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Biomedical subjects

A Lamprecht

Publications and source records attributed to A Lamprecht.

At least 37 records · Page 2Linked to original sources

Sudden loss of hearing and vestibular function, muscular weakness, and multiple white matter lesions in preschool children.

Sudden cochlear hearing loss, occurring successively or simultaneously in both ears, was observed in four unrelated preschool children. Vestibular testing could be carried out in three patients and showed complete bilateral loss of function. All patients had a mildly retarded motor development due to nonprogressive muscular weakness. On MR imaging all patients showed multiple periventricular and subcortical white matter lesions. These lesions were not clearly progressive in one patient examined repeatedly over 6 years. Virological, bacteriological, immunological, and metabolic examinations were normal in all patients. Muscle biopsy showed morphologically abnormal mitochondria in two and lipid storage in one patient. No indications have been found for a disturbed functioning of the muscle mitochondria. The identical pattern and course of the disease in these patients suggests a new nosological entity, the aetiology of which can only speculatively be attributed to a vascular process associated with a mitochondriopathy.

Biopsy↗

[Measuring evoked otoacoustic emissions at various times during intubation anesthesia].

Evoked otoacoustic emissions (EOAE) remain intraindividually similar in repeated measurements. After application of medication for general anesthesia no significant changes of EOAE could be seen. Damping of amplitude and energy, mostly seen in the late phase of general anesthesia, might be caused by possible middle ear pressure changes from nitrous oxide. The recording of EOAE under general anesthesia provides an additional method in objective infant hearing evaluation and expands our diagnostic tools in those cases when children do not cooperate.

Anesthesia, General↗

[Evoked otoacoustic emissions in normal and hearing impaired adults and children].

In adults and children, 203 normal ears, 124 cars with hearing-impairment and 37 ears with middle ear problems were studied concerning click-evoked oto-acoustic emissions. According to the results, this method by high specifity and sensitivity renders possible the recognition of low sensory hearing losses. Complete middle ear effusions too make themselves conspicuous by preventing the reception of these responses. The recording of click-evoked oto-acoustic emissions is a most promising auditory screening-method. A general use could essentially improve the early detection of hearing losses in infants. Additionally, in the pedaudiologic practice possibilities are indicated to employ this technique as a topodiagnostic instrument.

Adolescent↗

[Dysarthria, dysphagia or dyspnea as a reason for the initial consultation in pseudoparalytic myasthenia gravis and amyotrophic lateral sclerosis].

In myasthenia gravis and amyotrophic lateral sclerosis the ENT specialist or the phoniatrician may be consulted first, because in about 30 percent of all cases the initial symptoms are dysarthria, dysphagia or dyspnea. Three typical cases of each condition are presented. The quality of life of the patients can be improved considerably by early diagnosis and treatment. Special diagnostic and therapeutic procedures are described.

Adult↗

[Regulation of voice quality parameters at the beginning of phonation].

In 8 healthy probands, 6 patients with functional voice disorders, 3 singers and 3 deaf patients we studied the regulation of the voice parameters amplitude and fundamental frequency at the beginning of phonation. Analyses were performed under the following conditions: (1) normal conditions; (2) after troubling pharyngeal and/or glottic mucosal receptors by topical anaesthesia; (3) in the case of normal hearing during disturbance of the audiophonatory feedback mechanism by binaural masking, and (4) with a combination of the latter two situations. The results are discussed.

Deafness↗

Progressive erythrokeratodermia and cochlear hearing impairment. A case report and review of the literature.

A female child with congenital progressive erythrokeratodermia combined with sensory hearing loss observed through a period of 5 years is reported. She demonstrates symmetrical hyperkeratotic skin changes, verrucous plaques on her nose cheeks, ears, chin, knees, elbows, and heels. Electron microscopic studies of her skin did not reveal qualitative changes, her moderate to severe hearing impairment is of cochlear origin, moderately progressive, and particularly affecting the high frequencies. Up to now a vascularizing keratitis could not be detected. Her family history is not contributory. Twenty-eight similar cases from the literature, mainly reported as 'KID' syndrome, are reviewed. There are two familial instances. Autosomal dominant inheritance is assumed. We consider the acronymic designation 'KID' syndrome misleading, since the main features of the disorder are a progressive erythrokeratodermia, cochlear deafness, and non-obligatory vascularizing keratitis.

Child, Preschool↗

[Experiences with smell and taste studies in 798 patients].

We present our experiences of olfactometry and gustometry on 798 patients. The results show the necessity of combining tests for both taste and smell. They are essential components of the complete neuro-otological examination and should remain in the otorhinolaryngologist's repertoire.

Ageusia↗

[Changes of the voice in the Lombard reflex].

In a noisy background the vocal intensity increases (Lombard reflex). The same effect is produced by binaural masking. We demonstrate the regulation of the vocal intensity in the beginning of phonation in eight normal subjects by Hilbert transformation via an FFT analyser. The signals were recorded under normal conditions and with binaural masking. The results are discussed.

Adult↗

[Vestibular apparatus and hereditary hearing impairment].

Vestibular function tests in 33 cases of hereditary monosymptomatic symmetric sensorineural hearing loss do not show any significantly reduced excitability, but a mild difference between the groups with and without progression. A positive correlation is seen with the age of the children.

Child↗

Ataxia-deafness-retardation syndrome in three sisters.

Three sisters aged 16, 12 and 8 years from a consanguineous family presented with a progressive spinocerebellar ataxia combined with moderate mental retardation, progressive sensorineural hearing loss and signs of both upper and lower motor neuron disease. The patients represent the only known cases of the ataxia-deafness-retardation syndrome (McKusick #20885) except for the three brothers in the original description by Berman et al (1973). In the family described here transmission of the disease appears to be linked with occurrence of red hair colour.

Adolescent↗

[Mediastinal involvement in cancers of the subglottis, hypopharynx and cervical esophagus].

In carcinoma of subglottis, hypopharynx, and cervical oesophagus there is a high risk of mediastinal involvement by continuous infiltration of lymphogeneous metastatic spread because of tight neighbourhood. In progress of the disease we saw mediastinal involvement in 46.2% of subglottic carcinoma (n = 26), and in 44.1% of hypopharyngeal carcinoma (n = 79). Stomal recurrence after laryngectomy or tumour invasion of the oesophagus appeared to signify regularly mediastinal involvement. We recommend high attentiveness to the caudal borderline resection, to aspire complete oesophagectomy in cases of oesophageal cancer, and to include mediastinum into postoperative radiation area.

Esophageal Neoplasms↗

[Cochlear implantation in the child?].

Cochlear implants in early childhood may be recommended at this age which is critical for speech and language development. Implantation cannot yet be accepted in this group because of diagnostic difficulties, special rehabilitation problems and the frequency of middle ear infections with the possible risk of bacterial invasion of the cochlea. As children grow older, insufficient motivation may reduce the effects of rehabilitation procedures. A good cooperation with educators must be guaranteed. A further problem of cochlea implantation in children is posed by pre-operative counselling complicated by ethical and legal considerations. Cochlear implantation in children requires improved diagnostic tools to establish the etiology, objective testing of residual hearing and quantifications of nerve survival in addition to special rehabilitation programs.

Auditory Threshold↗

Hearing loss in facioscapulohumeral dystrophy.

Bilateral sloping high frequency hearing loss of 20-90 dB was found in six out of ten patients with infantile or adolescent onset FSHD. In all cases the basic defect could be traced to the cochlea. The outer hair cells of the basal turn are predominantly affected. In 20 patients with various other forms of muscular dystrophy or neuromuscular disorders with an FSH distribution, no sensorineural hearing loss was found. Myopathology of FSHD patients extended from mild to severe, often showing inflammatory infiltrates and type I fibre atrophy, without unequivocal differences between the two groups with and without hearing loss. It is concluded that cochlear dysfunction is a specific and frequent phenomenon of early onset FSHD.

Adolescent↗

[Quality of ructus voice following pharyngolaryngoesophagectomy].

The quality of voice production after pharyngolaryngoesophagectomy is examined in comparison with the oesophageal speech by speech tests and speech intelligibility tests. The functional effects of the specific anatomic conditions are discussed in consideration of radiological findings.

Carcinoma, Squamous Cell↗

[Phoniatric aspects in the rehabilitation of patients with cochlear implants].

The method employed in conventional rehabilitation programmes after cochlear implantation is described in detail. A cochlear implant is expected to provide a better acoustic speech discrimination by recognition of prosodic speech elements, such as rhythm, stress, intonation and rudimentary discrimination of phonemes. Furthermore we need to know about the influence of the new auditory control on speech and voice of the deaf. These changes are regularly documented by logopaedic examinations and phonetically adjusted tests. The indication of cochlear implantation in prelingually deaf and in children is discussed.

Cochlear Implants↗

[Perilymph fistulas--symptoms and indications for tympanoscopy].

In the last two years thirteen cases of perilymph fistula were treated by tympanoscopy and covering with connective tissue. Recovery of hearing was not observed in 4 totally deaf ears, but improvement was recorded in each of 9 cases with impaired hearing. Vertigo disappeared in 10 of 11 cases without any correlation with the degree of hearing loss. We perform tympanoscopy in suspected perilymph fistula, because prognosis is uncertain, spontaneous recovery with remaining defects may induce ménièriform syndrome, and pathway of infection should be closed. The diagnosis of a perilymph fistula must be solidified in cases of sudden deafness with vertigo, sudden hearing loss changing to the worse during medical treatment, sudden hearing loss without recovery during medical treatment, sudden hearing loss and subsequent onset of vertigo.

Adult↗

[Hearing loss in childhood combined with abnormalities].

Hearing loss was diagnosed in 923 of 1712 children attending our paedaudiology outpatient department over a period of two years. 14% of patients with hearing loss had one or more associated malformations, affecting the musculoskeletal system, the heart, the eye and central nervous system. Metabolic disorders were also found. In addition to specific otologic procedures such as surgery and hearing aids a close cooperation with several specialists, in particular human geneticists, is necessary.

Abnormalities, Multiple↗

[Multiple tumors in the upper aerodigestive tract--a retrospective study].

The authors observe an increasing incidence of multiple primary neoplasms in the upper aerodigestive tract. Out of 1231 patients with neoplasms, 110 (= 8.9%) developed another tumour. Following oral cavity tumours the incidence of second tumours was twice as high as in other localities. Privileged site of second tumours following cancer of the larynx is the lung, after oral cavity neoplasms the hypopharynx and larynx. The mean interval between first and secondary neoplasms was 4.4 years. In tumour follow-up therapy it is recommended to look not only for relapses and metastases but also for second tumours.

Germany, West↗