Section: 
Otiology
OtiologyFeatures of hearing aid for children with alpha-mannosidosis
N. A. Daikhes (1), A. S. Machalov (2), E. I. Nayandina (3), O. L. Bragina (4), A. O. Kuznetsov (5)
(1), (2), (3), (4), (5) National Medical Research Center for Otorhinolaryngology, Federal Medico–Biological Agency of Russia, Moscow, 123182, Russian Federation, (1), (2), (5) Pirogov Russian national research medical University, Moscow, 117513, Russian Federation, (2), (4) Russian Medical Academy of Continuous Professional Education, Moscow, 125993, Russian Federation
UDK: УДК 616.28-009:376:616.283-089.84-053.1
DOI: https://doi.org/10.18692/1810-4800-2025-6-77-86
ABSTRACT
Abstract. Introduction. One of the main clinical manifestations of alpha-mannosidosis is hearing impairment of varying forms and severity. The main method of correcting hearing impairment in patients with alpha-mannosidosis is the use of technical means of rehabilitation. Throughout the entire period of auditory and verbal rehabilitation for children with alpha-mannosidosis, specialized care and support is needed. Objective. Тo optimize the tactics of auditory and verbal rehabilitation of pediatric patients with hearing loss associated with alpha-mannosidosis. Patients and methods. 7 patients took part in the study on the basis of the Center for Otorhinolaryngology of Russia. As part of this study, an analysis of the results of comprehensive auditory and verbal rehabilitation was carried out. Results. Analysis of the results of comprehensive auditory and verbal rehabilitation revealed statistically significant improvements in speech perception and intelligibility in various acoustic environments, the emergence of vocalization, vocabulary development, the development of a conditioned motor response (CMR) to sound, and improved spatial orientation. Discussion. Effective rehabilitation of patients with alpha-mannosidosis requires a comprehensive approach, including medical monitoring, individualized rehabilitation programs, and ongoing specialist support.
Publication date:
23.12.2025
Keywords:
alpha-mannosidosis, sensorineural hearing loss, mixed hearing loss, quality of life, auditory and verbal rehabilitation For citation:
Daikhes N. A., Machalov A. S., Nayandina E. I., Bragina O. L., Kuznetsov A. O. Features of hearing aid for children with alpha-mannosidosis. Russian Otorhinolaryngology. 2025;24(6):77-86. (In Russ.) https://doi.org/10.18692/1810-4800-2025-6-77-86