Introduction: vHIT test to assess the functioning of three semicircular canals. Objective: This study was designed to objectively assess the functioning of the three semicircular canals in individual with severe to profound sensorineural hearing loss using vHIT respectively. Methods: Twenty adult participants (40 ears) having severe to profound hearing loss ranging in age from 15-40 years in gr...

With broadening candidacy criteria for cochlear implantation, a greater number of pediatric candidates have usable residual hearing in their nonimplanted ears. This population potentially stands to benefit from continued use of conventional amplification in their nonimplanted ears. The purposes of this investigation were to examine the speech and language development of pediatric cochlear impla...

Mutations in the gene (MYO7A) encoding myosin-VIIa, a member of the large superfamily of myosin motor proteins that move on cytoplasmic actin filaments, and in the USH2A gene, which encodes a novel protein resembling an extracellular matrix protein or a cell adhesion molecule, both cause Usher syndrome (USH), a clinically heterogeneous autosomal recessive disorder comprising hearing and visual ...

The DFNB1 subtype of autosomal recessive, nonsyndromic hearing impairment, caused by mutations affecting the GJB2 (connexin-26) [corrected] gene, is highly prevalent in most populations worldwide. DFNB1 hearing impairment is mostly severe or profound and usually appears before the acquisition of speech (prelingual onset), though a small number of hypomorphic missense mutations result in mild or...

The contribution of parental suspicion in the original identification of a 16 year cohort of 171 children with varying degrees of hearing impairment who were screened and identified in childhood was studied. Only a quarter of the children with permanent hearing loss were identified as a result of parental concern. The presence of parental suspicion preceding the audiological diagnosis was also ...

For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26-q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communicatio...

OBJECTIVE To characterize audiometric findings in children with a large vestibular aqueduct (LVA). DESIGN Retrospective review. METHODS Audiometric records of children with an isolated LVA, diagnosed by computed tomography of the temporal bone, from 1995 through 1998 were reviewed. RESULTS Nineteen children had an isolated LVA in one or both ears. In all, 26 ears with an isolated LVA were...