The understanding of the molecular genetics in sensorineural hearing loss (SNHL) has advanced rapidly during the last decade, but the molecular etiology of hearing impairment in the Portuguese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we analyzed the whole mitochondrial genome in 95 unrelated children with SNHL (53 nonsyn...

Sensorineural hearing loss is one of the most common neurosensory disorders in humans. The incidence of SNHL is estimated to be 1 in 500-1000 newborns. In more than half of these patients, the hearing loss is associated with genetic causes. In Japan, genetic testing for the patients with SNHL using the Invader assay to screen for 46 mutations in 13 deafness genes was approved by the Ministry of...

OBJECTIVE To elucidate clinical antecedents of sensorineural hearing loss (SNHL) in very preterm infants. DESIGN Case-control study. SUBJECTS Fifteen children < 33 weeks' gestation with significant SNHL born between 1 January 1990 and 31 December 1994, detected within 9 months of birth, and 30 matched control children. METHODOLOGY Perinatal variables in the two groups were compared using ...

Hajas et al. recently examined the prevalence of sensorineural hearing loss (SNHL) in patients with mixed connective tissue disease (MCTD) [1]. Auditory symptoms are relatively frequent, but apparently underestimated, in patients with connective tissue disease [2]. SNHL is rapidly progressive and accompanied by vertigo and/or tinnitus in some patients, but it is often asymptomatic. Since SNHL c...

The aim of this study was to provide more information on the causes of sensorineural hearing loss (SNHL) in children cochlear implant candidates in Croatia. The retrospective study included 270 children candidates for cochlear implantation between January 1997 and January 2005 at our institution. The medical assessment of the candidates included the history, physical examination, radiologic eva...

BACKGROUND Muckle-Wells syndrome (MWS) is a rare auto-inflammatory disease characterized by the presence of recurrent urticaria, deafness and amyloidosis. Progressive sensorineural hearing loss (SNHL) is reported to occur in up to 85% of patients occurring in the second and third decades and as early as the first decade in patients with a more severe phenotype, thus potentially having a signifi...

Although conventional structural MRI provides vital information in the evaluation of congenital sensorineural hearing loss (SNHL), it is relatively insensitive to white matter microstructure. Our objective was to evaluate possible changes in microstructure of the auditory pathway in children with congenital sensorineural hearing loss (SNHL), and the possible distinction between good and poor ou...

Listeners with hearing loss have difficulty processing sounds in noisy environments. This is most noticeable for speech perception, but is reflected in a basic auditory processing task: detecting a tonal signal in a noise background, i.e., simultaneous masking. It is unresolved whether the mechanisms underlying simultaneous masking arise from the auditory periphery or from the central auditory ...

The benefit provided to listeners with sensorineural hearing loss (SNHL) by an acoustic beamforming microphone array was determined in a speech-on-speech masking experiment. Normal-hearing controls were tested as well. For the SNHL listeners, prescription-determined gain was applied to the stimuli, and performance using the beamformer was compared with that obtained using bilateral amplificatio...

consanguineous marriage is strongly favored in many large human populations. in the most parts of south asia, consanguineous marriage account for 20%  to over 50% of the general population. the effect of consanguinity on hereditary deafness has been well studied and documented. many authors have suggested that approximately one half  of sensory neural hearing loss in children can be attributed ...