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[Labyrinth aspects of cranial Paget's disease].

We report the case of a 44-year-old woman suffering, since 5 years, from repetitive vertigo seizures, lasting less than one hour, together with light neuro-sensorial deafness, fullness of the left ear and left ear tinnitus. After analysis of audiologic and imagery tests a Meniere's syndrome secondary to Paget disease--confirm histologically through cortical cranial biopsy--was established.

Adult↗

Inner ear degeneration in Reye's syndrome.

A 9-year-old girl developed Reye's syndrome. On admission to the hospital on the fourth day of illness, responses for verbal stimuli had been absent, but were present for painful stimuli. Coma developed on the fourth day and was present until death ten days later. The fundus of both internal meatus showed vascular congestion of nerves with evidence of repeated hemorrhages. All structures within the endolymphatic system showed advanced degeneration, with exception of the tectorial membrane and cupulae. One ear showed vascular congestion, formation of thrombi, and free hemorrhage within the modiolus and spiral ganglion. In the other ear, congested vessels in nerves in the meatus were obstructed by swelling at the cribriform area. The degenerative changes corresponded closely to those produced experimentally in guinea pigs by interruption of arterial circulation to the inner ear.

Child↗

Successful treatment of sudden hearing loss in Cogan's syndrome with corticosteroids.

Cogan's syndrome (CS) is the association of acute nonsyphilitic interstitial keratitis and acute episodes of vertigo, tinnitus, and hearing loss. We have prospectively followed 6 patients with CS who were treated within 4 weeks after the acute onset of hearing loss. Within 1 to 2 weeks after the initiation of corticosteroid therapy, all 6 patients demonstrated improved hearing thresholds for pure tones and supra-threshold speech discrimination results. These patients have been followed an average of 2.5 years (range 0.25-9 years); all have only mild to moderate hearing impairment in the mid (1,000 to 2,000 Hz) and low (250 to 500 Hz) frequency sound ranges. Three of the patients have been tapered off steroids completely with no subsequent permanent decrement of hearing. Thus, early oral corticosteroid administration to patients with sudden hearing loss associated with Cogan's syndrome may preserve auditory function.

Acute Disease↗

Peripheral-vastibular spontaneous nystagmus. Analysis of reproducibility and methoidolgies.

UNLABELLED: With peripheral spontaneous nystagmus the intensity and its reproducibility was electronystagmorgraphically analyzed with eyes open under Frenzel's glasses and with eyes closed. RESULTS: 1. The maximum intensity of a spontaneous nystagmus with eyes closed is several times higher than under Frenzel's glasses. 2. A weak nystagmus can, under Frenzel's glasses, only be recognized under favorable conditions. A rather long and careful search in a totally dark room is necessary. 3. With purely peripheral lesions, the spontaneous nystagmus with closed eyes shows large intensity fluctuations, and it may disappear temporarily despite mental arithmetics. Contrary to this, with eyes open under Frenzel's glasses, the intensity fluctuation of the nystagmus is remarkably lower. Therefore, even experimental examinations with patients should be carried out electronystagmorgraphically under Frenzel's glasses. The absolute reaction is lower, but the content of information is higher because of the significantly better reproducibility.

Adult↗

Medical problems of space flight.

Several consistent medical problems have been encountered by astronauts during space flights. These include vestibular dysfunction, weight loss, increase in height, upward fluid shift, anemia, cardiovascular deconditioning, muscle atrophy, and bone loss. Almost all of these alterations can be attributed to the absence of gravitational force. Most are adaptive in nature and therefore reversible, but readaptation after returning to earth may cause further problems (e.g., in the case of vestibular dysfunction). The most recalcitrant and disturbing of all these problems is the relentless bone loss associated with negative calcium balance. This problem appears to be irreversible, and critical demineralization can occur after two years in a weightless state. Unless its mechanism is elucidated and preventive measures are taken, the bone loss may prove to be the medically limiting factor for the duration of space flight.

Anemia↗

Long-latency rotational evoked potentials in subjects with and without bilateral vestibular loss.

Human subjects with and without profound bilateral vestibular loss were evaluated using repetitive rotational stimulation about an earth-vertical axis and signal averaging of vertex-recorded potentials. The stimulus events to which averaging was synchronized were acceleration/deceleration pulses produced by abrupt reversals in direction of angular velocity. In control subjects the long-latency rotational evoked potentials elicited via this paradigm were robust and reproducible. The responses of subjects with vestibular loss did not differ remarkably from those of the control subjects. We conclude that the long-latency rotational evoked potential elicited using a conventional rotary chair is not primarily of vestibular origin.

Adolescent↗

Allergy for the otologist. External canal to inner ear.

Allergy may affect the outer, the middle, or the inner ear. Although the otologic manifestations of allergy are not by themselves diagnostic, the history, including family history and associated symptoms in other target organs, will often help lead to the correct diagnosis and institution of therapy. Patients with significant and chronic symptoms, including those with labyrinthine symptoms of allergy, will respond well to specific immunotherapy and/or dietary elimination.

Anti-Allergic Agents↗

Evidence for a vestibulo-cardiac reflex in man.

Changes in posture demand rapid cardiovascular adjustments to maintain blood pressure and volume distribution. We demonstrated a vestibulo-cardiac reflex in supine individuals by measuring electrocardiogram and arterial blood pressure after small backwards drops of the head triggered at varying intervals after the R-spike. In normal volunteers heart rate was accelerated by drops occurring within 500-600 ms of a beat, but no rapid effect was noted in patients with a vestibular defect. The speed at which the vestibular signal of head drop accelerated heart rate implies a direct reflex. Impairment of the vestibulo-cardiac reflex would help to explain the vaso-vagal consequences of labyrinthitis.

Adult↗

Aminoglycoside ototoxicity.

The author participated in two prospective studies of patients receiving aminoglycoside antibiotics. In the first study, 54 patients received amikacin, and 54 received gentamicin. In the second study, 61 patients received gentamicin, 50 received netilmicin, and 52 received tobramycin. The studies were randomized and the investigator was blinded when evaluating auditory and vestibular toxicity and nephrotoxicity. All patients had pure tone audiometric evaluations, and two thirds of the patients had vestibular function tests consisting of an electronystagmogram performed with 30 degrees C and 44 degrees C water. Nephrotoxicity was measured by changes in serum creatinine levels. The incidence of gentamicin toxicity in the first study was 11 per cent, and it was 18 per cent in the second study. Amikacin was ototoxic 12.9 per cent of the time, whereas the incidence of tobramycin ototoxicity was 11.5 per cent, and the incidence of netilmicin ototoxicity was 2 per cent. Cases of unilateral, delayed-onset, and reversible auditory and vestibular toxicities were seen in all drug treatment groups. Nephrotoxicity was rare with amikacin usage. Gentamicin, on the other hand, produced a 18.6 per cent, tobramycin a 25 per cent, and netilmicin a 21.3 per cent rate of nephrotoxicity.

Amikacin↗

Vestibular-evoked myogenic potentials in patients with dehiscence of the superior semicircular canal.

Recently Minor and co-workers described patients with sound- and pressure-induced vertigo due to dehiscence of bone overlying the superior semicircular canal. Identifying patients with this "new" vestibular entity is important, not only because the symptoms can be very incapacitating, but also because they are surgically treatable. We present symptoms and findings for three such patients. On exposure to sounds, especially in the frequency range 0.5-1 kHz, they showed vertical/torsional eye movements analogous to a stimulation of the superior semicircular canal. They also showed abnormally large sound-induced vestibular-evoked myogenic potentials (VEMP), i.e. the short latency sternomastoid muscle response considered to be of saccular origin. The VEMP also had a low threshold, especially in the frequency range 0.5-1 kHz. However, in response to saccular stimulation by skull taps, i.e. when the middle ear route was bypassed, the VEMP were not enlarged. This suggests that the relation between the sound-induced and the skull tap-induced responses can differentiate a large but normal VEMP from an abnormally large response due to dehiscence of bone overlying the labyrinth, because only the latter would produce large sound-induced VEMP compared to those induced by skull taps.

Adult↗

Chronic otitis media sequelae in skeletal material from medieval Denmark.

OBJECTIVES: Chronic otitis media sequelae (COMS) have been identified in archaeological skeletal materials from various ages. COMS reflecting episodes of upper respiratory tract infection may be used as a paleopathological indicator of general health. Estimation of the frequency of COMS may be useful in the gross evaluation of general standard of living. MATERIALS AND METHODS: Temporal bones and auditory ossicles from 659 individuals from two Danish medieval rural parish cemeteries, dated to 1050-1200 and 1150-1350, respectively, were examined otomicroscopically. RESULTS: Osseous fistulae from mastoid abscesses, remodelling of the hypotympanon, and erosion of the incus were among the convincing indications of COMS. A minimum frequency of COMS of 1% to 7% was found. The youngest material displayed the highest frequency of pathological changes. CONCLUSION: Indications of a rising incidence of infectious middle ear disease in early medieval Denmark were found. This may reflect a deterioration of living conditions from the 11th through the 14th centuries.

Bone Diseases↗

Surgical outcomes in patients with endolymphatic sac tumors.

OBJECTIVE: To determine the surgical outcomes in patients with endolymphatic sac tumors (ELSTs). STUDY DESIGN: Retrospective review of patients at a referral-based otology-neurotology practice. METHODS: A review of the records from the House Ear Clinic revealed 16 patients treated for ELSTs from 1971 to 2002. This article reports the treatment outcomes for the 14 patients for whom clinical data were available. RESULTS: Sensorineural hearing loss, tinnitus, and dizziness were the most common presenting signs and symptoms. Six patients presented with facial weakness, and three patients had symptoms characteristic of Menière's syndrome. One patient suffered from Von Hippel-Lindau disease. Patients underwent microsurgical removal and were followed for an average of 59.6 months. Patients that presented with normal facial function maintained excellent postoperative function, and hearing was preserved in two patients with small tumors. Two patients suffered persistent, progressive disease despite multiple attempts at microsurgical removal and radiotherapy. Both had incomplete resections of their initial tumors. A third patient developed a small recurrent tumor that was successfully managed by a second attempt at microsurgical removal. CONCLUSIONS: Taken together with other reports, these results suggest that ELSTs are best managed by complete surgical resection. This can generally be accomplished with minimal additional morbidity.

Adolescent↗