The importance of central auditory evaluation in Friedreich's ataxia

Abstract

Objective To assess central auditory function in Friedreich's ataxia. Methods A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments. Results The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed. Conclusion In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

Auditory and Olfactory Deficits in Essential Tremor – Review of the Current Evidence

“…A large number of studies have demonstrated an association between hearing loss and neurodegenerative diseases [ 21 22 23 24 25 26 27 28 29 30 31 32 33 ]. Hearing loss is a risk marker for cognitive decline and dementia [ 23 ].…”

“…However, according to another approach, similar patterns of sensorineural hearing loss (slightly worse in the PD group) were observed when comparing the control group and PD patients, which were typical for their age [ 29 ]. Auditory dysfunction is also shown in Huntington’s disease, diseases linked to the cerebellum, such as spinocerebellar ataxias, and Friedreich’s ataxia [ 30 31 32 33 ].…”

Pure-Tone Hearing Thresholds and Brainstem Auditory Evoked Potentials in Sporadic Ataxia

“…As for Friedreich ataxia, an autosomal recessive inherited disease, studies showed that 90% of the patients had hearing loss, considering this result as an important finding in the differential diagnosis for cerebellar ataxias. 10 For Zeigelboim et al, 11 who assessed 30 patients with Friedreich ataxia, audiometric testing unveiled 43.3% of altered cases, with bilateral prevalence in 36.7% of them.…”

“…The authors suggested that the involvement of the auditory pathways must have a genetic cause. Zeigelboim et al 11 assessed 30 patients with Friedreich ataxia and evidenced abnormalities in 56.6% of the cases, which were bilateral in 50%.…”

“…Some studies, 11 12 13 using electrophysiological tests in patients with cerebellar ataxia, already verified abnormalities in brainstem auditory evoked potentials (BAEPs) in > 50% of the evaluated subjects, while other studies 14 15 evidenced abnormality in 71 and 46.5%, respectively, in the BAEP assessment of autosomal recessive and dominant spinocerebellar ataxias. In the acoustic immittance measurement, Zeigelboim et al 11 reported disorders in 46.6% of the sample of patients with neurodegenerative diseases, and 44.1% of abnormality in patients with autosomal dominant spinocerebellar ataxia, 15 mainly related to acoustic reflexes.…”