11/127. Superficial siderosis of the central nervous system. A case report on examination by ECoG and DPOAE.This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.- - - - - - - - - - ranking = 1keywords = speech discrimination, discrimination, speech (Clic here for more details about this article) |
12/127. Word deafness after resection of a pineal body tumor in the presence of normal wave latencies of the auditory brain stem response.We studied the case of a 48-year-old woman who had resection of a pineal body tumor in terms of postoperative audiological function. Postoperative magnetic resonance imaging disclosed partial inferior colliculi destruction and medial geniculate body degeneration. A pure tone audiogram revealed only moderate sensorineural hearing loss, but her speech perception was totally impaired. The binaural sound localization function was also impaired. The auditory brain stem response (ABR) showed waves I, III, and V to have normal latencies. The amplitude of wave III was larger than that of wave V. These results support the view that the waves of the ABR are elicited from multiple sources in the auditory brain stem nuclei and tracts. This case suggests a substantial role for the inferior colliculus and medial geniculate body in the processing of speech perception and sound localization.- - - - - - - - - - ranking = 4.6468834483205keywords = speech perception, speech, perception (Clic here for more details about this article) |
13/127. Sudden cortical hearing loss for speech: a case report.A 35-yr-old male patient experienced a sudden loss of speech understanding due to a bilateral cerebrovascular disease. A detailed summary of audiological and neurological findings was presented. Findings indicated that the presence of Pa waves of the Middle Latency Response (MLR) may be a positive prognosis for improvement in hearing thresholds and speech understanding.- - - - - - - - - - ranking = 0.063228998739228keywords = speech (Clic here for more details about this article) |
14/127. Multichannel cochlear implantation in visually impaired patients.OBJECTIVE: To evaluate the outcome of cochlear implantation in patients with severe to profound hearing loss and visual impairment. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center with a large cochlear implant program. patients: Six adults and two children with severe or profound hearing loss and significant visual impairment underwent multichannel cochlear implantation. Follow-up period ranged from 6 months to 9 years. Case history, etiology of visual and hearing loss, and benefit from cochlear implant were evaluated. INTERVENTIONS: cochlear implantation and subsequent rehabilitation. MAIN OUTCOME MEASURES: speech perception measures were selected based on the patient age and cognitive abilities. Identical measures were used in each patient before and after implantation. RESULTS: As a group, patients did well after cochlear implantation. There was significant improvement in speech perception when compared with the score before implantation. CONCLUSIONS: cochlear implants can play a significant rehabilitative role in patients with severe visual and auditory impairment. Additional skills are required by the implant team for rehabilitation of patients with multiple sensory deficits.- - - - - - - - - - ranking = 2.3255471491805keywords = speech perception, speech, perception (Clic here for more details about this article) |
15/127. Fitting low ratio compression to people with severe and profound hearing losses.OBJECTIVE: In this study, we aimed to determine the extent to which wide dynamic range compression and compression limiting could benefit severely and profoundly hearing-impaired adults. DESIGN: Subjects were fitted with multi-memory hearing aids incorporating frequency independent input-controlled compression with a 2:1 compression ratio and output-controlled compression limiting. The input compression threshold (CT) was varied to establish the lowest level that maintained audibility for conversational intensity speech without acoustic feedback oscillation. Where a low (40 to 57 dB SPL) CT was possible, this was compared with a moderate (65 to 74 dB SPL) level. The preferred input compression setting was subsequently compared with linear, compression-limited amplification in the same aids. In the three cases where 2:1 input compression could not be used, because of feedback or loudness insufficiency problems, compression limiting was compared with peak clipping. Field trials were conducted over a 2- or 3-mo period to establish preferences. RESULTS: Nine of the 16 subjects preferred the inclusion of the higher level input compression, and one preferred lower level input compression. Four subjects preferred linear, compression-limited amplification, one favored peak clipping, and one could not be satisfied with any of the options provided. CONCLUSIONS: 2:1 input compression was useful, and preferred by 10 of the subjects, but for nine subjects the preferred CT was relatively high. With a group 4-frequency average loss of 87 dB HTL, the results demonstrate that fast-acting, low compression ratio systems can be useful for losses traditionally regarded as the domain of linear amplification.- - - - - - - - - - ranking = 0.010538166456538keywords = speech (Clic here for more details about this article) |
16/127. A contiguous deletion syndrome of X-linked agammaglobulinemia and sensorineural deafness.hearing loss in patients with X-linked agammaglobulinemia is often attributed to recurrent infections. However, recent genetic studies suggest a different etiology in some patients. We present three unrelated patients, 6, 9, and 14 years of age, with large deletions of the terminal portion of the Bruton tyrosine kinase (Btk) gene extending 4.2-19 kb beyond the 3' end of the gene. The dna immediately downstream of the 3' end of Btk contains the deafness-dystonia protein gene (DDP). Mutations in this gene have recently been shown to underlie the Mohr-Tranebjaerg syndrome, which is characterized by sensorineural deafness, dystonia, and mental deficiency. Besides the immunodeficiency, our patients exhibited progressive sensorineural deafness. The clue to an associated hearing problem was delayed development of speech in one patient and post-lingual deafness noticed between the age of 3-4 years in the other two. These patients have not yet exhibited significant associated neurologic deficits.- - - - - - - - - - ranking = 0.010538166456538keywords = speech (Clic here for more details about this article) |
17/127. An isolated and sporadic auditory neuropathy (auditory nerve disease): report of five patients.Five patients of various ages with difficulty in speech discrimination were evaluated. All showed evidence of abnormal auditory brainstem responses (ABRs) beginning with the VIIIth cranial nerve. Broad summating potentials were evoked on their electrocochleograms (EcochGs) and they all exhibited almost normal cochlear outer hair cell function by otoacoustic emissions (OAEs) recordings. Behavioural audiometric testing revealed a mild to moderate elevation of pure-tone threshold in all patients. The shape of their pure-tone losses varied, being predominantly low-frequency in four patients (rising slope pattern) and flat across all frequencies in one patient. speech intelligibility scores of all patients were poor and out of proportion to what would have been expected if threshold elevation of pure-tone was of cochlear origin (i.e. markedly poor scores on the speech audiogram with good scores on the auditory comprehension test). patients were neurologically normal when the hearing impairment was first manifested. We suggest that this type of hearing impairment is due to an isolated and sporadic disorder of auditory nerve function. It occurs in isolation and does not seem to be part of a generalized neuropathological process.- - - - - - - - - - ranking = 1.0105381664565keywords = speech discrimination, discrimination, speech (Clic here for more details about this article) |
18/127. Surgical evaluation of candidates for cochlear implants.The customary presentation of surgical procedures to patients in the united states consists of discussions on alternative treatment methods, risks of the procedure(s) under consideration, and potential benefits for the patient. Because the contents of the normal speech signal have not been defined in a way that permits a surgeon systematically to provide alternative auditory signals to a deaf patient, the burden is placed on the surgeon to make an arbitrary selection of candidates and available devices for cochlear prosthetic implantation. In an attempt to obtain some information regarding the ability of a deaf patient to use electrical signals to detect and understand speech, the Good Samaritan Hospital and Neurological Sciences Institute cochlear implant team has routinely performed tympanotomies using local anesthesia and has positioned temporary electrodes onto the round windows of implant candidates. The purpose of this paper is to review our experience with this procedure and to provide some observations that may be useful in a comprehensive preoperative evaluation for totally deaf patients who are being considered for cochlear implantation.- - - - - - - - - - ranking = 0.021076332913076keywords = speech (Clic here for more details about this article) |
19/127. False-positive magnetic resonance image in the diagnosis of small acoustic neuroma.A patient presented with sudden hearing loss on her first visit to our department. gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) of the posterior cranial fossa portrayed an intracanalicular tumour image (2-3 mm), and the pure tone average (PTA) and speech discrimination score (SDS) values were 65 dB and 60 per cent, respectively. Surgical intervention to remove the suspected tumour was scheduled by the translabyrinthine approach. Intracanalicular observations by the retrolabyrinthine approach revealed limited oedema on the inferior vestibular nerve with vascular dilation. The tumour image disappeared two years after the operation. Surgical findings and the post-operative course advocate that gadolinium-DTPA-enriched MRI image of an intracanalicular lesion such as arachnoiditis might produce a false-positive result.- - - - - - - - - - ranking = 1keywords = speech discrimination, discrimination, speech (Clic here for more details about this article) |
20/127. sign language in childhood epileptic aphasia (landau-kleffner syndrome).Acquired epileptic aphasia (AEA, or landau-kleffner syndrome) is a unique condition in which children can lose oral language (OL) comprehension and expression for a prolonged period. These children can benefit from visual forms of language, mainly sign language (SL), but the quality of SL has never been analyzed. The case is reported here of a boy with AEA who lost speech comprehension and expression from 3 years 6 months to 7 years and was educated in SL from the age of 6 years. His SL was evaluated at the age of 13 years and 6 months and compared with a control child with congenital sensorineural deafness. It was found that: (1) our patient achieved the same proficiency in SL as the control child with deafness; (2) SL learning did not compete with, but perhaps even hastened, the recovery of OL. Intact ability to learn a new linguistic code such as SL suggests that higher-order language areas were preserved and received input from a separate visual route, as shown by neuropsychological and functional imaging research in deaf and hearing signers.- - - - - - - - - - ranking = 0.010538166456538keywords = speech (Clic here for more details about this article) |
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