signifies a loss of the neuroepithelium, damage to the olfactory nerves, or injury to the olfactory centers in the brain resulting in the inability to smell. Conductive loss is caused by the inability of odorant molecules to reach the olfactory neuroepithelium. This would be the case with an acute upper respiratory infection with nasal inflammation or nasal polyps.
Common causes of sensorineural anosmia include postviral, traumatic, and neoplastic. Postvirally induced anosmia is quite common, accounting for up to a third of anosmia cases. Patients will typically report a very severe respiratory tract infection, and as their congestion resolves they do not regain their sense of smell. The etiologic factor is damage to the olfactory neuroepithelium by the viral agent. These patients are usually not completely anosmic and have a reduced sense of smell, hyposmia. These patients also tend to be older individuals, which may suggest that the anosmia may be related to a series of viral insults throughout life, resulting in progressive anosmia.
History of head trauma including prior surgery is another important cause of anosmia. Up to 5% of patients suffering head injury will have olfactory loss, depending on the severity of the head injury (43). The mechanism of injury is typically a frontal or occipital blow, which results in stretching or sheering of the olfactory nerves as they exit the cribriform plate. Loss of consciousness, concussion, and skull fracture are not necessary to precipitate anosmia. These patients are typically younger and are often completely anosmic.
Neoplasms of the olfactory tract are uncommon causes of anosmia. Intracranial abnormalities will generally present with other symptoms and not isolated smell disturbance. Imaging studies, CT scan of the paranasal sinuses and MRI scan of the olfactory tract, can yield information about the anatomy of the olfactory tracts but infrequently discover a lesion responsible for anosmia (44). Prior radiation therapy also often results in marked dysfunction of both smell and taste (45). Rare causes include endocrine disorders such as Kallmann syndrome (hypogonadotropic hypogonadism; see Chapter 81) and Turner syndrome (46). About 20% of patients presenting with anosmia do not have evidence of the preceding causes and after a careful workup will be classified as having an idiopathic cause.
Conductive anosmia can be because of a variety of causes: chronic rhinosinusitis, nasal polyposis, nasal septal deviation, or intranasal mass. History and physical examination are keys to successful diagnosis and treatment of conductive deficits.
Evaluation
As with many disorders, a complete history and physical examination can direct the healthcare provider to a potential cause and treatment plan. Physical examination should focus on the nasal anatomy (see Chapter 33). Inspection of the anterior nasal cavities may not be adequate to completely evaluate potential causes of anosmia. If anterior anatomic abnormalities are observed or if the cause of anosmia remains unclear, referral to an otolaryngologist is indicated. Anterior rhinoscopy can miss almost half of the cases where there a conductive cause. Nasal endoscopy is more sensitive in detecting intranasal abnormalities that may be causing decreased sense of smell (43).
An appropriate next step in anosmia evaluation would be to determine if the smell disturbance is reversible. This can be done clinically by using a short course of high-dose systemic corticosteroids in a tapered fashion (e.g., oral prednisone 60 mg daily for several days, followed by tapering doses to complete a 10-day course). If the olfactory deficit results from nasal inflammation, such as allergic rhinitis or chronic rhinosinusitis, then the olfactory deficit can be temporarily reversed. If the patient does not respond to this steroid challenge, imaging studies and consultation with an otolaryngologist may be warranted, especially if the history or examination do not suggest a cause. The best initial test is a CT of the sinuses. Sinus CT will completely delineate the nasal cavity and determine if there is adequate patency of the air passages and will evaluate for associated sinusitis. CT scans are poor for evaluating soft tissue and the anterior cranial fossa. When the cause of anosmia remains obscure, a magnetic resonance image may be needed to rule out anterior cranial fossa tumors, such as meningioma, or other soft tissue neoplasms.
Olfactory Testing
Olfactory testing may be performed by an otolaryngologist to determine threshold odor identification or odor intensity. Odor identification is often tested using the University of Pennsylvania Smell Identification Test. In this test, a patient releases 40 microencapsulated odorants by rubbing designated areas on a card and answers corresponding questions about the identities of the odors. The
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answers are graded using normative data based on gender and age.
Treatment and Management
Inflammatory nasal disease is a common entity affecting a patient's sense of smell. Some patients will have subacute sinusitis in which the nasal symptoms (congestion, facial pain, and nasal drainage) are subtle and not appreciated, and the primary complaint of the patient is anosmia or a distorted sense of smell. CT (see Evaluation) may show chronic sinusitis, and subsequent treatment of this sinusitis (seeChapter 33) can, in many cases, restore normal olfaction. Nasal polyposis treatment either with medications or surgical removal by an otolaryngologist may restore normal olfaction, although polyps often recur and olfaction may be compromised early as polyps regrow.
The prognosis for recovery of disorders of odorant conduction (polyps, septal deviation, or rhinosinusitis) causing poor delivery of olfactants to the neuroepithelium is good if the underlying nasal pathology can be remedied. Sensorineural loss caused by damage to the olfactory neurons after trauma or viral infection has a much less favorable prognosis. Approximately 30% of patients with posttraumatic anosmia improve partially or completely within the first year, most within the first 12 weeks (47).
Vitamin A and zinc are commonly prescribed for anosmia; however, their efficacy is not well supported (48). Newer and not yet standardized approaches for disorders of olfaction, such as olfactory epithelial biopsy by an otolaryngologist, with histopathologic and electron microscopic evaluation, are being studied in some centers.
It is important to counsel patients who suffer persistent anosmia about coping maneuvers, such as having appropriate gas and smoke detectors in their home and having adequate assistance with food preparation and evaluation for spoiled foods. Detecting the need to change diapers for small children and cleaning up after pets may be problematic for patients with anosmia. In food preparation, care must be taken not to overuse salt and sugar to add flavor because these additives may precipitate other medical problems. The use of temperature (i.e., hot or cold), texture, and spices in food preparation can be helpful to make food more palatable.
Hoarseness
Because the voice is so important to interpersonal communication, hoarseness causes a great deal of patient distress. Hoarseness is an imprecise term used to describe any alteration in a patient's normal voice quality. Otolaryngologists and speech pathologists use the termdysphonia to more accurately portray abnormal voice quality and localize the disease process to the laryngeal structures. Hoarseness is the symptom and dysphonia is the corresponding sign. A dysphonic patient will have a breathy, strained, rough, raspy, tremulous, or weak voice. A complaint of hoarseness should be evaluated thoughtfully, because it may represent a significant underlying pathology. Appropriate referral to an otolaryngologist for direct visualization of the laryngeal structures is warranted if hoarseness persists longer than a few weeks.
Pathophysiology
The evaluation of hoarseness depends on an understanding of the pertinent anatomy and normal physiology involved with voice production. The vocal tract can be thought of in three separate compartments: the lungs, the larynx, and the oral cavity. The lungs are the power source for voice production. The true vocal folds of the larynx are the anatomic site for sound production. Sound is generated by airflow through closely approximated vocal folds. The quality of the sound produced is determined by multiple factors, including the degree of vocal fold opposition, the tension in the laryngeal musculature, and the properties of the vocal fold epithelium itself (49). In the oral cavity and oropharynx, sound produced in the laryngeal structures is further modified by the tongue, lips, and teeth. Strictly speaking, the laryngeal phase of sound production is the site of origin of true hoarseness and dysphonia. The pulmonary portion of vocal production will determine the strength of the sound produced, and the oral cavity is primarily involved in articulation and the refinement of the sound to produce speech. An alteration in the oral phase of vocal production is more appropriately termed dysarthria.
The larynx is composed of a cartilaginous skeleton, including the epiglottis, thyroid cartilage, cricoid cartilage, and paired arytenoid cartilages. This cartilaginous framework provides for continuity of airflow from the trachea, through the glottis, to the oral tract. The soft tissue structures in the larynx are composed of laryngeal musculature, including the vocal fold abductors, adductors, and tensors. These muscular structures adjust the coaptation and tension of the vocal folds, which is a major determinant of the quality of sound production. Any alteration in the innervation or function of the laryngeal musculature will result in dysphonia. The mucosal epithelium for most of the larynx is made up of columnar epithelium and mucous-secreting goblet cells, which promote a moist environment for efficient voice production. The true vocal folds are lined with squamous epithelium, which overlies a loose lamina propria (50). This histologic arrangement allows the subglottic airflow to traverse the larynx and produce a mucosal wave in the overlying epithelium, generating sound. Increased viscosity of the loose lamina propria of the vocal folds from dehydration, inflammation, or scarring will result in the need for an increase in the pressure of the airflow through the larynx to establish the vibratory phase of the vocal folds. Adequate hydration of this gelatinous lamina
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propria layer and the maintenance of a lubricated mucosa of the laryngeal structures are important in determining the quality of laryngeal sound production.
Many local and systemic disorders alter these important elements of the laryngeal environment. With the exception of the cricothyroid muscle, which provides some vocal fold tension, the laryngeal muscles are innervated by the recurrent laryngeal nerves. It is an important anatomic consideration that the recurrent laryngeal nerves, after branching from the main trunks of the vagus nerves, travel in slightly different pathways to ultimately provide laryngeal innervation. The right recurrent laryngeal nerve loops around the right subclavian artery, whereas the left recurrent laryngeal nerve loops around the arch of the aorta, and both travel cephalad to enter the larynx below the thyroid cartilage. The left recurrent laryngeal nerve (but usually not the right) traverses the mediastinum and may be impacted by a variety of mediastinal lesions. Any lesion along the course of the recurrent laryngeal nerves may result in paralysis of the ipsilateral vocal fold. It is also important to know that the motor fibers of the vagus nerve originate in the nucleus ambiguous of the medulla, and any neurologic process at the brainstem level can impact vocal function (51). Sound production also requires the coordination of efforts between the expiratory thoracic musculature and laryngeal musculature.
Differential Diagnosis and Evaluation
Table 111.3 shows the differential diagnosis of hoarseness as broad. A careful history and physical examination, and sometimes visualization of the laryngeal structures (see Snoring, Evaluation, Hoarseness, and Pathophysiology), allows a diagnosis to be made in most cases.
History
Initial evaluation starts with characterizing a patient's voice complaints. A rough and raspy sound suggests a mucosal irregularity, whereas a breathiness or weakness of the voice suggests an incomplete closure of the true vocal folds (e.g., vocal fold paralysis or an endolaryngeal mass preventing apposition of the true vocal folds). It is also important to listen carefully to the patient to determine if the complaint of hoarseness is related to an articulation or resonance disturbance of the oral phase of speech or to a lack of speech volume resulting from an inadequate pulmonary phase of voice production. The duration of symptoms is important, because acute voice alteration may represent a self-limited process such as a viral upper respiratory tract infection, whereas chronic and progressive hoarseness is more concerning for an underlying condition. Fluctuation of the hoarseness implies that a fixed lesion (e.g., polyp, nodule, or tumor) is not present.
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TABLE 111.3 Common Problems That Can Cause Hoarseness |
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Any inciting event, such as recent voice abuse, neck trauma, or intubation, should be elicited. Recent voice abuse suggests that a vocal fold hemorrhage or nodules are the cause of the persistent hoarseness. Hoarseness that develops after a single event of excessive vocal use or coughing often represents an acute vocal fold hemorrhage which will resolve over the course of weeks. Vocal fold polyps are usually pedunculated masses and are often associated with smoking. They are located on the free edge of the true vocal fold and are extremely common benign lesions causing hoarseness in the adult patient. Neck trauma can result in arytenoid dislocation, laryngeal fractures, or even recurrent laryngeal nerve paralysis. Recent endotracheal intubation can also produce persistent hoarseness from arytenoid dislocation, recurrent nerve paresis (or paralysis), traumatic laryngeal granulomas, or atrophic changes of the glottis.
Voice complaints that are worse in the morning and resolve during the day are suggestive of laryngeal irritation from gastroesophageal reflux disease (GERD). Patients with laryngeal manifestations of GERD often do not have the classic symptoms of heartburn and indigestion (52). Associated symptoms should also be carefully investigated. Dysphagia or aspiration associated with hoarseness may indicate that a laryngeal tumor is present or that a neurologic process, such as recurrent laryngeal nerve paralysis, is the culprit. Persistent otalgia with a normal ear examination is also worrisome for referred pain from a supraglottic or glottic malignancy. Such pain is referred to the area of the ear via the vagus nerve, which, in addition to supplying the laryngeal structures, contributes to the innervation of the external ear canal.Allergic rhinitis or chronic sinusitis with postnasal drainage may also promote chronic laryngeal irritation and inflammation leading to hoarseness. It is important to elucidate past surgical history, because an endotracheal intubation may damage the larynx, and may place the recurrent laryngeal nerve at risk (Table 111.4).
Hypothyroidism may produce significant hoarseness because of edema of the laryngeal submucosal lamina propria (53). Medications also may be implicated in voice disturbances. Aspirin, nonsteroidal anti-inflammatory agents, and anticoagulants may promote vocal fold
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hemorrhage. Antihistamines and diuretics may produce upper airway dryness, which also will adversely affect the voice (54).
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TABLE 111.4 Surgical Procedures with a Risk of Laryngeal Denervation |
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Social history is also important in evaluating patients with hoarseness and dysphonia. Exposure to tobacco smoke, ethanol, or environmental pollutants directly causes laryngeal irritation and increases the risk for laryngeal carcinoma. Cigarette smoking and alcohol also contribute to the drying of secretions and chronic inflammation. An understanding of the patient's activities that involve the voice (e.g., a teacher, singer, or avid sports fan) is pertinent to diagnosis and to treatment. Patients who use their voice professionally often require early and aggressive intervention and specialized long-term care. The presence of a loud work environment or frequent contact with individuals who have hearing loss may require a patient to speak in an unnaturally loud voice, which can promote the formation of vocal fold nodules. Such patients should be referred to an otolaryngologist and may require therapy with a speech language pathologist.
Physical Examination
A focused head and neck examination should be performed on patients who present with a complaint of hoarseness. Otoscopic examination helps to identify whether associated ear complaints are due to otologic pathology or may be referred from a laryngeal process. Nasal examination may demonstrate abnormal secretions, polyps, or purulence, suggesting chronic rhinitis or sinusitis. Oral cavity and oropharyngeal examination may reveal “cobble stoning” or lymphoid hypertrophy of the posterior pharyngeal wall suggestive of chronic postnasal drainage. The neck should be carefully palpated for thyroid masses, diffuse thyromegaly, lymphadenopathy, or other lesions (e.g., carotid body tumor). Cranial nerve evaluation, to determine if abnormalities are present that may be associated with a vagal neuropathy, is also indicated. The presence or absence of manifestations of hypothyroidism (see Chapter 80) should be assessed.
When a current or recent upper respiratory tract infection is identified and hoarseness has been of a short duration (less than 2 weeks), it is reasonable to defer direct laryngeal examination. In this situation, hoarseness likely is a result of acute viral laryngitis, which should resolve in a few weeks. If it does not, visualization of the larynx is needed and referral to an otolaryngologist is appropriate.
Examination by an Otolaryngologist
A patient with hoarseness that has persisted for more than 2 to 3 weeks should undergo visualization of the laryngeal structures by an otolaryngologist. Any gross abnormality of the larynx, such as polyps, nodules, tumor, or vocal fold motion impairment, usually can be identified by indirect mirror examination. In approximately 15% of patients, the laryngeal structures are not fully visualized because of the patient's anatomy (e.g., an overhanging or retroflexed epiglottis) or an overactive gag reflex. In these cases, for complete visualization of laryngeal structures, the otolaryngologist will use transnasal flexible fiberoptic laryngoscopy. This procedure is easy to perform, is well tolerated, and usually lasts less than 5 minutes. This endoscopic examination may be recorded and documented by video. Occasionally, patients need to be taken to the operating room for direct laryngoscopy. This relatively brief procedure requires general endotracheal anesthesia and intubation but allows a very detailed microscopic examination of the entire larynx and biopsy of suspicious lesions.
Ancillary Testing
Thyroid function tests should be done if hypothyroidism is suspected (see Chapter 80). Video stroboscopy is a specialized examination performed by an otolaryngologist that requires a strobe light which is synchronized to the patient's voice (55). The larynx is visualized under a stroboscopic light source that displays slow motion and allows a very finely detailed examination of the mucosal wave of the true vocal fold. This examination may reveal subtle neurologic deficits with inadequate glottic closure, submucosal cysts, and mucosal abnormalities such as dysplasia or tumor. In the event that a vocal fold paralysis is noted and there is no previous surgical procedure that would put the recurrent laryngeal nerve at risk, an imaging study such as a CT or magnetic resonance image (from the skull base through the entire course of the recurrent laryngeal nerve), should be performed to rule out a neoplastic process. It is important that evaluation of left true vocal fold paralysis include the aortic arch, because this represents the course of the recurrent laryngeal nerve. If dysphagia is present, a barium swallow or esophagoscopy may be pertinent to evaluate for an esophageal neoplasm, which also may be implicated in recurrent laryngeal nerve paralysis.
Management
Treatment for hoarseness depends on its cause (Table 111.3). Inflammatory processes and acute laryngitis are
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often self-limited. It is important to note that true vocal folds have no lymphatic elements so resolution of edema and restoration of a truly normal voice may take some time. The patient should maintain adequate hydration and avoid the use of antihistamines and decongestants, which may have a drying effect and delay recovery. If the symptom persists and there is an associated sore throat, treatment for chronic or relapsing pharyngitis may be indicated (see Chapter 33).
Laryngeal complications of gastroesophageal reflux are best managed by use of proton pump inhibitors (PPI) in a higher dose than used for usual esophageal GERD, because this is an atypical or extraesophageal manifestation of GERD that requires more aggressive therapy (seeChapter 42). Patients should also be educated in reflux precautions: dietary modification, weight loss, smoking cessation, avoidance of activity immediately following meals, separation of the last meal from sleep by 3 to 4 hours, and elevating the head of bed. Patients will often improve after a 6- to 8-week course of PPI therapy, but a 6-month course of therapy is recommended for these symptoms to resolve completely and the laryngeal examination to normalize. Chronic laryngitis from toxic exposure such as cigarette smoke is best treated by smoking cessation or the elimination of other inciting agents. Vocal hygiene measures are important adjunctive treatments for all causes of hoarseness (see Vocal Hygiene).
Anatomic Lesions
The treatment of vocal fold polyps is simple surgical excision by suspension microlaryngoscopy. Vocal fold nodules often occur on the free edge of the vocal fold secondary to voice abuse. These nodules often resolve with appropriate speech therapy and voice rest. If vocal nodules progress to fibrotic lesions, they may need to be excised surgically, as resolution by conservative measures is unlikely. Postintubation lesions such as true vocal fold granulomas are present in the posterior glottis where the endotracheal tube was positioned. A granuloma can also form in the posterior glottis with gastroesophageal reflux or extreme vocal abuse or in patients with chronic and marked cough syndromes. These granulomas can usually be ameliorated by speech therapy and inhaled steroids combined with aggressive treatment for gastroesophageal reflux to reduce further mucosal injury. These lesions, unless they are obstructing the airway, rarely require surgical treatment. Human papilloma virus can cause recurrent respiratory papillomatosis that affects the glottis; although they are present in all age groups, they are usually seen in children. This viral illness has no curative medical therapy at present. The recommended therapy is frequent surgical excisions using a carbon dioxide laser, microsurgical debrider, and microlaryngeal instruments. These lesions will recur, and serial excisions are required. Rarely, papillomas can undergo malignant transformation.
Neoplastic lesions, such as laryngeal tumors, cause hoarseness from the tumor mass itself, interruption of the normal mucosal wave, or by contiguous spread and true vocal fold paresis or paralysis. These lesions are often easily detected on laryngeal examination and require operative biopsy for a tissue diagnosis. Treatment often requires surgical excision, irradiation, chemotherapy, or a combination of the three modalities.
Vocal fold paralysis with no obvious etiology (such as a previous surgical procedure that placed the recurrent nerve at risk) requires workup (see Examination by an Otolaryngologist and Ancillary Testing) to rule out an underlying neoplasm anywhere along the tract of the recurrent laryngeal nerve. If no lesions are found and the paralysis is accompanied by significant hoarseness with a harsh breathy voice or aspiration, a procedure can be performed to medialize this vocal fold toward the normal opposite side, either by an injection into the true vocal fold or by operative introduction of a spacer (medialization laryngoplasty) (56).
Vocal Hygiene
Patients with any process causing hoarseness should be educated about voice abuse. They should be advised to avoid straining their voices by using a loud voice or harsh whispering. It is important for these patients to maintain adequate hydration (e.g., six or more large glasses of water each day). Caffeine and alcohol should be avoided because of their drying and diuretic effects. Antihistamines and drugs with anticholinergic side effects should also be avoided, because they cause excessive dryness. Guaifenesin (e.g., Robitussin) may also be helpful as a mucolytic.
Hiccups
Hiccups are a common phenomenon that affects, at some time or another, most individuals. Usually, a bout of hiccups is a mild short-lived annoyance that resolves spontaneously. When hiccups are prolonged (more than 48 hours) or intractable (more than 1 month) it is important to rule out a serious underlying medical illness. Hiccups, sometimes referred to as singultus, involve the involuntary spasmodic contraction of the diaphragm. This spasmodic contraction of the diaphragm begins at inspiration, which is suddenly checked by closure of the glottis, giving the characteristic sound of hiccuping (57).
Pathophysiology
For unknown reasons, persistent hiccups in men are found to be caused by a specific organic etiology in over 90% of cases, whereas specific causes are less likely to be found
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in women after detailed evaluation (58). The afferent portion of the hiccup reflex arc arises from the phrenic and vagus nerves and the thoracic sympathetic chain. There is no discrete central connection for this reflex arc, although it appears to be located somewhere in the spinal cord, between segments C3 and C5. The phrenic nerve provides the efferent limb of the reflex arc. Fluoroscopy reveals that hiccups are most often unilateral, with the left diaphragm being more frequently involved than the right (59). The frequency of hiccups decreases as arterial PCO2 rises, and this is the physiologic basis for the popular hiccup treatment of breathing into a paper bag. The etiology of self-limited hiccups appears to be related mostly to ingestion of food and alcohol and gastric distention. These self-limited hiccups are thought to result from peripheral irritation of the branches of the vagus and phrenic nerve in the upper abdomen (57). Local irritation of the vagus nerve and diaphragm (e.g., by pneumonia, aortic aneurysm, pericarditis, abdominal abscesses, and various thoracic and abdominal tumors) appears to be an important mechanism for development of intractable hiccups. Additionally, central nervous system (CNS) lesions, such as multiple sclerosis, meningitis, and CNS neoplasms can also provoke hiccups. Psychogenic factors also may be the cause of intractable hiccups. Stress, conversion reaction, anxiety states, and malingering may all be psychogenic factors in the etiology of hiccups.
Evaluation
A focused history to determine the onset, precipitating factors, and duration of the hiccups is important. Associated medical events, such as trauma, surgery, or recent acute illness, should be elicited. Weight loss, fatigue, or night sweats suggest an underlying malignancy. The presence of hiccups during sleep usually indicates an underlying organic cause, whereas if the hiccups cease during sleep a psychogenic or idiopathic etiology is more likely (58). Any previous bouts of hiccups and the response to therapy should be reviewed and may suggest precipitants and effective treatments.
The head and neck, chest, and abdomen should be examined. The ears should be examined for any external auditory canal abnormality, which may trigger hiccups by irritating the vagally supplied external auditory canal skin. Pharyngitis or oropharyngeal inflammation may also be a trigger for hiccups. A cervical process, such as a thyroid tumor or malignant lymphadenopathy along the course of the recurrent laryngeal nerve in the neck, may also provide a trigger for these episodes. Examination of the chest is important to assess for infection, thoracic aortic aneurysm, pericarditis, or pulmonary or mediastinal tumor. The abdomen should be evaluated for an acute process such as bowel obstruction or abscess or an underlying neoplasm. A detailed neurologic examination should be performed, because early multiple sclerosis is thought to be one of the most frequent neurologic causes of intractable hiccups in young adults (60).
A chest radiograph may be helpful in evaluating patients with hiccups by ruling out pulmonary, mediastinal, or cardiac sources of phrenic, vagus nerve, or diaphragmatic irritation (61,62). Because hyponatremia may cause hiccups, the serum sodium concentration should be measured (63). Other studies may be indicated based on the findings identified by the history and physical examination.
Management
The most important consideration in the management of prolonged and intractable hiccups is to determine the etiology and correct any underlying pathology if possible. Once the instigating cause has resolved, the hiccup bout should also abate. In cases where the cause is idiopathic or not immediately apparent, therapies should be initiated that are specifically directed at terminating hiccups.
Initial efforts to treat hiccups usually involve physical maneuvers. These are performed in an attempt to interrupt the reflex arc. Many of these are folk remedies, such as swallowing rapidly and sequentially small sips of water or inducing a startle reaction. The previously mentioned breathing into a paper bag may also be tried. A more established maneuver that can be performed by the clinician is stimulation of the nasopharynx with a red rubber catheter; cessation rates of nearly 100% have been reported with this technique (59).
If physical maneuvers fail, then pharmacologic intervention should be attempted. Unfortunately, most studies related to the use of pharmacologic agents for hiccups have involved only small numbers of patients or relied on anecdotal clinical observations. Chlorpromazine hydrochloride is the most commonly used drug. The mechanism of action is unclear. However, a cure rate for intractable hiccups of almost 80% has been reported (64). Care must be taken when administering chlorpromazine intravenously (25 to 50 mg in 500 to 1,000 mL of normal saline over several hours) or intramuscularly (25 to 50 mg), because postural hypotension is common. If hiccup cessation is achieved with initial parenteral treatment, then 25 to 50 mg orally twice daily is recommended for 7 to 10 days (65).
Metoclopramide is the second drug of choice for intractable hiccups, with 10 mg given as an intravenous infusion over 1 to 2 minutes. If successful, an oral maintenance dose of 10 mg four times daily may be used for 7 to 10 days. Success rates of approximately 80% with metoclopramide have been reported (66). Many anticonvulsants also have been reported to be effective for treating hiccups, including phenytoin, phenobarbital, carbamazepine, and valproic acid. Phenytoin is the most efficacious in patients who have a central neurologic cause of their hiccups (67). In patients with multiple sclerosis, carbamazepine
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has been reported to be an effective agent for treating associated hiccups (68).
Less conventional therapies, such as hypnosis, psychotherapy, and acupuncture, may be tried if physical maneuvers and drug therapy fails (69,70). Surgical disruption of the phrenic nerve is considered only as a last resort. Before embarking on this mode of therapy, it is important for the otolaryngologist to identify which leaflet of the diaphragm is involved. An initial attempt at blocking the phrenic nerve with a local anesthetic usually should be performed to determine if phrenic nerve surgery would be fruitful.
Specific References*
For annotated General References and resources related to this chapter, visit http://www.hopkinsbayview.org/PAMreferences.
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