[Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury].

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.

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

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.

Hearing dynamics in patients with traumatic tympanic membrane perforation.

BACKGROUND: Hearing distortions from trauma to the ear could occur by direct perforation of the tympanic membrane. AIM: To characterize hearing thresholds in patients with traumatic tympanic membrane perforation and changes in hearing occurring in the course of treatment. STUDY DESIGN: Prospective analytical study. SETTING: Olabisi Onabanjo University Teaching Hospital, Sagamu, Nigeria. METHODOLOGY: We prospectively entered in a proforma the demographics of 60 patients with traumatic tympanic membrane perforation at the teaching hospital in Sagamu, Nigeria over a period of five years as well as their hearing assessment with pure tone audiometry at initial contact and six weeks post-injury. The type of hearing loss, pure tone average and air-bone gaps were recorded. Hearing changes between initial and second audiometric assessments were analyzed and compared. RESULTS: The data obtained from 60 patients with 73 traumatized ears were analyzed. In all, 64 (87.7%) of the ears had hearing loss while 33(45.2%) had conductive hearing loss. Injured ears had significantly worse hearing and higher air-bone gaps ABGs compared with non-traumatized ears. There was notable improvement in hearing thresholds and closure of air-bone gaps in the course of treatment, which was significantly more at the low frequencies compared with the high frequencies. CONCLUSION: Patients with traumatic tympanic membrane perforation majorly had conductive hearing loss in the injured ears with audiometric confirmed air-bone gaps and increased hearing thresholds which were not frequency dependent. There was appreciable improvement in hearing parameters over time, significantly more at the low frequencies.

Early pain relief from orthostatic headache and hearing changes in spontaneous intracranial hypotension after epidural blood patch.

Spontaneous intracranial hypotension (SIH) is a neurological condition characterized by orthostatic headache (OH), low cerebrospinal fluid (CSF) pressure and diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI). Hearing changes (HC) are also a common clinical finding. At present, epidural blood patch (EBP) is the most recommended treatment. Our study aimed at describing clinical variability of SIH patients. We also aimed at measuring the EBP efficacy on OH and HC in patients affected by SIH, by asking them to rate their levels of discomfort on a VAS. 28 consecutive patients were recruited. All of them complained about OH, 16 of them also reported HC. They were all treated with EBP. Two clinical psychologists interviewed them before and after the procedure, asking to rate the intensity of their OH and HC on a VAS at different time points: the day before the procedure, between 24 and 48 h after it and 2 months after treatment. Before EBP, patients rated their OH as 5 (IQR 2-7) and their HC as 4 (IQR 2-5.75). 24/48 h after EBP, a significant improvement in OH (median 0, IQR 0-0; p < 0.001) and HC (median 1, IQR 0-2; p < 0.05) was found. At follow up assessments, all patients reported a complete relief from their OH and four out of 16 patients only still reported mild HC. Our data show for the first time the early and durable efficacy of EBP on OH and HC in patients affected by SIH.