Audio-vestibular Findings in a Patient with Pelizaeus- Merzbacher Disease.

Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive rare disease condition in which audiological deficit is also observed. A 4-year-old male child with PMD underwent an audiological evaluation. The results suggested normal middle ear and outer hair cells functioning, with only peak I of the auditory brainstem response present until 30 dBnHL. Further, the cervical vestibular evoked myogenic potential showed delayed latencies with normal amplitudes. In this case report, we attempt to explain the audio-vestibular test results and correlate them with the pathophysiology. This is the first report on the cervical vestibular myogenic potentials in patients with PMD.

Correction: Comprehensive Normative Data for Objective Vestibular Tests.

[This corrects the article DOI: 10.7759/cureus.40080.].

Does Binaural Stimulation Enhance Temporal Processing in Young Children?

Background and objective Temporal processing abilities help perceive signal changes over time. Efficient temporal processing is necessary for pitch perception, voice identification, and speech perception. It plays a significant role in language development. Internal redundancy of the central auditory nervous plays a role in processing sensory information. There is a need to gain more insights into the maturation of neural hardwiring that supports binaural temporal processing at a young age. The purpose of the present study was to evaluate the difference between monaural and binaural temporal processing in children aged 7-11 years. Methods Temporal processing was assessed using gap detection and temporal modulation transfer function (TMTF) tests. The tests were administered in 40 typically developing children with normal clinical auditory sensitivity. The maximum likelihood procedure (MLP), a MATLAB toolbox, was employed to deliver the stimulus. A multivariate analysis followed by post hoc analysis was performed to analyze the data. Results There was a significant difference between binaural and monoaural stimulation in children aged 7-11 years. However, there was no statistically significant difference between the right and left ears for gap detection threshold (GDT) and TMTF across all test frequencies. Conclusion Based on our findings, binaural stimulation enhances temporal processing in young children.

Comprehensive Normative Data for Objective Vestibular Tests.

INTRODUCTION:  Vestibular dysfunction is a debilitating disorder frequently encountered in neurological and otological settings. The vestibular system is a complex network between peripheral and central mechanisms. This innate complexity of the vestibular system necessitates objective test procedures for evidence-based diagnostic formulations and intervention. Objective tests aid in the evaluation of both peripheral and central vestibular pathologies. Establishing and availability of comprehensive normative data for these objective tests is crucial for clinicians and researchers alike. MATERIALS AND METHODS: This is a prospective study involving 120 participants (both males and females) aged between 18 and 55 years. All participants were right-handed individuals and had no significant medical history. On pre-set protocols, cVEMP (cervical vestibular evoked myogenic potential), oVEMP (ocular vestibular evoked myogenic potential), vHIT (video head impulse test), and VNG (videonystagmography) were done. RESULTS: While all participants (n=120) underwent cVEMP, oVEMP, vHIT, saccade, smooth pursuit, and optokinetic tests, only 109 participants consented to the caloric test. Each test's mean, standard deviation, median, quartile, and third quartiles have been recorded. A right-left comparison yielded no significant difference on cVEMP, oVEMP, caloric test, smooth pursuit, and optokinetic test. However, few vHIT and saccade parameters did reveal significant differences. DISCUSSION: This study presents comprehensive normative data for cVEMP, oVEMP, vHIT, caloric test on VNG, and oculomotor tests (smooth pursuit, saccade, optokinetic) on VNG. The test results were in concordance with previously published data. The significant difference between the right and left sides in vHIT may be because of the monocular goggles used for the testing. CONCLUSION: This study brings out the normative data for various vestibular tests on individuals aged between 18-55 years. This information could aid both clinicians and researchers working in the field of vestibular science.

Measure of Central Vestibular Compensation: A Review.

The central vestibular compensation reduces vestibular symptoms and helps individuals improve balance affected by vestibular dysfunction. The video head impulse test provides an opportunity to study central vestibular compensation objectively. This study aims to methodically present existing information about the video head impulse test as a measure to evaluate central vestibular compensation in patients with unilateral vestibular dysfunction. Literature review comprised 12 research articles selected based on pre-set criteria and timeline (January 2010 to June 2020). The findings indicate that the appropriate video head impulse test measures to evaluate central vestibular compensation after the occurrence of temporary unilateral vestibular dysfunction are the improvement in vestibulo-ocular reflex gain. And, for permanent unilateral vestibular dysfunction are reduction in catch-up saccades percentage, velocity, amplitude, latency, and Perez and Rey score.

A systematic review on the effect of low-dose radiation on hearing.

Numerous studies have documented the adverse effects of high-dose radiation on hearing in patients. On the other hand, radiographers are exposed to a low dose of ionizing radiation, and the effect of a low dose of radiation on hearing is quite abstruse. Therefore, the present systematic review aimed to elucidate the effect of low-dose ionizing radiation on hearing. Two authors independently carried out a comprehensive data search in three electronic databases, including PUBMED/MEDLINE, CINAHL, and SCOPUS. Eligible articles were independently assessed for quality by two authors. Cochrane Risk of Bias tool was used assess quality of the included studies. Two articles met the low-dose radiation exposure criteria given by Atomic Energy Regulatory Board (AERB) and National Council on Radiation Protection (NCRP) guidelines. Both studies observed the behavioral symptoms, pure-tone hearing sensitivity at the standard, extended high frequencies, and the middle ear functioning in low-dose radiation-exposed individuals and compared with age and gender-matched controls. One study assessed the cochlear function using transient-evoked otoacoustic emissions (TEOAE). Both studies reported that behavioral symptoms of auditory dysfunction and hearing thresholds at extended high frequencies were higher in radiation-exposed individuals than in the controls. The current systematic review concludes that the low-dose ionizing radiation may affect the hearing adversely. Nevertheless, further studies with robust research design are required to explicate the cause and effect relationship between the occupational low-dose ionizing radiation exposure and hearing.

Audiovestibular findings in a 6 year old child with pilocytic astrocytoma - a case report.

Objective: Pilocytic astrocytoma commonly occurs in children and depending on the extension of the lesion may cause varied audiovestibular dysfunctions. However, audiovestibular findings are scarcely reported in the literature.Design: Audiovestibular testing was performed on a single subject on two occasions pre-surgically.Study sample: A 6 year old girl with pilocytic astrocytoma.Results: All audiological tests revealed normal findings except for the cervical vestibular evoked myogenic potential testing (cVEMP). The amplitude of cVEMP was higher in the lesion side indicating a hypersensitive vestibulocollic reflex pathway functioning.Conclusions: This case study reported a unique finding of hypersensitive cVEMP findings in the lesion side in a patient with pilocytic astrocytoma. The pathophysiological basis for this hypersensitivity is attributed to anatomical connections between the cerebellum and the vestibular nuclei through the inferior cerebellar peduncle.

Audiological Profile of Adult Persons with Auditory Neuropathy Spectrum Disorders.

BACKGROUND AND OBJECTIVES: The auditory profile of a large number of persons with late onset auditory neuropathy spectrum disorder (ANSD) is recently described in the Indian context. The purpose of study was 1) to profile data on routine audiological parameters, cortical evoked potentials, and temporal processing, 2) to analyze the benefit from hearing aids for persons with ANSD, and 3) to understand the association between benefit from hearing aids and auditory profile. SUBJECTS AND METHODS: Thirty-eight adults with late onset ANSD and a matched group of 40 normally hearing adults participated in the study. Basic audiological tests, recording of cortical evoked potentials, and temporal processing tests were carried out on both groups of participant while only persons with ANSD were fitted with hearing aid. RESULTS: Subjects in the two groups were significantly different on all the audiological parameters. ANSD group seemed to benefit from hearing aids variably. The mean amplitude of N2 was significantly different between normally-hearing participants and patients with ANSD. CONCLUSIONS: Residual temporal processing, particularly amplitude modulation detection seems to be associated with benefit from hearing aids in patients with ANSD.

Cortical Evoked Potentials and Hearing Aids in Individuals with Auditory Dys-Synchrony.

OBJECTIVE: The purpose of the present study was to investigate the relationship between cortical processing of speech and benefit from hearing aids in individuals with auditory dys-synchrony. MATERIALS AND METHODS: Data were collected from 38 individuals with auditory dys-synchrony. Participants were selected based on hearing thresholds, middle ear reflexes, otoacoustic emissions, and auditory brain stem responses. Cortical-evoked potentials were recorded for click and speech. Participants with auditory dys-synchrony were fitted with bilateral multichannel wide dynamic range compression hearing aids. Aided and unaided speech identification scores for 40 words were obtained for each participant. RESULTS: Hierarchical cluster analysis using Ward's method clearly showed four subgroups of participants with auditory dys-synchrony based on the hearing aid benefit score (aided minus unaided speech identification score). The difference in the mean aided and unaided speech identification scores was significantly different in participants with auditory dys-synchrony. However, the mean unaided speech identification scores were not significantly different between the four subgroups. The N2 amplitude and P1 latency of the speech-evoked cortical potentials were significantly different between the four subgroups formed based on hearing aid benefit scores. CONCLUSION: The results indicated that subgroups of individuals with auditory dys-synchrony who benefit from hearing aids exist. Individuals who benefitted from hearing aids showed decreased N2 amplitudes compared with those who did not. N2 amplitude is associated with greater suppression of background noise while processing speech.