Audiological Findings in Children with PLA2G6-Associated Neurodegeneration.
J Am Acad Audiol
; 2022 Nov 29.
Article
em En
| MEDLINE
| ID: mdl-35705187
ABSTRACT
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.
Texto completo:
1
Base de dados:
MEDLINE
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article