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Cochlear implantation in the chronically diseased ear.

PURPOSE OF REVIEW: The presence of chronic ear disease in patients with profound SNHL presents a challenge to the otologic surgeon and was considered, in the past, a contraindication to cochlear implantation. This review discusses options for cochlear implantation in patients with chronically diseased ears. RECENT FINDINGS: Several management options are available for cochlear implantation in patients with chronic suppurative otitis media (CSOM) and severe to profound sensorineural hearing loss (SNHL). CONCLUSION: Cochlear implantation can be safely achieved in patients with CSOM. The approach chosen should be individualized based on clinical findings.

Adolescent↗

Practical versus theoretical management of autoimmune inner ear disease.

Autoimmune inner ear disease is an uncommon but distinct clinical entity. Our ignorance of the immune mediating pathways, need of further animal model experimentation, variability of laboratory test results and of patient treatment responses illustrate how poorly we understand this disorder. The purpose of this review is to compare practical vs theoretical management of autoimmune inner ear disease, based upon our current knowledge of the disease process and upon a review of clinical experience at the Cleveland Clinic Foundation. Representative case histories are presented. The following preliminary conclusions are discussed: Autoimmune inner ear disease can present as a systemic or localized otologic immune disorder. Hearing loss can begin at any age, with unilateral or bilateral sudden onset, fluctuating or progressive symptoms, with or without associated dizziness. The pathogenesis of autoimmune inner ear disease is probably multifactorial (cellular and humoral). The sensitivity and specificity of different laboratory tests vary greatly, but even the most sensitive tests may be falsely normal when symptoms are not acute or when the patient is taking immunosuppressant medication. The mainstay of autoimmune inner ear treatment is steroids: however, cytotoxic drugs are recommended when there is no response to steroid treatment. Apheresis is reserved for selected cases. Hearing improvement can be dramatic even after 2 months of profound deafness. Flare-ups of autoimmune ear disease are best managed by increasing steroid dosage or adding cytotoxic medications. Unfortunately, some patients will develop progressive hearing loss despite vigorous treatment.

Adolescent↗

Sulfoglucuronosyl glycolipids as putative antigens for autoimmune inner ear disease.

Autoimmune inner ear disease is diagnosed based on clinical history of fluctuating but progressive sensorineural hearing loss (SNHL) with or without vestibular symptoms occurring over weeks to months. An initial response to steroids or immunosuppressive drugs usually reverses the hearing loss. In search of specific diagnostic and therapeutic markers for autoimmune inner ear diseases, we investigated serum anti-glycolipid antibody activities in these patients by two different methods, HPTLC-immunoblotting and ELISA. We found that 37 out of 74 patients of clinically diagnosed autoimmune inner ear disease (30 of sensorineural hearing loss (SNHL) (group I), 14 of vestibular symptoms only (group II), 30 of Menieres symptoms (with both hearing loss and vestibular symptoms) (group III)) showed positive anti-sulfoglucuronosyl lactosaminyl paragloboside (SGLPG) antibody titers (p < 0.001). On the other hand, anti-sulfoglucuronosyl paragloboside (SGPG) titers were not elevated in these conditions. In contrast, only 3 out of 56 pathological control and 2 out of 28 healthy volunteers had measurable anti-SGLPG antibody titers. We further analyzed the localization of SGLPG in the auditory pathway and found that the antigens existed exclusively in inner ear and the eighth nerve, but not in pons, cerebellum, nor cerebrum. We conclude that the anti-SGLPG antibody represents a novel diagnostic marker for autoimmune inner ear disease and may participate in the pathogenesis of this disease.

Autoantibodies↗

Pathophysiological mechanisms in immune inner ear disease.

Immune inner ear disease is a somewhat controversial entity which describes cochleovestibular dysfunction that is related to immune-mediated mechanisms. The diagnosis of this disease is based on clinical presentation and response to various treatment protocols. Unfortunately, the presentation is variable and the treatment empirical, and this has caused much confusion in the diagnosis and management of the condition. To elucidate the variable nature of the disease, it is important to understand that more than one mechanism of immune injury may be involved. This paper attempts to classify clinical and experimental cases of immune inner ear disease with regard to the Gell and Coombs classification scheme of immune-mediated injury. By understanding the different pathophysiological mechanisms involved, the clinician should be better able to diagnose and manage this difficult problem in a directed fashion. The ramifications of the proposed classification system on the diagnosis, treatment and future research of immune inner ear disease are discussed.

Autoimmune Diseases↗

Laboratory diagnosis of immune inner ear disease.

Immune inner ear disease is a relatively new and distinct clinical entity that produces unexplained, rapidly progressive, bilateral sensorineural hearing loss. The diagnosis is based on clinical manifestations, positive immune laboratory testing, and beneficial response to a trial of corticosteroids. Immune laboratory tests are used to confirm a presumptive clinical diagnosis. The two tests most commonly used are the lymphocyte transformation test and Western blot immune assay. The rationale behind these tests is presented, and the role they play in laboratory diagnosis of immune inner ear disease is discussed.

Antibodies↗

Role of pneumatic otoscopy in the evaluation of tympanic membrane and middle ear diseases.

Many middle ear diseases show visible changes on the tympanic membrane which can be identified with pneumatic otoscopy. With practice and patience the technique can be easily performed even on infants and very young children and can reveal what may guide therapeutic approach. Careful positioning of the patient and proper insertion of a fitting ear speculum are of paramount importance. Mobility of the tympanic membrane may be absent in certain conditions and decreased or increased in other conditions. White masses are sometimes observed in the tympanic membrane or in the middle ear. These may be normal bony structures, tympanosclerosis or cholesteatoma and pneumatic otoscopy may help differentiate these conditions.

Age Factors↗

Changing patterns of contact allergy in chronic inflammatory ear disease.

Chronic inflammatory ear disease comprises a wide range of conditions. Although contact allergy to topical medicaments is common in such conditions, the causes of these reactions have not been examined in a large series for over a decade. Our aim was to investigate the major sensitizers recorded in 179 patients over the last 17 years. Medicament contact allergic dermatitis was diagnosed in 45 patients (25%), of whom 34 (76%) demonstrated a relevant allergy to neomycin, 28 (62%) to framycetin, 11 (31%) to gentamicin, 8 (18%) to quinoline mix and 5 (11%) to caine mix. Of all neomycin-allergic patients, 28 (76%) were also allergic to framycetin and 12 (43%) to gentamicin. Comparing the first 60 patients (1985-94) to the most recent 60 (1998-2002) identified a significant recent reduction in the number of patients allergic to neomycin (P = 0.002) and framycetin (P = 0.050), with a non-significant reduction in the total number diagnosed as medicament-allergic (P = 0.066). We have therefore demonstrated a high frequency of medicament contact allergy in chronic inflammatory ear disease, supporting the routine use of patch testing in these patients. We have also shown a significant recent reduction in the number of such patients with allergy to neomycin and framycetin.

Adolescent↗

Physical and behavioral aspects of middle ear disease in school children.

Middle ear disease is today's primary aural health problem in school-age children. Until recently, pediatric health researchers were aware only of the medical consequences that middle ear disease had no children. Except in a few cases of chronic otitis media, the effects of middle ear disease were considered transitory. However, current research on middle ear disease suggests permanent changes in hearing sensitivity, reduced development of the auditory neural network and developmental delays in speech, language and cognitive skills dependent on hearing. This article presents a review of recent studies on the physical and behavioral aspects of middle ear disease and its sequelae in school children.

Auditory Pathways↗

New concepts in the pathophysiology and management of middle ear disease in childhood.

Middle ear disease encompasses acute otitis media, recurrent otitis media, and otitis media with effusion. For many children, middle ear disease occurs early in life, is chronic and recurrent and can impair language development and/or school performance. Risk factors for recurrent otitis media include early disease onset, bottle feeding, daycare attendance, exposure to cigarette smoke and immunological defects or immaturity. Antimicrobial therapy in acute otitis media is associated with earlier resolution and a reduction in the frequency of persistent disease and suppurative complications. An antimicrobial agent should be selected according to its activity, tolerability and concentrations achieved in the middle ear. At present, amoxicillin remains the drug of choice for acute otitis media. Antimicrobial prophylaxis is also effective, but only for the duration of therapy. Future challenges for the management of middle ear disease include the treatment of penicillin-resistant Streptococcus pneumoniae, effective prevention of relapses, and the development of a vaccine for S. pneumoniae that has the appropriate serotypes and is immunogenic in young children.

Anti-Bacterial Agents↗

Relationship between three inner ear antigens with different molecular weights and autoimmune inner ear disease.

Crude inner ear antigen (CIEAg) can induce autoimmune inner ear disease (AIED) although it is not known which subcomponent of CIEAg is involved. In this study, we investigated the relationship between 3 purified inner ear antigens (31, 42-45 and 60 kD proteins) and AIED, and determined their distribution in normal guinea pig cochlea. Three groups of guinea pigs were immunized with the three inner ear antigens and one group served as a control. The hearing thresholds, serum IgG level and morphological changes in the inner ear were observed. The expression of the three antigens in the cochlea was detected using immunohistochemical techniques. No obvious changes in hearing thresholds or inner ear morphology were observed between the control and 42-45 kD groups. Animals immunized with the 31 or 60 kD proteins showed a significant increase in hearing thresholds (p < 0.05 vs control), accompanied by morphological changes in the inner ear. The serum IgG level was increased significantly (p < 0.05) in all immunized animals. The 31 kD protein was distributed in the cochlear nerve and spiral ganglion, while the 42-45 and 60 kD proteins were distributed widely, being found in the spiral ganglion, organ of Corti, stria vascularis and spiral ligament. These results suggest that two subcomponents of CIEAg (the 31 and 60 kD proteins) may induce AIED independently, that several inner ear antigens may contribute to the pathogenesis of AIED and that the 31 kD protein is of high tissue specificity and may be used as a marker protein for the clinical diagnosis of AIED.

Animals↗

Ciprofloxacin for the treatment of chronic ear disease.

The treatment of chronic ear disease is often difficult and frustrating. Patients typically present with a history of chronic, persistent otorrhea that has failed to respond to multiple topical and oral antibiotics. Organisms that are resistant to multiple antibiotics are common. Ciprofloxacin has been shown to be effective against a wide range of gram-negative and gram-positive organisms. To evaluate the role of ciprofloxacin in the treatment of chronic ear disease, 21 patients who failed routine therapy for chronic ear disease were prospectively treated with oral ciprofloxacin. Prior to therapy, all ear cultures grew Pseudomonas aeruginosa, Staphylococcus aureus or other gram-negative organisms. Ninety-five percent of patients completing therapy showed either improvement or cure. Only one patient failed to improve. Ciprofloxacin has been shown to be effective in the management of chronic ear disease.

Adult↗

Inflammatory cells in chronic middle ear disease. Value of lymphocyte subset determination in ear surgery.

Lymphoid cell subsets were studied in biopsy specimens of uninfected and infected ear canal epidermis, tympanic membrane, cholesteatoma epithelium, and middle ear and antrum granulation tissue. All specimens showing uninfected squamous epithelium, including those of cholesteatoma membrane, contained only a few lymphoid cells. Biopsies of infected squamous epithelium showed enhanced Ts/c activity in the subepithelial layer. Granulation tissue showed normal Th/i activity. B-cells and NK cells appeared in small numbers. Lymphoid cell subset analysis is indicated in ears reconstructed with allograft material to detect signs of rejection. It is of little value in cases of chronic inflammatory middle ear disease which have not previously been operated upon.

Cholesteatoma↗