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Biomedical subjects

K Lüerssen

Publications and source records attributed to K Lüerssen.

7 recordsLinked to original sources
PubMed2006

[Auditory processing disorder versus attention deficit/hyperactivity disorder. A dysfunction complex or different entities?].

BACKGROUND: It has been suggested that auditory processing disorders (APD) and attention deficit/hyperactivity disorders (ADHD) may be the same entity with slightly differing symptoms. For testing this theory we compared parents' ratings of APD and ADHD behavioural characteristics. METHODS: A retrospective study was carried out with parents rating 312 children (113 girls and 199 boys; 6.0-9.11 years old, nonverbal intelligence IQ 95 or above) using APD and ADHD questionnaires. RESULTS: Significant, albeit weak correlations between several APD and ADHD subscale scores were found. Correlations between selective listening and attention behaviours approximated clinical relevance (rho >0.4). Factor analysis revealed two factors with APD behaviour loading on one and ADHD behaviour on a second different factor. Using a categorical approach, it appears that ADHD children have significantly more APD problems than children without ADHD. DISCUSSION: These data are consistent with the hypothesis that APD and ADHD overlap partly while still being distinct entities. In addition to dimensional aspects, the parents' rating may provide a guideline for establishing a diagnosis based on categorical distinctions.

Attention Deficit Disorder with Hyperactivity↗
PubMed2005

Osteopathia striata with cranial sclerosis and hearing loss.

Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis, it represents a separate entity that is not limited to the bones, but may also affect other structures, leading to an abnormal face, cleft palate, deafness, heart defects and vertebral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness. So far, only a few patients have been described in the literature, demonstrating autosomal dominant inheritance. Here, we report a spontaneous mutation of inheritance. The parents and three sisters of the affected child are healthy. In 78.6% of the reported cases with osteopathia striata and hearing loss, a conductive hearing loss was described. Surgical attempts to mobilize the middle-ear bones were unsuccessful because of swelling and augmented growth of the bones in the middle ears. Here the mobilization of the eardrum was not attempted because the middle ear cavities were epidermized. This is the second case in the literature in which a myringotomy was not done because the middle ear was completely epidermized. As a possible alternative, children with osteopathia striata and cranial sclerosis with hearing loss should be fitted with hearing aids because surgery of the middle ear probably will be difficult or perhaps not possible.

Abnormalities, Multiple↗
PubMed2004

[Niikawa-Kuroki (Kabuki) syndrome and hearing impairment].

The Niikawa-Kuroki syndrome is a rare syndrome characterized by multiple congenital anomalies, mental retardation, postnatal growth deficiency, dermatoglyphic abnormalities and a characteristic facial appearance. More than 100 cases of the syndrome have been described in Europe. Here we report a 10 year old girl with this syndrome. Recurrent infections of the middle ear as well as delayed motor and speech development were present. The physiognomy since early infancy showed a flat profil, long palpebral fissures, long eyelashes, high arched eyebrows, missing lateral incisors and persistent fetal pads on the fingers. The patient was referred to us because of hearing impairment. Her parents also described a delayed motor development, mental retardation and a language impairment. Audiometry demonstrated a pantonal hearing loss. Since children affected by this syndrome may also have a severe hearing impairment early testing is mandatory.

Abnormalities, Multiple↗
PubMed2004

[Small deletion--large effect].

Velocardiofacial syndrome (VCFS) or Shprintzen's syndrome leads to cleft palate (69%), heart defects (74%), and characteristic facial dysmorphies as well as learning difficulties (70-90%). There is phenotypic overlap with DiGeorge syndrome (DGA). In 1992, it was shown that patients with VCFS had a partial 22q11 monosomy. The site and size of the deletion in many VCFS patients do not differ from patients with DGS. For the otolaryngologist, it is important to check for cardiac defects if the characteristic middle ear effects and possibly submucosal cleft palate are present. If a combination of these exist, it is advisable to carry out a genetic examination of the child in order to determine whether VCFS is present or not. This is the only way of providing an early diagnosis of this syndrome.

Child↗
PubMed2003

[Long-term results after velopharyngoplasty].

BACKGROUND: Children with cleft palate frequently demonstrate speech and resonance disorders following primary cleft repair. In some patients no improvement can be achieved with conservative therapy and a velopharyngoplasty (VPP) may be indicated. This study was performed to evaluate the long-term results after VPP. MATERIAL AND METHODS: Twenty-six patients were followed up on an average 9 years after VPP (mean age: 16+/-5 years). Apart from the phoniatric-pedaudiologic findings, the speech intelligibility and nasality were judged by speech pathologists and by laymen. The extent of the speech handicaps was evaluated with a questionnaire. The nasalance was measured with NasalView. The vowels, two test sentences, and three reading texts (LT(1-3)) were used as test materials. RESULTS: The phoniatric-pedaudiologic examination revealed a clear improvement of hearing, language, and speech function. Compared to the previous results, decreased nasality and improved ability in articulation were detected. The judgment of laymen was also positive. The speech intelligibility was mostly evaluated as favorable. A speech handicap was present in only a few patients. The evaluation using NasalView showed significantly increased nasalance values for the LT(2) ( p=0.030). Moreover there were lower nasalance values for all the reading texts compared to the control group (LT(1) p=0.257, LT(2) p=0.408, LT(3) p=0.187). CONCLUSION: A clear improvement can be achieved with VPP in patients with a high degree of therapy-resistant nasality. In many cases even normal colloquial language is possible. The evaluation of nasalance has proved to be successful for rating surgical outcome.

Adolescent↗