Polio And Hearing Loss

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...

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...

Aspirin nsaid hypersensitivity

Three manifestations of sensitivity to NSAIDs are of importance to the head and neck. They include urticaria angioedema, anaphylaxis, and rhinoconjunctivitis asthma. These appear to occur separately, and, in most instances, cross-reaction with other drugs in the class is common. By definition, NSAID hypersensitivity is present in patients who react adversely to the administration of this class of drug. It was originally described by Widal as a symptom complex of aspirin sensitivity, asthma, and...

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...

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...

References

Ponikau JU, Sherris DA, Kern EB, et al. The diagnosis and incidence of allergic fungal sinusitis. Mayo Clin Proc 1999 74(9) 877-884. 2. Branovan DI. Pathophysiology of rhinosinusitis. In Rice DH, Schaefer SD, eds. Endoscopic Paranasal Sinus Surgery. 3rd ed. Philadelphia, PA Lippincott Williams & Wilkins, 2004 53-68. 3. Furguson BJ, Johnson JT. Infectious causes of rhinosinusitis. In Cummings CW, Flint PW, Haughey BH, eds. Cummings Otolaryngology-Head and Neck Surgery. Vol. 2. 4th ed....

Systemic Lupus Erythematosus

Rheumatoid Vocal Nodules

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,...

Genetic Diseases

Congenital hearing loss is common, and about one-half of cases are thought to be genetic. Genetic hearing loss is typically classified as syndromic or nonsyndromic. When hearing loss consistently coexists with other pathologic findings, it is considered part of a syndrome when it exists in isolation, it is nonsyndromic. As our focus is on systemic diseases, nonsyndromic hearing loss, which accounts for two-thirds of hereditary hearing loss, will not be discussed extensively. Mutations in the...

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...

Clinical Manifestations Primary Lesion

A primary lesion is the most common manifestation of malignant melanoma of the head and neck region. This lesion can vary in appearance from the classical black-pigmented, raised lesion to an enlarging, skin-colored (amelanotic) mole (Fig. 1A and B). Melanoma can arise from a preexisting nevus or normal skin. The appearance of melanoma may be FIGURE 1 Primary melanoma has a wide range of appearances. (A) Superficial-spreading melanoma. (B) Amelanotic nodular melanoma of the scalp. (C) Lentigo...

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...

Treatment

Autoimmune disorders such as those discussed in this chapter are generally treated with immunosuppressive therapy. Corticosteroids are a mainstay of therapy in all of these disorders, but doses may vary widely, depending on the severity of disease manifestations. Among other immunosuppressives, those with more serious potential side effects are reserved for more severe disease manifestations. Often, however, the dermatologic manifestations of SLE and DM can be treated by hydroxychloroquine....

Systemic Sclerosis

Systemic sclerosis is a disorder of the small arteries, resulting in proliferation of fibrosis affecting the skin and multiple end organs. There are two subtypes of systemic sclerosis, diffuse and limited cutaneous. Diffuse cutaneous systemic sclerosis is characterized by rapid progression with skin involvement proximal to the elbows and knees. There is usually the early onset of Raynaud's phenomenon and early visceral involvement. Limited cutaneous systemic sclerosis is characterized by a...

Info

FIGURE 5 The technique of anterior ethmoid artery ligation via a Lynch incision. intractable epistaxis. Since that time, multiple reports utilizing this basic technique with various catheters and embolic materials have been reported. The use of this technique is seen in many areas including arteriovenous malformations, pseudoaneurysms, bleeding varices, shrinking of fibroids, and vascular tumors of the head and neck. Diagnostic angiography with embolization is commonly performed under local...

Tularemia

Tularemia is a zoonosis caused by Francisella tularensis, a small gram-negative bacillus. It is most commonly contracted by humans through bites of insects and through handling of infected animals, e.g., rabbits, hares, or muskrats. The clinical form infecting humans is ulceroglandular, with an ulcerative lesion on the skin and regional lymphadenitis. Epidemiology. Although tularemia may be common in endemic areas, the variation in incidence is wide. Highest incidence in endemic areas occurs in...

Dermatomyositis Polymyositis

DM also may include head and neck dermatologic manifestations that can have considerable diagnostic importance. Among these is the virtually pathognomonic heliotrope rash (Fig. 7). This lesion, which leads to edema and discoloration of the eyelids, takes its name from the deep purple flower of the heliotrope plant, although the lesion may vary in color from pink to red to brown. DM may also lead to an erythematous rash on many locations on the body, including the face and neck (Fig. 8). Nasal...

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...

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...

Pathologypathophysiology

Most CSD begins with a scratch from the claw or tooth of a kitten younger than six months of age. It can also be inflicted by an adult cat, or from contact of the animal's saliva with broken skin or the eye. Previous investigations into the responsible organism identified a family of a-proteobacteria based on 16S ribosomal RNA gene sequences (6). Currently it is believed that Bartonella henselae, a Gram-negative bacterium, is the causative organism in CSD. In California, about 40 of cats carry...

Differential Diagnosis

In addition to the clinical symptoms of anemia, iron deficiency produces a microcytic, hypochromic anemia. Serum iron is low. Total iron-binding capacity is elevated. A similar clinical appearance of the tongue mucosa may be seen in Vitamin B complex deficiencies and pernicious anemia (Fig. 43). Atrophic candidiasis (Fig. 45) may also produce a sensitive, erythematous, depapillated lingual mucosal surface. In severe xerostomia, the tongue may exhibit atrophy of the lingual papillae and erythema...

Diagnosis And Treatment

Individuals with HHT should receive an initial workup at the time of diagnosis, followed by preventive care and surveillance. Recommended workup for patients with HHT includes complete blood count (CBC), evaluation for stool occult blood, brain magnetic resonance imaging to evaluate for cerebral AVMs, contrast echocardiography to screen for pulmonary shunting followed by chest computed tomography to evaluate pulmonary AVMs if shunting is found, and evaluation for liver involvement by listening...

Clinical Manifestations

Classic symptoms of infectious mononucleosis are the triad of fever, pharyngitis, and cervical lymphadenopathy. In addition to fever, systemic symptoms include fatigue and generalized lymphadenopathy. Oral hairy leukoplakia, a collection of hairy or corrugated white lesions located on the lateral surface of the tongue, is common. Sometimes splenomegaly or mild hepatitis may develop. Uncommon manifestations include heart problems, jaundice, pneumonitis, blood dyscrasia, and cerebritis (2). In...

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...

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...

Epidemiology

Mucormycosis is extremely rare, making it difficult to calculate incidence accurately. In a population-based epidemiological assessment, the cumulative incidence of invasive mycotic infections was 178.3 cases per million per year, with zygomycosis comprising 1.7 infections per million per year based on data from the San Francisco Bay Area (4). A recent review of mucormycosis cases at one U.S. cancer center showed that it was present in 0.7 of patients at autopsy and in 20 patients per 100,000...

Melkersson Rosenthal Syndrome

Melkersson-Rosenthal syndrome is characterized by a triad of recurrent episodes of facial paralysis and facial edema along with a fissured tongue. Other neurologic sequelae have been documented, including headache, trigeminal neuralgia, cranial nerve dysfunction, and autonomic dysfunction. Although the etiology is unknown, recent investigation points toward a granulomatous disease leading to recurrent attacks of edema and inflammation of the face and oral cavity with a predilection for facial...

Neoplasm

Cancer Tonsillar Fossa

Although neoplasms are not a cause of acute or chronic pharyngitis, tumors arising in the oropharynx often present with signs and symptoms that most commonly indicate an infectious etiology. Patients treated for infectious pharyngitis, who do not improve, warrant further investigation to identify a possible neoplasm. Common presenting symptoms of oropharyngeal cancer include unilateral sore throat, dysphagia, odynophagia, weight loss, and otalgia. On physical exam, an asymmetric pharyngeal mass...

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...

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,...

Head and Neck Manifestations

Tuberculous Lymphadenitis (Scrofula). This represents the most common form of extrapulmonary TB (13), and in 80 to 90 of cases, it is the only site of infection. In HIVnegative patients, it is usually bilateral and posterior cervical in location, presenting as an erythematous, painless mass along the anterior border of the sternocleidomastoid, typically without systemic symptoms (11). The tuberculin skin test (TST) is positive in more than 75 of patients. In HIV-positive patients, multiple...

Syphilis

Syphilis is a sexually transmitted disease resulting from infection by the spirochete Treponema pallidum. It is the oldest documented venereal disease, dating back to the 1400s. A more detailed discussion of syphilis appears in Chapter 15. Epidemiology. The United States has seen cyclic rises in the incidence of syphilis since nearly eliminating the disease in the late 1950s. Since 1990, the rate of primary and secondary syphilis has decreased nearly 90 to 2.5 cases per 100,000 people. Of the...

Cutaneous Bullous Diseases

Many cutaneous disorders have associated esophageal involvement. The more common of these disorders include epidermolysis bullosa, cicatricial pemphigoid (CP), lichen planus, and pemphigus vulgaris. Epidermolysis bullosa is a relatively rare cutaneous disease mediated by circulating IgG antibodies directed against type VII collagen. Clinically, patients develop intradermal blistering lesions with scarring at sites of trauma (hands and feet are most common). The proximal third of the esophagus,...

Complicationsprognosis

The prognosis for patients with AIED is quite variable. Part of this variability can be explained by the diverse nature of the disorder. Autoimmune injury in general can be from direct antibody injury toward specific epitopes, from immune complex deposits, or from cellmediated attacks by T cells. Depending on the cause of the autoimmune process and the speed with which it is treated, the damage to the cochlea and vestibule can be reversible or permanent. Cogan's syndrome, for example, carries a...

Epstein Barr Herpes Simplex and Herpes Zoster Infections

Department of Head and Neck Surgery, Kaiser Permanente Medical Center, Oakland, California, U.S.A. & Introduction 127 & Epstein-Barr Virus 127 & Definition 127 & Epidemiology 127 & Pathogenesis 127 & Clinical Manifestations 127 & Diagnosis 128 & Treatment 128 & Complications and Prognosis 128 & Summary 129 & Herpes Simplex Virus Type 1 129 & Introduction 129 & Definition 130 & Epidemiology 130 & Pathogenesis 130 & Clinical Manifestations 131 &...

Cytomegalovirus

Cytomegalovirus (CMV) is a large DNA virus of the Herpes virus group. It is estimated that 50 to 80 of adults have prior evidence of CMV infection (58). The infection is usually subclinical when contracted by immunocompetent infants and adults and infrequently may lead to a mononucleosis-type syndrome. However, significant sequelae exist from in-utero infections as well as infections in immunocompromised patients. Neurologic involvement almost always is associated with immunosuppression or...

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...

Scleroderma

As in SLE, changes in the skin are the most obvious manifestations of scleroderma in the head and neck. In systemic sclerosis, the skin changes can begin with a sometimes pruritic, edematous phase, as inflammatory cells and fibroblasts are activated and cytokines are released. The skin in the affected areas tightens, loses flexibility, and thickens. In systemic sclerosis, characteristic facies may be noted, with a pursed-lip appearance and reduction in the oral aperture (Fig. 4). After several...

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,...

Pathogenesis

Multiple aspects of the immune system have been shown to be abnormal in Adamantiades-Behget's, including lowered neutrophil and endothelial cell activation thresholds, abnormal T-cell responses (including yS T-cells), increased cytokine expression and immune complex formation, increased Fas-ligand expression, abnormal complement activation proteins, and disrupted coagulation pathways (4,6). The driving process behind these multiple abnormalities is unknown. Infections have been thought to lead...

Loco Regional Disease Nodal Assessment

After initial pathological assessment by punch or excisional biopsy, the next step is treatment of the primary site and staging of the regional lymph nodes. Treatment for the primary tumor consists of wide local excision. Local recurrence can be as high as 40 for primary melanomas that are not reexcised after biopsy (3). Melanoma-in-situ is treated by reexcision with at least 0.5-cm margins around the primary lesion or biopsy scar. When primary invasive melanomas are < 1 mm thick or Clark's...

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...

Amyloidosis

Amyloidosis is a disease resulting from abnormal protein deposits in extracellular tissue. The disease can be localized or systemic and can be further subclassified as primary, secondary, and familial. In primary amyloidosis, the protein fibrils form without a known cause. Secondary amyloidosis results from multiple myeloma or other chronic diseases that generate an abnormal amount of protein breakdown. Familial amyloidosis refers to a hereditary version of the disorder. Presentation is...

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...

Definition

AIED can be defined as a fluctuating or rapidly progressing sensorineural hearing loss that is responsive to immunosuppressive therapy. Vestibular dysfunction may or may not be present. In some cases, a concurrent systemic autoimmune disorder exists at the time of diagnosis. Therefore, AIED can exist in isolation as an organ-specific disease (primary AIED) or can occur as a nonspecific injury as part of a systemic autoimmune disease (secondary AIED). Currently, there is no diagnostic laboratory...

Cytologic Testing

Demonstration of fungal elements from cytologic preparations (i.e., sputa, inflammatory fluid aspirates from abscesses or sinusitis infection, and genitourinary and gynecologic specimens) may be difficult, due to the difficulty in extracting fungal elements from invaded tissues (8). Cultures of blood and cerebrospinal fluid (CSF) are negative. CSF, if inadvertently examined, may show an increased opening pressure, modest neutrophilic pleocytosis, normal or slightly elevated protein levels, or...

Sarcoidosis

Sarcoidosis is a chronic systemic granulomatous disease of unknown etiology, characterized by the presence of noncaseating granulomas in any organ system of the body. Involvement of the head and neck is noted in 10 to 15 of patients (17). Please see Chapter 6 for a detailed discussion of sarcoidosis. Epidemiology. Sarcoid has a slight female preponderance and occurs in all age groups, although more commonly in young adults. Pathogenesis. An etiology for sarcoid has not been established....

Osteopetroses

Osteopetroses are rare genetic osseous dysplasias characterized by bony sclerosis and abnormal bone metabolism with increased bone density. The skull is frequently involved and various cranial neuropathies have been described secondary to bony impingement of the skull foramina. The incidence of cranial neuropathies in osteopetroses has been estimated at 16 . Although the facial nerve is the most common cranial nerve involved, the optic and vestibulocochlear nerves are also affected (122). One...

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...

Summary

Systemic rheumatic disease may present with a variety of manifestations in the head and neck regions. Especially important among these are the dermatologic findings of SLE, DM, and scleroderma. Rashes characteristic of these disorders may also arise later in the course of the diseases. Some of the more frequently reported manifestations to be aware of are autoimmune hearing loss, especially in SLE esophageal dysmotility in scleroderma oropharyngeal and esophageal involvement in DM PM and...

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...

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...

Desquamative Gingivitis

Desquamative gingivitis is a term used to describe a clinical reaction pattern that may be seen in a number of disease processes. It refers to a clinical condition in which the gingiva exhibits atrophy, erosion, and ulceration and possibly a positive Nikolsky sign (27,28). The term is used in a nonspecific manner, as are the terms leukoplakia and erythroplakia, which refer to white and red lesions, respectively, which cannot be attributed to any specific disease process. The designation...

Chagas Disease

Carlos Chagas described the tropical parasitic infection caused by Trypanosoma cruzi initially in 1909, while working in a remote area collecting bugs for malaria research. The protozoan T. cruzi is endemic to Central and South America and constitutes a significant public health concern in much of South America. The life cycle of T. cruzi involves transmission to humans from the feces of an insect vector. The acute phase is characterized by fever, malaise, and generalized lymphadenopathy. In...

Introduction

Mucormycosis (zygomycosis and phycomycosis) is one of the most acute, fulminant fungal infections known. Invasive fungal infections are major medical complications in immunocompromised patients (Table 1). The recent rise in the incidence of cancer and the increased use of newer medical treatment modalities, including organ transplantations, have resulted in growing numbers of highly immunosuppressed individuals. Although aspergillosis and candidiasis are among the most common invasive mycoses...

Infection Fungal

Candida albicans is a common organism found in the oral cavity flora that causes candidiasis in certain clinical situations. Candida has a capsule and forms true hyphae and pseudohyphae. It adheres to mucosal surfaces and is capable of superficial mucosal invasion. Factors that contribute to oral candida proliferation include uncontrolled diabetes mellitus, antibiotic therapy, and any condition that causes immunosuppression. Clinical features of candidiasis include white, cheesy...

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...

Case Example

A 51-year-old woman was transferred from a small community emergency department for subspecialty evaluation. The patient had presented to that facility the previous night complaining of headache and fever for three days with a sudden loss of vision in her left eye. During our evaluation, the patient reported that one week earlier, she fell, striking the left side of her face and sustaining several small abrasions. She denied other facial trauma. Further history revealed a general malaise and...

Stevensjohnson syndrome

In 1922, Stevens and Johnson described two boys who had an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis (27). Stevens-Johnson syndrome (SJS) (Figs 2 and 3) is a severe mucocutaneous disorder that is usually drug induced and associated with high morbidity and poor prognosis. Identifying patients with SJS is crucial, because these patients should be referred to intensive care units or burn centers for management. FIGURE 2...

Pathogenesis Infection Bacterial

At birth, the upper respiratory tract is colonized with multiple organisms and the normal bacterial flora is established. It consists primarily of Gram-positive aerobic organisms and anaerobic organisms. Infectious pharyngitis represents a change in this normal flora. Streptococcus. Streptococcus species are Gram-positive cocci bacteria arranged most commonly in chains. They are ubiquitous inhabitants of the oral and nasal cavities. Members of this bacterial family are classified...

S f A

Less common but clearly identifiable causes of angioedema include drug use and alteration of complement metabolism. A recent prospective study noted that angioedema due to angiotensin-converting enzyme inhibitor (ACE-I) use occurred in 86 of 12,557 patients (0.68 ) (12). ACE-I-induced angioedema was about three times more likely to occur among black patients (1.62 vs. 0.55 for white patients), and slightly more likely in women (0.84 vs. 0.54 for men). Approximately 50 of patients with...

Infection Viral

Adenovirus is the most common cause of viral pharyngitis. It is a double-stranded DNA virus. Serotypes 3,4, and 7 are frequently associated with viral pharyngitis. It is transmitted by either respiratory droplets or direct contact. School-aged children are most commonly affected. The classic presentation includes fever, sore throat, coryza, and red eyes. Adenovirus is cytolytic to the epithelial cells it invades and induces a localized inflammatory response in the surrounding...

Pathology

Rhabdomyosarcoma is classified into three cell types embryonal, alveolar (which has botryoid and spindle cell variants), and pleomorphic. The embryonal subtype accounts for 60 to 70 of childhood cases and is the most common subtype. It is also the most common subtype presenting in the head and neck (20-22). Rhabdomyosarcomas are malignant tumors of skeletal muscle. They are part of the larger group of soft-tissue sarcomas. Sarcomas of bony origin are classified separately. Like most malignant...

Jugular foramen syndrome

Jugular foramen syndrome (JFS) is an uncommon clinical entity that often presents a diagnostic and management challenge to the otolaryngologist. The purpose of this section is to define this syndrome and elucidate the epidemiology, pathogenesis, clinical manifestations, diagnostic workup, and general treatment options for the syndrome. Various combinations of palsies of cranial nerves IX, X, and XI resulting from lesions in the area of the jugular foramen have been referred to as JFS. Eponyms...

Head and Neck

Ulcerative Lesions Skin

Oral ulcerations represent the most common finding of disease and are seen in over 95 of patients with Adamantiades-Behget's. They are required for diagnosis (Table 1), although some argue that in rare cases, oral ulcers need not be present for the diagnosis to be made. The oral ulcerations seen in Adamantiades-Behget' s may be present on the tongue, lips, gingival surfaces, buccal mucosa, soft palate, or posterior pharynx (Fig. 1). In general, they are shallow and painful, resolving in...

Diagnosis

The vast majority of patients with hearing loss need only an audiogram to confirm the diagnosis. As stated earlier, most of these patients will be diagnosed with diseases limited to the inner ear such as presbycusis, noise-induced hearing loss, and ototoxicity. Asymmetry in hearing loss warrants the performance of magnetic resonance imaging (MRI) scan with paramagnetic enhancement to rule out a retrocochlear etiology. In the pediatric patient with unilateral sensorineural hearing loss, a CT...

Crohns Disease

Crohn's disease is a systemic inflammatory disorder affecting the entire GI tract. Although Crohn's disease most commonly affects the distal alimentary tract, esophageal involvement does occur. One study of patients without esophageal symptoms showed evidence of esophageal involvement in 5 by upper endoscopy (21). The typical findings are small, punctuate ulcerations in the esophageal mucosa. Rarely, fissures may form leading to fistula formation with adjacent organs. When patients are...

Rhinocerebral Mucormycosis

RCM represents one-third to one-half of all cases of Zygomycosis. The process originates in the nose and paranasal sinuses following inspiration of fungal spores. It is estimated that 70 of the cases of rhinocerebral zygomycosis occur in the setting of DKA (7). Disease starts with symptoms consistent with sinusitis. Low-grade fever, dull sinus pain, drainage, and soft-tissue swelling are initially seen, followed in a few days by double vision, increasing fever, and obtundation. Examination...

FIGURE 16 Oral hairy leukoplakia associated with EBV Source From

(PCR) for CMV viral load in serum can be used, but the clinical utility of the test is not yet clear. Whether treatment is beneficial for oral ulcers caused by CMV is not clear. Ulcerative esophagitis-causing symptoms can be treated with IV antivirals such as ganciclovir, foscarnet, or cidofovir, followed by oral maintenance, usually with oral valganciclovir. Epstein-Barr Virus and Oral Hairy Leukoplakia. OHL has been encountered since the early epidemic and has been strongly linked to...

Pernicious Anemia

Pernicious anemia is associated with atrophic gastritis. The marked loss of gastric parietal cells results in a deficiency of intrinsic factor, which is required to transport Vitamin B12 across the intestinal mucosa. The resulting deficiency of Vitamin B12, which is necessary FIGURE 43 Pernicious anemia. This elderly female complained of a painful tongue. The mucosa of the anterior portion of the tongue was severely atrophic and totally devoid of lingual papillae. Posteriorly, the mucosa was...

F

Coccidioides Immitis Structure

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...

Myeloproliferative Disorders

PRV is frequently discovered incidentally when a complete blood count is performed for another reason. When symptoms are present, they are usually nonspecific. Fatigue, headache, and diaphoresis are common. Pruritis, often following a hot shower, is a frequent complaint. Up to 15 of patients may present with a thrombotic episode. Thrombotic cerebrovascular accidents, coronary artery thrombosis, Budd-Chiari syndrome, and pulmonary embolus all occur. Cavernous sinus thrombosis may also occur in...

Infectious Diseases

Hearing loss is an uncommon presenting symptom of a systemic infectious disease but may develop during the course of several viral and bacterial infections. Pathogens may infect the inner ear and auditory pathways in utero, resulting in congenital syndromes that often include deafness, or infection may be acquired after birth. As the fluids of the inner ear are not easily accessible for sampling, the implication of viral pathogens as the causes of sensorineural hearing loss has often depended...

Human Immunodeficiency Virus

Infection with HIV is often heralded by presenting signs and symptoms involving systems of the head and neck. Hence, otolaryngologists must remain cognizant of this when evaluating patients whose findings may constitute those attributable to HIV infection, for which there is no definitive cure. HIV is discussed in greater detail in Chapter 16. Definition. HIV infection induces a disabling of T-cell immunocompetency, resulting in a constellation of findings associated with AIDS. Infection may be...

Stomatitis

Typical Location For Intraoral Herpes

Stomatitis refers to an inflammatory process involving the mucous membrane of the mouth that may manifest itself through a variety of signs and symptoms including erythema, vesiculation, bulla formation, desquamation, sloughing, ulceration, pseudomembrane formation, and associated discomfort. Stomatitis may arise due to factors that may be of either local, isolated conditions or of systemic origin. For example, a solitary oral ulcer with a history of a recurrent pattern may be classified as...

Lymphoma Involving Specific Head and Neck Sites

Images Follicular Lymphoma Neck

Lymphoma involving the thyroid gland is rare, accounting for only 2 to 3 of all cases of lymphoma and less than 10 of thyroid malignancies. Women are affected more frequently than men are by a ratio of 2.7 1. The median age at presentation is over 60 years. The most common subtypes are DLBCL and follicular Grade-3 lymphoma, accounting for at least 80 of cases. A rare but interesting subtype is marginal zone B-cell lymphoma, which arises from MALT. In many cases, this lymphoma...

Inflammatory Autoimmune

Pemphigus is a rare disease that affects mucosal membranes. Although the term pemphigus may be erroneously used interchangeably with the condition bullous pemphigoid, it is a separate entity warranting a separate discussion. Pemphigus is characterized by vesicular lesions and bulla. There are multiple subtypes, with pemphigus vulgaris and pemphigus foliaceus being the most common (7). Other subtypes include pemphigus vegetans, pemphigus erythematosus, pemphigus herpetiformis,...

Infectious Diseases Mycobacterial Infection

Atypical Mycobacteria Lymph Node

The incidence of mycobacterial infections has undergone a varied course over the past 50 years. For an approximate 30-year period following 1950, the incidence in mycobacterial infection declined annually in excess of 5 . This was followed by a paradoxical sharp increase at the rate of approximately 20 during the late 1980s and early 1990s, due to multiple factors such as acquired immunodeficiency syndrome (AIDS), drug resistance, and population influx from endemic areas. With improved...

Neuromuscular Disorders

Many neuromuscular diseases cause dysphagia in fact, the presenting symptom is often dysphagia. This is not surprising, considering the complex neuromuscular coordination required to execute a normal swallow. Although stroke patients develop sudden dysphagia, patients with other degenerative and nondegenerative neuromuscular diseases have a more insidious onset of dysphagia. Motor neuron diseases causing bulbar palsy or pseudobulbar palsy usually develop a progressive dysphagia and dysarthria...

Erythema Multiforme

EM is an acute, widely distributed hypersensitivity reaction associated with circulating immune complexes that are deposited in the basement membranes of the superficial vessels of the skin and mucosa. Subsequent complement activation produces vasculitis and thrombosis, leading to tissue ischemia and necrosis of the adjacent epithelium. The intensity of the skin and mucosal reaction varies from a localized minimal erythematous FIGURE 40 Erythema multiforme. Young adult male with rapid onset of...

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...

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...

Mixed Connective Tissue Disease

Mixed connective tissue disease is characterized by a combination of overlapping features of systemic lupus erythematous, scleroderma, and polymyositis. Typical presentation is with Raynaud's phenomenon, arthralgias, inflammatory myopathy, lymphadenitis, skin or mucosal lesions, and serositis. A key distinguishing factor of the disorder is a high titer of antibody to ribonucleoprotein, a finding absent in any of these three disorders SLE, scleroderma, and polymyositis . Neurologic dysfunction...

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...

Wegeners Granulomatosis

Wegener's granulomatosis is an idiopathic vasculitis of small arteries, arterioles, and capillaries primarily affecting the upper aerodigestive tract, lungs, and kidneys. Typical presentation is in middle-aged patients with a slight male predominance. Otologic manifestations are common and range from 19 to 45 of cases 103 . They include conductive hearing loss, sensorineural hearing loss, otalgia, otorrhea, and serous otitis media. Neurologic involvement is frequent with nearly half of patients...

Crohns Disease and Ulcerative Colitis

Department of Medicine, Division of Gastroenterology, Inflammatory Bowel Disease Center, Mount Sinai School of Medicine, New York, New York, U.S.A. Clinical Manifestations 299 Crohn's disease CD and ulcerative colitis, collectively part of the inflammatory bowel diseases IBD , are both diseases of the gastrointestinal tract that arise in genetically susceptible individuals. Both CD and ulcerative colitis, though clinically different entities, are thought to be part of a spectrum of diseases...

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...

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 occurs...

Mucous Membrane Pemphigoid

Pemphigoid Bullous Mucosa

MMP is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the hemidesmosome destroy the epithelial-connective tissue attachment at the level of the basement membrane, producing a subepithelial separation 24,25 . The protein targets of the autoantibodies include BP-1, BP-2, and laminin-5 epiligrin , all components of the epithelial anchoring apparatus. MMP is a generalized term for a group of closely related disease processes 26 . The term oral...

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...

Cerebrovascular Disease

Cerebrovascular disease is the most common disorder causing neurogenic oral and oropharyngeal dysphagia 3 . Swallowing disorders have been reported in 27 to 50 of patients suffering from CVAs 4 . The incidence is not limited to severe strokes, because even patients with mild-to-moderate strokes have a poorer long-term outcome directly attributable to their swallowing disorder 5 . Given the complex neurophysiology associated with swallowing, a wide range of stroke locations can result in...

Migraine Syndrome

Migraine Aura Fortification

Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, California, U.S.A. Clinical Manifestations 336 Complications and Prognosis 340 Migraine is a syndrome characterized by periodic headaches. Often, however, patients experience other symptoms, including hearing loss, tinnitus, and dizziness and in some cases, these can be the only symptoms. Migraine is nearly always familial and occurs in complex patterns and settings. The association of migraine and...

Mycobacterial Infections

Tuberculosis TB, from Mycobacterium tuberculosis and or Mycobacterium bovis is a major cause of morbidity and mortality in HIV disease. It usually presents as reactivation of a pulmonary primary focus, with a risk of 7 to 10 per year for HIV-infected persons regardless of CD4 lymphocyte count, versus 10 per lifetime for HIV-negative persons. There can be involvement of the lungs, central nervous system CNS , or other organs, with rhinosinusitis, diffuse or localized scrofula...

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...