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Methotrexate in the management of immune mediated cochleovesitibular disorders: clinical experience with 53 patients.

OBJECTIVE: To describe our experience with low dose weekly methotrexate (MTX) in the management of immune mediated cochleovestibular disorder (IMCVD). METHODS: Between 1991 and 1999, we treated 53 patients with IMCVD with MTX. Patients were selected on the basis of progressive vestibular symptoms that had responded to corticosteroids and in most cases, relapsed. MTX was initiated at a dose of 7.5 mg weekly and increased to doses up to 25 mg weekly as needed. Response was assessed by audiologic studies and history of change in tinnitus and vertigo. MTX was discontinued after 4-6 mo in patients showing no improvement, and after 12-18 mo in patients with improved and stable symptoms. RESULTS: Three patients were still in early therapy and had not improved. Of the 50 remaining patients, significant improvement was seen in vertigo in 27/39 (69%) patients, hearing loss in 25/47 (53%), and tinnitus in 11/42 (26%). Overall improvement in symptoms was seen in 35/50 (70%) patients. Four patients stopped MTX due to toxicity, and 11 due to lack of response. In 28 patients, MTX was stopped after 12-18 mo when symptoms had stabilized, and restarted in 5 of these after relapse. Seven patients remain on therapy with improved and stable symptoms after 17.3 mo. CONCLUSION: In this open label experience, a majority of patients with IMCVD improved with weekly low dose MTX therapy with minimal toxicity.

Adolescent↗

Acute measles infection in the hamster cochlea.

Measles virus has been implicated in sudden sensorineural hearing loss (SNHL) in adults as well as in children. Furthermore, sensorineural hearing loss following live measles virus vaccination has been reported. As of yet, however, there has been only few reports on human temporal bone pathology due to measles, and on experimental animal models of measles infection. This study was undertaken to examine acute measles infection in adult hamster cochlea morphologically and immunohistochemically for precise understanding of this viral infection. Atrophy of the stria vascularis, loss of the organ of Corti, "rolled-up" tectorial membrane, and cell infiltration with a positive immunofluorescent reaction primarily within the scale media indicating endolymphatic labyrinthitis were the principal findings. These results were generally consistent with previous ones on human temporal bone pathology not only due to measles but also to SNHL. We consider measles virus to be one of the possible pathogens, even if low in frequency, causing profound and irreversible hearing loss, including SNHL. Completion of measles vaccination without complication and selective or mass revaccination may be necessary to prevent such hearing loss.

Acute Disease↗

Meniere's disease: prevalence of contralateral ear involvement.

OBJECTIVE: Determine the prevalence and time interval for conversion from unilateral to bilateral involvement in Meniere's disease and cochlear hydrops. STUDY DESIGN AND SETTING: Retrospective chart review in a tertiary otologic referral center. PATIENTS: 232 patients diagnosed with Meniere's Disease (n=186) or cochlear hydrops (n=46) between 1959 and 2001, who visited the clinic over a five-year period between 1997-2001 and have at least 2 audiograms more than 12 months apart. MAIN OUTCOME MEASURES: Prevalence of cochlear hydrops relative to Meniere's Disease, rate of progression from unilateral to bilateral involvement; interval between unilateral onset of symptoms and bilateral involvement; and rate of progression from cochlear hydrops to Meniere's disease. RESULTS: Initial diagnosis was Meniere's disease in 71% and cochlear hydrops in 29% of all 950 hydropic patients presenting between 1997 and 2001. In the study sample, Meniere's disease was bilateral at presentation in 11%; an additional 12% (14% of unilaterals) became bilateral during the follow-up period. At presentation, 6.5% of cochlear hydrops patients were bilateral, with another 26% becoming bilateral. Conversion from cochlear hydrops to Meniere's disease occurred in 33% and some of these are included among the bilateral. The average time interval for conversion from unilateral to bilateral Meniere's was 7.6 years (SD=7.0 years). CONCLUSION: Most otologists are aware of the potential for contralateral ear involvement and conversion from cochlear hydrops to Meniere's disease after diagnosis. These changes are significant, require long-term follow-up for detection, and may necessitate further treatment. Patients should be counseled regarding this potential when interventions are considered, especially with respect to ablative treatments.

Adult↗

Cochlear endolymphatic shunt for Meniere's disease.

We have now performed the cochlear endolymphatic shunt operation for Meniere's disease on 102 ears and have follow-up data on ninety cases. In this fourth report of results of the procedure, we show that after follow-up ranging from one month to six years (average 22.3 months), definitive vertigo was relieved in 72% of the cases and that hearing at the time of most recent audiogram (average 12.6 months postoperatively) was worse (AAO criteria) in 45% of the cases.

Adolescent↗

Transmeatal cochlear laser (TCL) treatment of cochlear dysfunction: a feasibility study for chronic tinnitus.

Low-level-laser-therapy (LLLT) targeting the inner ear has been discussed as a therapeutic procedure for cochlear dysfunction such as chronic cochlear tinnitus or sensorineural hearing loss. Former studies demonstrate dose-dependent biological and physiological effects of LLLT such as enhanced recovery of peripheral nerve injuries, which could be of therapeutic interest in cochlear dysfunction. To date, in patients with chronic tinnitus mastoidal and transmeatal irradiation has been performed without systematic dosimetric assessment. However, light-dosimetric studies on human temporal bones demonstrated that controlled application of laserlight to the human cochlea depends on defined radiator position within the external auditory meatus. This feasibility study first presents a laser application system enabling dose-controlled transmeatal cochlear laser-irradiation (TCL), as well as preliminary clinical results in patients with chronic cochlear tinnitus. The novel laser TCL-system, consisting of four diode lasers (lambda=635 nm-830 nm) and a new specific head-set applicator, was developed on the basis of dosimetric data from a former light-dosimetric study. In a preliminary clinical study, the TCL-system was applied to 35 patients with chronic tinnitus and sensorineural hearing loss. The chronic symptoms persisted after standard therapeutic procedures for at least six months, while retrocochlear or middle-ear pathologies have been ruled out. The patients were randomised and received five single diode laser treatments (lambda=635 nm, 7.8 mW cw, n=17 and lambda=830 nm, 20 mW cw, n=18) with a space irradiation of 4 J/cm2 site of maximal cochlear injury. For evaluation of laser-induced effects complete otolaryngologic examinations with audiometry, tinnitus masking and matching, and a tinnitus-self-assessment were performed before, during and after the laser-irradiation. The first clinical use of the TCL-system has been well tolerated without side-effects and produced no observable damage to the external, middle or inner ear. Changes of tinnitus loudness and tinnitus matching have been described. After a follow-up period of six months tinnitus loudness was attenuated in 13 of 35 irradiated patients, while two of 35 patients reported their tinnitus as totally absent. Hearing threshold levels and middle ear function remained unchanged. Further investigations by large double-blind placebo-controlled studies are mandatory for clinical evaluation of the presented TCL-system and its therapeutic effectiveness in acute and chronic cochlear dysfunction.

Chronic Disease↗

Intratympanic steroids: do they acutely improve hearing in cases of cochlear hydrops?

OBJECTIVE: To study the acute effects on hearing of intratympanic dexamethasone in patients with cochlear hydrops. STUDY DESIGN: Retrospective review. METHODS: Patients who met established criteria for the diagnosis of Ménière's disease or had a history of fluctuating hearing loss and met hearing loss criteria for Ménière's disease, indicating cochlear hydrops, underwent a series of one to three intratympanic injections of dexamethasone in the affected ear. Follow-up audiograms were obtained 1 week after each injection and, in many patients, several months after injection. RESULTS: Fifty patients met inclusion criteria and were studied. Using the American Academy of Otolaryngology-Head and Neck Surgery reporting guidelines, hearing improved acutely in 20 of the 50 patients (40%), was worse in 2 (4%), and did not change in 28 (56%). For those who improved, the average decrease in threshold was 14.2 dB. Whether the patient had typical Ménière's disease or cochlear hydrops did not affect the response to therapy. There were no significant complications from the injections. CONCLUSIONS: Intratympanic administration of dexamethasone may acutely affect sensorineural hearing loss associated with endolymphatic hydrops. A prospective, controlled study is required.

Administration, Topical↗

Canavan's leukodystrophy is associated with defects in cochlear neurodevelopment and deafness.

The authors present the temporal bone histopathology of two siblings (4 months old and 6 months old at autopsy) with Canavan's disease, an autosomal recessive leukodystrophy that is variably associated with sensorineural hearing loss. The histopathology demonstrated bilateral absence of the organ of Corti throughout the apical and basal cochlea and mild secondary atrophy of the spiral ganglia neurons. The vestibular end organs and ganglia were normal. These findings implicate a role of aminoacylase II in the neurodevelopment of the organ of Corti.

Atrophy↗

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↗

A comparison of PORP, TORP, and incus homograft for ossicular reconstruction in chronic ear surgery.

This report presents the results of 210 cases over a 10-year period using PORPs, TORPs, and notched incus homografts (NIH), for ossicular reconstruction in chronic ear surgery. There were 192 adults and 18 children. The surgical technique utilized temporalis fascia in an underlay technique with canal skin covering the outer surface of the fascia. Intact canal wall mastoid-tympanoplasty, as a one-stage procedure, was used for most cases. Homograft nasal cartilage was placed between the Plasti-Pore prosthesis and the graft. Notched incus homografts were prepared prior to surgery and stored in 4% formalin. There were 149 mastoid-tympanoplasties and 61 tympanoplasties performed. Revision of our cases was performed in 16.6%. Within 3 months of surgery, 86% of adults, and 44% (8/18) of children had dry, healed ears free of disease. The graft take rate was 96%. In adults, a total of 99 NIH, 50 TORPs, and 43 PORPs were implanted. In adults, the closure of the air-bone gap to 20 dB or less occurred in 58% using TORPs, 67% using PORPs, 76% using NIH-Partial replacement, and 20% using NIH-Total replacement. Excluding the cases that failed for reasons other than conductive hearing loss, the results improved to 69% for TORPs, 77% for PORPs, 77% for NIH-P, and 27% for NIH-T. In adults, the extrusion rate was 5.5% for Plasti-Pore and 3% for NIH. In children, the extrusion rate was 17% for Plasti-Pore prostheses. From this study, it appears that PORPs and TORPs with homograft nasal cartilage are satisfactory prostheses for chronic ear surgery in adults. In children, Plasti-Pore prostheses should be avoided unless the ear is healed, aerated, and stable. NIHs are good prostheses when the stapes is intact, but they are inferior to the TORP when placed on the footplate. Also, the NIH requires preparation prior to surgery and may be difficult to obtain. We plan to continue using PORPs and TORPs in chronic ear surgery until a better technique is found, or the complication rate becomes unacceptable.

Adult↗

Bioactive ceramics versus proplast implants in ossiculoplasty.

During the last decade, the surgical use of alloplasts has become more and more widespread among otologists. Yet, based upon long-term clinical surveys and histologic findings, a debate has recently arisen about the biocompatibility and structural stability of these materials. Outcomes, one to five years postoperatively, are reported here for 188 patients who underwent ossiculoplasty with either Proplast (N = 118) or Ceravital (N = 70) columellas. Ceravital performed 10% better than Proplast with regard to hearing improvement. However, almost 25% of the Ceravital patients did not attain an appreciable auditory gain;even compared with Proplast's 35% figure, this is still too high a figure. The bioactive ceramic yielded an extrusion rate of 8%, whereas 18% of the Proplast prostheses with cartilage interposition were rejected. Causes of failure other than extrusion were detected in a non-negligible number of cases. Actually, columellar ossiculoplasty requires the most realistic expectations and the wisest surgical strategy. Besides the biophysical behavior of the implant, control of the primary disease and the achievement of the best prereconstructive anatomic set-up are critical prerequisites to a fully satisfactory outcome. In this context, staging of the procedure for functional aims seems to play a major role that has perhaps been neglected too often.

Adolescent↗