Definitions

Fungal sinusitis, when used loosely, can be a misleading term. It actually refers to a spectrum of fungal-associated diseases of the nose and paranasal sinuses, each with a unique presentation and management implications (Table 1). When communicating with TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses ( is used to indicate controversy) TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses ( is used to indicate controversy) topical nasal steroids, antifungals...

AIDSHuman Immunodeficiency Virus

Human immunodeficiency virus (HIV) is a retrovirus that is transmitted through blood or other body fluids. As the HIV destroys the CD4+ helper T-cells, the patient inevitably becomes immunodeficient, at which point the patient is considered to have acquired immunodeficiency syndrome, better known as AIDS. The conversion to the diagnosis of AIDS is met when the CD4+ count drops below 200 or when the patient exhibits any immunodeficient-defining symptom such as Kaposi's sarcoma, candidiasis, CMV...

Airways and Lungs

Respiratory tract symptoms may occur in about 9 of patients with GCA and are the initial manifestation in 4 of patients (13). GCA should be considered in an elderly patient with new onset of cough, hoarseness, or throat pain without obvious cause. In a series of 16 patients with respiratory tract manifestations, cough was noted in 11 patients, while 15 patients had a sore or tender throat, 3 had hoarseness, 1 had a sensation of choking, 4 had a sore tongue, and 2 complained of chest pain (13)....

Allergy and Dermatitis

There is clear evidence that otitis media with effusion is highly related to an allergic diathesis. When this converts to chronic draining otitis media, the allergic component would seem to still be relevant, although direct evidence is scant (17-19). Therefore, the surgeon must consider allergy evaluation, based on a patient history of other allergic diatheses, especially of the unified respiratory epithelium. Patients with chronic draining ear and allergic rhinitis, chronic rhinosinusitis,...

Angioedema

Angioedema is an uncommon and usually self-limited swelling of the deep dermis. It can, however, be complicated by potentially serious adverse consequences, including death. Although IgE-mediated mechanisms are not the major cause of angioedema, they are among the few that can be clearly identified, and thus this topic will be discussed here. Angioedema occurs together with urticaria approximately 85 of the time in 15 of instances, it occurs alone. Angioedema (Fig. 1) is the abrupt and...

Autoimmune Diseases

Autoimmune inflammation of the inner ear may occur in isolation as an organ-specific process or may be part of a systemic autoimmune syndrome (20). Evidence is mounting to support the existence of an organ-specific cochlear autoimmune disease. The presence of antibodies to cochlear antigens, such as the 68 kD inner-ear antigen, as well as an impressive response to immunosuppressive medications, implicate autoimmunity as the underlying pathologic process in some patients with idiopathic...

Clinical Manifestations

The clinical findings in acute KD evolve over the first week after onset of fever and include the features described in Table 1. The onset of fever is usually abrupt, without a prodrome, and has a spiking remitting pattern throughout the day. More than 90 of patients develop bilateral, symmetrical dilatation of vessels in the bulbar conjunctiva with limbal sparing, which creates a halo effect around the iris (24). There is no exudate, and conjunctival biopsy reveals no local inflammatory...

Clinical Manifestationssystemic

An extensive discussion of the systemic manifestations of HIV AIDS would be quite lengthy and beyond the scope of this chapter. Note will be made of the PHI, which presents with fever, myalgias, pharyngitis, arthralgias, headache, and malaise. Nonpruritic maculo-papular rash of the face and trunk is reported in 30 to 70 of cases (10) Generalized lymphadenopathy, nausea, vomiting, neurological complications, oral ulceration, and candidiasis might be observed. Since PHI can present before...

Complications Prognosis And Outcome

Observations on the wide array of head and neck manifestations seen in this group of rheumatic diseases have been published as case reports and short case series, and little is available from the literature to assess long-term outcomes. Some clinical events, such as the facial rashes in SLE, serve a diagnostic purpose and are rarely seen later as the disease is treated or as it unfolds. Many times, these head and neck manifestations are overlooked by both the patient and the physician because...

Complications And Prognosis

Existing longitudinal studies of persons with SS show that the clinical course of SS is slowly progressive and is life altering, rather than life threatening, for most patients. One study found no overall increased mortality in a population of individuals with SS when compared to controls (12). Those with SS also do not have increased cardiovascular mortality, as do individuals with SLE, despite the apparent overlap in signs and symptoms. Salivary glands in SS begin with focal lymphocytic...

Complications Prognosis And Prevention

Mucor is a disease with protean findings and diverse outcomes. These infections are often fatal, although patients with limited sinonasal disease may have a better prognosis, especially with early diagnosis, aggressive surgical resection, and aggressive antifungal therapy. There is a markedly poorer prognosis for those patients with hemiplegia, facial necrosis, and nasal deformity (12). Among patients with RCM, 70 of survivors are left with residual defects (14). In a meta-analysis by Yohai et...

Diabetes Mellitus

Gastrointestinal (GI) symptoms such as nausea, vomiting, and diarrhea are relatively common in diabetes mellitus. Population-based studies in Australia have shown that esophageal symptoms are also more common in diabetics than in nondiabetic control patients (18). The underlying pathophysiology is presumed to be a neuropathy there is a progressive axonal atrophy and segmental demyelination of the parasympathetic fibers in the esophagus. The most typical motility abnormality is ineffective...

Diagnosis And Treatment The Infectious Etiologies

Both the external ear and the middle ear are susceptible to infection and both can present with otorrhea. External-ear infections (acute otitis externa) are most often caused by irritation to the ear, either from manipulation (fingers, Q-tips, etc.) or from environmental factors (water, debris, etc.). External-ear infections will present with ear pain and drainage. In bacterial otitis externa, the discharge is typically purulent. The main pathogens are...

Diagnosis

The diagnosis of CS is based on the presence of both ocular inflammation and vestibulo-auditory dysfunction. IK and other anterior ocular segment problems are diagnosed by slit-lamp examination, while posterior ocular segment disorders are recognized by ophthalmo-scopy. Retinal vascular abnormalities may be revealed using fluorescein angiography. Vestibuloauditory disease may result from a toxic metabolic disturbance or a peripheral (labyrinth, vestibule, or cochlea) or central (brainstem,...

Differential Diagnosis

In NUG, the interdental papillae undergo necrosis beginning at the tips and extending toward the crest of the dental alveolar bone. As a result of the soft-tissue necrosis, the interdental gingival papillae exhibit a punched out blunted, crateriform area covered by a fibrinonecrotic membrane (Fig. 39). The gingiva are exquisitely sensitive and often exhibit spontaneous bleeding. A fetid odor accompanies the soft-tissue necrosis. There may be associated systemic symptoms of fever, malaise, and...

Epidemiology

In the United States, roughly 22,000 cases of CSD are diagnosed annually, with 2000 resulting in hospital admissions. Over 90 of individuals with the illness report some form of contact with cats, often kittens. The male female ratio is 3 2. In 80 to 90 of cases, patients are younger than 21 years (7). TABLE 2 Cat-Scratch Disease Key Points Benign and self-limited infection with a course lasting 6-12 wk Bartonella henselae usually introduced into host via scratch from the claw or tooth of a...

Epidemiology Of Tinnitus

It has been found through epidemiologic studies that although chronic tinnitus is quite common and is found in both men and women, young and old, it is typically the elderly male who has spent a lifetime working in an industrial job who is most commonly diagnosed with tinnitus (1), pp. 16-41 . Interestingly, across multiple epidemiologic studies from different countries, the prevalence of tinnitus shows a strong correlation with level of hearing loss. The more severe the hearing loss, the...

F

FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source Courtesy of the International AIDS Society-U.S. A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source...

FIGURE 1 Linear gingival erythema Source Courtesy of the International AIDS SocietyUSA From Refs 3 4

Also known as HIV gingivitis, a fiery red linear discoloration of the gingival margin can be seen in HIV disease, even without significant plaque formation (Fig. 1). Patients complain of spontaneous bleeding or are asymptomatic. Referral to an HIV dental specialist is recommended and usually involves debridement, local care, and systemic antibiotics. Necrotizing ulcerative periodontitis and stomatitis. Rarely encountered in the asymptomatic HIV-infected individual,...

Fungal Infections in the Immunocompromised Host

For the most part, the Mucorales are considered to be opportunistic pathogens. They require a breakdown in the immune defenses, particularly disease processes that lead to neutropenia or neutrophil dysfunction. Although neutrophil dysfunction induced by ketoacidosis underlies the majority of cases of human zygomycosis, neutropenia induced by bone marrow suppression during chemotherapy or immunosuppression induced following transplantation is causing a growing proportion of cases. Specifically,...

Imaging Studies in Mucormycosis

Maxillofacial CT scan is used for initial investigation in rhinocerebral infection. The CT scan may demonstrate ethmoid and sphenoid mucosal thickening or sinusitis as well as orbital or intracranial extension and is valuable in planning surgical debridement. Magnetic resonance imaging (MRI) with enhancement may be helpful in assessing patients with allergic fungal sinusitis and in patients in whom invasive fungal sinusitis is suspected. MRI is helpful in evaluating CNS spread in invasive...

Introduction

Due to the complexity of the balance mechanism in the human body, a variety of systemic diseases can create a sense of dizziness or imbalance. In order to maintain postural control and gaze stability, the body employs sensory input, central integration, and motor output to create appropriate responses to changes in the visual world and support surface. Accordingly, diseases that alter or destroy one or more sensory inputs to the brain, interfere with brainstem and cortical integration and...

Leukemia

AML is treated with intensive chemotherapy. The standard regimen is an anthracycline plus Ara-C (the three plus seven regimen). Following remission, consolidation, often with high-dose ARA-C, is administered. Depending on risk stratification, allogeneic or autologous transplant may be appropriate in selected patients. Acute promyelocytic leukemia (FAB M3) is treated with ATRA in addition to chemotherapy. Induction is followed by consolidation and then a maintenance regimen. There is no benefit...

Malignant Melanoma

Morton John Wayne Cancer Institute at Saint John's Health Center, Santa Monica, California, U.S.A. Division of Otolaryngology, Head and Neck Oncology Program, Moores Cancer Center, University of California, San Diego, California, U.S.A. Clinical Manifestations 274 Loco-Regional Disease (Nodal Assessment) 278 Complications and Prognosis 281 Mucosal Melanomas Special Considerations 281

Mixed Connective Tissue Disease

Mixed connective tissue disease (MCTD) is a relatively rare syndrome with clinical features that overlap with systemic sclerosis, systemic lupus erythematosus, idiopathic inflammatory myopathies, and rheumatoid arthritis (Chapter 1). The typical clinical features are Raynaud's phenomenon, polyarthritis, myalgias, swelling of the hands, and esophageal dysfunction. Unlike systemic sclerosis, patients with MCTD may respond to corticosteroids. The primary clinical esophageal symptoms are heartburn...

Mucosal Melanomasspecial Considerations

Mucosal melanomas of the head and neck are rare, with only about 1000 cases reported. Of more than 84,000 melanoma cases in the National Cancer Data Base, only 1.3 were mucosal, and 55 of that subgroup arose in the head and neck (15). The majority of head and neck mucosal melanomas are sinonasal in origin, and the others are primarily from the oral mucosa. Among the sinonasal tumors, 81 arise in the nose and 19 in the sinuses, although the size of some of these tumors makes it difficult to be...

Myeloproliferative Disorders

PRV is suspected when an elevated hematocrit is found. Confirmation of an elevated red blood cell mass has been advocated as necessary but is becoming increasingly difficult to obtain in most laboratories, and there are concerns regarding its sensitivity. For females and males, a hematocrit above 50 and above 56 , respectively, is associated with an elevated red-cell mass, and this measurement can sometimes be used as a proxy for the red-cell mass. Secondary causes of polycythemia must always...

Pathophysiology

Patients with RP demonstrate both cell-mediated and humoral immunity against extracellular matrix components of cartilage, including type II, IX, and XI collagens, matrillin-1, cartilage oligomeric matrix protein, and proteoglycans. Evidence for cellmediated autoimmunity includes demonstration of T cells directed against type II collagen in patients with RP. Additionally, T cell clones have been isolated from an RP patient which were specific for an immunodominant epitope of type II collagen...

Pemphigus And Pemphigoid

Pemphigus and pemphigoid are autoimmune diseases that produce blistering of skin and or mucosa. In pemphigus, intraepithelial blistering is caused by destruction of desmogleins that connect epithelial cells. Its most common variant, pemphigus vulgaris, begins with ulcerating lesions in the mouth, as the outer epithelium of the blister sloughs. Later, skin is also involved. In pemphigoid, the basement membrane is attacked, resulting in subepithelial blisters. Mucosal involvement nearly always...

Porphyrias

Porphyrias are a group of metabolic disorders characterized by defects in the synthesis of heme, a metalloporphyrin that is the product of porphyrin metabolism. Presentation of the disorder typically involves abdominal pain, vomiting, constipation, hypertension, tachycardia, photosensitivity, psychosis, and neuropathy. Neuropathy is estimated to affect 10 to 40 of patients and is primarily motor (126). Cranial neuropathies have been described but facial paralysis is rare (127). Although the...

References

Pharyngitis. In Bailey BJ, Calhoun KH, Derkay CS, et al., eds. Head and Neck Surgery-Otolaryngology. Vol. 1. 3rd ed. Philadelphia, PA LippincottRaven, 2001 543-554. 2. Cummings CW, Flint PW, Haughey BH, et al. Anatomy. In Cummings CW, Flint PW, Haughey BH, et al., eds. Otolaryngology-Head and Neck Surgery. 4th ed. Philadelphia, PA Mosby, Inc., 2005 4135-4165. 3. Bisno AL. Acute pharyngitis. N Engl J Med 2001 344(3) 205-211. 4. Huovinen P. Causes, diagnosis...

Summary

With the increasing prevalence of diabetes and immunosuppressive conditions, zygomy-cosis has emerged as an important fungal infection. The increasing number of organ transplantations and new immunosuppressant therapies also may be changing the demographics of this disease. In the setting of diabetes mellitus, hematological or solid-organ malignancies, transplantation, neutropenia, steroid therapy, and other immunocompromising conditions, the Mucorales tend to produce angioinvasive disease....

Rheumatoid Arthritis

RA, discussed at length in Chapter 1, is a symmetric autoimmune polyarticular arthritis. It also affects nonarticular structures. Patients may have vasculitis, pulmonary fibrosis, and inflammatory changes in ligaments, tendons, and fascia. Of patients with RA, 25 to 30 have cricoarytenoid arthritis, with symptoms of hoarseness, globus, odynophagia, and pain with speaking or coughing. If the joints become fixed in an adducted position, the airway can be severely obstructed. Cricoarytenoid

Sarcoidosis

This is an idiopathic chronic granulomatous disease that occasionally affects the larynx. Peak onset is from the age of 20 to 40 years. There is a higher incidence in African Americans, Puerto Ricans, and Scandinavians, and it is sometimes familial. The pathogenesis of sarcoidosis is unknown. One theory is that it results from a mycobacterial infection. It is usually systemic, most commonly including the lungs and hilar lymph nodes. Isolated local involvement is rare, so head and neck...

Systemic Lupus Erythematosus

FIGURE 2 Rheumatoid nodule on left vocal fold with reactive changes on right vocal fold. SLE is a common autoimmune connective-tissue disease affecting 1 in 1000. It is much more prevalent in young females, with a female-to-male incidence of 9 1. It affects many organ systems. Skin rash is a very common presentation, typically appearing in the malar areas following sun exposure. Oral ulcerations develop in 40 of patients. Other systemic manifestations include myocarditis, nephritis,...

TABLE 1 Mechanisms of Hoarseness

Mucosal inflammation edema Mass lesions of the vocal fold Mechanical limitation of vocal fold motion Laryngeal paralysis Neuromotor dysfunction Psychogenic factors with epistaxis and or chronic sinusitis. The larynx is involved in 6 to 25 of cases, usually with exophytic granulation tissue in the subglottis and sometimes the vocal folds. The inflammation can progress to subglottic stenosis. There is a male predominance and onset is most common in the fifth and sixth decades. The classic...

TABLE 3 Immunotherapy Protocol for AFS

Surgical exenteration of disease from the sinuses and pathologic confirmation of the diagnosis. 2. Allergy evaluation and testing (RAST or quantitative skin test) for typical nonfungal antigens 3. Instruct patient in avoidance measures for molds. 4. Prepare a vial of all positive nonfungal antigens and a second vial of all positive fungal antigens. Perform a skin test with each. 5. Administer immunotherapy weekly, with dose advancement as tolerated, placing one injection 6. Observe the patient...

TABLE 3A International Prognostic Index Prognostic factors

Performance status > ECOG1 LDH > normal Extranodal sites > 1 Stage 3 or 4 Note The table shows the identified prognostic variables. TABLE 3B CR with Initial Therapy and the Chance of OS at Five Years Risk group Number of risk factors CR OS Abbreviations CR, complete response rate OS, overall survival. TABLE 4A Follicular Lymphoma International Prognostic Indexa Prognostic factors Ann Arbor Stage 3-4 Number of nodal sites > 4 LDH > normal Hemoglobin < 12 aThe table shows the...

Treatment

Treatment of VZV has no universally accepted protocol. The goal of treatment should be to inactivate the virus, control pain, reduce severity of the vesicular eruption, and reduce complications (including postherpetic neuralgia). Oral treatment with antiviral agents acyclovir (14), valacyclovir (15), or famciclovir (16) should be continued for 7 to 10 days. Patients should receive 800 mg acyclovir orally five times a day, 500 mg famciclovir orally three times a day, or 1 g valacyclovir orally...

Treatment Complications and Prognosis

NUG generally responds to initial therapy of debridement, antimicrobial oral rinses, and antibiotic therapy (37). When the process heals, the altered gingival anatomy resulting from tissue loss through necrosis creates anatomic forms that predispose to recurrence of NUG and to the development of chronic periodontitis. NUG may progress from gingival soft-tissue involvement to produce loss of periodontal attachment (necrotizing ulcerative periodontitis) and may extend to involve the adjacent oral...

Viral Infections

Primary infection with HIV usually presents as a mono-nucleosis-like syndrome after an incubation period of one to four weeks, as previously mentioned. The clinician should consider acute HIV infection in a mononucleosis-like presentation with negative serologies. The symptoms reported, by decreasing order of frequency, are fever (95 ), lymphadenopathy, pharyngitis, maculopapular rash, myalgias arthralgias, nausea, vomiting or diarrhea, headache, hepatosplenomegaly,...

Multiple Sclerosis

Multiple sclerosis is an autoimmune disorder characterized by patches of demyelination throughout the nervous system. Presentation is highly variable but typically involves a neurologic deficit such as paresthesia, visual disturbance, motor weakness, or autonomic dysfunction. The disease has a highly variable clinical spectrum, with some patients entering into remission and others deteriorating with recurrent and unremitting exacerbations of neurologic dysfunction. Diagnosis is confirmed by...

Cat Scratch Disease

Cat-scratch disease is caused by a systemic infection with Bartonella henselae, a bacteria colonizing cat saliva. The infection typically presents as a skin lesion at the site of a cat scratch with ensuing local lymphadenopathy. Infection is manifested by lymphadenopathy, but infected individuals may display fever, fatigue, anorexia, or headaches. It is most commonly a pediatric disorder, affecting individuals usually younger than 21 years of age. Neurologic involvement is estimated to affect 2...

Pathogenesis

There are a variety of sites within the auditory apparatus that may be affected by multi-systemic diseases. The external and middle ear serve primarily to amplify the sound stimulus. Particularly critical to this function are the tympanic membrane and middle ear bones (ossicles). The inner ear functions to convert the acoustic energy transmitted to the cochlea into an electrical signal that is transmitted to the central nervous system (CNS) via the eighth cranial nerve afferent fibers. This...

TABLE 4 Eye Disease in GCA

Permanent visual loss Unilateral Bilateral Without amaurosis fugax After amaurosis fugax Abbreviation GCA, giant cell arteritis. Source From Ref. 15. Ischemic damage to the optic nerve or retina can cause unilateral or bilateral tonic pupils, usually with dilatation. Horner's syndrome has also been described in patients with GCA as the result of paralysis of the ocular motor or abducens nerve, ischemia of the cavernous sinus, or as a central process in the brain stem (14). Other ocular symptoms...

Allergic rhinosinusitis

Allergic rhinosinusitis is a disorder expressed in the tissues of the nares and upper airway, manifested by local edema and inflammatory infiltration of the submucosa and mucosa, and associated with increased secretion of mucoid materials with accompanying inflammatory leukocytes. These processes lead to obstruction of airflow and of mucus drainage, and, therefore, may be complicated by postobstructive infectious complications. By definition, allergic disease is dependent on the host generation...

CoGans Syndrome Introductiondefinition

The principal features of CS are interstitial keratitis (IK) and vestibuloauditory neuronitis. This syndrome was reported in 1945 by David Cogan, an ophthalmologist at the Harvard Medical School, who meticulously described the medical history and findings of four patients with recurrent nonsyphilitic IK and Meniere's-like vestibuloauditory symptoms. The ocular component of CS subsequently expanded to include other types of eye inflammation, such as conjunctivitis, episcleritis, scleritis, and...

Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis, or Lou Gehrig's disease, is a progressive degeneration of selected upper and lower motor neurons, of unknown etiology. Patients present with dysfunction of the voluntary muscles, most often having problems with ambulation as well as difficulties with tongue movements, causing dysphagia and dysarthria. This dysfunction inevitably progresses to respiratory difficulties as the disease worsens, but cognitive, sensory, and autonomic nerve functions are spared. Eye...

TABLE 2 Causes of Dry Mouth

Continuous positive airway pressure for sleep apnea Use of systemic anticholinergic medication Dehydration Congenitally missing salivary glands Acute bacterial viral illnesses affecting the salivary glands Radiation therapy to the head and neck Bell's palsy Excessive thirst (i.e., diabetes) Anxiety depression stress Past treatment with I131 component, while others also use subjective criteria. As a result of these differing criteria, the prevalence estimates of SS have ranged from 2 to 10...

Basic Inner Ear Immunology

Due to substantial research efforts, it is now well accepted that the inner ear is fully capable of generating an immune response and that this immune response can be destructive to the Autoimmune Inner Ear Disease TABLE 1 Epidemiology of AIED Year Number of patients Mean age (range) Women Men Hughes et al. (3) Moscicki et al. (4) Rauch (5) Sismanis et al. (6) Lasak et al. (7) Harris et al. (8) Broughton et al. (9) Loveman (10) Cohen etal. (11) Matteson et al. (12) 44 (8-77) 1-80 47 (4-72) 51...

Other Manifestations

A complete discussion of the clinical features of sarcoidosis is beyond the scope of this chapter. Briefly, sarcoidosis most commonly affects the lungs, where it may be asymptomatic or cause symptoms of dyspnea, dry cough, wheezing, and chest pain. Lung rales are heard in less than 20 of patients. The diagnosis may be aided by chest radiograph and highresolution chest CT scan showing bilateral, symmetrical lymphadenopathy with or without pulmonary interstitial opacities. Sarcoidosis may also...

Pathologypathophysiology

C. diphtheria is a member of a group of irregular, non-spore-forming, aerobic Grampositive bacilli characterized by growth in facultative anaerobic conditions and catalase-and oxidase-positivity. Although other coryneform bacteria are ubiquitous in nature, C. diphtheria exclusively exists on human mucous membranes and skin. Transmission is via infected respiratory droplets or skin wound for two to four weeks in untreated infected individuals, although antibiotic treatment greatly reduces the...

Leukemias

Leukemias are classified as either acute or chronic. Within the acute category, the major types are AML and ALL. The equivalent chronic types are CML (discussed under MPDs) and CLL. CLL is the most common leukemia in adults, whereas in children, ALL is most frequently seen. The acute leukemias are characterized by the proliferation and accumulation of immature blast cells. The blasts accumulate in the bone marrow, resulting in the failure of normal hematopoiesis. The WBC is usually elevated at...

Medications

Many treatments have been advocated for sudden sensorineural hearing loss, which is presumed to be of viral (or rarely vascular) origin. The diagnosis of autoimmune inner-ear disease is still predicated by a documented response to corticosteroid administration (20). Treatment strategies have varied between reports, but, in general, a dose of prednisone, 40 to 60 mg in the adult for two weeks, is an appropriate therapeutic trial. Substantial improvement on this dosage regimen would then warrant...

Complications

Complications relate to site-specific manifestations of the disorder and or consequences of treatment with chemotherapy or radiation. Infectious complications are common, most often following treatment of acute leukemia. The most feared are fungal infections, especially with Aspergillus species (68) or mucormycoses (69). The incidence increases with prolonged periods of neutropenia or with immunosuppression given for the treatment of graft versus host disease following allogeneic stem-cell...

Definition

SJS was first described in 1922 by two American physicians, Stevens and Johnson, who reported an acute mucocutaneous syndrome characterized by severe purulent conjunctivitis, severe stomatitis, extensive mucosal necrosis, and skin lesions of erythema multiforme (EM) in two young boys. SJS was later designated EM major by Bernard Thomas in 1950, but this designation is not currently accepted, as SJS and EM are now considered distinct disorders. SJS is a rapidly progressive mucocutaneous eruption...

Clinical Manifestations Lymphoma

Most often, lymphoma is suspected following the finding of painless lymphadenopathy. Occasionally, with the more aggressive types, there may be associated pain and redness affecting the overlying skin. Differentiation from infection is important and patients have often received a course of antibiotics prior to undergoing a biopsy. Depending on the location, there may be symptoms related to organ compromise. Chest pain, pressure, or shortness of breath may occur with intrathoracic disease. The...

Otic Diseases

Acute otitis media is a suppurative infection of the middle-ear cavity. Facial nerve involvement is considered a complication of the infection and often occurs from direct pathogen invasion of a dehiscent portion of the facial nerve course, most commonly in the horizontal segment of the facial nerve. Treatment consists of systemic antibiotic therapy against the most common pathogens Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus pneumoniae. Drainage of the...

Info

Balough, and Darrell Hunsaker Department of Otolaryngology, Naval Medical Research Command, Naval Medical Center, San Diego, California, U.S.A. Diagnosis and Treatment 363 The Infectious Etiologies 363 Acute Bacterial Otitis Externa 363 Noninvasive Fungal Otitis Externa 364 Infectious Otitis Media 364 Infections in Immunocompromised Individuals 364 Complications and Prognosis 366

Preface

In thinking about a contribution we could make to the modern practice of medicine that had not yet been achieved by others, we remembered what constituted the complete physician at the time of Sir William Osler A working knowledge of multiple disciplines and of disease processes that affect many organ systems. This was perhaps even more important in that era of medicine when laboratory and imaging modalities were essentially nonexistent, forcing physicians to use their powers of history-taking,...

Pathology

FIGURE 1 (Left) A section of ear from a patient with relapsing polychondritis showing necrosis of cartilage and inflammatory cell infiltration (hematoxylin-eosin, medium power). (Right) A marked loss of metachromasia is observed in cartilage from the ear, indicating disappearance or breakdown of chondroitin sulfate (azure A, medium power). Source American College of Rheumatology. The immunologic attack on cartilage matrix components helps to explain the distribution of tissue inflammation and...

Acknowledgment

Figures are from the ACR slide collection. 1. Gergely P Jr, Poor G. Relapsing polychondritis. Best Pract Res Clin Rheumatol 2004 18(5) 723-738. 2. Knipp S, Bier H, Horneff G, et al. Relapsing polychondritis in childhood case report and short review. Rheumatol Int 2000 19(6) 231-234. 3. Zeuner M, Straub RH, Rauh G, et al. Relapsing polychondritis clinical and immunogenetic analysis of 62 patients. J Rheumatol 1997 24(1) 96-101. 4. Lang B, Rothenfusser A, Lanchbury JS. Susceptibility to relapsing...

Systemic Manifestations

Facial lesions due to conditions associated with TB have been described these include erythema induratum of Bazin, papulonecrotic tuberculids, and others. M. tuberculosis DNA has been detected in erythema induratum skin lesions by PCR, and erythema nodosum has been attributed to primary TB. Skin involvement may result from exogenous inoculation, spread from an adjacent focus to the overlying skin, or hematogenous spread, often seen in patients with AIDS and tuberculous bacteremia....

TABLE 2 Laboratory Findings Suggestive of Kawasaki Disease

CRP > 3.0mg dL ESR > 40 mm hr Albumin < 3.0mg dL Anemia for age Elevated alanine aminotransferase and g-glutamyl transpeptidase Platelet count > 450,000 after first week of fever White blood cell count > 15,000 L Urine white blood cell count > 10 cells hpf CSF pleocytosis with normal CSF protein Changes on echocardiogram including z score of LAD or RCA > 2.5, lack of vessel tapering, perivascular brightness, decreased LV function, mitral regurgitation, and pericardial effusion...

Prognosis

The prognosis of RP varies, depending upon the pattern and severity of chondral inflammation. Most patients (84 ) will have intermittent flares lasting days to weeks (18). Many patients have chronic indolent symptoms between flares, and most will develop some degree of disability. A minority of patients will have a benign course free of visceral involvement or a fulminant course resulting in death. The most common complications are loss of hearing and olfaction, reduced vision (8 ), phonation...

Polyarteritis Nodosum

Polyarteritis nodosum is a vasculitis involving medium-sized arteries, most commonly at their bifurcations. Presentation usually involves the gastrointestinal tract as postprandial abdominal pain with nausea. Patients may also demonstrate kidney dysfunction, subcutaneous palpable nodules, fevers, livedo reticularis, and mononeuritis multiplex. Unlike Wegener's granulomatosis, lung involvement is not typically seen. Nervous system involvement is most often a peripheral neuropathy. Although...

Sjogrens Syndrome

Sj gren's syndrome is an autoimmune disorder of unknown etiology. It occurs in isolation, known as primary Sj gren's syndrome, or secondary to a connective tissue disease. It primarily affects women over 50 years of age. Typical presentation involves dryness of the eyes, termed xeropthalmia, along with dryness of the mouth, termed xerostomia. The pathogenesis of the disorder involves a monocytic infiltration and damage to the salivary and lacrimal glands. Typical symptoms include dysphagia,...

Tangier Disease

Tangier disease is an autosomal-recessive disorder of lipoprotein metabolism that results in the deposition of xanthomatous cells in lymph nodes, tonsils, palate, spleen, and liver. Epidemiology. The disorder was initially observed on Tangier Island in the Chesapeake Bay area of the United States. There appears to be gender predilection with the disease occurring in all age groups. Pathogenesis. Tangier disease results in a deficiency of high-density lipoproteins and low levels of apoproteins....

Fungus Ball

Fungal colonization of the maxillary sinus occasionally manifests as a fungus ball. This clinical entity is, on occasion, referred to as mycetoma however, this is an inappropriate term, because mycetoma refers to a locally destructive subcutaneous fungal infection that can involve muscle and bone. A maxillary sinus fungal ball, on the other hand, is not invasive. It is typically unilateral, may or may not be symptomatic, and occurs in an immunocompetent host. MR imaging may demonstrate...

Mycoplasma

Mycoplasma pneumoniae is a small bacterium that lacks a cell wall and commonly causes upper respiratory tract infections and pneumoniae. The overall incidence of infection in the United States has been estimated at 1.7 cases 1000 people year (38) with 6.8 of all pneumonia caused by this pathogen (39). Symptoms include fever, cough, malaise, and headache, but as many as 25 of patients are asymptomatic. Extrapulmonary manifestations do occur, with neurologic sequelae estimated in 0.1 of all cases...

Anatomy

The facial nerve is one of 12 cranial nerves. It contains motor nerve fibers responsible for facial expression as well as other nerve fibers involved in sensation, taste, and secretory function. The motor pathway begins within the premotor and motor cortex. Upper motor neurons then connect the premotor and motor cortex to the facial nucleus located within the pons. Lower motor neurons exit from the facial nucleus and continue on to innervate the various mimetic muscles of facial expression. It...

Pagets Disease

Paget's disease is a common osteodystrophy characterized by abnormal bone metabolism where focal areas of bone are resorbed, then replaced in excess. It is estimated to affect 7 of males and 4 of females over the age of 55 (119). Although 85 of cases of Paget's disease are asymptomatic, typical presentations of pain, arthritis, fractures, and high-output cardiac failure are characteristic (120). Skull enlargement with bony impingement of neural foramina can lead to neuropathy, but this appears...

Fungal Culture

The Mucorales grow well on both nonselective and fungal-selective media. The growth of the Mucorales tends to be rapid, with mycelial elements expanding to cover the entire plate in only a few (1-7) days. Organisms of the order Mucorales are characterized by an erect aerial mycelium that is described as fibrous or cotton candy-like. The mycelium tends to be quite high, with some isolates reaching the lid of the petri dish at mature growth. It is this vigorous growth characteristic that is...

Other Etiologies

While the vast majority of otorrhea is infectious in etiology, there are a number of other important causes. Trauma can cause otorrhea in several different fashions. Head injury or surgery can create a CSF leak from the middle fossa or posterior fossa into the mastoid. The CSF can then pass into the middle ear and escape through the tympanic memberane (TM), if it, too, is damaged or opened by the traumatic event or surgery. More commonly, trauma will be associated with bloody otorrhea due to...

Polio

Polio, also termed poliomyelitis, is a small RNA enterovirus transmitted by fecal-oral contamination. It is highly contagious and often remains asymptomatic. When patients are symptomatic, presentation varies but typically includes mild flu-like symptoms of fever, odynophagia, and diarrhea. A minority of cases progress to aseptic meningitis with paralysis of the extremities and respiratory musculature. Facial paralysis is commonly associated with polio and can be an isolated finding in the...

Evaluation of Tissue Specimens

The diagnosis of zygomycosis is easily made on tissue section from the margin of a necrotic lesion. Involved tissue demonstrates focal areas of infection and may appear nodular or may produce extensive areas of necrosis with accompanying hemorrhage into the tissue. Specifically, histology demonstrates invasion along the elastic lamina of blood vessels, with hyphae of the fungus extending into and occluding the lumens of the blood vessels they have invaded, followed by thrombosis and tissue...

Mononucleosis

Mononucleosis is caused by an infection with the Epstein-Barr virus (EBV), a DNA virus in the Herpes virus family. It typically presents with fever, sore throat, malaise, lymphadeno-pathy, and hepatosplenomegaly. It is estimated that 90 of adults have serologic evidence of prior EBV infection. Diagnosis is confirmed by the clinical picture, characteristic hematologic changes, and immunologic findings. Hematologic changes include atypical lymphocytosis of peripheral monocyte cells. Immunologic...

Spondyloarthropathy

Spondyloarthropathies, including ankylosing spondylitis, are accompanied by acute anterior uveitis in about one-third of patients during the course of their disease. Anterior uveitis is typically acute in onset and unilateral it lasts four to eight weeks, and subsides without sequelae, if treated early (13). Cervical spondylitis is the other common head and neck manifestation of spondyloarthropathy. Examination of the neck may reveal decreased range of motion in all planes. Temporomandibular...

Saprophytic fungal disease

Saprophytic fungal disease is a superficial growth of fungus on mucopurulent crusts within the sinonasal cavity. This is occasionally seen on the crusts that form after sinus surgery. The hyphae are not found invading the nasal mucosa or deeper tissues. Treatment involves simple removal of the crusts along with the resident hyphae. The prognosis is excellent and residual crusts can be loosened and washed free with the addition of nasal irrigation. There is no role for steroids or antifungal...

Foreword

This book provides a great wealth of the most current information for today's readers and will serve as a respected resource for decades to come. The two editors, one from otolaryngology-head and neck surgery and the other from rheumatology, are distinguished scientist-clinicians who have made important contributions to our knowledge of basic and clinical immunology. They have designed a work with a unique perspective First, to present the fundamental information on a multitude of diseases that...

Staging

The diagnosis, staging, and treatment of primary melanoma are intimately related. Melanoma is staged by the characteristics of the primary tumor, nodes, and metastases (TNM). The TNM system for melanoma staging correlates with prognosis (Table 1) (7). Primary TABLE 1 AJCC Staging for Melanoma and Corresponding Survival TABLE 1 AJCC Staging for Melanoma and Corresponding Survival Abbreviations AJCC. American Joint Committee on Cancer TNM, primary tumor, nodes, and metastases LN, lymph node SC,...

Associated Diseases

More than 30 of patients with RP have another associated systemic disease, including necrotizing vasculitis, autoimmune rheumatic disease (rheumatoid arthritis and others), hematologic disorders (especially myelodysplastic syndromes), endocrine disease (Graves' disease and others), inflammatory bowel disease, or another autoimmune disease (Table 1). Additionally, isolated cases have been described following ear piercing and intravenous substance abuse (6,7). How these associated conditions lead...

Infectious Uveitis

Toxoplasmic chorioretinitis may be diagnosed as a congenital or postnatally acquired disease due to primary infection or reactivation of latent infection. Toxoplasmosis causes an acute focal retinitis in which the hallmark is a thickened cream-colored retina accompanied by an overlying intense vitreal inflammatory reaction (headlight in the fog appearance). Recurrent lesions are usually seen at the borders of chorioretinal scars (Fig. 1C). Primary ocular infections are less common than...

Mumps

Mumps is caused by infection with an RNA paramyxovirus. The most common presentation is parotid gland swelling and pain the testicles, pancreas, kidneys, eyes, and ovaries are also often involved. As mumps has a predilection for neural tissue, neurologic involvement is present in 5 to 30 of cases and may manifest as meningitis, encephalitis, or cranial neuropathies (14). Among cranial nerve involvement, the facial and cochleovestibular nerves are most commonly affected. Some investigators...

Congenital Syphilis

Fetal infection with syphilis is highly dependent on the mother's stage of disease. Mothers with untreated primary or secondary disease are extremely likely to pass the infection, while those with latent disease have rates of transmission as low as 2 . Appropriate treatment of the mother during pregnancy generally prevents fetal infection. When infection does occur, the outcome can range from spontaneous abortion to a healthy-appearing child at birth with only latent infection. Similar to...

Adamantiades Behets Disease TABLE 3 Treatment of Behets Disease

Mild mucocutaneous disease Topical anesthetics Topical or intralesional corticosteroids Topical tacrolimus Topical tetracycline solutions Oral tetracycline or macrolide agents Topical amlexanox (paste) Sucralfate Oral colchicine Oral dapsone (25-200 mg daily) Antimalarials Rebamipide (mucoprotective agent) Severe mucocutaneous disease Thalidomide Oral corticosteroids (0.5-1.0 mg kg day) Methotrexate Azathioprine Interferon-a Anti-TNF-a agents Systemic disease Systemic corticosteroids...

TABLE 2 Clinical Manifestations in Churg Strauss Syndrome

Prevalence during course of disease ( ) Hemorrhage Glomerulonephritis Eye Skin Pericardial effusion Myocardial involvement Abbreviation GI, gastrointestinal. Source Adapted from Ref. 30. FIGURE 9 Churg-Strauss syndrome nasal polyp. FIGURE 9 Churg-Strauss syndrome nasal polyp.

Mucormycosis in Children

In addition to cases involving immunosuppressed children, mucormycosis also has been observed in neonates (especially very-low-birth-weight premature infants weighing less than 1000 g), burn patients, and children with a history of incidental trauma. As with adult patients, the occurrence of mucormycosis depends on host immunity, but the mechanisms of increased susceptibility in certain hosts remain perplexing. In a pooled review by Kline of 41 cases of RCM in children aged 2 months to 18...

Tuberculosis

Tuberculosis is caused by an infection with the bacterium Mycobacterium tuberculae. In 2004, 14,517 cases of tuberculosis were reported in the United States (17). The infection is spread via respiratory droplets. Pulmonary symptoms predominate, but any organ system can be affected. Spread to the ear can be via direct extension through the nasopharynx, from hematogenous spread, or on rare occasions, via direct implantation through a preexisting tympanic perforation. Tubercular otitis media...

Systemic Disease

Patients with metastatic melanoma may receive a number of treatment modalities. Solitary or few metastases in solid organs such as lung, small bowel, or liver can be treated with surgical excision. This is particularly effective for patients with a prolonged disease-free interval, characterized by a tumor doubling time of > 60 days. Brain metastases can be managed with surgical excision or gamma knife radiation. Systemic immunotherapy and chemotherapy are options. Immunotherapy with...

Tetanus

Tetanus results from the neurotoxin secreted by the bacterium Clostridium tetani. On average, the Centers for Disease Control (CDC) has reported 90 cases per year in the United States since vaccinations were implemented to prevent the infection (68). The most consistent early symptom is trismus. Without treatment, the infection is lethal in 30 of patients. A rare localized form of tetanus, termed cephalic tetanus, causes facial paralysis and usually results from an ipsilateral facial wound....

Prosthetics

Hearing-aid amplification remains the mainstay of rehabilitation for patients with sensorineural hearing loss. Unfortunately, hearings aids are amplifiers, not clarifiers, and, as such, leave patients less than satisfied. Cochlear implants have revolutionized the management of patients with severe sensorineural hearing loss or deafness. They are the only prosthesis that successfully replaces sensory function. Many objective studies have proven their value in the treatment of pediatric patients'...

Perioperative Management

The stress of surgery, even if minor, may induce a sickle crisis. It is therefore important for the otolaryngologist to be aware of the perioperative management of a patient with sickle cell disease. Sickle cell trait does not cause an increase in operative morbidity. On the other hand, sickle cell disease has a high incidence of perioperative problems. Complications related to sickle cell events, such as acute pain syndrome, are increased after surgery. Sickle cell disease may not increase the...

Prognosis for Lymphomas

Prognostic indices have been developed for the indolent and aggressive lymphomas. The International Prognostic Index (IPI) (65) is utilized to evaluate a patient's risk status for the aggressive lymphomas. The Follicular Lymphoma IPI (FLIPI) (66) is similarly utilized for the indolent follicular lymphomas. Using the IPI or FLIPI to determine therapy is the subject of ongoing clinical trials. The IPI and FLIPI criteria are shown in Tables 3 and 4, respectively. It is important to note that the...

Prognosis And The Role Of The Surgeon

The prognosis of this most potent 20th century epidemic has changed from a disease with an almost 100 fatality rate to a manageable syndrome within 20 years. This represents a change that is unprecedented in the history of medicine. Reports from around the developed world have documented the efficacy and safety of potent antiretroviral therapy and the huge impact (Fig. 22) this has had to the disease prognosis and survival. Unfortunately, as of 2006, the epidemic continues to be rampant in the...

TABLE 3 Diseases That Can Share Signs and Symptoms with SS

Amyloidosis Sarcoidosis Graft vs. host disease HIV disease (diffuse infiltrative lymphocytosis syndrome) Abbreviation SS, Sj gren's syndrome. TABLE 4 A Revised Classification Criteria for SS by the 2002 American-European Consensus Group I. Ocular symptoms a positive response to at least one of the following questions a. Have you had daily, persistent, troublesome dry eyes for more than 3 mo b. Do you have a recurrent sensation of sand or gravel in the eyes c. Do you use tear substitutes more...

Uveitis Introduction

Uveitis can occur alone, but is commonly the presenting feature of a systemic disease due to both infectious and noninfectious etiologies. It can be acute, recurrent, and chronic. Chronic uveitis in particular has a high incidence of vision-threatening complications (1). Approximately 10 of patients over 65 years of age with uveitis develop blindness. The number of people with blindness from uveitis is similar to that from diabetic retinopathy, despite the fact that uveitis is 250 times less...

FIGURE 2 Necrotizing ulcerative periodontitis Source Courtesy of the International AIDS SocietyUSA From Refs 3 4

With severe malnutrition and coexisting chronic infections in developing countries. Anaerobic indigenous flora seems to play a role in maintaining the destructive character of the lesion, but the pathogenesis is unclear. Treatment is best left to an HIV dental specialist and includes debridement of necrotic tissue, aggressive local care with antiseptics and chlorhexidine mouthwashes, and systemic antibiotics with some degree of anaerobic coverage, such as doxycycline, amoxicillin clavulanate,...

Fibrous Dysplasia

Fibrous dysplasia is another osteodystrophy or developmental disturbance of bone formation and is also discussed in Chapter 21. In fibrous dysplasia, bone is resorbed and then replaced with haphazardly arranged fibrous tissue. Fibrous dysplasia is classified as monostotic, polyostotic, or McCune-Albright syndrome. McCune-Albright syndrome is a polyostotic fibrous dysplasia along with endocrine abnormalities consisting of skin changes over the neck and trunk, precocious puberty, accelerated...

Sjgrens Syndrome

Sj gren's syndrome is an autoimmune inflammatory disorder characterized by the destruction of the salivary and lacrimal glands, resulting in xerostomia and keratocon-junctivitis. Sj gren's syndrome can be a primary disorder or it can present in association with other autoimmune disorders. Autoantibodies anti-Ro and anti-La are found in 60 and 40 , respectively, of patients with Sj gren's. The histologic findings are consistent with the pathophysiology of an autoimmune disease directed at...