Over the last decade, a number of distinct mutations in the mtDNA (mitochondrial DNA) have been found to be associated with both syndromic and non-syndromic forms of hearing impairment. Their real incidence as a cause of deafness is poorly understood and generally underestimated. Among the known mtDNA mutations, the A1555G mutation in the 12S gene has been identified to be one of the most commo...

Objective To assess long-term hearing outcomes in patients treated with third-generation bisphosphonates for otosclerosis-related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review. Methods We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate ...

INTRODUCTION Aminoglycosides (AGs) have been widely used for potential life-threatening bacterial infections. Although AGs are well known for their ototoxic side effects, some AGs such as amikacin are considered less harmful to auditory functions; thus, auditory monitoring is mostly neglected during treatment with these drugs. OBJECTIVE To reflect the potential auditory hazards of repeated am...

Mitochondrial diseases (MD) are a clinically heterogeneous group of disorders that arise as a result of dysfunction of the mitochondrial respiratory chain. Sensorineural hearing loss (SNHL) is often associated to mitochondrial dysfunctions both in syndromic, nonsyndromic forms. SNHL has been described in association to different mitochondrial multisystemic syndromes, often characterized by an i...

OBJECTIVES/HYPOTHESIS The present study aimed to examine whether the response patterns to the chimeric lexical tone tokens, combined with their pure tone audiometry (PTA) results, could separate listeners with sensorineural hearing loss (SNHL) from listeners with auditory neuropathy spectrum disorder (ANSD). STUDY DESIGN Case-control study. METHODS Forty-three SNHL subjects and 46 ANSD subj...

The classic view of sensorineural hearing loss (SNHL) is that the "primary" targets are hair cells, and that cochlear-nerve loss is "secondary" to hair cell degeneration. Our recent work in mouse and guinea pig has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of cochlear-...

OBJECTIVE Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child. STUDY DESIGN Retrospective review. SETTING Tertiary care university hospital. PATIENTS A total of 270 children refer...

cavernous hemangioma is a rare benign tumor of the internal auditory canal (iac) of which fourteen cases have been reported so far. tinnitus and progressive sensorineural hearing loss (snhl) are the chief complaints of the patients. audiological and radiological planes, ctscan, and magnetic resonance image (mri) studies are helpful in diagnosis. the only choice of treatment is surgery with elec...

Background/objectives Hearing loss (HL) is associated with certain diseases and affects health, resulting in a low quality of life. Some components of the metabolic syndrome (MetS) coincide with the risk factors for sensorineural hearing loss (SNHL). To date, very few studies have examined the link between MetS and HL. The aim of the current study was to try to understand the potential associat...

Vestibular schwannomas (VSs) are the most common tumours of the cerebellopontine angle. Ninety-five percent of people with VS present with sensorineural hearing loss (SNHL); the mechanism of this SNHL is currently unknown. To establish the first model to study the role of VS-secreted factors in causing SNHL, murine cochlear explant cultures were treated with human tumour secretions from thirtee...