Extranodal natural killer/T cell lymphoma, nasal type (ENKL) is a rare and aggressive tumour that can, clinically and histologically, mimic infectious and inflammatory conditions, presenting a diagnostic challenge. The authors report the case of a 69-year-old Portuguese woman previously misdiagnosed with chronic recurrent sinusitis. Despite maximal medical and surgical treatments, the disease was refractory and progressed. The patient had undergone multiple biopsies when the histopathological diagnosis of ENKL was made, 5 months after the initial complaints. Multiagent chemotherapy was offered, but during the first cycle, the patient developed severe infection and pancytopenia, which culminated in her death. This case highlights the need to consider a neoplastic cause when faced with aggressive sinonasal disease not responsive to maximal treatment and the difficulties in establishing the diagnosis of ENKL, with multiples biopsies of deep-tissue usually being required.
Chondrosarcoma of the larynx is a rare, slow-growing malignant tumor. One hundred and fifty cases of this entity have been reported in the literature. In 75% of cases the site of involvement was the cricoid cartilage, while 20% have involved the thyroid cartilage. The tumor presents as a space-occupying lesion in the subglottic region, or as a neck mass. An insidious, slowly progressing disease course associated with hoarseness and dyspnea accounts for usual delays in arriving at a diagnosis. We report our experiences with six cases of this entity and discuss a diagnostic and therapeutic approach. Whenever possible a conservative surgical management should be attempted.
Objectives
To evaluate hearing outcome of salvage treatment with intratympanic steroids (ITS) in idiopathic sudden sensorineural hearing loss (ISSNHL) refractory to initial systemic steroid (SS) therapy.
Material and methods
A retrospective medical chart review was conducted on 54 consecutive patients with ISSNHL refractory to SS. Salvage treatment with a low dose intratympanic dexamethasone (4 mg/ml) was offered after one week of primary treatment. Patients were divided into two groups: 25 patients accepted ITS (treatment group) and 29 patients did not undergo additional treatment (control group). A pure tone average (PTA) gain of at least 10 dB was considered hearing improvement.
Results
Hearing improvement rate was higher in ITS group compared to control group (40% vs. 13.8%, p = 0.035). A mean PTA improvement of 8.6 ± 9.8 dB was observed in the ITS group and, whereas the control group had an average hearing gain of 0.7 ± 2 dB (p < 0.001). Audiometric analysis revealed a significant hearing gain in ITS group at all tested frequencies compared to control group (p < 0.05). Analysis of the selected variables, identified intratympanic steroid treatment as the only independent prognostic factor for hearing improvement (OR = 4.2, 95% CI: 1.1–15.7; p = 0.04).
Conclusion
Intratympanic low dose dexamethasone is effective in patients with incomplete hearing recovery after primary systemic steroid treatment.
Pneumolabyrinth is the entrapment of air within the inner ear and is a rare complication of stapes surgery. We report the case of a patient submitted to stapedectomy who, 4 weeks later, suddenly developed right hearing loss, ipsilateral tinnitus and vertigo. On the physical examination, the patient showed no signs of vestibular deficits. Audiometry was compatible with right profound mixed hearing loss and high-resolution CT of the temporal bone revealed the presence of pneumolabyrinth. During exploratory tympanotomy, the prosthesis was found dislodged; the communication between the middle and inner ear was closed with vein graft and a new prosthesis was placed. Following surgery, vestibular symptoms was abolished and the patient experienced great improvement of the hearing thresholds.
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