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- Nasal septum perforation as the presenting sign of lupus erythematosusPublication . Mascarenhas, R; Tellechea, O; Oliveira, H; Reis, JP; Cordeiro, M; Miguéis, JNasal septum perforation is an uncommon and not well known feature of lupus erythematosus (LE). In general, it occurs during exacerbations and in a context of systemic vasculitis. Very rarely it can be a presenting sign, accompanying more usual manifestations of LE. We report the case of a 30-year-old woman who presented with a 2-year history of painful, slowly progressive nasal septum perforation. Laboratory study disclosed positive antinuclear antibodies, circulating immune complexes, hypocomplementemia, nuclear epidermal deposition of IgG in normal skin and transitory positive antiphospholipid antibodies. Symmetric peripheral joint arthritis, photosensitivity and diffuse alopecia subsequently developed. This case seems unique in that the nasal septum perforation occurred as an isolated presenting sign; it emphasizes the value of this feature in the diagnosis of LE.
- USPIO-enhanced magnetic resonance imaging for nodal staging in patients with head and neck cancerPublication . Curvo-Semedo, L; Diniz, M; Miguéis, J; Julião, MJ; Martins, P; Pinto, A; Caseiro-Alves, FPURPOSE: To determine the accuracy of ultrasmall superparamagnetic iron oxide (USPIO)-enhanced magnetic resonance imaging (MRI) for nodal staging in patients with head and neck cancer. MATERIALS AND METHODS: Twenty patients with carcinomas of the upper aerodigestive tract were prospectively enrolled. MRI was performed before and 24-36 hours after intravenous infusion of an USPIO agent, ferumoxtran-10 (Sinerem; Guerbet, France; and Combidex; Advanced Magnetics) at a dose of 2.6 mg Fe/kg using T2-weighted spin-echo and gradient-echo sequences. Surgery was performed the same day or the day after the ferumoxtran-10-enhanced MR examination. Based on MRI, selected nodes were surgically removed and directly correlated with pathology using hematoxylin-eosin (H&E) and Perls stainings. RESULTS: A total of 63 nodes were studied; 36 were nonmetastatic, 25 metastatic, and two inflammatory. Ferumoxtran-10-enhanced MRI allowed diagnosis of 24 metastatic and 30 nonmetastatic nodes, yielding a sensitivity of 96%, a specificity of 78.9%, a positive predictive value of 75%, and a negative predictive value of 96.8%, compared to 64%, 78.9%, 66.6%, and 76.9%, respectively, for nonenhanced MRI. Accuracy of ferumoxtran-10-enhanced MRI was 85.7%. The gradient-echo T2-weighted sequence was the most accurate to detect signal loss in nonmetastatic nodes. CONCLUSION: USPIO-enhanced MRI is useful for nodal staging of patients with head and neck cancers
- The Portuguese version of Mini-Tinnitus Questionnaire: brief screening test for assessment of tinnitus-induced stress.Publication . Cerejeira, R; Cerejeira, J; Paiva, S; Gonçalves, P; Firmino, H; Quartilho, MJ; Vaz-Serra, A; Paiva, AHYPOTHESIS: The Portuguese version of Mini-Tinnitus Questionnaire (Mini-TQ) is as valid as the English version to assess tinnitus-associated distress in the Portuguese-speaking population. BACKGROUND: Tinnitus is a major symptom in ENT practice affecting subjects in all demographic groups. Our objective is to validate a Portuguese version of Mini-TQ (Mini-TQ-pv) to be used in clinical practice and research. METHODS: Mini-TQ-pv was administered to 51 patients with chronic tinnitus. Statistical analysis was done to determine the psychometric properties of the instrument. RESULTS: After double translation, face and content validity were confirmed by high internal consistency (Cronbach alpha = 0.861) and significant correlation between individual items and total score. The questionnaire was easy and quick to administer (2.57 min). CONCLUSION: We provide a suitable Mini-TQ-pv to be used in the assessment of Portuguese-speaking patients with tinnitus
- Tympanoplasty in children: A review of 91 casesPublication . Ribeiro, JC; Cerejeira, R; Silvestre, N; Romão, J; Paiva, AOBJECTIVES: There is a marked diversity in the reported success rates for achieving an intact tympanic membrane following tympanoplasty. Controversy exists about the factors thought to influence surgical outcome. These facts have important implications for the selection of patients who would benefit the most. This study reviews the factors thought to determine the anatomical and functional success of tympanoplasty in children. MATERIALS AND METHODS: Retrospective study of the anatomical and functional results of 91 tympanoplasties performed in children. Age, gender, size and site of perforation, status of operated and contralateral ear, underlying cause of the perforations, surgical technique, pre-operative and post-operative hearing levels, post-operative follow-up time and post-operative complications were recorded. We divided our population into two groups according to the expected eustachian tube maturity (younger group (N=24): ≤10 years old, older group (N=67): >10 years old). All patients were evaluated in terms of anatomical and functional outcome and complications. RESULTS: Anatomical success was achieved in 85.7% and functional success was 76.9% after a mean follow-up of 25.6 ± 17.1 months. Anatomical success (intact tympanic membrane) was achieved in 83% of younger vs 87% of older patients (p=n.s.). Functional (air bone gap closure) success was 75% in the younger group vs 78% in the older group (p=n.s.). There were no significant differences in post-operative gain at different frequencies (500, 1000, 2000 and 3000 Hz) between the two groups. A previous adenoidectomy in children older than 10 years seems to be an independent predictor of functional success The incidence of minor and major complications were 29% in patients aged ≤10 and 21% in those older than 10 (p=n.s.). We report 12.9% minor post-operative complications in successful cases: injury to the chorda tympani nerve (5.7%), wound infection (2.9%), otitis externa (2.9%) and transient vertigo (1.4%). Among the 21 reperforations observed, 92.3% occurred before 1 year. CONCLUSIONS: This study shows that tympanoplasty is a valid treatment modality for tympanic membrane perforation in the pediatric population. A tympanic membrane perforation can be closed at any age. There is no age limit below which perforation should not be closed. A previous adenoidectomy in children older than 10 years seems to be an independent predictor of functional success.
- Evaluation protocol for amusia: portuguese samplePublication . Peixoto, MC; Martins, J; Teixeira, P; Alves, M; Bastos, J; Ribeiro, JCAmusia is a disorder that affects the processing of music. Part of this processing happens in the primary auditory cortex. The study of this condition allows us to evaluate the central auditory pathways. OBJECTIVE: To explore the diagnostic evaluation tests of amusia. METHOD: The authors propose an evaluation protocol for patients with suspected amusia (after brain injury or complaints of poor musical perception), in parallel with the assessment of central auditory processing, already implemented in the department. The Montreal Evaluation of Battery of amusia was the basis for the selection of the tests. From this comprehensive battery of tests we selected some of the musical examples to evaluate different musical aspects, including memory and perception of music, ability concerning musical recognition and discrimination. In terms of memory there is a test for assessing delayed memory, adapted to the Portuguese culture. Prospective study. RESULTS AND CONCLUSIONS: Although still experimental, with the possibility of adjustments in the assessment, we believe that this assessment, combined with the study of central auditory processing, will allow us to understand some central lesions, congenital or acquired hearing perception limitations.
- Otologic Wegener's granulomatosisPublication . Mesquita-Oliveira, P; Silvestre, N; Silva, C; Romão, J
- Downbeat nystagmus elicited by eyelid closurePublication . Lemos, J; Pereira, D; Amorim, M; Santiago, B; Paiva, A; Cunha, LWe describe a patient with downbeat nystagmus (DBN) evoked only by eye closure. Brain and spinal cord magnetic resonance imaging revealed a T2 paramedian lesion in the left lower basis pontis and other white matter lesions consistent with multiple sclerosis. One potential mechanism for DBN in this case involves transverse ephaptic spread of excitation from areas that subserve coordinated lid closure to the decussating ventral tegmental tract.
- Complications of ear mold impressions: two case reportsPublication . Silva, C; Amorim, AM; Gapo, C; Paiva, A
- Accelerated age-related olfactory decline among type 1 Usher patientsPublication . Ribeiro, JC; Oliveiros, B; Pereira, P; António, N; Hummel, T; Paiva, A; Silva, EUsher Syndrome (USH) is a rare disease with hearing loss, retinitis pigmentosa and, sometimes, vestibular dysfunction. A phenotype heterogeneity is reported. Recent evidence indicates that USH is likely to belong to an emerging class of sensory ciliopathies. Olfaction has recently been implicated in ciliopathies, but the scarce literature about olfaction in USH show conflicting results. We aim to evaluate olfactory impairment as a possible clinical manifestation of USH. Prospective clinical study that included 65 patients with USH and 65 normal age-gender-smoking-habits pair matched subjects. A cross culturally validated version of the Sniffin' Sticks olfaction test was used. Young patients with USH have significantly better olfactory scores than healthy controls. We observe that USH type 1 have a faster ageing olfactory decrease than what happens in healthy subjects, leading to significantly lower olfactory scores in older USH1 patients. Moreover, USH type 1 patients showed significantly higher olfactory scores than USH type 2, what can help distinguishing them. Olfaction represents an attractive tool for USH type classification and pre diagnostic screening due to the low cost and non-invasive nature of the testing. Olfactory dysfunction should be considered among the spectrum of clinical manifestations of Usher syndrome.
- A pulsatile pharyngeal wall: case report and clinical relevancePublication . Simões, J; Miguéis, J; Miguéis, A