BACKGROUND we aimed to assess both cross-sectional and temporal links between measured hearing impairment and self-perceived hearing handicap, and health outcomes. METHODS in total, 811 Blue Mountains Hearing Study participants (Sydney, Australia) aged ≥55 years were examined twice (1997-99 and 2002-04). Hearing levels were measured with pure-tone audiometry. The shortened version of the hear...

Different strains of mice provide a valuable research tool for studying both hereditary and acquired forms of deafness. The cd/1 strain has been found to demonstrate hereditary cochlear pathology. The characteristics of hearing loss in cd/1 mice have not previously been reported. In this investigation auditory thresholds were obtained by measuring evoked brain stem responses in subjects of thre...

The genetic heterogeneity of hereditary hearing loss is thus far represented by hundreds of genes encoding a large variety of proteins. Mutations in these genes have been discovered for patients with different modes of inheritance and types of hearing loss, ranging from syndromic to non-syndromic and mild to profound. In many cases, the mechanisms whereby the mutations lead to hearing loss have...

BACKGROUND As in all western countries, Australia's older population experiences high levels of hearing impairment coupled with relatively low levels of hearing device usage. Poor hearing diminishes the quality of life of affected individuals and their families. This paper discusses how to improve Australian hearing health policies in order to better combat this impairment amongst older Austral...

BACKGROUND Hereditary forms of hearing loss are classified as syndromic, when deafness is associated with other clinical features, or non-syndromic, when deafness occurs without other clinical features. Many types of syndromic deafness have been described, some of which have been mapped to specific chromosomal regions. METHODS Here we describe a family with progressive sensorineural hearing l...

This review concentrates on factors causing permanent hearing impairment in childhood, described in some European studies. The emphasis is on comparative studies, based on strict criteria and terminology upon which longitudinal changes in the pattern of factors causing hearing impairment can be demonstrated in well-defined cohorts of children. The high proportion of 20-39 percent of unknown cau...

BACKGROUND Mitochondrial DNA (mtDNA) mutations are known to cause Leber hereditary optic neuropathy (LHON). However, the co-occurrence of double pathogenic mutations with different pathological significance in pedigrees is a rare event. METHODS Detailed clinical investigation and complete mtDNA sequencing analysis was performed for two Indian families with LHON. The haplogroup was constructed...

PURPOSE Spatial hearing refers to the ability to understand speech and identify sounds in various environments. We assessed the validity of the Korean version of the Spatial Hearing Questionnaire (K-SHQ). MATERIALS AND METHODS We performed forward translation of the original English SHQ to Korean and backward translation from the Korean to English. Forty-eight patients who were able to read a...