Gap prepulse inhibition of acoustic startle (GPIAS) method has been used effectively for the objective assessment of tinnitus in animals. Among two types of enclosures for the GPIAS, the unconstrained type carries less risk of animal death due to the absence of binding stress in the enclosure, and lack of need for alteration to animal size variation as it grows. However, animals' voluntary movements, which have no relation to the startles evoked by acoustic stimuli, are problematic, as they cannot be excluded in the case of the unconstrained enclosure based GPIAS measurement system. In order to discount voluntary movements which are not associated with external acoustic stimuli, we propose the conditional random interstimulus interval (CR ISI) method for unconstrained enclosure based GPIAS measurement. With the proposed ISI method, the unconstrained enclosure based acoustic startle response measurement system has been implemented in this paper. As a result, the effectiveness of the proposed CR ISI method has been verified and compared with those of conventional ISI methods through animal experiments using SD-rats. The experimental results showed that abnormal startle responses and invalid GPIAS values caused by motion were prevented when our proposed CR ISI method was applied to our implemented system. It was also verified that our proposed CR ISI method is advantageous in reducing the total experimental time for acquiring normal startle responses and valid GPIAS values, compared to conventional ISI methods, since our proposed CR ISI can begin the acoustic stimulation only when the animal gets stable and motionless.
Acoustic Stimulation ; Acoustics ; Animal Experimentation ; Animals ; Integrin alpha2 ; Methods ; Prepulse Inhibition ; Reflex, Startle ; Tinnitus

BACKGROUND AND OBJECTIVES: The suppression head impulse (SHIMP) test has emerged as one of the new vestibular function tests. The SHIMP test is an analysis of anti-compensatory saccadic movements in gazing moving targets as the head moves. In this study, we investigated the accuracy and sensitivity of the test. SUBJECTS AND METHOD: We analyzed the results of SHIMP test (gain, amplitude, and latency) in 24 cases, which included normal, vestibular neuritis, benign paroxysmal positional vertigo (BPPV), Meniere's disease, bilateral vestibular function loss, temporal bone fracture, tuberculosis meningitis and acoustic schwannoma patients. We also carried out a physical exam, audiologic study, video electronystagmography (vENG) and a conventional video head impulse test to evaluate the feasibility of SHIMP test in cases of vesitibulopathy. RESULTS: In patients with vestibulopathy, the analysis of SHIMP test indicated high sensitivity and accuracy, which were supported by accompanying audio-vestibular studies. Patients suspected with BPPV, vestibular neuronitis and Meniere's disease showed low vestibulo ocular reflex (VOR) gain and delayed anticompensatory saccade with smaller and more scattered amplitudes than the control. Patients with bilateral vestibular function loss, temporal bone fracture, tuberculosis meningitis, and acoustic schwannoma showed delayed latency, and small amplitude or few anticompensatory saccades. Finally, changes in the SHIMP test values might be a hint that patients had recovered from vestibular neuronitis and tuberculosis meningitis. CONCLUSION: We found the feasibility of SHIMP test in detecting the pathologic condition of VOR in vestibulopathy patients. We argue that the usefulness of SHIMP test might be extended to evaluating the effectiveness of rehabilitation.
Benign Paroxysmal Positional Vertigo ; Electronystagmography ; Head Impulse Test ; Head ; Humans ; Meniere Disease ; Methods ; Neuroma, Acoustic ; Reflex, Vestibulo-Ocular ; Rehabilitation ; Saccades ; Temporal Bone ; Tuberculosis, Meningeal ; Vestibular Function Tests ; Vestibular Neuronitis

BACKGROUND AND OBJECTIVES: Although ethnicity effect on wideband absorbance (WBA) findings was evident for adults, its effect on neonates has not been established yet. This study aimed to investigate the influence of ethnicity on WBA measured at 0 daPa from neonates with healthy middle ear functions. SUBJECTS AND METHODS: Participants were 99 normal, healthy, full-term newborn babies with chronological age between 11 and 128 hours of age (mean=46.73, standard deviation=26.36). A cross-sectional study design was used to measure WBA at 16 one-third octave frequency points from 99 neonates comprising of three ethnic groups: Malays (n=58), Chinese (n=13) and Indians (n=28). A total of 165 ears (83.3%) that passed a battery of tests involving distortion product otoacoustic emissions, 1 kHz tympanometry and acoustic stapedial reflex were further tested using WBA. Moreover, body size measurements were recorded from each participant. RESULTS: The Malays and Indians neonates showed almost identical WBA response across the frequency range while the Chinese babies showed lower absorbance values between 1.25 kHz and 5 kHz. However, the differences observed in WBA between the three ethnic groups were not statistically significant (p=0.23). Additionally, there were no statistically significant difference in birth weight, height and head circumference among the three ethnic groups. CONCLUSIONS: This study showed that Malays, Chinese and Indians neonates were not significantly different in their WBA responses. In conclusion, to apply for the ethnic-specific norms is not warranted when testing neonates from population constitute of these three ethnicities.
Acoustic Impedance Tests ; Acoustics ; Adult ; Asian Continental Ancestry Group ; Birth Weight ; Body Size ; Cross-Sectional Studies ; Ear ; Ear, Middle ; Ethnic Groups ; Head ; Humans ; Infant, Newborn ; Malaysia ; Reflex

BACKGROUND AND OBJECTIVES: To analyse the audiometric profile and the pedigree of a large family with otosclerosis to understand the inheritance pattern and its implication in clinical management of the disease. SUBJECTS AND METHODS: Pedigree analysis was performed on the basis of family history and audiometric tests. Pure tone audiometry, tympanometry, and acoustic reflexes were evaluated for the family members. Audiometric analysis was also carried out for the individuals who have already underwent corrective surgery at the time of study. RESULTS: Out of 112 family members, 17 were affected individuals, and 11 of them were surgically confirmed. Hearing loss (HL) started unilaterally and progressed to bilateral form. Otosclerosis was presented in early 20’s in the first and second generations but it was delayed to mid-late 30’s in the fourth generation. An affected female was diagnosed with otosclerosis during her pregnancy. Though the disease was familial, a mother of four affected offspring in this family did not develop otosclerosis until she died at the age of 84. CONCLUSIONS: The five-generation family, which was analysed in the present study, exhibited autosomal dominant inheritance of otosclerosis with reduced penetrance. Bilateral HL and pregnancy-aggravated otosclerosis were observed in this family. It was found for the first time that the age of onset of the disease delayed in the successive generations. The current study indicated the importance of detailed pedigree analysis for better clinical management of otosclerosis.
Acoustic Impedance Tests ; Age of Onset ; Audiometry ; Family Characteristics ; Female ; Hearing Loss ; Hearing Loss, Conductive ; Humans ; Inheritance Patterns ; Mothers ; Otosclerosis ; Pedigree ; Penetrance ; Pregnancy ; Reflex, Acoustic ; Wills

OBJECTIVES: Baso-apical gradients exist in various cochlear structures including medial olivocochlear (MOC) efferent system. This study investigated the cochlear regional differentials in the function and morphology of the MOC system, and addressed the functional implications of regional MOC efferent terminals (ETs) in the mouse cochlea. METHODS: In CBA/J mice, MOC reflex (MOCR) was assessed based on the distortion product otoacoustic emission in the absence and presence of contralateral acoustic stimulation. High, middle, and low frequencies were grouped according to a mouse place-frequency map. Cochlear whole mounts were immunostained for ETs with anti-α-synuclein and examined using confocal laser scanning microscopy. The diameters of ETs and the number of ETs per outer hair cell were measured from the z-stack images of the basal, middle and apical regions, respectively. RESULTS: The middle cochlear region expressed large, clustered MOC ETs with strong MOCR, the base expressed small, less clustered ETs with strong MOCR, and the apex expressed large, but less clustered ETs with weak MOCR. CONCLUSION: The mouse cochlea demonstrated regional differentials in the function and morphology of the MOC system. Strong MOCR along with superior MOC morphology in the middle region may contribute to ‘signal detection in noise,’ the primary efferent function, in the best hearing frequencies. Strong MOCR in spite of inferior MOC morphology in the base may reflect the importance of ‘protection from noise trauma’ in the high frequencies.
Acoustic Stimulation ; Animals ; Cochlea* ; Hair ; Hearing ; Mice* ; Microscopy, Confocal ; Noise ; Reflex

BACKGROUND AND PURPOSE: Tumors involving the cerebellopontine angle (CPA) pose a diagnostic challenge due to their diverse manifestations. Head impulse tests (HITs) have been used to evaluate vestibular function, but few studies have explored the head impulse gain of the vestibulo-ocular reflex (VOR) in patients with a vestibular schwannoma. This study tested whether the head impulse gain of the VOR is an indicator of the size of a unilateral CPA tumor. METHODS: Twenty-eight patients (21 women; age=64+/-12 years, mean+/-SD) with a unilateral CPA tumor underwent a recording of the HITs using a magnetic search coil technique. Patients were classified into non-compressing (T1-T3) and compressing (T4) groups according to the Hannover classification. RESULTS: Most (23/28, 82%) of the patients showed abnormal HITs for the semicircular canals on the lesion side. The bilateral abnormality in HITs was more common in the compressing group than the non-compressing group (80% vs. 8%, Pearson's chi-square test: p<0.001). The tumor size was inversely correlated with the head impulse gain of the VOR in either direction. CONCLUSIONS: Bilaterally abnormal HITs indicate that a patient has a large unilateral CPA tumor. The abnormal HITs in the contralesional direction may be explained either by adaptation or by compression and resultant dysfunction of the cerebellar and brainstem structures. The serial evaluation of HITs may provide information on tumor growth, and thereby reduce the number of costly brain scans required when following up patients with CPA tumors.
Brain ; Brain Stem ; Cerebellopontine Angle* ; Classification ; Female ; Head Impulse Test* ; Head* ; Humans ; Neuroma, Acoustic* ; Reflex, Vestibulo-Ocular ; Semicircular Canals ; Vertigo

Biotinidase deficiency is a disorder inherited autosomal recessively showing evidence of hearing loss and optic atrophy in addition to seizures, hypotonia, and ataxia. In the present study, a 2-year-old boy with Biotinidase deficiency is presented in which clinical symptoms have been reported with auditory neuropathy/auditory dyssynchrony (AN/AD). In this case, transient-evoked otoacoustic emissions showed bilaterally normal responses representing normal function of outer hair cells. In contrast, acoustic reflex test showed absent reflexes bilaterally, and visual reinforcement audiometry and auditory brainstem responses indicated severe to profound hearing loss in both ears. These results suggest AN/AD in patients with Biotinidase deficiency.
Ataxia ; Audiometry ; Biotinidase Deficiency* ; Biotinidase* ; Child, Preschool ; Ear ; Evoked Potentials, Auditory, Brain Stem ; Hair ; Hearing Loss ; Humans ; Male ; Muscle Hypotonia ; Optic Atrophy ; Reflex, Abnormal ; Reflex, Acoustic ; Seizures

OBJECTIVES: The aim of this study was to investigate hearing and outer cells function in patients with psoriatic arthritis. Our investigation was a prospective case control study. METHODS: A total of 31 psoriatic arthritis patients (62 ears) and 31 healthy control subjects (62 ears) were enrolled in the study. We investigated hearing changes of patients and controls via pure tone audiometry, speech discrimination scores, tympanometry, acoustic reflex, and transient product otoacoustic emission. RESULTS: The mean age of psoriatic arthritis patients was 36.1+/-8.5 years (range, 14 to 62 years). The average age of the control group was 37.9+/-8.1 years (range, 16 to 62 years). There were statistically significant differences between pure tone audiometry in all frequencies and right and left emission at the 4.0 and 1.0 in psoriatic arthritis patients versus controls (P<0.05). This difference was evident, especially at high frequencies. There was no statistically significant difference between the ages and genders of the patient and control groups (P>0.05). Both audiological and otoacoustic emissions were not significantly different between right and left ear (P>0.05). CONCLUSION: Based on the audiological and otoacoustic findings; it is likely that the cochlear outer hair cells become subtly damaged in psoriatic arthritis patients, consequently leading to changes in hearing thresholds. These data suggest that it is important to screen psoriatic arthritis patients for hearing changes with otoacoustic emissions and audiologic tests regularly.
Acoustic Impedance Tests ; Arthritis, Psoriatic* ; Audiometry ; Audiometry, Pure-Tone ; Audiometry, Speech ; Case-Control Studies ; Cochlea* ; Discrimination (Psychology) ; Ear ; Hair* ; Hearing* ; Humans ; Otoacoustic Emissions, Spontaneous ; Prospective Studies ; Reflex, Acoustic

Tinnitus is a common auditory phenomenon associated with many otological diseases, and is usually subjective. Objective tinnitus can be generated by para-auditory structures, usually derived from vascular or myogenic sources, or the eustachian tube. We present a rare case of intermittent unilateral tinnitus associated with eye blinking. Otoendoscopic examination showed that the external auditory canals and tympanic membranes were normal; however, rhythmic movements of both tympanic membranes, concomitant with the tinnitus, were evident whenever the patient blinked. The tympanometry and stapedial reflexes measured via impedance audiometry exhibited saw-tooth patterns; movement of the tympanic membrane was associated with eyelid blinking. The patient was managed conservatively, with reassurance and medication, and the condition became well-controlled. Here, we present this educational case and review the literature.
Acoustic Impedance Tests ; Blinking* ; Ear Canal ; Ear Diseases ; Eustachian Tube ; Eyelids ; Humans ; Reflex ; Tinnitus* ; Tympanic Membrane

Mass and stiffness affect on the peculiar characteristics of transmission of the middle ear and the distinctive behavior of the cochlear mechanics. Applying the principle of the mass and stiffness, the band-pass characteristic transfer function of the middle ear has been explained. The greatest transfer function of the middle ear, approximately 24-29 dB, is observed at 1-2 kHz in both cat and human species. However, at lower frequencies, the transfer function was disturbed by the stiffness of the middle ear primarily due to middle ear cavity. At higher frequencies, the transfer function was disturbed by the stiffness of the middle ear primarily due to middle ear bones. Several examples, such as an acoustic reflex, otitis media, and otosclerosis are discussed. For understanding the traveling wave of the basilar membrane, different place tuning at certain stimulus frequencies, contrastingly shaped basilar membrane to the cochlear duct, and the structural and physical characteristics of the whole cochlear partition were reviewed in terms of changing width, mass, and stiffness from the base to apex. Being about ten times wider, more massive, and one hundredfold stiffer at the base than the apex, the nature of the cochlear partition to absorb high-frequency energy changes in fluid pressure declines toward the apex. Consequently, at the base of the cochlea, high frequencies stimuli are decoded while low frequencies stimuli are decoded at the apex of the cochlea. Due to these characteristics of the cochlear partition, the direction of the traveling wave was also proved to be in the fashion of base-to-apex always.
Animals ; Basilar Membrane ; Cats ; Cochlea ; Cochlear Duct ; Ear, Middle* ; Humans ; Mechanics ; Otitis Media ; Otosclerosis ; Reflex, Acoustic