Objective: To explore the etiology, clinical aspects, diagnosis and therapeutic strategies of acute low-tone sensorineural hearing loss (ALHL).
Methods: Thirty patients (31 ears) with ALHL were selected for this study. Detailed history collection, otological examination and systematic audiological evaluations were conducted. The hearing tests included pure tone audiometry, acoustic immittance, auditory brainstem response (ABR) and otoacoustic emissions (OAE). All cases received therapeutic trial of corticosteroid for 15 days with 6 to 14 months' following-up.
Results: ALHL mainly affected young people. Low-tone tinnitus, a sensation of ear fullness and hearing impairment were the frequent complains. Otological examinations showed normal results. Mild to moderate sensorineural hearing loss at low frequencies and type "A" tympanograms were found in all patients. Acoustic stapedial reflexes were elicited in 26 of 31 affected ears, and 14 of them had positive results on the Metz test. ABR responses were normal in all 20 tested ears. In 14 out of 20 ears, TEOAEs were absent and DPOAE grams at low frequencies (0.5, 0.75 kHz) were abnormal on the first visit. After steroid therapy, 24 ears demonstrated complete recovery, but 4 ears showed partial recovery and 3 ears unchanged. The total improvement rate was 90.3%.
Conclusions: ALHL patients are clinically characterized by low-tone tinnitus, aural fullness and hearing loss, which mainly involved unilateral ear. Audiological findings indicate a cochlear impairment, which only invades low frequency region. The basic pathological feature may be endolymphatic hydrops involves immune response. Conflicting data exist on whether ALHL is an independent disorder or a subtype of Meniere's disease. Ideal therapeutic strategy has not been established by now and corticosteroid is probably an effective agent.
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J Appl Genet
January 2025
Genetics Research Center, University of Social Welfare and Rehabilitation Sciences, Koodakyar Avenue, Daneshjoo Blvd, Evin, Tehran, 1985713834, Iran.
Perrault syndrome (PS) is an extremely rare autosomal recessive condition characterized primarily by bilateral sensorineural hearing loss in both genders and primary or secondary ovarian failure in females. Neurological features such as cerebral ataxia, peripheral neuropathy, epilepsy, and intellectual disability are frequent manifestations of PS. To date, six genes have been reported to cause PS, and nearly 100 families have been identified worldwide with this syndrome.
View Article and Find Full Text PDFAudiol Res
December 2024
ENT & Audiology Unit, Department of Neurosciences, University Hospital of Ferrara, 44124 Ferrara, Italy.
Objectives: The present study aims to identify potential predictive factors for developing sensorineural hearing loss (SNHL) in individuals with congenital Cytomegalovirus (cCMV) infection.
Methods: A retrospective study was performed on 50 subjects with cCMV infection (symptomatic and asymptomatic), followed at the Audiology Service of Sant'Anna Hospital (University Hospital of Ferrara). The following data were analyzed: the type of maternal Cytomegalovirus (CMV) infection (primary versus non-primary), time of in utero infection, systemic signs and symptoms or laboratory test anomalies due to cCMV infection, and signs and symptoms of central nervous system (CNS) involvement at birth.
Audiol Res
December 2024
Department of Audiology and Speech Language Pathology, Kasturba Medical College Mangalore, Manipal Academy of Higher Education, Manipal 576104, Karnataka, India.
Hearing loss in children can have a detrimental impact on their development, thus lowering the psychological well-being of parents. This study examined the amount of parental stress, learned helplessness, and perceived social support in mothers of children with hearing loss (MCHL) and mothers of typically developing children (MTDC), as well as the relationship between various possible contributing factors to parental stress such as learned helplessness and perceived social support. Three questionnaires measured parental stress (Parental Stress Scale; PSS), learned helplessness (Learned Helplessness Scale; LHS), and perceived social support (Perceived Social Support-Friends PSS-Fr and Perceived Social Support-Family PSS-Fa Scale) in 100 MCHL and 90 MTDC.
View Article and Find Full Text PDFInt Arch Otorhinolaryngol
January 2025
Department of Otorhinolaryngology, Head and Neck Surgery, Army College of Medical Sciences, Brar Square, Delhi Cantonment, New Delhi, India.
Stapedotomy is the standard of care in the surgical management of clinical otosclerosis. It is a precise and technically demanding craft requiring impeccable surgical skills. Both conventional and laser-assisted procedures aim to achieve closure of the air-bone gap (ABG) with minimum collateral damage.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
January 2025
Department of Pathology and Pathophysiology, School of Medicine, Nanjing University of Chinese Medicine, Nanjing210023, China.
To compare the efficacy and safety of postauricular injection (PI) and intratympanic injection (II) of glucocorticoids (GC) in the initial treatment of sudden sensorineural hearing loss (SHL). Electronic databases retrieval (PubMed, Web of Science, CNKI, VIP, WANFANG) was performed to identify all randomized controlled trials about PI and II of GC in the initial treatment of SHL between 2015 and 2024. Meta-analysis was performed on the studies met the inclusion criteria by RevMan5.
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