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1.
Audiol Res ; 11(1): 22-30, 2021 Jan 21.
Article in English | MEDLINE | ID: mdl-33494464

ABSTRACT

The purpose of the study was to examine the subjective and objective potential advantage for speech understanding in noise achieved by cochlear implant (CI) recipients when using the autosensitivity control (ASC) input signal processing in combination with the adaptive dynamic range optimization (ADRO). Eighteen subjects (8 females, 10 males, mean age 17.7 ± 6.7) were enrolled in a prospective open blinded comparative study between the ASC + ADRO condition vs. the ADRO alone; 16 were sequential binaural and 2 were monoaural CI recipients. All patients had been wearing their CI for at least 3 years, had no additional disabilities, had an age-appropriate receptive and expressive language. Word recognition performances in noise (at signal-to-noise ratio +5 dB HL) were significantly better in the ADRO-alone condition than in the ADRO + ASC condition. (p = 0.03) These objective outcomes were in agreement with the subjective reports. No significant difference was found in quiet. Our results, apparently in contrast with other reports in the literature, suggest that the decision of adding the slow-acting automatic reduction in microphone sensitivity provided by ASC should be limited to selected CI recipients.

2.
Am J Med Genet A ; 182(10): 2345-2358, 2020 10.
Article in English | MEDLINE | ID: mdl-32820599

ABSTRACT

Alport syndrome (AS) is caused by mutations in collagen IV, which is widespread in the basement membranes of many organs, including the kidneys, eyes, and ears. Whereas the effects of collagen IV changes in the cochlea are well known, no changes have been described in the posterior labyrinth. The aim of this study was to investigate both the auditory and the vestibular function of a group of individuals with AS. Seventeen patients, aged 9-52, underwent audiological tests including pure-tone and speech audiometry, immittance test and otoacoustic emissions and vestibular tests including video head impulse test, rotatory test, and vestibular evoked myogenic potentials. Hearing loss affected 25% of the males and 27.3% of the females with X-linked AS. It was sensorineural with a cochlear localization and a variable severity. 50% of the males and 45.4% of the females had a hearing impairment in the high-frequency range. Otoacoustic emissions were absent in about one-third of the individuals. A peripheral vestibular dysfunction was present in 75% of the males and 45.4% of the females, with no complaints of vertigo or dizziness. The vestibular impairment was compensated and the vestibulo-ocular reflex asymmetry was more evident in rotatory tests carried out at lower than higher speeds; a vestibular hypofunction was present in all hearing impaired ears although it was also found in subjects with normal hearing. A posterior labyrinth injury should be hypothesized in AS even when the patient does not manifest hearing disorders or evident signs of renal failure.


Subject(s)
Audiometry, Pure-Tone , Cochlea/physiopathology , Hearing Loss/genetics , Nephritis, Hereditary/genetics , Adolescent , Adult , Child , Female , Head Impulse Test , Hearing Loss/diagnostic imaging , Hearing Loss/physiopathology , Humans , Male , Middle Aged , Nephritis, Hereditary/diagnosis , Nephritis, Hereditary/physiopathology , Otoacoustic Emissions, Spontaneous/physiology , Vestibule, Labyrinth/diagnostic imaging , Vestibule, Labyrinth/physiopathology , Young Adult
3.
Ann Otol Rhinol Laryngol ; 126(4): 322-327, 2017 Apr.
Article in English | MEDLINE | ID: mdl-28290230

ABSTRACT

OBJECTIVE: To investigate the prevalence of otological complications derived from primary ciliary dyskinesia (PCD) in adulthood. METHODS: Twenty-three patients with diagnosed PCD underwent medical history aimed at recording the presence of ear, nose, and throat manifestations (ENT) and any surgical treatments. The ENT objectivity was annotated, and then patients were subjected to audiometric test, tympanometry, registration of otoacoustic emission, and vestibular evaluation. RESULTS: Otitis media with chronic middle ear effusion (OME) during childhood was reported in 52% of the subjects, no patient had undergone ear surgery, and only 2 patients had an episode of otitis in the last year. Eleven of 23 patients showed normal hearing, 11 had a conductive hearing impairment, and 1 showed a severe sensorineural hearing loss unrelated to the syndrome. The bilateral stapedial reflex was only found in all cases of normoacusia and type A tympanogram, distortion product otoacoustic emissions (DPOAE) were present in 8 patients, and no patient had vestibular alterations. CONCLUSION: Our study confirms a very frequent prevalence of OME in PCD during childhood. Careful monitoring of otological complications of the syndrome is always desirable, also given the high presence in adults of other manifestations in the upper airways, such as chronic rhinosinusitis and nasal polyposis.


Subject(s)
Hearing Loss, Conductive/epidemiology , Kartagener Syndrome/epidemiology , Myringosclerosis/epidemiology , Otitis Media with Effusion/epidemiology , Acoustic Impedance Tests , Adult , Audiometry, Pure-Tone , Chronic Disease , Female , Hearing Loss, Conductive/physiopathology , Humans , Kartagener Syndrome/physiopathology , Male , Middle Aged , Otoacoustic Emissions, Spontaneous/physiology , Prevalence , Reflex/physiology , Stapedius/physiopathology
4.
Int J Pediatr Otorhinolaryngol ; 78(11): 1861-5, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25193583

ABSTRACT

OBJECTIVE: To assess if young subjects affected by Williams syndrome (WS) with normal middle ear functionality and normal hearing thresholds might have subtle auditory dysfunctions that could be detected by using clinically available measurements. METHODS: Otoscopy, acoustic reflexes, tympanometry, pure-tone audiometry, and distortion product otoacoustic emissions (DPOAEs) were measured in a group of 13 WS subjects and in 13 age-matched, typically developing control subjects. Participants were required to have normal otoscopy, A-type tympanogram, normal acoustic reflex thresholds, and pure-tone thresholds≤15 dB HL at 0.5, 1, and 2 kHz bilaterally. To limit the possible influence of middle ear status on DPOAE recordings, we analyzed only data from ears with pure-tone thresholds≤15 dB HL across all octave frequencies in the range 0.25-8 kHz, middle ear pressure (MEP)>-50 daPa, static compliance (SC) in the range 0.3-1.2 cm3, and ear canal volume (ECV) in the range 0.2-2 ml, and we performed analysis of covariance to remove the possible effects of middle ear variables on DPOAEs. RESULTS: No differences in mean hearing thresholds, SC, ECV, and gradient were observed between the two groups, whereas significantly lower MEP values were found in WS subjects as well as significantly decreased DPOAEs up to 3.2 kHz after adjusting for differences in middle ear status. CONCLUSIONS: Results revealed that WS subjects with normal hearing thresholds (≤15 dB HL) and normal middle ear functionality (MEP>-50 daPa, SC in the range 0.3-1.2 cm3, ECV in the range 0.2-2 ml) might have subtle auditory dysfunctions that can be detected by using clinically available methods. Overall, this study points out the importance of using otoacoustic emissions as a complement to routine audiological examinations in individuals with WS to detect, before the onset of hearing loss, possible subtle auditory dysfunctions so that patients can be early identified, better monitored, and promptly treated.


Subject(s)
Williams Syndrome/physiopathology , Acoustic Impedance Tests , Adolescent , Adult , Audiometry, Pure-Tone , Auditory Threshold/physiology , Case-Control Studies , Child , Ear, Middle/physiology , Female , Humans , Male , Otoacoustic Emissions, Spontaneous/physiology , Otoscopy , Pressure , Reflex, Acoustic/physiology , Young Adult
5.
Eur Arch Otorhinolaryngol ; 271(7): 2069-77, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24557440

ABSTRACT

Although many studies have been carried out regarding postural stability during pediatric age, reliable information and a complete analysis of all age groups are still lacking. The purpose of this study was to verify the test-retest reliability of posturographic parameters in four sensory conditions and provide normative values for children and young adolescents. 289 subjects, aged 6-14 years, were assessed by means of the static posturography system SVeP. 173 elementary school pupils (114 males and 59 females, aged 6-10 years; mean age 8.80 ± 1.53) and 116 middle school students (60 males and 56 females, aged 11-14 years; mean age 12.6 ± 0.9) underwent static posturography in two consecutive trials with four testing conditions: eyes open and eyes closed with and without foam pads. The participants were divided into nine age groups. Thirty healthy young adults were also recruited for comparison. The analysis of test-retest reliability demonstrated an excellent reliability of velocity measurement and a moderate reliability of area measurement. Velocity and area decreased significantly with age in all sensory conditions, indicating an improvement in postural control from childhood to adolescence. Postural stability had not reached the adult level by the age of 13-14 years. Reliable information regarding postural stability can be obtained in children and young adolescents by means of stabilometric parameters. These data can be used as a reference for early detection of atypical postural development and for the assessment of dizziness and balance disorders in children.


Subject(s)
Postural Balance/physiology , Adolescent , Age Factors , Body Height , Body Weight , Case-Control Studies , Child , Female , Humans , Male , Reference Values , Reproducibility of Results , Vision, Ocular/physiology , Young Adult
6.
Eur J Med Genet ; 56(9): 490-6, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23886711

ABSTRACT

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in most patients.


Subject(s)
Hearing Loss/physiopathology , Sound , Williams Syndrome/physiopathology , Adolescent , Audiology , Child , Child, Preschool , Female , Follow-Up Studies , Hearing Loss/diagnosis , Humans , Male , Sensory Thresholds , Williams Syndrome/diagnosis
7.
Am J Otolaryngol ; 34(4): 355-6, 2013.
Article in English | MEDLINE | ID: mdl-23374485

ABSTRACT

We report the case of a 63-year-old female with definite unilateral Ménière disease, osteoarthritis of the distal finger joints with mucous cysts and Heberden's nodes, and constipation with recurrent abdominal pain whose symptoms remitted after 6months of a restrictive gluten-free diet.


Subject(s)
Celiac Disease/complications , Diet, Gluten-Free , Meniere Disease/diet therapy , Meniere Disease/etiology , Celiac Disease/diagnosis , Celiac Disease/therapy , Female , Follow-Up Studies , Glutens/adverse effects , Glutens/immunology , Humans , Middle Aged , Risk Assessment , Treatment Outcome
8.
Gait Posture ; 38(2): 221-5, 2013 Jun.
Article in English | MEDLINE | ID: mdl-23219788

ABSTRACT

Impaired control of balance has been described in Williams syndrome (WS). The aim of this study was to investigate balance function by means of clinical and instrumental tests in order to measure postural sway in people with WS in an objective way. 23 WS patients (11 males, 12 females, mean age 17.52 ± 8.33 years) and 23 healthy subjects (11 males, 12 females, mean age 17.74 ± 8.93 years) performed static posturography with eyes open and closed, on a firm surface and on foam pads. The WS patients had higher mean length, velocity and surface values than controls under all of the test conditions, and their length and surface values were significantly higher in the eyes open test. The cognitive abilities of the WS patients were not related to their stabilometric performance. The greatest differences between the WS patients and the controls were found mainly in the older subjects. WS patients are more unstable than healthy subjects of the same age, particularly when they use visual information to maintain their balance: i.e. under conditions of normal everyday life. Possible explanations may be the ophthalmologic problems and the visuospatial difficulties attributed to a neural processing abnormality involving the dorsal stream impairment model. The balance function of WS patients is different from that of normal developing subjects, especially after adolescence when postural control is generally complete. This suggests an atypical developmental trajectory.


Subject(s)
Cerebellar Diseases/physiopathology , Postural Balance/physiology , Williams Syndrome/physiopathology , Adolescent , Adult , Case-Control Studies , Cerebellar Diseases/etiology , Child , Female , Humans , Male , Williams Syndrome/complications , Young Adult
9.
Am J Med Genet A ; 158A(4): 759-71, 2012 Apr.
Article in English | MEDLINE | ID: mdl-22411878

ABSTRACT

The aim of this study was to investigate, in a clinical setting, the auditory function of a group of individuals affected by Williams syndrome (WS). Sixty-nine patients with WS, aged 2-30, underwent comprehensive audiological testing including air/bone conduction behavioral audiometry, speech audiometry, tympanometry and measurement of the acoustic reflex, transient evoked otoacoustic emissions and brainstem auditory evoked responses. Hearing loss, defined by a pure-tone average above 15 dB HL, affected 22.6% of the patients studied with traditional audiometry and was mostly slight in severity. Hearing loss was conductive in 9.4% of patients, mainly children with otitis media with effusion, and sensorineural in 13.2% of patients. However, 30% of the ears studied had a hearing impairment in the high frequency range (high-frequency pure-tone audiometry above 15 dB HL), higher in participants above 15 years (46.15%) than in the younger ones (23.45%). Contralateral stapedial reflexes were present in all patients with A-type tympanograms. Transient otoacoustic emissions were absent in 44% of the ears of patients with normal hearing. Brainstem auditory evoked responses fell within normal ranges thus confirming the absence of retrocochlear dysfunction. Although hearing loss does not seem to be frequent, a cochlear fragility, especially in the high frequency range, related to outer hair cells is characteristic of WS. Therefore we strongly recommend monitoring patients affected by WS using annual audiometric tests and performing otoacoustic emissions in order to identify a subclinical cochlear dysfunction which might benefit from an audiological follow up before the possible onset of hearing loss.


Subject(s)
Hearing Loss, Conductive , Hearing Loss, High-Frequency , Hearing Loss, Sensorineural , Williams Syndrome/pathology , Williams Syndrome/physiopathology , Acoustic Impedance Tests , Adolescent , Adult , Audiometry , Audiometry, Pure-Tone , Child , Child, Preschool , Cochlea/pathology , Evoked Potentials, Auditory, Brain Stem/physiology , Female , Hearing/physiology , Humans , Male , Otoacoustic Emissions, Spontaneous/physiology , Reflex, Acoustic/physiology , Young Adult
10.
Hear Res ; 272(1-2): 157-67, 2011 Feb.
Article in English | MEDLINE | ID: mdl-20969939

ABSTRACT

The aim of this study was to investigate the functionality of cochlear active mechanisms in normal-hearing subjects affected by Williams syndrome (WS). Transient evoked otoacoustic emissions (TEOAEs) were recorded in a group of young WS subjects and a group of typically developing control subjects, all having normal-hearing thresholds and normal middle-ear functionality. We also analysed the narrow-band frequency components of TEOAEs, extracted from the broad-band TEOAE recordings by using a time-frequency analysis algorithm based on the Wavelet transform. We observed that TEOAEs and the frequency components extracted from TEOAEs measured in WS subjects had significantly lower energy compared to the controls. Also, the narrow-band frequency components of TEOAEs measured in WS subjects had slightly increased latency compared to the controls. Overall, results would suggest a subtle (i.e., sub-clinical) dysfunction of the cochlear active mechanisms in WS subjects with otherwise normal hearing. Also, results point out the relevance of using otoacoustic emissions in the audiological evaluation and monitoring of WS subjects to early identify possible subtle auditory dysfunctions, before the onset of mild or moderate hearing loss that could exacerbate language or cognitive impairments associated with WS.


Subject(s)
Cochlea/physiopathology , Hearing , Otoacoustic Emissions, Spontaneous , Williams Syndrome/physiopathology , Acoustic Impedance Tests , Acoustic Stimulation , Adolescent , Analysis of Variance , Audiometry, Pure-Tone , Auditory Threshold , Case-Control Studies , Child , Female , Humans , Male , Phenotype , Reaction Time , Signal Processing, Computer-Assisted , Sound Spectrography , Time Factors , Young Adult
11.
Int Tinnitus J ; 8(2): 111-4, 2002.
Article in English | MEDLINE | ID: mdl-14763222

ABSTRACT

In this report, we summarize our clinical experience with intratympanic dexamethasone treatment (IDT) for control of tinnitus. From March 2000 through February 2001, we observed 54 patients (23 women, 31 men; mean age, 49.6 +/- 7.2 years; range, 24-71 years) suffering from subjective idiopathic tinnitus (SIT). After common audiological tests had been performed; all patients underwent specific topodiagnostic tests to verify the cochlear SIT genesis. The 50 subjects with positive results from a furosemide test and negative results from caraverine and carbamazepine tests were selected for the IDT, consisting of transtympanic perfusion of 4 mg dexamethasone to the round window via the middle ear. The treatment was repeated three times daily for 3 consecutive months. Its short-term effects were evaluated 2 weeks after the last perfusion. In 17 of 50 of these patients (34%), the SIT disappeared; 20 of the 50 (40%) reported a significant decrease of the symptom; and the remaining 13 of the 50 (26%) did not experience any improvement. Therefore, we believe that IDT represents an effective drug delivery system for SIT control, as long as the condition arises from inner ear disorders only and treatment occurs within 3 months of symptom onset.


Subject(s)
Anti-Inflammatory Agents/therapeutic use , Dexamethasone/therapeutic use , Tinnitus/drug therapy , Adult , Aged , Anti-Inflammatory Agents/administration & dosage , Dexamethasone/administration & dosage , Drug Delivery Systems , Ear, Inner/drug effects , Ear, Middle , Female , Humans , Injections , Labyrinthine Fluids/drug effects , Male , Middle Aged , Patient Selection , Retrospective Studies , Round Window, Ear/metabolism , Treatment Outcome
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