Audiological Findings in Children with PLA2G6-Associated Neurodegeneration.

BACKGROUND: Audiological manifestations of patients with PLA2G6-associated neurodegeneration are limited. OBJECTIVE: To analyze the audiological findings in a cohort of 13 children with infantile neuroaxonal dystrophy (INAD). METHOD: Patients underwent a battery of audiological tests including tympanometry, distortion product otoacoustic emissions, impedance audiometry, and Brainstem Auditory Evoked Potentials (BAEPs). RESULTS: Audiological studies revealed abnormal findings in 10 out of 13 children (77%). The findings indicated sensorineural hearing loss in six and auditory neuropathy spectrum disorder in four children. CONCLUSION: This study may extend the auditory findings for INAD. Additional studies on quality of life and cognitive-brain degeneration related to this disease are required before making appropriate recommendations for aural rehabilitation.

The Triad of Cognition, Language, and Communication in Traumatic Brain Injury: A Correlational Study.

Objectives Traumatic brain injury (TBI) is associated with deficits in cognition, language, and communication along with physical and psychosocial consequences. This study aimed at evaluating the association between cognition, language, and communication in a cohort of patients with TBI in southern India using standardized language specific assessment tools. Materials and Methods The present correlational study included 30 patients with TBI of any severity, that is, mild, moderate, and severe within the postinjury period of 6 to 36 months. Comprehensive evaluation of cognition, language, and communication was done using National Institute of Mental Health and Neurosciences Neuropsychology Battery, Indian adapted versions of Western Aphasia Battery and La Trobe Communication Questionnaire (LCQ) respectively. Data Analysis Measures of descriptive statistics, that is, mean, median, percentages, and frequencies, were used. Raw scores, that is, aphasia quotient (AQ), and subdomain scores for LCQ, that is, LCQ-quality, quantity, relation, manner, and cognition, were calculated. Composite scores for the three neuropsychological domains, that is, attention and processing speed, executive function, learning and memory, were evaluated. Partial correlation was computed for identifying the association between the three variables by controlling for age, education, duration, and severity of injury. Results A significant correlation was found between AQ and measures of attention and executive functioning and all measures of LCQ except LCQ-manner. Further, a significant correlation was found between executive functioning and LCQ-total, LCQ-quantity, and LCQ cognition. No correlation was found between measures of attention and memory with communication. Conclusion A good language and executive functioning are associated with better communication abilities in patients with TBI thus emphasizing on the importance of a multidisciplinary team for the management of cognitive-communication impairments following a TBI.

Speech Characteristics of Patients with Parkinson's Disease-Does Dopaminergic Medications Have a Role?

Objective The present study aimed to investigate the effects of dopaminergic medication on voice, speech motor functions, and motor impairment in patients with Parkinson's disease (PD). Materials and Methods Twenty-five individuals (16 males and 9 females) with PD underwent comprehensive assessment of voice, speech, and motor functions in levodopa medication ON and medication OFF conditions. Age- and gender-matched healthy controls were recruited to compare speech and acoustic parameters. Multi-Dimensional Voice Program (MDVP) from Computerized Speech Laboratory (Model: 4500) was utilized for acoustic analysis of voice and the Voice Handicap Index (VHI) for the self-assessment of vocal function. Frenchay Dysarthria Assessment (FDA-2) and Unified Parkinson's Disease Rating Scale-III (UPDRS III) were used to evaluate speech motor and motor functions, respectively. Statistical Analysis The mean and standard deviation were used as descriptive statistics measures. Raw scores were obtained for FDA-2, DRS, VHI, MDVP parameters, and UPDRS-III in either medication condition. The Wilcoxon signed-rank test was performed to determine the statistical significance of the above measures in both genders across the medication conditions. Spearman's rank correlation coefficient was used to determine the relationship between motor speech function and motor impairment and between VHI and MDVP parameters across both medication conditions. The interrater reliability rating was established using Cohen's kappa. Results An improvement in lip and laryngeal functioning was found in the medication ON over medication OFF state in both males and females with PD. A few frequency and amplitude-related measures improved in the medication-ON state over the medication-OFF state. UPDRS-III scores reduced from the OFF state to the ON state, and no change in dysarthria severity or VHI was found in either gender or medication condition. No correlation was found between speech motor function and motor function or between VHI and acoustic parameters of voice in either medication condition. Conclusions Improvement in motor symptoms with levodopa was predominantly observed when compared with the minor improvements in a few aspects of speech motor function and vocal parameters. The results of this study suggest the need for speech therapy as a nonpharmacological treatment method for speech impairments in PD.

Erratum: Speech Characteristics of Patients with Parkinson's Disease-Does Dopaminergic Medications Have a Role?

[This corrects the article DOI: 10.1055/s-0041-1735249.].