Familial early-onset progressive vestibulopathy without hearing impairment.

Abstract:

:A family with early-onset vestibulopathy is presented. The 34-year-old father had experienced brief attacks of vertigo and currently suffers from unsteadiness and oscillopsia during head movements. His two young sons also experience brief attacks of spontaneous vertigo. In vestibular testing all three subjects showed reduced caloric responses. However, only the father showed reduced otolith function (as reflected by the vestibular-evoked myogenic potentials). Further, all three subjects had walked before the age of 1 year and none of them had had any auditory symptoms. It is suggested that they have familial early-onset progressive vestibulopathy affecting the canals before the otoliths but sparing cochlear function.

journal_name

Acta Otolaryngol

journal_title

Acta oto-laryngologica

authors

Brantberg K

doi

10.1080/00016480310002500

subject

Has Abstract

pub_date

2003-08-01 00:00:00

pages

713-7

issue

6

eissn

0001-6489

issn

1651-2251

journal_volume

123

pub_type

杂志文章
  • A new concept of vertex ERA and EEG analysis applying inverse filtering.

    abstract::Evidence for a common anatomical and physiological substrate for the generation of the background EEG and the vertex-evoked response led to the hypothesis that the EEG can be interpreted as the output from a time varying filter driven by (a) a noise generator responsible for the background activity, and (b) a pulse ge...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016487709128834

    authors: Salomon G,Barfod J

    更新日期:1977-01-01 00:00:00

  • Cochlear pathology of the circling mouse: a new mouse model of DFNB6.

    abstract:CONCLUSION:The circling mouse (cir/cir) has phenotypes which follow the pattern of neuroepithelial defects of deafness from 10 days after birth. The cir mouse is defective in Tmie gene, the function of which should be further elucidated. OBJECTIVES:We previously reported a recessive mutation of deafness called circlin...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016480600827071

    authors: Chung WH,Kim KR,Cho YS,Cho DY,Woo JH,Ryoo ZY,Cho KI,Hong SH

    更新日期:2007-03-01 00:00:00

  • CD73 expression in lymph node metastases in patients with head and neck cancer.

    abstract:INTRODUCTION:CD73 is an enzyme crucial in the metabolism of immunosuppressive adenosine. In cancer, it has various functions including tumor growth and metastases. Squamous cell carcinoma of the head and neck has an overall poor prognosis, also due to early spread of metastatic cells. MATERIALS AND METHODS:Tumor and l...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016489.2017.1378436

    authors: Mandapathil M,Boduc M,Netzer C,Güldner C,Roessler M,Wallicek-Dworschak U,Jahns E,Stuck B

    更新日期:2018-02-01 00:00:00

  • Early detection of hearing impairment in patients with diabetes mellitus with otoacoustic emission. A systematic review and meta-analysis.

    abstract:CONCLUSIONS:The distortion product otoacoustic emission (DPOAE) amplitudes in diabetics were significantly lower than those in controls when pure tone audiometry (PTA) was within normal limits, implying cochlear function impairment in the early stage of diabetes mellitus. Retrocochlear damage was suggested by elongated...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章,meta分析,评审

    doi:10.1080/00016489.2016.1223344

    authors: Hao J,Fu X,Zhang C,Zhang X,Zhao S,Li Y

    更新日期:2017-02-01 00:00:00

  • Reduction of acoustically-induced auditory impairment by inhalation of carbogen gas. I. Permanent noise-induced cochlear damage.

    abstract::The possible beneficial effects of carbogen gas (95% O2/5% CO2) inhalation on noise-induced permanent cochlear impairment were investigated. Groups of guinea pigs were exposed to daily 3 1/2-hour presentations of 120 dB of broad-band noise in an atmosphere of either carbogen gas or normal air. Other groups received ca...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016488209130889

    authors: Brown JJ,Vernon JA,Fenwick JA

    更新日期:1982-05-01 00:00:00

  • The vestibular blood flow during CO2-breathing in rabbits.

    abstract::The microsphere method was used to measure the inner ear blood flow in albino rabbits before and during artificial ventilation with 5% CO2 in air. An increase in blood flow resulted in both the cochlear and the vestibular parts of the inner ear. ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016488709134692

    authors: Larsen HC,Angelborg C

    更新日期:1987-01-01 00:00:00

  • Epiglottitis and necrotizing fasciitis: a life-threatening complication of infectious mononucleosis.

    abstract::Life-threatening cervical complications associated with infectious mononucleosis are rare. The combination of acute epiglottitis and subsequent necrotizing fasciitis of the head and neck in a patient with infectious mononucleosis has not been reported to date.A 47-year-old female with infectious mononucleosis and epig...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016480510027475

    authors: Caballero M,Sabater F,Traserra J,Alòs L,Bernal-Sprekelsen M

    更新日期:2005-10-01 00:00:00

  • SISI test and adaptation. II. Subjects with conductive hearing defects.

    abstract::The effect of adaptation of the ear on the SISI test was studied at 500 Hz and 2000 Hz in 43 patients with chronic otitis and in 20 patients with otosclerosis. The mean adaptation was slightly over 10 dB, except in the otosclerosis group at 2000 Hz, for which the value was nearly 20 dB after continuous stimulation at ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016487509121302

    authors: Rahko T

    更新日期:1975-07-01 00:00:00

  • Recovery period of sinonasal quality of life and its associated factors after endoscopic endonasal approach for anterior skull base tumors.

    abstract:BACKGROUND:Although endoscopic endonasal approach (EEA) for skull base tumors showed a good prognosis in sinonasal quality of life (QOL), what factors have influence on QOL is still in question. Aim/Objectives: To investigate the recovery of sinonasal QOL after EEA for anterior skull base tumors and find its prognostic...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016489.2019.1574982

    authors: Ahn JC,Cho SW,Kim DK,Han DH,Kim DY,Rhee CS,Lee CH,Kim YH,Paek SH,Won TB

    更新日期:2019-05-01 00:00:00

  • Subclinical deviation of the subjective visual vertical in patients affected by a primary headache.

    abstract:CONCLUSION:Our results suggest that patients with migraine or tension-type headache have subclinical deviations of the subjective visual vertical, which may be associated with their subjective imbalance. OBJECTIVES:Patients affected by migraine or tension-type headache often complain of unsteadiness. However, they rar...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016480802032785

    authors: Asai M,Aoki M,Hayashi H,Yamada N,Mizuta K,Ito Y

    更新日期:2009-01-01 00:00:00

  • Extracellular ATP-induced Ca2+ mobilization of type I spiral ganglion cells from the guinea pig cochlea.

    abstract::Intracellular calcium concentrations ([Ca2+]i) in type I cochlear spiral ganglion cells (SGCs) of the guinea pig were measured by digital imaging microscopy and the Ca(2+)-sensitive dye Fura-2. Extracellular ATP induced elevation of [Ca2+]i in type I SGCs in a concentration-dependent manner. The ATP-induced elevation ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489709113435

    authors: Cho H,Harada N,Yamashita T

    更新日期:1997-07-01 00:00:00

  • Up-regulated expression of Dicer reveals poor prognosis in laryngeal squamous cell carcinoma.

    abstract:CONCLUSIONS:Increased expression of Dicer may be a prognostic biomarker for patients with laryngeal squamous cell carcinoma (LSCC). OBJECTIVES:Recent studies have shown that many microRNAs (miRNAs) play an important role in the development and progression of human cancers. Dicer, one of the most important enzymes of t...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489.2014.920962

    authors: Gao C,Li X,Tong B,Wu K,Liu Y,Anniko M,Duan M

    更新日期:2014-09-01 00:00:00

  • The totally implantable middle ear device 'Esteem' for rehabilitation of severe sensorineural hearing loss.

    abstract:CONCLUSION:The Esteem(®) device proved to offer beneficial results in subjects suffering from high frequency, severe bilateral sensorineural hearing loss (SNHL) and may be considered as an alternative procedure to conventional hearing aids (HAs) or electroacoustic stimulation (EAS) systems. OBJECTIVES:Since the Esteem...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489.2010.536994

    authors: Barbara M,Biagini M,Monini S

    更新日期:2011-04-01 00:00:00

  • Diclofenac (Voltaren). Pain-relieving effect after tonsillectomy.

    abstract::The efficacy of diclofenac suppositories was estimated in a two-centre, double-blind, placebo-controlled study comprising 97 patients (47 in the diclofenac group and 50 in the placebo group). The series from the two centres and patients in the two treatment groups were comparable. Immediately postoperatively, the pati...

    journal_title:Acta oto-laryngologica

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.3109/00016488409107553

    authors: Dommerby H,Rasmussen OR

    更新日期:1984-07-01 00:00:00

  • Automated image analysis of proliferating cells in carcinoma of the larynx.

    abstract:OBJECTIVE:To determine the role of cell proliferation neoplastic progression in the larynx and possibly derive criteria of clinical significance using automated quantitative image analysis. MATERIAL AND METHODS:In a retrospective study involving archival material, the occurrence and location, size, shape and staining ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/0001648021000028135

    authors: Laitakari J,Harrison D,Stenbäck F

    更新日期:2003-08-01 00:00:00

  • Middle ear transmission losses caused by tympanic membrane perforations in cats.

    abstract::Losses (determined by measuring microphonic responses) were essentially identical, regardless of whether produced by admittance of sound into the middle ear via perforations in the tympanic membrane (TM) or in the bulla. With SPLs monitored directly at the TM, losses increased at 12 dB/oct with inverse frequency for f...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016487609119970

    authors: Tonndorf J,McArdle F,Kruger B

    更新日期:1976-03-01 00:00:00

  • Immunohistochemical analysis of EGFR and hyaluronan in tongue cancer and the development of regional recurrence in patients initially diagnosed N0.

    abstract:OBJECTIVE:To investigate whether the extent of expression of hyaluronan (HA) and epidermal growth factor receptor (EGFR) in squamous cell carcinoma of the mobile tongue can predict the risk of cervical metastasis and survival. STUDY DESIGN:Retrospective histopathologic study. METHODS:Surgical specimens from 64 patien...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016489.2017.1292049

    authors: Lindell Jonsson E,Nylander K,Hallén L,Laurell G

    更新日期:2017-08-01 00:00:00

  • Development of otorhinological care of the child.

    abstract::During the last third of the 20th century, pediatric otolaryngology became a defined specialty in many nations, resulting in focused training, fellowships, societies, journals, textbooks, etc. This development occurred as a result of an interaction between the changing sociological and economic status of the child and...

    journal_title:Acta oto-laryngologica

    pub_type: 历史文章,杂志文章

    doi:10.1080/00016480310000575

    authors: Ruben RJ

    更新日期:2004-05-01 00:00:00

  • Microdrill stapedotomy for otosclerosis with small and large preoperative air-bone gap: a retrospective comparison of results.

    abstract::Background: In otosclerosis mixed hearing loss is the most frequent symptom and arises when the focus involves the stapes footplate. Surgeons usually prefer to wait a minimum air-bone gap of 25 - 35 dB before surgery.Objectives: To evaluate the outcome of microdrill stapedotomy for otosclerosis in patients with a preo...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016489.2020.1764618

    authors: Canale A,Albera A,Macocco F,Caranzano F,Albera R

    更新日期:2020-09-01 00:00:00

  • Gene silencing with siRNA targeting E6/E7 as a therapeutic intervention against head and neck cancer-containing HPV16 cell lines.

    abstract:CONCLUSION:Our results indicate that siRNA E6 and/or E7 may have potential as a gene-specific therapy for human papillomavirus (HPV) type 16 (HPV16)-related squamous cell carcinoma of the head and neck (HNSCC). OBJECTIVES:To evaluate the effectiveness of siRNA targeting E6 and/or E7 on the in vitro and in vivo growth ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489.2013.773405

    authors: Adhim Z,Otsuki N,Kitamoto J,Morishita N,Kawabata M,Shirakawa T,Nibu K

    更新日期:2013-07-01 00:00:00

  • Interleukin 2 receptors on squamous cell carcinomas of the head and neck. Characterization and functional role.

    abstract::IL2Rs, in the presence of IL2, mediate activation and proliferation of human normal lymphocytes and modulate functional changes of some human leukocytic malignancies. IL2Rs have been demonstrated also on human neoplastic and fetal non-hematologic cells in vitro, although their functional role has not been described. W...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.1080/00016489.1992.11665436

    authors: Cortesina G,Sacchi M,Galeazzi E,Johnson JT,Whiteside TL

    更新日期:1992-01-01 00:00:00

  • Patients' perceptions of vocal problems: results from a survey in the Tokyo area.

    abstract::Laryngoscopy in cases of sulcus vocalis reveals bowed vocal folds, resulting in a spindle-shaped chink with glottal incompetence. The anatomic and functional problems and resulting incomplete glottal closure during phonation lead to the presenting symptoms of breathy hoarseness, decrease in maximum phonation time (MPT...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章,评审

    doi:10.3109/00016489.2014.999873

    authors: Tsunoda K,Fujimaki Y,Nito T,Yamasoba T,Tayama N,Makiyama K,Murofushi T,Tsunoda A,Ohshima K,Nishiyama K,Kano S,Niimi S

    更新日期:2015-06-01 00:00:00

  • Auditory plasticity in cochlear implant patients.

    abstract::Dynamic range (DR) of cochlear implant electrodes and speech recognition scores (SRS) were chronologically studied in 23 post-lingually deaf subjects over a period of one year. DR significantly increased over one year (ANOVA, p = 0.03). When DR was compared among three groups of electrodes which were located in the in...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489609137829

    authors: Kubo T,Iwaki T,Ohkusa M,Doi K,Uno A,Yamamoto K,Fujii K

    更新日期:1996-03-01 00:00:00

  • The effect of vasopressin upon the cochlear potentials in the guinea pig.

    abstract::The change in the EP, CM, SP and AP during perilymphatic perfusion of vasopressin (antidiuretic hormone) was examined in the guinea pig. The EP was recorded with a microelectrode through the spiral ligament of the second turn. The CM, SP and AP were measured with the differential electrodes in the basal turn. The perf...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016488609132830

    authors: Mori N,Ohta K,Matsunaga T

    更新日期:1986-03-01 00:00:00

  • Analysis of curves Na-24 efflux from membrane labyrinth containing stria vascularis.

    abstract::The efflux of Na-24 was investigated in isolated labyrinths from a membrane section containing stria vascularis and spiral ligament in artificial endolymph medium. The efflux curves were calculated and compartmental analyses were made. The theoretical possibility of a exchange of sodium between compartments connected ...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016487509121308

    authors: Mnich Z

    更新日期:1975-07-01 00:00:00

  • Effect of chewing gum on static posturography in patients with balance disorders.

    abstract:CONCLUSION:The chewing gum indirectly affects postural control by influencing vestibular function to stabilize posture during upright standing. OBJECTIVES:This study aimed to evaluate the effect of chewing gum on static posturography in patients. METHODS:The subjects were 26 patients with chronic balance disorders. T...

    journal_title:Acta oto-laryngologica

    pub_type: 临床试验,杂志文章

    doi:10.3109/00016489.2011.607846

    authors: Goto F,Kushiro K,Tsutsumi T

    更新日期:2011-11-01 00:00:00

  • MASPIN tumour-suppressing activity in head and neck squamous cell carcinoma: emerging evidence and therapeutic perspectives.

    abstract:CONCLUSIONS:A large series confirmation may suggest elective neck dissection in cN0 MASPIN-negative oral squamous cell carcinomas (SCCs). Re-activated nuclear MASPIN in association with anti-angiogenic or cytotoxic drugs may be extremely effective in the treatment of laryngeal SCC. OBJECTIVES:MASPIN is a serpin showin...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章,评审

    doi:10.1080/00016480802256079

    authors: Marioni G,Staffieri C,Staffieri A,De Filippis C,Blandamura S

    更新日期:2009-05-01 00:00:00

  • The alteration of ultrastructure and immunoreactivity of human embryonic organ of Corti tissue culture after exposure to aminoglycoside (neomycin) ototoxicity.

    abstract::Using electron microscopy and GABA immunohistochemistry we evaluated the effects on human embyronic organ of Corti tissue culture of exposure to the ototoxic aminoglycoside antibiotic neomycin at a dose of 1 mM for 48 to 96 hrs. Neomycin induces the formation of multilamellar myeloid structures. These lesions, found o...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489609137929

    authors: Mu MY,Pao X,Jie T,Yu YJ

    更新日期:1996-11-01 00:00:00

  • Inner ear injury caused by air intrusion to the scala vestibuli of the cochlea.

    abstract::In a previous communication, we demonstrated that the introduction of air into the scala tympani of the cochlea causes a decrease of cochlear potentials; however, the change in endocochlear dc potential (EP) was mild and the decreased cochlear microphonics (CM) and compound action potentials (CAP) were, at least parti...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016489309135892

    authors: Kobayashi T,Sakurada T,Ohyama K,Takasaka M

    更新日期:1993-11-01 00:00:00

  • Prognostic factors for squamous cell carcinoma of the temporal bone: extensive bone involvement or extensive soft tissue involvement?

    abstract:CONCLUSION:Extensive bone erosion correlated with a worse prognosis of the squamous cell carcinoma (SCC) of the temporal bone but extensive soft tissue involvement did not correlate with prognosis in this study. Resectability of the tumor seems to be major prognostic factor of temporal bone SCC. OBJECTIVE:Prognostic f...

    journal_title:Acta oto-laryngologica

    pub_type: 杂志文章

    doi:10.3109/00016480802642096

    authors: Ito M,Hatano M,Yoshizaki T

    更新日期:2009-11-01 00:00:00