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Biomedical subjects

E J Theunissen

Publications and source records attributed to E J Theunissen.

16 recordsLinked to original sources
PubMed2001

Hereditary cochleovestibular dysfunction due to a COCH gene mutation (DFNA9): a follow-up study of a family.

Cochleovestibular impairment was evaluated, in relation to age, in a longitudinal follow-up study on a Dutch family with a DFNA9 trait caused by a Pro51Ser mutation in the COCH gene on chromosome 14q12-q13. Fourteen cases were genotyped. The onset age of progressive impairment reported by the mutation carriers was between age 35 and 45 years. Pure-tone thresholds deteriorated by about 2-7 dB per year (mean 3.8 dB per year) in a variable, often asymmetrical, fashion. One mutation carrier developed recurrent episodes of vertigo accompanied by nausea and vomiting, resembling Ménière's disease. Two others developed special susceptibility for motion sickness and appeared to have a hyperactive vestibulo-ocular reflex. More advanced stages of vestibular impairment, i.e. vestibular hyporeflexia and complete vestibular areflexia, were eventually found in a number of cases. DFNA9/COCH should be considered as a possible cause in patients developing combined progressive cochlear and vestibular impairment, or suspected hereditary Ménière-like disease, from around middle age.

Adult↗
PubMed2000

[Screening of hearing in children up to 18 months of age].

In the Netherlands the hearing screening of young children is carried out at the age of nine months by the Ewing or 'Compact Amsterdam paedo-audiometric screener' (CAPAS) test. These screening tests do not always lead to the necessary follow-up diagnostics, mainly because the different disciplines disagree. Recently a consensus concerning the follow-up of children with a poor test result was reached by the Youth Health Care institutions, general practitioners, paediatricians, ENT physicians and audiologic centres. Each party has its own responsibilities and commits itself to complete the diagnostics before the age of 18 months. Until new screening methods have been validated it is advised to refer all children with poor screening test results for special diagnostics, if necessary after they have been treated for otitis media with effusion. Currently the most appropriate method for final diagnosis is the measurement of oto-acoustic emissions.

Audiometry, Evoked Response↗
PubMed1995

Vestibular hyperreactivity and hyperventilation after whiplash injury.

Vestibular, oculomotor and respiratory tests were performed on 32 patients after whiplash injury caused by a rear-end car collision. Oculomotor functions were generally normal. The cervico-ocular reflex was usually absent or displayed the low gain typical of normal subjects. There was no nystagmic response to static neck torsion. The vestibulo-ocular reflex showed vestibular hyperreactivity (VH) in a significantly large number of cases (n = 17; 53%). The respiratory test results were also typical of the hyperventilation syndrome (HVS) in a significantly large number of cases (n = 12; 38%). The findings of VH and the HVS were not significantly correlated within the patient group. However, the combination of VH and the HVS occurred significantly more often (n = 7; 22%) than could be accounted for by combined false positivity. Most of the significant findings were due to high relative frequencies in the women: 11 out of the 17 women (65%) showed VH, 8 (47%) had the HVS and 5 (29%) showed a combination of VH and the HVS. The findings were not correlated with the patient's age or the time interval between the accident and the examination. VH might have been the result of plastic adaptation to limited head mobility secondary to neck pain. Behavioural and emotional distress might offer alternative explanations for both VH and the HVS.

Accidents, Traffic↗
PubMed1995

Hyperactive VOR and hyperventilation after whiplash injury.

Otoneurological and respiratory tests were performed on 32 patients after whiplash injury caused by a rear-end car collision. Oculomotor and cervico-ocular test results were generally normal. In a significantly large number of case, the vestibulo-ocular reflex (VOR) was hyperactive (n = 17; 53%) and the respiratory test results were typical of the hyperventilation syndrome (HVS) (n = 12; 38%). Hyperactive VOR and the HVS occurred significantly more often in combination (n = 7; 22%) than could be accounted for by combined false positivity. Most of the significant findings were due to high relative frequencies in the women. The hyperactive VOR might have been the result of plastic adaptation to limited head mobility secondary to neck pain. Behavioural and emotional distress might offer alternative explanations for both the hyperactive VOR and HVS.

Adult↗
PubMed1994

Translabyrinthine and transotic surgery for acoustic neuroma.

The results and complications of translabyrinthine and transotic surgery for petrous apex lesions between 1980 and 1992 are presented. An acoustic neuroma was found in 52 patients. In 1988, the translabyrinthine approach was modified into the transotic approach and replaced the former technique. There was no mortality in this series, but two patients had mild brainstem infarcts and there was post-operative bleeding into the cerebellopontine angle in one. Cerebrospinal fluid (CSF) leakage was seen in six patients and meningitis in two. Three suffered deep vein thrombosis in their legs. There was one case each of herniation of the cerebellum and gastric bleeding. Post-operative facial nerve function was good in 88%, moderate in 10% and poor in 2%. In the case of acoustic neuromas the aim was total tumour removal, but if there was a serious risk of damaging the nerve anatomically, near total or subtotal removal was performed. During the study period, there was a gradual decrease in facial nerve morbidity and surgical complications. This was attributed to increasing experience, the modified wider approach and better post-operative care.

Adolescent↗
PubMed1993

The vestibulo-ocular reflex in pupils at a Dutch school for the hearing impaired; findings relating to acquired causes.

The vestibulo-ocular reflex was evaluated with caloric tests and velocity step (VS) tests in 121 pupils at a school for the hearing-impaired. Vestibular impairment was found in a total of 50 pupils (41%). It was found in 17 out of the 33 (52%) pupils with acquired causes of hearing impairment and significantly more often in pupils with a pure tone hearing threshold of more than 90 dB than in those with a lower threshold. In the group with prenatal causes, 5 out of 8 rubella cases showed vestibular impairment. Four of these cases had impaired VS responses and normal caloric responses. The high proportion of vestibular impairment in the group with postnatal causes was due to 5 cases with meningitis, all of whom showed caloric weakness. Kernicterus was significantly associated with vestibular hyperreactivity (5 cases out of 7), presumably due to a central dysfunction.

Adolescent↗
PubMed1991

Cavernous hemangioma of the internal acoustic canal.

A case report of a 39-year-old man suffering from left-sided, progressive hearing loss is presented. As well, the patient noted ipsilateral recurrent facial paralysis. MRI succeeded in confirming the presence of a tumor in the internal acoustic canal 18 months after the first MRI.

Adult↗
PubMed1989

Familial vestibulocerebellar dysfunction: a new syndrome?

Three members of a single family with the symptom of "motion sickness" showed rebound nystagmus, saccadic pursuit eye movements, defective optokinetic slow phase velocity and lack of fixation suppression of vestibularly induced nystagmus. One of them showed vestibular hyperreactivity and a gradual build-up of the optokinetic response. In absence of other abnormalities, these findings can be localized to the vestibulocerebellum (flocculo-nodular lobe).

Adult↗
PubMed1989

Hereditary vestibulo-cochlear dysfunction and vascular disorders.

A family is described with a progressive autosomal dominant vestibulocochlear dysfunction resulting in a Dandy syndrome, head movement dependent oscillopsia and hearing loss. The history was negative for other neurological or otological diseases (including infectious diseases) or use of neuro-ototoxic drugs, except for a high incidence of vascular disorders (hypertension, stroke, and heart infarction).

Adult↗
PubMed1989

Compensation of total loss of vestibulo-ocular reflex by enhanced optokinetic response.

The gain in the optokinetic nystagmus reflex (OKR) of 27 labyrinthine-defective patients was compared with that of 27 subjects without any otoneurological abnormality, who were matched by age and sex. The patients without labyrinth function exhibited significantly higher OKR gains than the control subjects, especially so at higher age. The observed enhancement of OKR gain in the absence of vestibular function is ascribed to the effect of optokinetic training brought about by enhanced slippage of retinal images due to lack of an efficient vestibulo-ocular reflex.

Adaptation, Physiological↗
PubMed1988

The velocity step test. Its use in the evaluation of the effects of drugs in dizzy patients.

The effect of flunarizine (10 mg/day) was studied in open and double-blind clinical trials. Patients presenting the symptom of dizziness were diagnosed as having the hyperventilation syndrome. They showed vestibular hyperreactivity in the velocity step test. This test was performed before and after 4-6 weeks of treatment. The results of treatment were compared with a control or placebo group. In an open trial, 12 of 14 patients reported alleviation of dizziness during treatment and showed a significant decrease in right-left asymmetry of their velocity step responses. In a double-blind trial, no significant difference in effects or side effects was found between the drug and placebo groups.

Adolescent↗
PubMed1986

Vestibular hyperreactivity and hyperventilation.

In a group of 26 patients with a hyperventilation syndrome, 77% showed vestibular hyperreactivity of velocity step responses, mostly due to an increase in gain of the vestibulo-ocular reflex (VOR), apparent from an increase in initial velocity, but also due to an increase in the time constant. Similar effects were found among 11 normal subjects after forced hyperventilation. In another group of 44 patients whose primary complaint was dizziness and who showed vestibular hyperreactivity without having any other demonstrable abnormality, a hyperventilation syndrome was found in 75%. This suggests that the velocity step test without hyperventilation provocation is a useful test for the detection of a hyperventilation syndrome.

Adolescent↗
PubMed1986

Flunarizine treatment in dizzy patients with vestibular hyperreactivity and hyperventilation.

Flunarizine (10 mg/day) was given in an open pilot study to 14 patients with the primary complaint of dizziness, who showed vestibular hyperreactivity in the velocity step (VS) test and were proven to have a hyperventilation syndrome (HVS) without any other disease. The VS test was repeated after 4-6 weeks of treatment. Twelve of these HVS patients reported alleviation of dizziness. Objective evaluation of the results of treatment was attempted by defining suitable response parameters and comparing their values to those obtained in a control group of 14 healthy volunteers tested twice with the same interval. A significant decrease in right-left asymmetry in the VS responses of the patients on drug was found.

Adolescent↗
PubMedDate not supplied

Contralateral hyperactive caloric response in unilateral labyrinthine weakness.

In a series of 600 patients, 10 showed unilateral hyperactive caloric responses without a trivial explanation such as spontaneous nystagmus or directional preponderance. All 10 had unilateral weakness on the contralateral side, which was accompanied in 9 by cochlear hearing loss. It is assumed that the phenomenon of unilateral hyperreactivity in these cases can be explained by release from commissural inhibition on the basis of temporary vestibular decompensation.

Caloric Tests↗