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PubMed2000

[The mechanisms of hearing and hearing loss].

The ear is constituted of an apparatus for transmission, the external ear and the middle ear, and of an apparatus for perception, the inner ear or cochlea and the acoustic nerves. A disorder can occur at each of these levels, leading to different types of hearing loss: defective transmission through a disorder of the external canal and/or by disorder of the tympanic-middle ear bone apparatus; endocochlear hearing loss through disorder of the ciliary cells of the Corti ganglion and/or by metabolic disorder or by pressure modification (such as Meniere's disease) of the endolymph compartment of the inner ear; retrocochlear hearing loss through disorders of the 8th nerve; central hearing loss through disorder of the temporal cortex.

Auditory Perception↗
PubMed1981

The summating potential and Meniere's disease. I. Summating potential amplitude in Meniere and non-Meniere ears.

We investigated the relationship between Meniere's disease and the abnormally enlarged summating potential (SP) in a series of patients and normal subjects. Cochlear and auditory nerve responses to rectangular-pulse clicks, delivered at a rate of 8/s and an intensity of 115 dB peak-equivalent sound pressure level, were recorded from ear canal skin. We performed a parabolic regression for SP amplitudes as a function of auditory nerve action potential (AP) amplitudes in normal ears and obtained the 95% confidence interval (+/- 2 SD) for normal scatter around the fitted curve. Sixty-eight percent of SP amplitudes from ears with Meniere's disease exceeded the upper normal confidence limit. In contrast, only 7% of the cochlear ears and none of the retrocochlear ears had SP amplitudes above this normal upper limit. Cochlear microphonic potentials and APs from the ears with Meniere's disease also tended to be larger than normal, but this tendency was not strong enough to be of diagnostic use.

Action Potentials↗
PubMedDate not supplied

Evoked oto-acoustic emissions from adults and infants: clinical applications.

Evoked oto-acoustic emissions (EOAEs), i.e. sounds emitted by the cochlea in response to a click, are recordable in the ear canal by a miniature microphone. They reflect an active cochlear biomechanism based upon OHC function, which accounts for the exquisite properties of sensitivity and frequency selectivity of the cochlea. EOAE recordings can therefore be considered an objective, easy, rapid and non-invasive audiological procedure which can be used to investigate the most sensitive part of the cochlea. From our experience of more than 300 recordings, we propose several clinical applications of EOAEs: 1) objective assessment of sensorineural hearing loss; 2) staging Meniere's disease by recording glycerol-induced changes; 3) diagnosis of retrocochlear pathology, and 4) screening of auditory function in infants.

Acoustic Stimulation↗
PubMed2002

The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease.

BACKGROUND: Gadolinium-enhanced magnetic resonance imaging (GdMRI) is routinely used in the evaluation and management of suspected retrocochlear pathology such as vestibular schwannoma. However, its value in the evaluation and diagnosis of cochlear pathology associated with sensorineural hearing loss (SNHL) has been less clear. STUDY DESIGN: Retrospective review of case histories and imaging studies of patients with SNHL and cochlear enhancement on GdMRI diagnosed between 1998 and 2000. RESULTS: Five patients with SNHL who required gadolinium administration to establish the diagnosis of endocochlear disease were identified. Diagnosed lesions included an intralabyrinthine schwannoma, intracochlear hemorrhage, radiation-induced ischemic change, autoimmune labyrinthitis, and meningogenic labyrinthitis. In these illustrative cases, the GdMRI demonstrated intrinsic high signal or contrast enhancement within the cochlea and labyrinth in the absence of a retrocochlear mass. In one patient with meningogenic labyrinthitis, cochlear enhancement on MRI led to prompt cochlear implantation before the potential development of cochlear ossification. CONCLUSION: Our experience suggests that GdMRI plays a crucial role in the diagnosis of cochlear pathology associated with sensorineural hearing loss and may directly impact patient management.

Adult↗
PubMed2005

Treatment of vertigo.

Vertigo is the illusion of motion, usually rotational motion. As patients age, vertigo becomes an increasingly common presenting complaint. The most common causes of this condition are benign paroxysmal positional vertigo, acute vestibular neuronitis or labyrinthitis, Ménière's disease, migraine, and anxiety disorders. Less common causes include vertebrobasilar ischemia and retrocochlear tumors. The distinction between peripheral and central vertigo usually can be made clinically and guides management decisions. Most patients with vertigo do not require extensive diagnostic testing and can be treated in the primary care setting. Benign paroxysmal positional vertigo usually improves with a canalith repositioning procedure. Acute vestibular neuronitis or labyrinthitis improves with initial stabilizing measures and a vestibular suppressant medication, followed by vestibular rehabilitation exercises. Meniere's disease often responds to the combination of a low-salt diet and diuretics. Vertiginous migraine headaches generally improve with dietary changes, a tricyclic antidepressant, and a beta blocker or calcium channel blocker. Vertigo associated with anxiety usually responds to a selective serotonin reuptake inhibitor.

Diagnosis, Differential↗
PubMed2001

[Evaluation of sensorineural hearing loss in childhood].

OBJECTIVE: To explore the clinical and audiological characteristics of sensorineural hearing loss (SHL) with pathological changes both in cochlea and retrocochlear in children and evaluate the relationship between SHL and the lesions in the central nervous system (CNS). METHODS: Three hundred and ten cases (500 ears) of SHL accepted between 1998 and 2000 were studied. The age of patients was ranged from 1 month to 6 years old. According to the evaluation of function of CNS by pediatric neurologist, all cases were divided into two groups: SHL with CNS disease and SHL without CNS disease. Some same age children without hearing loss were subjected as control group. All children were tested using both auditory brainstem responses (ABR) and distortion product otoacoustic emissions (DPOAE). RESULTS: 1. The rate of SHL accompanied with CNS diseases was very high in these children. 2. Patients with kernicterus-cerebral palsy usually had serious hearing loss caused by acoustic nerve lesion at retrocochlear, but their cochlea function was injured slightly. Patients with external hydrocephalus had only slight acoustic nerve lesion at retrocochlear, and patients with other CNS diseases usually had no change for their cochlea function. 3. In the group of SHL caused by cochlea lesion, amplitudes of DPOAE decreased obviously when the threshold of wave V of ABR was up to 60 dB nHL, and amplitudes of DPOAE seriously decreased or disappeared when the threshold of wave V was up to above 70 dB nHL. CONCLUSION: The patients with SHL are usually accompanied with CNS diseases in childhood, and their hearing loss appears very difference from audiological characteristics. We suggest that it is necessary to test both ABR and DPOAE in these patients, and it is important that pediatric neurologist join in audiologist team for our clinical study.

Brain Edema↗
PubMed2005

Trends in the diagnosis and the management of Meniere's disease: results of a survey.

OBJECTIVE: To determine the practices of the American Neurotology Society (ANS) membership in the evaluation and treatment of the Meniere's patient. STUDY DESIGN: Prospective. INTERVENTION: Questionnaire. MAIN OUTCOME MEASURE: Respondents' response to questions pertaining to the diagnostic and therapeutic practices in the management of Meniere's disease. RESULTS: Three hundred members of ANS were mailed a 15-item questionnaire. Two hundred three responded, for a 67.7% response rate. For the diagnosis of Meniere's disease, 1 in 3 practitioners relied solely on history, physical exam, and audiometry, whereas 2 in 3 relied in part on adjunctive tests, such as electrocochleography (ECOG) and electronystagmography (ENG). Two in 3 practitioners pursued retrocochlear studies on initial evaluation, with the overwhelming majority using MRI. In treating Meniere's disease, conservative medical management was preferred. For medically recalcitrant Meniere's disease, endolymphatic sac surgery (ESS) was the most commonly employed initial intervention (50%), followed by transtympanic gentamicin (38%). Currently, <10% routinely recommend the Meniett device. Eighty-three percent include ESS as a therapeutic option for medically recalcitrant Meniere's disease. The vast majority continue to perform surgical labyrinthectomies and vestibular nerve sections for Meniere's disease. CONCLUSIONS: Meniere's disease continues to pose a difficult diagnostic and therapeutic problem, resulting in heterogeneous approaches to both evaluation and treatment. Despite the 1995 American Academy of Otolaryngology guidelines in the diagnosis of Meniere's disease, most clinicians rely in part on ENG or ECOG in diagnosing Meniere's disease. Furthermore, despite the passing of 20 years since the publications claiming a purely placebo effect, ESS is the most commonly employed initial surgical treatment for Meniere's disease.

Audiometry, Evoked Response↗
PubMedDate not supplied

Clonal expansion of T-cell receptor beta gene segment in the retrocochlear lesions of EAE mice.

It has been reported that the T cell receptor V beta 8.2 (TcrbV8.2) gene segment is predominantly expressed in encephalomyelitic T cells responding to myelin basic protein (MBP) in experimental allergic encephalomyelitis (EAE) mice. We have demonstrated retrocochlear hearing loss in EAE mice in previous studies. Administration of a monoclonal antibody specific to the T cell receptor V beta 8 (TcrbV8) subfamily prevented both this type of hearing loss and the central nerve disease. In this study, we examined the role of the TcrbV8.2 gene segment in the retrocochlear lesions of EAE mice. A clonal expression of T cell receptor beta chain gene segment (TcrbV8.2-TcrbD2-TcrbJ2.7) was identified in the retrocochlear lesions. The TcrbV8.2 gene segment appears to recombine only with TcrbJ2.1 (32.1%) and TcrbJ2.7 (67.9%) gene segments. The TcrbJ2.7 gene segment has also been previously identified as the dominant TcrbJ gene in the lymph nodes of EAE mice. Only TcrbD2, with a length of 4 amino acids, was observed recombining with these TcrbV8.2 sequences. G and C nucleotides are predominantly expressed at the N regions between the V-D and D-J junctions. This dominant TcrbV gene segment (TcrbV8.2-TcrbD2-TcrbJ2.7) observed in the retrocochlear lesions has been identified in the MBP-specific T cells from the lymph nodes of EAE mice. These results suggest that a small subset of antigen-specific T cells migrate to, and expand at, the retrocochlear lesions, which leads to hearing loss.

Amino Acid Sequence↗
PubMedDate not supplied

Sensorineural hearing loss in rheumatoid arthritis.

Hearing function was tested in 20 patients affected by rheumatoid arthritis. Audiological examination was performed by pure tone audiometry thresholds, tympanometry, a stapedial reflex threshold test and auditory brainstem responses (ABR). Hearing impairment was observed in 55% of patients. Conductive hearing loss and the absence of stapedius reflex were never recorded. Five patients with abnormal audiograms had normal ABR and normal stapedial reflex thresholds while 6 patients showed abnormalities in their audiograms, stapedial reflex thresholds test and ABR. Abnormal audiometric results associated with normal ABR are compatible with cochlea involvement, while abnormal audiometric results associated with an altered ABR and stapedial reflex test may be due to retrocochlear involvement. Sensorineural hearing loss appeared to significantly correlate with active disease and with the presence of rheumatoid factor.

Adolescent↗
PubMedDate not supplied

Cochlear and retrocochlear immune-mediated inner ear disorders. Pathogenetic mechanisms and diagnostic tools.

Three different forms of immune-mediated sensorineural hearing loss are described. The pathogeneses of these three cases with severe audiovestibular deficits are completely different. To make an appropriate diagnosis remains a dilemma. Autoimmunity plays a certain role, but is not always present. Vascular and neural tissue can become involved. Immune-mediated forms of sensorineural hearing loss can be of cochlear or retrocochlear origin.

Adolescent↗
PubMed1996

[Dysfunction of medial olivocochlear system and its audiological test].

The function of medial olivocochlear system (MOC) in patients with three kinds of auditory diseases was tested in order to investigate the relationship between MOC and retrocochlear impairment. Suppressive effect of evoked otoacoustic emissions (OAEs) by contralateral white noise was used as the indicator to evaluate the function of MOC in patients with: (1) sensorineural deafness with definite otoacoustic emissions, (2) hyperacusis and (3) tinnitus. Dysfunction of MOC was found in nine cases out of sixteen patients with sensorineural deafness and six cases out of fifteen patients with tinnitus. Two cases of hyperacusis also showed MOC dysfunction. It was concluded that MOC dysfunction may exist in some patients with sensorineural hearing loss and tinnitus, and in patients with hyperacusis as well. It seemed likely that dysfunction of MOC may played a role in causing tinnitus and hyperacusis in these patients. Testing the function of MOC may provide a helpful way for differential diagnosis of cochlear and retrocochlear impairment in auditory system.

Adolescent↗
PubMedDate not supplied

Adamantiades-Behçet's disease with inner ear involvement.

Adamantiades-Behçet's disease is a chronic recurrent inflammatory disorder involving the small and large vessels. Typical loci of manifestations are the mucous membranes, skin and eyes, as well as the joints and central nervous system. Other organs are not commonly involved. We present two patients, one with ocular and the other with mucocutaneous manifestation of Adamantiades-Behçet's disease. In addition, the first patient reported three episodes of sudden hearing loss while under immunosuppressive therapy for his eye involvement. The second, therapy-naive patient complained of tinnitus in his left ear. Careful examination revealed vestibular involvement in the first patient and retrocochlear involvement in the second. Inner ear involvement is an uncommon manifestation of Adamantiades-Behçet's disease. In case of relevant signs or history, such as hearing disturbance, tinnitus and/or vertigo, patients should be examined for inner ear involvement.

Adult↗
PubMed1989

Sudden sensorineural hearing loss as a presentation of multiple sclerosis.

While auditory involvement in multiple sclerosis is not infrequent, sensorineural hearing loss as the initial manifestation of this disease is rare. Auditory brain-stem evoked responses, speech audiometry, acoustic reflexes, and electronystagmography yield significant objective information concerning brain-stem involvement. Magnetic resonance imaging objectively documents white matter demyelinization. The acute onset of the retrocochlear type of auditory dysfunction without other evidence of central nervous system involvement can be an early manifestation of this disease. Two patients with multiple sclerosis had sudden sensorineural hearing loss, with one patient experiencing this as the sole initial complaint.

Acute Disease↗
PubMed1975

Acoustic reflex and reflex decay. Occurrence in patients with cochlear and eighth nerve lesions.

Acoustic reflex and reflex decay tests were administered to 50 normal ears, 50 ears with hearing loss due to noise trauma, 50 ears that had Meniere disease, and 28 ears that had involvement of the eighth nerve. In one normal ear, ten noise trauma ears, 11 Meniere disease ears, and 24 eighth nerve lesion ears to reflexes or reflex decay that were suggestive or retrocochlear lesions were observed. Acoustic reflex and reflex decay results are also compared to tone decay results for these patients.

Acoustic Stimulation↗
PubMed1991

[Neuro-otological study on Behçet disease].

It seems acceptable that pathological brain stem lesions exist in the patient with Neuro-Behçet disease (NB). However, only few reports have been published in this field. We report the neuro-otological findings in 33 patients who fulfilled the diagnostic criteria of Behçet disease completely or incompletely. The patients were classified into 2 groups: one is group of NB and the other is a group of Behçet disease without NB (non-NB). Furthermore, the latter was divided into 2 subgroups of patients with dysequilibrium and patients with normal equilibrium. They underwent equilibrium function tests, such as body balance test, gaze test, spontaneous and positional nystagmus test, eye tracking test (ETT) and optokinetic pattern test (OKP) by using electro-nystagmography (ENG). The following results were obtained. 1. Cerebellar and brain stem lesions were confirmed in all cases of NB group. 2. Most cases (6 of 8 cases) in the subgroup of dysequilibrium of the non-NB group showed peripheral vestibular disturbance. The other two patients were diagnosed to be vertebro-basilar insufficiency (VBI) and vascular Behçet disease with CNS disorder, respectively. 3. Nine of 13 patients in the normal equilibrium subgroup of the non-NB group were diagnosed to be neuro-otologically normal. However, we found two patients with peripheral vestibular disturbance and CNS disorder associated with hearing disturbance of the retrocochlear origin, respectively. 4. The neuro-otological examination is assumed to be useful to diagnose neuro-Behçet disease, to reveal the lesion site and the state of dysequilibrium in patients with Behçet disease.

Adolescent↗