Browsing by Author "Ribeiro, JC"
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- 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.
- 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.
- 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.