Tony Sirimanna

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Exogenous enzyme replacement therapy achieves satisfactory biomedical correction in Gaucher type 1 disease and may halt or reverse neurological progression in type 3, while it does not appear to influence the outcome in type 2. In view of the therapeutic possibilities, early detection and monitoring of type 3 Gaucher disease, as well as evaluation of the(More)
A short-term longitudinal study was conducted to investigate possible benefits of cochlear implant (CI) use on the development of phonological awareness in deaf children. Nineteen CI users were tested on 2 occasions. Two groups of deaf children using hearing aids were tested once: 11 profoundly deaf and 10 severely deaf children. A battery of tests was(More)
OBJECTIVE To identify the clinical and radiologic characteristics of aplasia and hypoplasia of the eighth nerve. STUDY DESIGN Retrospective case-note review. SETTING Cochlear implant program. PATIENTS All children at the authors' institution in whom the cochlear implant assessment failed because of absence or hypoplasia of the eighth nerve. (More)
Children with listening difficulties, but normal audiometry, may be diagnosed with APD. The diagnosis is typically based on poor performance on tests of perception of both non-speech and speech stimuli. However, non-speech test results correlate only weakly with evaluations of speech-in-noise processing, cognitive skills, and caregiver evaluations of(More)
The aim of this pilot study was to investigate whether children with a suspected auditory processing disorder (sAPD) in the presence of normal hearing, differ significantly from normal age-matched controls on particular parameters of auditory event-related potentials. We assessed nine children (mean age 9.5 years) in whom the clinical profile and the(More)
OBJECTIVE Auditory processing disorder (APD) is diagnosed on the basis of listening difficulties despite normal audiogram, although the cause is unknown. This study examined the hypothesis that the underlying cause of APD is a modality-specific deficit in auditory temporal processing and also considered how far the auditory impairments in APD differ from(More)
Hearing thresholds in children with a cleft palate prior to cleft palate repair are not widely documented, and audiological criteria for short-term ventilation tube insertion do not exist. The aims of this prospective study are to estimate hearing thresholds in 40 children with a cleft palate by 3-month developmental age with auditory brainstem responses(More)
AIM Recently, we reported a previously unrecognized symptom constellation comprising epilepsy, ataxia, sensorineural deafness, and tubulopathy (EAST syndrome) associated with recessive mutations in the KCNJ10 gene. Here, we provide a detailed characterization of the clinical features of the syndrome to aid patient management with respect to diagnosis,(More)
AIM To examine the yield of computed tomography (CT) of the temporal bones when investigating sensorineural hearing loss (SNHL) and to identify factors associated with CT findings. METHODS Retrospective analysis of 116 consecutively investigated children with bilateral SNHL at the audiology department of Great Ormond Street Hospital, London. Main outcome(More)
The contribution of air conduction auditory brainstem response (AC-ABR) testing in the paediatric population is widely accepted in clinical audiology. However, this does not allow for differentiation between conductive and sensorineural hearing loss. The purpose of this paper is to review the role of bone conduction auditory brainstem responses (BC-ABR). It(More)