1/127. Successful cochlear implantation in a patient with melas syndrome.OBJECTIVE: To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration. STUDY DESIGN: A patient with a degenerative CNS disease (melas syndrome) undergoing evaluation for cochlear implantation is described. SETTING: This study took place at a tertiary care center. PATIENT: A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had cortical blindness and profound sensorineural hearing loss was evaluated and rehabilitated with cochlear implantation. INTERVENTIONS: Pure-tone audiogram, behavioral responses to promontory stimulation electrical auditory brainstem response, and electrically evoked middle-latency responses (MLRs) were used to assess eighth nerve, auditory brainstem, and cortical auditory pathways. cochlear implantation with Cochlear Corporation mini 22 implant was performed. RESULTS: Repeatable electrically evoked MLRs and behavioral responses to promontory stimulation documented the presence of auditory cortical responses. Successful implantation resulted in open set speech recognition and communication using the auditory/oral mode. CONCLUSION: This report describes successful implantation in a patient with melas syndrome and demonstrates the ability to preoperatively confirm the integrity of brainstem and cortical auditory pathways despite significant CNS degeneration.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
2/127. Case report and discussion of hearing preservation after translabyrinthine excision of small acoustic tumors.OBJECTIVE: Since 1991, three separate reports have shown how hearing may be salvaged after translabyrinthine excision of small acoustic tumors. The authors submit yet another report of a complete translabyrinthine excision of a 1.4-cm intracanalicular acoustic tumor with modest hearing preservation. An attempt is made to retrace the steps of the operation and recognize and discuss what particular events may have safeguarded the viability of the cochlea. With the availability of cochlear implantation, there should be added incentive to preserve the cochlear neurones if hair cells cannot be saved. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at a primary care hospital. INTERVENTION: Therapeutic and rehabilitative measures were performed. MAIN OUTCOME MEASURES: Hearing preservation was measured. CASE REPORT: A 55-year-old woman presented with a left-sided hearing loss and a 1.4-cm left acoustic tumor completely filling the internal auditory canal (speech reception threshold [SRT] 30 dB, discrimination [Pb] 28%). A successful translabyrinthine excision of the tumor was performed in November 1995. A 1-year postoperative audiogram showed a mixed hearing loss in the left ear with SRT 85 dB and Pb 0%. Average pure-tone threshold for 500 Hz, 1 kHz, and 3 kHz was 50 dB and aided SRT 40 dB with Pb 64%. Postoperative magnetic resonance imaging confirmed complete excision of the tumor. CONCLUSION: An exceptional case of hearing preservation after translabyrinthine excision of a small acoustic tumor illustrates how it may be possible to preserve cochlear hair cells and neurones simultaneously in certain selected cases. A review of the surgical events shows the value of sealing the cochlear duct with bone wax, selectively removing the vestibular nerves with the tumor by sharp dissection, and safeguarding the meatal segment of the anterior inferior cerebellar artery by a limited dural incision.- - - - - - - - - - ranking = 3.8129169323637keywords = discrimination, speech (Clic here for more details about this article) |
3/127. Effects of acknowledging a hearing loss on social interactions.An important part of the counseling process is to offer guidance to people with hearing impairments on accepting their disability and adjusting to the negative attitudes held by some communications partners. The purpose of this study was to determine subjects' (1) preferences for interacting with individuals with a hearing impairment who acknowledged their hearing loss, and (2) perceptions of personality, employability, intelligence, and adjustment related to acknowledgment of hearing loss. Two male students with hearing impairments were videotaped under two experimental conditions. During one condition students acknowledged their hearing losses, spoke about adjustment, concerns, etc., while during the other condition they did not acknowledge their hearing loss. Results indicated that subjects preferred speakers when they discussed their hearing loss. Speakers who acknowledged their hearing loss also received more favorable ratings on personality, employability, and adjustment. Clinical implications for aural rehabilitation programs are discussed.- - - - - - - - - - ranking = 0.19979045016115keywords = perception (Clic here for more details about this article) |
4/127. Ototoxicity associated with vinblastine.OBJECTIVE: To describe a patient with ototoxicity associated with vinblastine chemotherapy. CASE SUMMARY: A 29-year-old white man with recurrent Hodgkin's disease received doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy once every two weeks for 12 cycles. He reported tinnitus after each treatment, with an onset of about six hours and a duration of seven to 10 days. This interfered with reading, watching television, and general concentration. Symptoms returned to baseline prior to the beginning of each subsequent cycle. Audiograms performed before and after several cycles showed mild sensorineural hearing loss in the high-decibel range, but no loss of speech recognition. DISCUSSION: No reported cases of ototoxicity or tinnitus from ABVD were found. All concomitant medications were eliminated as possible causes either due to lack of temporal association with the symptoms or no reports of ototoxicity in the literature. vincristine, a more commonly used vinca alkaloid very similar to vinblastine, was noted to have caused several cases of sensorineural hearing loss. CONCLUSIONS: This case suggests that vinblastine may cause ototoxicity.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
5/127. Neuroaudiological effects in a case of fatal dimethylmercury poisoning.OBJECTIVE: The audiological examination of this patient was to determine the degree and type of hearing loss that was incurred from apparent dimethylmercury poisoning. DESIGN: This was a single subject case study design. Audiologic tests were selected to help determine sensory from neural and/or central auditory system dysfunction. RESULTS: This patient demonstrated an inability to understand speech, both in formal and informal assessments, yet relatively good hearing sensitivity for pure tones bilaterally. Distortion product otoacoustic emissions showed only minimal deficits in each ear. The auditory brain stem response was abnormal bilaterally, indicating neural and/or central involvement. CONCLUSION: Dimethylmercury poisoning, in this case, resulted in compromise of the auditory neural system with little effect on the sensory (cochlea) mechanism.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
6/127. Cutaneous-evoked tinnitus. I. Phenomenology, psychophysics and functional imaging.DC00166e and acute unilateral deafferentation of the auditory periphery (auditory and vestibular afferents) can induce changes in the central nervous system that may result in unique forms of tinnitus. These tinnitus perceptions can be controlled (turned on and off) or modulated (changed in pitch or loudness) by performing certain overt behaviors in other sensory/motor systems. Clinical reports from our laboratory and several other independent sources indicate that static change in eye gaze, from a neutral head-referenced position, is one such behavior that can evoke, modulate and/or suppress these phantom auditory events. This report deals with a new clinical entity and a form of tinnitus that can be evoked directly by cutaneous stimulation of the upper hand and fingertip regions. In 2 adults, cutaneous-evoked tinnitus was reported following neurosurgery for space-occupying lesions at the base of the skull and posterior craniofossa, where hearing and vestibular functions were lost completely and acutely in one ear (unilateral deafferentation) and facial nerve paralysis (unilateral deefferentation) was present either immediately following neurosurgery or had occurred as a delayed-onset event. Herein, we focus on the phenomenology of this discovery, provide perceptual correlates using contemporary psychophysical methods and document in one individual cutaneous-evoked tinnitus-related neural activity using functional magnetic resonance imaging. In a companion paper, neuroanatomical and physiological interactions between auditory and somatosensory systems, possible mechanistic accounts and relevant functional neuroimaging studies are reviewed.- - - - - - - - - - ranking = 0.19979045016115keywords = perception (Clic here for more details about this article) |
7/127. Sensorineural hearing loss associated with Byler disease.Progressive familial intrahepatic cholestasis, sometimes described as Byler disease, is a lethal liver disease and its inheritance is autosomal recessive. There is a previous report on the occasional association between this disease and sensorineural hearing loss without any audiological findings. We report here two siblings, an 18-year-old female and a 16-year-old male, suffering from Byler disease and hearing loss. Pure tone, Bekesy and speech audiometries and auditory brain stem response examination were performed. Audiometric data showed hearing characteristics of cochlear origin, high-frequency loss and progressiveness. This sensorineural hearing loss possibly results from a genetic mutation. The mechanism of cochlear disorder in patients with Byler disease is unknown, however, a novel gene responsible for deafness might be found to be related to Byler disease.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
8/127. Altered phonatory physiology with short-term deactivation of children's cochlear implants.OBJECTIVES: The purposes of this investigation were 1) to determine whether short-term auditory deprivation results in systematic phonatory changes for prelingually deafened children who use cochlear implants (CIs) and 2) to determine whether such changes are similar to those that have been reported for postlingually deafened adults. DESIGN: Participants were two 6-yr-old children with CIs. Both children had been prelingually deafened, had good to excellent speech production and speech perception skills, and had been using their CIs for 2.5 yr. A single-subject design was used. Intraoral air pressure (Po), phonatory air flow (Vl), electroglottograph (EGG) cycle width, fundamental frequency (F0), and intensity were measured during syllable production over two baseline days and three experimental days. Data were collected twice on each baseline day while the children wore their CIs, with a 1 hr break between data collection sessions. On experimental days, data were collected while the children wore their CIs (ON condition) and after their CIs had been removed for 1 hr (OFF condition). RESULTS: Both children demonstrated highly variable phonatory behaviors in baseline. The child with the more proficient speech production and perception skills showed consistent and significant reductions in Po, F0, and intensity in the OFF condition. These findings were dissimilar to those that occurred with repeated testing in the baseline condition and so were attributed to the sudden loss of auditory feedback. The other child showed a consistent and significant increase in mean Vl in the OFF condition. However, this child exhibited a similar finding with repeated testing in the baseline condition. Therefore, increased Vl in the OFF condition may have represented a practice effect. She also showed a small and consistent decrease in F0 in the OFF condition when F0 was derived from acoustic data, but this effect was not reliable in another data set when F0 was derived from the EGG signal. Our results with prelingually deafened children were inconsistent with reports of increased intensity and F0 in the absence of auditory feedback for postlingually deafened adults with CIs. CONCLUSIONS: Some prelingually deafened children who are successful CI users appear to use auditory feedback to self-monitor phonation. We suggest that the participant in our investigation who showed systematic phonatory changes in response to diminished auditory feedback was using auditory feedback primarily to stabilize her phonatory behaviors. She may not have had adequate experience with auditory feedback or adequate flexibility in her use of feedback mechanisms to implement the phonatory compensations that late-deafened adults use when auditory feedback suddenly is diminished. We further suggest that the phonatory changes that she exhibited during short-term auditory deprivation reflected disruption of her typical speaking strategies or apprehension about speaking when her ability to self-monitor auditorily was compromised.- - - - - - - - - - ranking = 222.67853632523keywords = speech perception, speech, perception (Clic here for more details about this article) |
9/127. cochlear implantation of auditory neuropathy.Auditory neuropathy (AN) is a hearing disorder that presents with a grossly abnormal or absent neural response as measured by evoked potentials in the presence of normal outer hair cell function evidenced by present otoacoustic emissions or cochlear microphonics. rehabilitation for patients with AN is challenging due to abnormal temporal encoding at the auditory nerve leading to severely impaired speech perception. Although patients with AN may demonstrate improvement in thresholds with amplification, temporal encoding dysfunction, and consequently speech perception degradation, is not alleviated by amplification. Another issue is the heterogeneity of the AN population in terms of audiologic and neurologic findings, in addition to uncertain etiology and pathophysiology. For children with prelingual onset of AN, development of auditory and oral communication skills is particularly compromised. All children with hearing loss in the severe-to-profound range who do not benefit from conventional amplification can be considered candidates for a cochlear implant (CI). This paper presents a case study of a child with AN who received a CI. Whereas no synchronous neural response auditory brainstem response could be elicited to acoustic stimuli, an electrically evoked auditory nerve action potential was evident following implantation, suggesting restoration to some degree of neural synchrony. Significant improvement in speech perception was found post-CI. Recommendation to implant all patients with AN would be premature, but these findings suggest that electrical stimulation in some cases of auditory neuropathy can be a viable option.- - - - - - - - - - ranking = 661.43623762522keywords = speech perception, speech, perception (Clic here for more details about this article) |
10/127. Auditory neuropathy: case study with hyperbilirubinemia.Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5-year period that includes audiologic data and communication development results.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
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