Positional Nystagmus of Central Origin

2006 ◽  
Vol 17 (02) ◽  
pp. 085-092 ◽  
Author(s):  
Frederick E. Cobb ◽  
Lauren B. Friedman

Audiometric, electrophysiologic, and radiographic findings for a 68-year-old male with an "imbalance" concern are presented. This paper has a two-fold purpose: (1) to present an unusual electronystagmography case study and (2) to highlight the importance of test conditions in lesion localization. The specific disease pathophysiology remains obscure. Repeated hearing tests documented a known hearing impairment with worsening word-recognition ability of the right ear. An initial electronystagmographic exam was normal except for a mild ageotropic direction-changing positional nystagmus with eyes open and fixed. No repeatable click-evoked auditory brainstem response waveforms could be collected. A magnetic resonance imaging of the brain documents diffuse ischemic white matter disease. A repeated vestibular examination some months later supports the initial findings. The case illustrates the importance of following diagnostic protocol, of repeated measures, and of using both a visual fixation and a nonfixation condition for select electronystagmographic subtests.

2015 ◽  
Vol 26 (03) ◽  
pp. 311-324 ◽  
Author(s):  
Kamakshi V. Gopal ◽  
Binu P. Thomas ◽  
Deng Mao ◽  
Hanzhang Lu

Background: Tinnitus, or ringing in the ears, is an extremely common ear disorder. However, it is a phenomenon that is very poorly understood and has limited treatment options. Purpose: The goals of this case study were to identify if the antioxidant acetyl-L-carnitine (ALCAR) provides relief from tinnitus, and to identify if subjective satisfaction after carnitine treatment is accompanied by changes in audiological and imaging measures. Research Design: Case Study. Patient Case: A 41-yr-old female with a history of hearing loss and tinnitus was interested in exploring the benefits of antioxidant therapy in reducing her tinnitus. The patient was evaluated using a standard audiological/tinnitus test battery and magnetic resonance imaging (MRI) recordings before carnitine treatment. After her physician's approval, the patient took 500 mg of ALCAR twice a day for 30 consecutive days. The audiological and MRI measures were repeated after ALCAR treatment. Data Collection and Analysis: Pure-tone audiometry, tympanometry, distortion-product otoacoustic emissions, tinnitus questionnaires (Tinnitus Handicap Inventory and Tinnitus Reaction Questionnaire), auditory brainstem response, functional MRI (fMRI), functional connectivity MRI, and cerebral blood flow evaluations were conducted before intake of ALCAR and were repeated 30 days after ALCAR treatment. Results: The patient’s pretreatment pure-tone audiogram indicated a mild sensorineural hearing loss at 6 kHz in the right ear and 4 kHz in the left ear. Posttreatment evaluation indicated marginal improvement in the patient’s pure-tone thresholds, but was sufficient to be classified as being clinically normal in both ears. Distortion-product otoacoustic emissions results showed increased overall emissions after ALCAR treatment. Subjective report from the patient indicated that her tinnitus was less annoying and barely noticeable during the day after treatment, and the posttreatment tinnitus questionnaire scores supported her statement. Auditory brainstem response peak V amplitude growth between stimulus intensity levels of 40–80 dB nHL indicated a reduction in growth for the posttreatment condition compared with the pretreatment condition. This was attributed to a possible active gating mechanism involving the auditory brainstem after ALCAR treatment. Posttreatment fMRI recordings in response to acoustic stimuli indicated a statistically significant reduction in brain activity in several regions of the brain, including the auditory cortex. Cerebral blood flow showed increased flow in the auditory cortex after treatment. The functional connectivity MRI indicated increased connectivity between the right and left auditory cortex, but a decrease in connectivity between the auditory cortex and some regions of the “default mode network,” namely the medial prefrontal cortex and posterior cingulate cortex. Conclusions: The changes observed in the objective and subjective test measures after ALCAR treatment, along with the patient’s personal observations, indicate that carnitine intake may be a valuable pharmacological option in the treatment of tinnitus.


2019 ◽  
Vol 28 (1) ◽  
pp. 114-124
Author(s):  
Linda W. Norrix ◽  
Julie Thein ◽  
David Velenovsky

Purpose Low residual noise (RN) levels are critically important when obtaining electrophysiological recordings of threshold auditory brainstem responses. In this study, we examine the effectiveness and efficiency of Kalman-weighted averaging (KWA) implemented on the Vivosonic Integrity System and artifact rejection (AR) implemented on the Intelligent Hearing Systems SmartEP system for obtaining low RN levels. Method Sixteen adults participated. Electrophysiological measures were obtained using simultaneous recordings by the Vivosonic and Intelligent Hearing Systems for subjects in 2 relaxed conditions and 4 active motor conditions. Three averaging times were used for the relaxed states (1, 1.5, and 3 min) and for the active states (1.5, 3, and 6 min). Repeated-measures analyses of variance were used to examine RN levels as a function of noise reduction strategy (i.e., KWA, AR) and averaging time. Results Lower RN levels were obtained using KWA than AR in both the relaxed and active motor states. Thus, KWA was more effective than was AR under the conditions examined in this study. Using KWA, approximately 3 min of averaging was needed in the relaxed condition to obtain an average RN level of 0.025 μV. In contrast, in the active motor conditions, approximately 6 min of averaging was required using KWA. Mean RN levels of 0.025 μV were not attained using AR. Conclusions When patients are not physiologically quiet, low RN levels are more likely to be obtained and more efficiently obtained using KWA than AR. However, even when using KWA, in active motor states, 6 min of averaging or more may be required to obtain threshold responses. Averaging time needed and whether a low RN level can be attained will depend on the level of motor activity exhibited by the patient.


1994 ◽  
Vol 159 (11) ◽  
pp. 676-680 ◽  
Author(s):  
Linda L. Pierson ◽  
Kenneth J. Gerhardt ◽  
Robert M. Abrams ◽  
Scott K. Griffiths ◽  
Aemil J.M. Peters

2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Diala Hussein ◽  
Büşra Altın ◽  
Münir Demir Bajin

Abstract Background H syndrome is an autosomal recessive disorder caused by mutations in SLC29A3. Hyperpigmentation, hypertrichosis, hyperglycemia, and hearing loss are some characteristics of this disorder, and it has a prevalence of < 1/1000. The aim of this report is to spread awareness among otologists, audiologists, and pediatricians about this syndrome and its audiological features. Case presentation An 8-year-old male with a diagnosed H syndrome registered to our clinic with a complaint of hearing loss. The patient was diagnosed with hearing loss in a different clinic using only the air-conducted click auditory brainstem response test which showed wave V at 60 dB nHL for the right ear and at 80 dB nHL for the left ear. The initially performed pure tone audiometry (PTA) test in our clinic revealed a bilateral asymmetric hearing loss with a moderate sensorineural hearing loss in the right ear and a profound mixed hearing loss in the left ear. The performed air conducted click auditory brainstem response (ABR) result showed wave V at 55 dB nHL for the right ear and at 70 dB nHL for the left ear. Then, the repeated PTA test revealed a mild-severe sensorineural sloping hearing loss in the right ear and a severe sensorineural hearing loss in the left ear. Conclusion Although hearing thresholds in H syndrome could be within normal limits in some patients, sensorineural hearing loss is an important characteristic feature for this syndrome. Sensorineural hearing loss could be progressive or of sudden onset and ranges from mild to profound. Thus, it must be taken into consideration to apply the audiological follow-up regularly and paying attention to the patient’s complaints; also, a regular follow-up for language development of children with H syndrome and for the hearing aids is advised.


2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Theodoros Varakliotis ◽  
Federico Maspes ◽  
Vittoria Di Rubbo ◽  
Sara Cisternino ◽  
Maria Lauriello ◽  
...  

We report a case of a 54-year old female patient, complaining for chronic dizziness, hearing loss, tension headaches without aura, postural instability and gait dysfunction. The patient referred having these symptoms from 1992, but the last few months she experienced a noticeable aggravation of the symptoms. A magnetic resonance imaging test revealed a triventricular hydrocephalus, not associated with signs of intracranial hypertension decompensation. The ENT-Audiology evaluation revealed a bilateral sensorineural hearing loss with a conductive component, video-nystagmography resulted in an areflexia of the right ear and a reduced vestibular activity for the left ear. Auditory brainstem response test was also carried out and showed pathologic findings for the latencies of the waves I-III, III-V and I-V bilaterally but more significant in the right ear. On January 2016 the patient had endoscopic third ventriculostomy. On the follow up the patient referred an important subjective improvement regarding instability and gait dysfunction. In this paper we study the correlation between hydrocephalus, hearing loss and vestibular dysfunction.


2018 ◽  
Vol 29 (08) ◽  
pp. 685-695
Author(s):  
Ishara Ramkissoon ◽  
Mihika Batavia

AbstractPopulation health is impacted by environmental secondhand smoke (SHS) exposure. Although the negative health effects of SHS exposure include respiratory problems in children (nonsmokers) as seen in cigarette smokers, other health impacts such as sensory function are not assumed to be the same for both passive nonsmokers and smokers. However, hearing loss was recently reported in adolescents and aging adults with SHS exposure, suggesting that SHS might impact auditory function similarly to cigarette smoking. Specific effects of SHS exposure on the central auditory system have not been fully described.To measure auditory function via pure-tone audiometry and evoked potentials in young nonsmoking adults aged 18–23 yr who reported exposure to environmental SHS.Participants were selected for the SHS-exposed (SHS-E) group first, followed by age and gender matched individuals for the SHS-unexposed (SHS-U) group. Self-reported nonsmoker status was confirmed by biochemical analysis of urine for cotinine level.Potential participants (N = 208) completed a questionnaire about health, smoking history, SHS exposure, and hearing ability. Individuals with any neurological conditions, alcohol/drug dependencies, excessive noise exposure, using certain medications, or current smokers were excluded. Twenty-two nonsmokers in excellent health consented to participate. Participants in the SHS-E group reported SHS exposure in home, work, or social settings for an extensive time period. Participants in the SHS-U group did not live with smokers and reported no SHS exposure, medication use, tinnitus, or any chemical exposures. Statistical analysis was conducted on data from 20 participants, 10 per group with a mean age of 20 yr.Participants underwent auditory procedures in one session in an IAC sound-treated room, including otoscopy, tympanometry, pure-tone threshold evaluation, auditory brainstem response per ear, and a three-channel auditory middle latency response in the right ear. The primary study outcomes were hearing thresholds measured (dB HL) at five frequencies, and evoked potential wave latencies (I, III, V, Na, Pa, Nb, Pb) and amplitudes (V–I, Na–Pa, Pa–Nb, Nb–Pb). It was hypothesized that SHS-exposed individuals would have poorer hearing sensitivity (threshold >25 dB HL) and abnormal central auditory function (longer latencies; smaller amplitudes) based on evoked potentials. Statistical analyses focused on identification of group differences in hearing and central auditory function.All participants had normal hearing sensitivity (thresholds ≤25 dB HL) with no significant group differences. The V/I amplitude ratio in the right ear was significantly decreased in SHS-exposed individuals (p < 0.05). Auditory brainstem response latencies were not significantly different between participant groups or ears. Wave Pb latency was significantly increased in SHS-exposed individuals (p < 0.01). Auditory middle latency response relative amplitudes were significantly different from each other at every electrode site (Cz, Fz, C4) but not between groups. Overall, the Na–Pa complex was highest in amplitude at all three electrode sites.This preliminary study indicated toxic effects of SHS exposure by evoked potentials with decreased V/I amplitude ratio and longer (delayed) Pb latency in young adults. Further studies should corroborate these findings to facilitate clinical recommendations.


2007 ◽  
Vol 18 (10) ◽  
pp. 863-871 ◽  
Author(s):  
Nancy Vaughan ◽  
Kenneth James ◽  
Daniel McDermott ◽  
Susan Griest ◽  
Stephen Fausti

A number of studies have found statistically significant delays in auditory brainstem latencies of patients with diabetes compared with non-diabetic controls. However, the mechanisms ascribed as responsible for the latency differences between diabetic and non-diabetic patients vary among studies, and the latency differences, while significant, are small. In this 5-year prospective study, auditory brainstem response testing was conducted with 416 non-diabetic and 375 diabetic veterans from the Portland Veterans Affairs Medical Center as part of a larger study. Patients with diabetes had significantly delayed latencies of Wave III and V in the right ear and significantly prolonged interpeak I-III and I-V latencies in both ears. Stimulus polarity difference yielded slightly different results. None of the diabetes-related clinical characteristics were associated with the latency differences between the subject groups after accounting for hearing loss and age. Effect size was calculated and clinical significance of these differences is discussed. Un número de estudios han encontrado retardos estadísticamente significativos en las latencias auditivas del tallo cerebral en pacientes diabéticos comparados con controles no diabéticos. Sin embargo, los mecanismos atribuidos como responsables de las diferencias en las latencias entre pacientes diabéticos y no diabéticos varían entre estudios, y las diferencias en las latencias, aunque significativas, fueron pequeñas. En este estudio prospectivo a 5 años, se condujeron las pruebas de respuestas auditivas del tallo cerebral en 416 veteranos no diabéticos y 375 diabéticos, del Centro Médico de Asuntos de Veteranos de Portland, como parte de un estudio mayor. Los pacientes con diabetes tuvieron latencias significativamente retardadas para las ondas III y V en el oído derecho, y latencias inter-pico I-III y I-V significativamente retardadas en ambos oídos. Diferencias en la polaridad del estímulo rindieron resultados levemente diferentes. Ninguna de las características clínicas relacionadas con la diabetes se asoció con las diferencias en las latencias entre los grupos de sujetos, luego de considerar la pérdida auditiva y la edad. Se calculó el efecto del tamaño y se discutió el significado clínico de estas diferencias.


2020 ◽  
Vol 30 (2) ◽  
pp. 260-265
Author(s):  
Bruna Mauer Lopes ◽  
Claudine Devicari Bueno ◽  
Dayane Domeneghini Didoné ◽  
Pricila Sleifer

Objective: To compare the application time of the Automated Auditory Brainstem Response (A-ABR) between the click and CE-Chirp® stimuli. Methods: Forty-six newborns were evaluated without risk indicators for hearing loss and presenting transient evoked otoacoustic emissions (TEOAE). The A-ABR was performed with Interacoustics® Titan equipment in a hospital, with the click and CE-Chirp® stimuli at the same time. Descriptive statistical analyses and inferential statistics analyses (Student's t-test calculation for mean comparisons among independent samples) were used for the variables age, gender, examination time, laterality and test stimulus used. Results: Of the 46 neonates in the sample, 23 were male and 23 female. The mean age of the sample was 23.1 days. The mean procedure time using the Click stimulus was 85.9 seconds for the right ear and 86.1 seconds for the left ear, whereas for the use of the CE-Chirp® stimulus the results obtained for the right and left ear were28.4 seconds and 27.9 seconds, respectively. There was a statistically significant difference between the mean times obtained through the CE-Chirp® and Click stimuli for both ears (p=0.000). There was no statistically significant difference in the comparison between the right and left ears or between females and males. Conclusion: It was found that the mean duration of the A-ABR procedure using the CE- Chirp® stimulus is three times lower than with the Click stimulus.


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