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At least 289 records · Page 16Linked to original sources
PubMed1983

An unusual otological manifestation of Usher's syndrome in four siblings.

A family in which four out of ten siblings affected by Usher's syndrome, with progressive hearing loss, is described. Two of the siblings had hearing loss while of school age, but two others first exhibited otological symptoms in their thirties. One of the siblings had retinitis pigmentosa, diagnosed before the hearing impairment became evident. The family seems to exhibit type III Usher's syndrome with unusual manifestations.

Adult↗
PubMed1991

Neurophysiological and neuro-otological study of homozygous beta-thalassemia under long-term desferrioxamine (DFO) treatment.

This report presents data on visual evoked potentials (VEPs) and brainstem auditory evoked potentials (BAEPs), as well as neurologic, ophthalmologic and otologic assessments performed on 120 patients with beta-thalassemia major undergoing long-term DFO treatment. A total of 32 patients showed abnormal VEPs and 14 abnormal BAEPs; seven had both VEP and BAEP abnormalities; 12 had sensorineural hearing loss (SNHL); 18 had conductive hearing loss, while 14 showed a combination of SNHL and conductive hearing loss. After DFO administration was modified (taking in consideration the serum ferritin levels) patients with abnormal findings were retested. The values of 15 patients of 23 who underwent VEP examinations had been normalized. Eleven of 15 who repeated the BAEP test had also gained normal values. The audiogram had not returned to normal in any patient with SNHL. In a second repetition of the examinations, no change was observed. It is concluded that in a great percentage of thalassemics at least one of the above examinations shows abnormal values. These abnormalities are mostly reversible, and probably reflect a dysfunction of the visual or auditory system, due either to DFO neurotoxicity or to iron overload or both.

Adult↗
PubMed1997

Normative auditory brainstem response data for hearing threshold and neuro-otological diagnosis in the dog.

There is growing interest in the application of auditory brainstem response (ABR) audiometry for hearing assessment in dogs. The technique is far from standardised, however, resulting in large discrepancies between studies. This study aimed to obtain normative data, under clearly defined conditions, for two breeds of significantly different size; head size being a potential factor determining ABR latency values. The subjects, 20 dalmatians and 20 Jack Russell terriers, were sedated prior to ABR testing, and subcutaneous scalp electrodes used to detect the evoked potential elicited by a click stimulus presented via insert earphones. The mean ABR thresholds for the two breeds, 0 and -5 decibels re normal hearing level (dB nHL), respectively, were very similar to those for humans. The latency values of the main ABR waves and the interval between them were statistically significantly smaller for the smaller breed, but there was no correlation with head size within either breed. The results provide a baseline to assist with confirmation of hearing impairment and neuro-otological diagnosis in the dog.

Animals↗
PubMed1987

Testing the vestibular-ocular reflexes: abnormalities of the otolith contribution in patients with neuro-otological disease.

Conventional vestibular rotation testing with the head centered on the axis stimulates the semicircular canals evoking compensatory eye movements. If the head is placed forwards of the axis in an eccentric position the otoliths are also stimulated by a tangential linear acceleration acting laterally to the skull. In normal subjects the additional otolithic stimulus evokes compensatory eye movements with a higher gain than with head centred, particularly for high frequency (greater than 0.1 Hz) stimuli. The responses with head centred and eccentric in various patients with known/suspected neuro-otological abnormalities have been compared. Patients with vestibular neurinectomies who have asymmetrical head centred responses showed greater asymmetry with head eccentric at higher stimulus frequencies. Some patients with cerebellar lesions showed abnormally enhanced or depressed and asymmetrical responses with head eccentric in comparison with head centred responses, which could be normal. The enhancing effects could be specific to low frequency stimuli. All patients who showed abnormal responses with head eccentric also had positional nystagmus provoked by the gravity acceleration vector when the head was tilted laterally. The direction of the positional nystagmus with respect to the gravity vector was not necessarily the same as the direction of the effect on eye movements of lateral acceleration during eccentric oscillation. Patients with benign paroxysmal vertigo or chronic linear vertigo in whom otolithic abnormalities are suspected were not found to have abnormal responses with head eccentric. We conclude that this method of testing may be useful in elucidating pathophysiology but is not a decisive clinical test for the presence of disordered otolith function.

Acceleration↗
PubMed1992

Psychiatric morbidity in patients with peripheral vestibular disorder: a clinical and neuro-otological study.

This study reports the psychiatric morbidity in 54 patients with objective evidence of peripheral vestibular disorder seen three to five years after their original referral. A third of the patients were free from vestibular symptoms at follow up and a further third had experienced some improvement. Two thirds of the patients had experienced psychiatric symptoms during this period, although only 50% were rated above the cut off point for significant psychiatric disturbance when interviewed. Panic disorder with or without agoraphobia and major depression were the commonest psychiatric diagnoses. Patients with classical "labyrinthine" symptoms had a more severe canal paresis than the rest, but the degree of the abnormalities in the neuro-otological tests was unrelated to outcome or to psychiatric morbidity. On the other hand, there was a significant correlation between the presence of vestibular symptoms and psychiatric morbidity, which in turn correlated with measures of anxiety, perceived stress and previous psychiatric illness.

Adolescent↗
PubMed1981

Otological aspects of the earpit-deafness syndrome.

The results of a systematic study of the otological aspects in 13 cases of earpit-deafness syndrome are reported. The audiometric, radiological and vestibular findings as well as the results of exploratory tympanotomies with and without stapedectomies are discussed together with the results reported in the literature. A convincing explanation of the poor results of exploratory tympanotomies in cases with mixed hearing loss is not furnished. If the hearing loss is confined to conduction and ankylosis of the stapes or a disconnection of the ossicular chain is suspected, exploratory tympanotomy can be expected to be successful.

Acoustic Impedance Tests↗
PubMed1988

The Otorhinolaryngology Department in Helsinki: organization and otologic research.

The organization and the otologic research in the Otorhinolaryngology Department of the University of Helsinki are described. The department has 4 surgical wards, each with 22 beds, and a new surgical wing with 6 full-sized operating rooms. The ear research is centered on middle ear histopathology and immunology, focusing especially on secretory and chronic otitis media. There is an audiological and vestibular unit, the latter is expanding rapidly. Acoustic neuroma surgery is performed as team work with a neurosurgeon. The department takes part in a cochlear implant and Audiant prosthesis program.

Audiology↗
PubMed1989

Assessment of head shaking test in neuro-otological practice.

The head shaking test (HST) is an important test in neuro-otological diagnosis. In our study of 277 patients complaining of dizziness we verified this importance. The results thus obtained were compared with the results recorded in 73 normal subjects. Head shaking nystagmus was observed in 73 patients. Of these 73 cases, 42 involved central diseases and 31 cases involved peripheral diseases. Head shaking nystagmus was observed in 7 subjects of the control group. A highly significant correlation was noted between head shaking nystagmus and positional nystagmus, between head shaking nystagmus and the caloric test and between head shaking nystagmus and all the other spontaneous types of nystagmus that were investigated (eyes open in light/gaze straight ahead; eyes open behind Frenzel's glasses; eyes closed; eyes open in darkness). No correlation was found between head shaking nystagmus and cervical nystagmus and positioning nystagmus. The sensitivity of head shaking nystagmus, in comparison with other types of spontaneous nystagmus, was found to be slightly higher in cases with peripheral lesions than in those with central lesions. These data confirm the diagnostic importance of this simple test. It is also important to keep in mind that the HST has no importance for topodiagnostic purposes.

Adolescent↗
PubMedDate not supplied

Diltiazem for prevention of acoustical trauma during otologic surgery.

100 patients were tested in a prospective, randomized, double-blinded study to assess the perioperative efficacy of a calcium channel blocker (diltiazem) in preventing acoustical trauma during otologic surgery. The patients were randomly divided into a therapy group (diltiazem) and a control group (placebo). Bone conduction hearing thresholds were examined preoperatively and again 1 day and 3 months postoperatively. Frequency-dependent changes in postoperative bone conduction and the number of patients with various degrees of postoperative hearing loss in both groups were statistically analyzed. The results indicated only a small postoperative hearing loss after ear surgery in both groups. There was a tendency for better results in the therapy group, but this was not statistically significant. Despite favorable results in animal trials utilizing different types of noise and the prophylactic application of diltiazem, further studies in humans are needed to determine the role of calcium antagonist drugs in the prophylaxis or treatment of acoustical trauma.

Adult↗
PubMedDate not supplied

Clinical significance of rebound nystagmus in neuro-otological diagnosis.

The present study is concerned with a clinical analysis of 17 patients with "rebound nystagmus" examined over a period of three years. All of them have shown a short duration second degree nystagmus evoked by changes in the direction of fixation, from the lateral to straight ahead gaze. This nystagmus was a fixation nystagmus, that is to say, it was enhanced in the presence of active optic fixation and inhibited in its absence. Almost all the patients (16 out of 17) had cerebellar signs on neurological examination (in one subject rebound nystagmus was the first sign suggesting cerebellar involvement and appeared several months before any other cerebellar sign was present). Rebound nystagmus was far more common than the other neuro-otological signs suggesting cerebellar dysfunction (vestibular hyperexcitability, dysrhythmia in postcaloric nystagmus and ocular dysmetria). In three out of four patients with unilateral lesions rebound nystagmus was ipsilateral with respect to the side of the lesion. Postmortem studies were carried out upon five cases and showed either pathological changes in the cerebellum or a lesion involving the cerebellar peduncles in the brain stem.

Cerebellar Diseases↗
PubMedDate not supplied

The round window membrane: otological observations.

The membrane of the round window serves as a barrier between the middle and inner ear and has a well-established role in cochlear physiology. It is important in many otological conditions: otitis media, otosclerosis, trauma, sequelae to drugs reaching the middle ear, tumors, congenital dysplasia, sudden deafness. We here describe the first electron microscopic findings in round window membranes from adult humans, and various conditions in reference to these findings. Since surgical lesions are made in this membrane during cochlear implantation, and by some otologists in treating vertigo, preliminary findings of a lesioning experiment in animals are presented. Interestingly, early healing resulted from a mesothelial cellular reaction in the adjacent scala tympani, with formation of clots, while in the middle ear the epithelial cellular layer sealed the edges of the perforation, leading to formation of an indentation ("pocket"). Subsequently, the middle layer of the membrane contributed to healing.

Adult↗
PubMedDate not supplied

Sir Astley Cooper and his otological papers.

In 1800, the year of his election as Surgeon to Guy's Hospital (London), and the following year, Astley Cooper presented two communications to the Royal Society based on his tympanic membrane observations and his treatment of certain forms of deafness by myringotomy. For these contributions he was awarded the Copley Medal of the Royal Society. The first paper concerned itself with the effect of a perforated tympanic membrane on hearing. Contrary to the then accepted opinion, Cooper found the patient to have excellent hearing. The second paper reported on myringotomy in patients who appeared to lack sufficient atmospheric pressure within the tympanic cavity because of closure of the eustachian tube. The relevance of Cooper's observations 184 years ago is of some significance in relation to the practice of modern otology.

Deafness↗
PubMedDate not supplied

Otologic complications following temporomandibular joint arthroscopy.

The recent application of arthroscopic surgical techniques to the temporomandibular joint (TMJ) has facilitated the diagnosis and treatment of TMJ disorders. However, as TMJ arthroscopy is performed more frequently, new complications are being recognized. We report three patients who developed severe otologic complications following TMJ arthroscopy. Two sustained complete or severe sensorineural hearing loss and severe vertigo from trauma to the ipsilateral ear. The third patient had complete facial paralysis from trauma to the facial nerve in the middle ear and a conductive hearing loss from incus dislocation. Complete hearing loss and facial paralysis from trauma to the main trunk of the facial nerve have not been reported previously as complications of TMJ arthroscopy.

Adult↗
PubMed1989

Otologic histopathology of Fabry's disease.

Fabry's disease is a rare progressive X-linked recessive disorder of glycosphingolipid metabolism. The accumulation of glycosphingolipids occurs in virtually all areas of the body, including the endothelial, perithelial, and smooth-muscle cells of blood vessels, the ganglion cells of the autonomic nervous system, and the glomeruli and tubules of the kidney. Although otologic symptoms have been described in these patients, to our knowledge there have been no temporal bone histopathologic reports. We describe the clinical histories, audiometric results, and temporal bone findings of two patients with this rare disorder. Both patients demonstrated a bilateral sloping sensorineural hearing loss audiometrically. Middle ear findings of seropurulent effusions and hyperplastic mucosa were seen in all four temporal bones. Strial and spiral ligament atrophy in all turns, and hair cell loss mainly in the basal turns, were also common findings. The number of spiral ganglion cells was reduced in all temporal bones; however, evidence of glycosphingolipid accumulation was not observed in the spiral ganglia.

Audiometry↗
PubMed1990

Nasopharyngeal mass: a manifestation of inflammatory otologic disease.

A nasopharyngeal mass with cranial neuropathies usually indicates an advanced neoplastic process. We present three patients with these findings and concurrent invasive Pseudomonas otitis in whom repeated nasopharyngeal biopsies were negative for tumor. All of the nasopharyngeal masses resolved following treatment of the otitis. Mechanisms of disease spread from the temporal bone to the nasopharynx are discussed. Clinicians may choose to modify diagnostic and therapeutic approaches to the nasopharyngeal mass in patients with concurrent invasive otologic disease.

Aged↗
PubMed1988

Neuro-otological findings in normal children.

The findings of a neuro-otological evaluation of 100 normal school children and 21 adults are presented. The results show changes in the vestibulo-ocular reflex with age which are of importance in the interpretation of future tests. They also give a fascinating insight into the maturation processes taking place in the growing child.

Adolescent↗
PubMedDate not supplied

Aminoglycoside antibiotics in the treatment of otologic infections.

Aminoglycoside antibiotics can be used successfully in the treatment of otologic infections caused by gram-negative bacteria. They can be particularly valuable, sometimes in combination with a beta-lactam antibiotic such as carbenicillin, in malignant external otitis, acute middle ear infections caused by gram-negative organisms, and central nervous system complications of cholesteatomas. On the basis of susceptibility tests and of the pharmacology of these drugs, we administer appropriate therapeutic doses of one of the following antibiotics: kanamycin, gentamicin, tobramycin, or amikacin. All of these drugs may be ototoxic and nephrotoxic.

Aminoglycosides↗
PubMed1986

The jugular bulb in otologic surgery: anatomic, clinical, and surgical considerations.

The jugular bulb varies widely in position and dimensions. In certain pathologic conditions there is an increased incidence of jugular bulb enlargement, but such enlargement also occurs without an obvious underlying cause. Jugular bulb enlargment may lead to clinical symptoms for the patient and particular difficulties for the otologic surgeon, not only in the middle ear but also in neurotologic approaches. The different clinical aspects and problems associated with jugular bulb enlargement are discussed with illustrative cases and temporal bone histologic findings.

Adult↗