1,721,011 research outputs found
La sindrome di Crouzon: aspetti clinici oro-maxillo-facciali.Presentazione di un caso clinico.
No full textBenefit of cochlear implantation in a patient with Myhre syndrome
No full textMyhre syndrome is a rare disorder characterised by short stature, skeletal anomalies, facial dysmorphism and hearing loss (HL), resulting from heterozygous mutations of the SMAD4 gene. We describe the benefits of cochlear implant (CI) in a patient with sensorineural HL carrying a mutation (NM_005359.6: c.1498A>G; p.lle500Val) within the SMAD4 gene, detected by whole-exome sequencing. The CI was inserted through the round window despite otospongiotic abnormalities. Pure-tone audiometry improved up to 20 dBHL. Speech perception in noise (Simplified Noise Reduction - SNR +10) increased from 0% pre implantation with hearing aids to 50% post implantation. The postoperative setting of the electrical stimulation limits yielded an asymmetric map, with lower levels for central electrodes and higher levels for lateral ones. Action potential could not be evoked via medial electrodes, suggesting a cochlear nerve dysfunction. Outcomes related to quality of life and cognitive impairment improved. CI was shown to be an effective auditory rehabilitation strategy
Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation
No full textVery little information has been published on the clinical outcome of auditory brainstem implants (ABI). The present paper evaluates results obtained in a patient affected by a bilateral acoustic neuroma in type II neurofibromatosis who received an implant during removal of the residual tumor. One year later surgical revision of the ABI was necessary because no auditory sensation was obtained after ABI activation. Twelve months after the surgical revision, 12 electrodes out of 15 evoked auditory sensation. The results of rehabilitation were compared with those obtained in a group of eight postlingually deaf patients with cochlear implants (CI). Twelve months postoperatively the CI patients identified 97.7 +/- 5.1% of bisyllabic words in a closed set while the ABI patient identified 86%. CI patients recognized 87.1 +/- 11.3% of sentences and 81.3 +/- 14.8% of words with contextual cues while the ABI patient recognized 75% and 65% respectively. Speech recognition improved more slowly in the ABI patient than in the CI patients and his scores for open-set words and sentences without lip reading and contextual cues were poorer. Although the results obtained in the ABI patient were not as good as those obtained in the CI patients, the ABI patient said his quality of life was improved
An unusual complication of cochlear implant: benign paroxysmal positional vertigo
No full textThree days after the initial fitting of the cochlear device a 40-year-old woman complained of severe rotational vertigo following head movements associated with neurovegetative symptoms. Otoneurological evaluation revealed a horizontal paroxysmal positional nystagmus beating towards the lowermost ear, induced by rolling the patient's head from supine both to the right or to the left lateral position suggesting the diagnosis of benign paroxysmal positional vertigo of the left horizontal semicircular canal. The nystagmus characteristics were the same whether the cochlear device was switched on or off. The hypothesis of an otolith dislodging due to the electrical stimulation during the initial fitting is discussed
Musical training software for children with cochlear implants
No full textAlthough the voice in a free field has an excellent recruitment by a cochlear implant (CI), the situation is different for music because it is a much more complex process, where perceiving the pitch discrimination becomes important to appreciate it. The aim of this study is to determine the music perception abilities among children with Cis and to verify the benefit of a training period for specific musical frequency discrimination. Our main goals were to prepare a computer tool for pitch discrimination training and to assess musical improvements. Ten children, aged between 5 and 12 years, with optimal phoneme recognition in quiet and with no disabilities associated with deafness, were selected to join the training. Each patient received, before training period, two types of exams: a pitch discrimination test, consisting of discovering if two notes were different or not; and a music test consisting of two identification tasks (melodic and full version) of one music-item among 5 popular childhood songs. After assessment, a music training software was designed and utilised individually at home for a period of six months. The results following complete training showed significantly higher performance in the task of frequency discrimination. After a proper musical training identification, frequency discrimination performance was significantly higher (p < 0.001). The same considerations can be made in the identification of the songs presented in their melodic (p = 0.0151) and full songs version (p = 0.0071). Cases where children did not reach the most difficult level may be due to insufficient time devoted to training (ideal time estimated at 2-3 hours per week). In conclusion, this study shows that is possible to assess musical enhancement and to achieve improvements in frequency discrimination, following pitch discrimination training
Tonotopic organization of human auditory cortex analyzed by SPET
No full textSingle photon emission tomography (SPET) was used to map blood flow increases in the temporal and parietal cortex of 16 normally-hearing subjects after auditory stimulation. Eight subjects were stimulated with a multifrequency 40 dB HL pure tone at 250, 500, 1000, 2000, 4000 Hz, each frequency varying every 30 s. Single 500 Hz pure tones at 40 dB HL were delivered to the remainder of the subjects. Five bilaterally deaf subjects were used as controls. Marked cerebral flow increase following acoustic stimulation with a significantly prevalent activation of the contralateral temporal cortex was achieved (p < 0.001). According to the tonotopic organization of the human auditory cortex, low monofrequency stimulation activated the most lateral sagittal tomograms (from 48.75 to 56.25 mm laterally to the brain midline) only, while multifrequency stimuli activated all sagittal tomograms (from 18.75 to 56.25 mm).
On the basis of these results, it is likely that SPET is able to give real information on the cortical distribution of the auditory frequency range, taking into account the number and position of the activated slices. Further clinical investigations in order to define the relationships among blood flow cortical increases, stimulus intensity and auditory threshold, are in progress
Postural control in horizontal benign paroxismal positional vertigo
No full textSixteen patients affected by benign paroxysmal positional vertigo of the horizontal semicircular canal (BPPV-HSC) were investigated by means of dynamic posturography (DP) and during bithermal caloric stimula- tion. Data were compared to data from 40 patients with benign paroxysmal positional vertigo of the posterior semicircular canal (BPPV-PSC) and 20 healthy controls. No postural deficit was observed before or after a libera- tive Lempert’s manoeuvre when patients were compared to control subjects. BPPV-PSC postural scores were sig- nificantly impaired compared to scores from the BPPV- HSC group. A residual significant postural impairment was also observed after a successful liberative manoeuvre in the BPPV-PSC group. Electronystagmographic record- ings before recovery revealed significant hypoexcitability of the affected ear in 8/16 patients of the BPPV-HSC group. After the liberative manoeuvre, a symmetric bilat- eral response to caloric stimulation was recorded in all pa- tients. Three main conclusions can be drawn from the pre- sent data. First, disorders of the horizontal semicircular canal do not change postural control. Second, dynamic posturography can detect the postural imbalance due to posterior semicircular canal dysfunction even after resolu- tion of paroxysmal vertigo attacks. Third, utricular dys- function can be ruled out as a cause of the residual pos- tural deficit observed in BPPV-PSC patients. Therefore the recovery delay observed even 1 month after the liber- ative manoeuvre in the BPPV-PSC-group might be due to the persistence of small amounts of residual debris in the canal, to paralysis of ampullar receptors, or to the time needed for central vestibular re-adaptatio
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