Speech, language, and cognition in preschool children with epilepsy

from Developmental Medicine and Child Neurology

We studied expressive and receptive language, oral motor ability, attention, memory, and intelligence in 20 6-year- old children with epilepsy (14 females, six males; mean age 6y 5mo, range 6y-6y 11mo) without learning disability, cerebral palsy (CP), and/or autism, and in 30 reference children without epilepsy (18 females, 12 males; mean age 6y 5mo, range 6y-6y 11mo). Ten children had partial, six primarily generalized, and four unclassified epilepsy. Fourteen were having monotherapy and six were taking two or more antiepileptic drugs; 13 children were free from seizures 3 months before the assessment. Results show no statistically significant difference between the groups concerning Verbal IQ, expressive and receptive grammar, and receptive vocabulary. The children with epilepsy had a significantly lower Performance IQ and lower scores in tests of oral motor ability, articulation, emerging literacy, auditory attention, short-term memory, and rapid word retrieval. Parent ratings revealed no significant difference in communicative ability. Polytherapy and early onset of epilepsy influenced some results. Preschool children with epilepsy without learning disability, CP, and/or autism may have receptive verbal ability within the normal range but visuoperceptual, auditory attentional, and speech-language difficulties that could affect school achievement. Careful testing of children with epilepsy who appear to be functioning within the normal range is needed because this may reveal specific impairments that require appropriate professional input.

Language Conflict in the Bilingual Brain

from Cerebral Cortex

The large majority of humankind is more or less fluent in 2 or even more languages. This raises the fundamental question how the language network in the brain is organized such that the correct target language is selected at a particular occasion. Here we present behavioral and functional magnetic resonance imaging data showing that bilingual processing leads to language conflict in the bilingual brain even when the bilinguals’ task only required target language knowledge. This finding demonstrates that the bilingual brain cannot avoid language conflict, because words from the target and nontarget languages become automatically activated during reading. Importantly, stimulus-based language conflict was found in brain regions in the LIPC associated with phonological and semantic processing, whereas response-based language conflict was only found in the pre-supplementary motor area/anterior cingulate cortex when language conflict leads to response conflicts.

Cochlear implantation and connexin expression in the child with keratitis–ichthyosis–deafness syndrome

from the International Journal of Pediatric Otorhinolaryngology

We report one child with keratitis–ichthyosis–deafness (KID) syndrome. The child showed sparse, short scalp and body hairs, ichthyotic skin, bilateral sensorineural hearing loss, and visual loss. These are associated with a nucleotide substitution, 148G>A, which results in amino acid change in codon 50 of gap junctional protein connexin (Cx) 26. Immunohistochemistry of skins showed up-regulation of Cx26 and down-regulation of Cx43. He has benefited from cochlear implantation for hearing even though low visual function and skin problems with high risk of infections. This is the very rare report about Cx expression in skins and cochlear implantation in KID syndrome.

2nd Phonak Virtual Conference

from the National Health Service (UK)

For further information contact:

ilearn@phonak.com

Where:Online
When:19 May 2008 10:00 – 21 May 2008 16:00

British Society of Audiology Annual Conference

from the National Health Service (UK)

For further information contact:

Jan Deevey
Tel. 0118 966 0622
or e mail jan@thebsa.org.uk

Where:Nottingham
When:09 Sep 2008 09:00 – 11 Sep 2008 16:30

A Novel SLC26A4 (PDS) Deafness Mutation Retained in the Endoplasmic Reticulum

from Archives of Otolaryngology–Head and Neck Surgery

Objectives To identify mutations in the SLC26A4 gene in individuals with nonsyndromic hearing loss and enlarged vestibular aqueduct, to design a predicted model of the pendrin protein, and to characterize novel mutations by means of localization in mammalian cells and effect of the mutation on the predicted model.

Design Validation of the mutation by its exclusion in more than 300 individuals with normal hearing.

Setting A laboratory of genetics of hearing loss research, clinical genetics laboratories, an otolaryngology department at Tel Aviv University, and medical centers in Israel.

Patients A patient with nonsyndromic hearing loss and enlarged vestibular aqueduct, 203 deaf probands, and 310 controls with normal hearing.

Interventions Sequencing the SLC26A4 gene in the patient with nonsyndromic hearing loss and enlarged vestibular aqueduct. Transfection of yellow fluorescent protein (YFP) constructs into mammalian COS7 cells. Designing a computational model of the human SLC26A4 protein.

Main Outcome Measure Detection of a novel c.1458_1459insT SLC26A4 mutation.

Results A computational model of the human pendrin protein suggests that the novel c.1458_1459insT mutation leads to a prematurely truncated protein, p.Ile487TyrfsX39. Mammalian COS7 cells transfected with the YFP-1458_1459insT construct showed mislocalization of the mutant protein.

Conclusions A novel SLC26A4 mutation was detected in Israel. Because current estimates demonstrate that SLC26A4 mutations are involved in up to 4% of nonsyndromic deafness, our findings emphasize the importance of adding a molecular test for the SLC26A4 gene in the diagnosis of deafness, particularly when bone abnormalities are involved, to the list of genes screened in Israel and elsewhere in the world.

Implantation in the first few months of life – What do we know, and what are we doing?

from the National Health Service (UK)

For further information contact:

The Ear Foundation
Event Tel: Tel: +44 (0)115 942 7800
Event Fax:
Event Website: Web: www.earfoundation.org.uk

Where:Nottingham When:27 Jun 2008 10:00 – 16:00

Hearing problems at working age: Audiometric features and the aetiology of hearing impairment

from Audiological Medicine

Sixty patients, 37 women and 23 men, aged 30- 65 years, were included in the study. All had adult-onset hearing loss to an extent that necessitated hearing-aid fitting. The objective was to describe the audiometric features and extent of hearing loss, and to determine the aetiology of hearing impairments in hard-of-hearing patients in working life. The extent of hearing loss varied considerably; about one-third of the patients had moderate to severe hearing loss in both ears, about one-third had bilateral, mild hearing loss, and somewhat less than one-third had unilateral or asymmetrical hearing loss, with mild loss or even normal hearing in one ear. In 77% of the cases a diagnosis, verified or tentative, could be established. Middle ear disease with conductive or mixed hearing loss was found in 20% of the cases. Noise-induced hearing loss was diagnosed in 10% of the cases. The most common, but highly tentative, diagnosis was hereditary hearing loss. Including cases of otosclerosis, genetic hearing loss was suspected in 50% of the patients. Other diagnoses were occasionally encountered. Most of the patients had gently sloping audiograms of the better ear, followed by steeply sloping and U-shaped audiograms. It is concluded that a thorough audiological and medical investigation is an essential part of the process of aural rehabilitation.

The effects of a sound awareness pre-fitting intervention: A randomized controlled trial

from Audiological Medicine

The objective of the study was to evaluate the effects of an individual pre-fitting intervention for first-time hearing aid users. Thirty-eight hearing impaired adults were randomly assigned to a sound awareness pre-fitting intervention (n =19) or to a control group (n =19). The purpose of the sound awareness training was to facilitate the users’ acclimatization to amplified sound. The pre-fitting intervention consisted of three visits and was followed by conventional hearing aid fitting that was identical for both groups. Standardized questionnaires were administered before and after the pre-fitting intervention, after the conventional hearing aid fitting, and at a one-year follow-up. The follow-up also included a clinical assessment by means of a telephone interview performed by an independent audiologist. The pre-intervention did not result in any major improvement over and above the control group. However, improvements were found for both groups following hearing aid fitting. In addition, most participants were considered as successful users in the interview. Future research should target individuals in need of extended hearing aid rehabilitation.

Temporomandibular Disorder and New Aural Symptoms

from Archives of Otolaryngology–Head and Neck Surgery

Objectives To report the prevalence and demographics of temporomandibular disorder (TMD) within a population of clinic patients and to describe the prevalence of revisited and new, previously unstudied, aural symptoms described by a sample of these patients with TMD (hereinafter “TMD patients”).

Design A retrospective evaluation of patient records was completed to determine the percentage and the demographics of TMD patients in a clinical setting. A prospective analysis was done on the self-reported prevalence of previously studied and new aural symptoms of 78 TMD study patients compared with 78 control patients without TMD.

Setting A private otolaryngology practice in a rural Arizona town.

Patients Patients with TMD and aural symptoms.

Results Ten percent of all new otolaryngology clinic patients were diagnosed as having TMD. Of the 78 patients, 27 (35%) listed the ear as one of their sites of pain. The prevalence of each of the 8 aural symptoms assessed was significantly higher in TMD patients compared with controls (P < .001). A warm and/or fluid sensation in the ear and a stuffed cotton sensation in the ear were the most indicative symptoms of TMD because they had the highest relative risk ratios in TMD patients. Aural symptoms of loud noise sensitivity and cold air/wind sensitivity are also relevant and were approximately 5 times more frequent in TMD subjects than in controls.

Conclusions Patients with TMD are a significant component of otolaryngology practice. There are previously uninvestigated aural symptoms that occur much more frequently in TMD patients than in patients without TMD.