Search results for " Sensorineural"
showing 10 items of 62 documents
Familial insulin resistant diabetes associated with acanthosis nigricans, polycystic ovaries, hypogonadism, pigmentary retinopathy, labyrinthine deaf…
1993
Two sibs, whose parents are first cousins, had diabetes mellitus with hyperinsulinism, insensitive insulin receptors, and acanthosis nigricans. Both patients had pigmentary retinopathy, secondary cataracts, labyrinthine deafness, mental retardation, and cerebral atrophy. They were disproportionately short with relatively broad hands and feet and slightly coarse face. The young woman had secondary amenorrhea and polycystic ovaries and the boy gynecomastia and hypergonadotrophic hypogonadism. This appears to be the second family with a new autosomal recessive disorder differing from Alstrom syndrome by the presence of mental retardation and absence of renal insufficiency. Impaired insulin rec…
Canonical Babbling and Early Hearing and Language Development of Normal Hearing Children and Children with Cochlear Implants
2010
(2010). Canonical Babbling and Early Hearing and Language Development of Normal Hearing Children and Children with Cochlear Implants. Cochlear Implants International: Vol. 11, Proceedings of the 9th European Symposium on Paediatric Cochlear Implantation, Warsaw, 2009, pp. 375-378.
Manifestation of speech and language disorders in children with hearing impairment compared with children with specific language disorders.
2010
Children with hearing impairment (HI) often suffer from speech and language disorders. We wondered if the manifestation of these disorders resembled the ones in children with specific language impairment (SLI). Using matched pairs, we compared the manifestation of a speech and language disorder in 5- and 6-year-old children with HI and SLI. We looked at receptive language skills using the Reynell scales, the lexicon, syntax and morphology, output phonology, and phonological short-term memory. Receptive language skills were more impaired in HI children. No significant differences were recorded for all other domains. We conclude that language deficits that are at least partially caused by the…
Auditory Neuropathy in Children
2001
Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory brainstem evoked potentials and normal cochlear outer hair cell function. A variety of processes is thought to be involved in its pathophysiology and their influence on hearing may be different. We present here the diagnostic sequence and management of two new cases of auditory neuropathy in breastfeeding children.
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report.
2013
Objective: To evaluate independent etiologic factor associated with sensorineural hearing loss (SNHL) in newborn at risk; to study the role of their interaction especially in NICU infants who present often multiple risk factors for SNHL. Methods: The main risk factors for SNHL reported by JCIH 2007 were evaluated on 508 infant at risk ranging from 4 to 20 weeks of life, transferred to the Audiology Department of Palermo from the main births centers of Western Sicily. After a global audiological assessment, performed with TEOAE, tympanometry and ABR, the prevalence and the effect of risk factors was statistically studied through univariate and multivariate analysis on the total population (n…
The Jervell and Lange-Nielsen syndrome.
1991
Deafness and electrocardiographic changes (prolongation of the Q-T interval and inversion of the T wave) with a clinical picture of syncopal attacks and sudden death, were described as a distinct syndrome by Jervell and Lange-Nielsen in 1957. The syndrome is inherited as an autosomal recessive trait. In this study, all the cases reported since 1957 and their proposed prevalence are reviewed. The authors describe the 4 cases they have studied, all of which presented congenital sensorineural hearing loss and electrocardiographic changes characteristic of the syndrome. The relatively high number of cases they have encountered casts doubt on literature that states that the syndrome occurs more …
Comparative Study Between Unilateral and Bilateral Cochlear Implantation in Children of 1 and 2 Years of Age
2016
Abstract Introduction The studies that have evaluated the effectiveness of bilateral cochlear implantation in children suggest an improvement in hearing about sound localisation and speech discrimination. In this paper we show the differences in audio-linguistic achievements with early bilateral cochlear implantation versus unilateral, and differences between simultaneous and sequential bilateral implantation. Material and methods We present 88 children with bilateral profound sensorineural hearing loss, treated with bilateral cochlear implantation in 32 cases and unilateral in 56 cases, during the first 12 months (27 children) of life and between 12 and 24 months (61 children). We conducte…
Evolution of otosclerosis to cochlear implantation.
2011
Abstract Introduction Otosclerosis is an osteodystrophy of the labyrinthine capsule producing conductive hearing loss. If the process invades the cochlea, a sensorineural hearing loss usually takes place. The cochlear implant is a good alternative in these patients. Objective To ascertain the behaviour of cochlear implantation (CI) in otosclerosis. Material and methods We reviewed a database of 250 patients who underwent CI, performing a retrospective study of 13 patients with clinical, audiological and/or imaging findings of bilateral otosclerosis. The 26 ears were studied as to their natural history, previous surgeries, evolution to profound hearing loss, computed tomography (CT) images, …
Bacterial Cytolysin Perturbs Round Window Membrane Permeability Barrier In Vivo: Possible Cause of Sensorineural Hearing Loss in Acute Otitis Media
1998
ABSTRACT The passage of radioiodinated streptolysin-O (SLO) and albumin through the round window membrane (RWM) was studied in vivo. When applied to the middle ear, SLO became quantitatively entrapped in this compartment and no passage to the cochlea occurred. However, flux of radioiodinated albumin through the toxin-damaged RWM was observed. We propose that the passage of noxious macromolecules, such as proteases, from a purulent middle-ear effusion may be facilitated by pore-forming toxins, resulting in cochlear damage and sensorineural hearing loss.
Risk Factors for Sensorineural Hearing Loss and Auditory Maturation in Children Admitted to Neonatal Intensive Care Units: Who Recovered?
2022
Background: Newborns admitted to neonatal intensive care units (NICUs) are at higher risk of developing sensorineural hearing loss (SNHL), which may improve over time. The aim of this study was to describe the prevalence of the main risk factors for SNHL in a NICU cohort, focusing on children who underwent auditory maturation. Methods: An observational study of 378 children admitted to NICUs, who were followed for at least 18 months, with periodic audiologic assessments. Results: Out of 378 patients, 338 had normal hearing and 40 were hearing-impaired; we found a higher percentage of extremely preterm (EPT) and extremely low-birthweight (ELBW) infants in SNHL children (p < 0.05). Sevente…