Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury / 临床耳鼻咽喉头颈外科杂志
Journal of Clinical Otorhinolaryngology Head and Neck Surgery
; (12): 44-49, 2024.
Article
em Zh
| WPRIM
| ID: wpr-1011100
Biblioteca responsável:
WPRO
ABSTRACT
Objective:To investigate long-term auditory changes and characteristics of Alport syndrome(AS) patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33(25 males, 8 females, aged 3.4-27.8 years) were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal disease(ESRD), predominantly males (6/7). The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group(P=0.013). There was no correlation between the progression of renal disease and long-term hearing level(P>0.05). kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing loss(P=0.048), and there was no correlation with the severity of hearing loss(P>0.05). Among 11 cases, theCOL4A5mutationwas most common (8 out of 11), but there was no significant correlation between the mutation type and hearing phenotype(P>0.05). Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Estudos Retrospectivos
/
Surdez
/
Perda Auditiva
/
Rim
/
Falência Renal Crônica
/
Mutação
/
Nefrite Hereditária
Limite:
Child
/
Female
/
Humans
/
Male
Idioma:
Zh
Revista:
Journal of Clinical Otorhinolaryngology Head and Neck Surgery
Ano de publicação:
2024
Tipo de documento:
Article