Stefania Barozzi

Reliability of postural control measures in children and young adolescents.

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 6–14xa0years, were assessed by means of the static posturography system SVeP. 173 elementary school pupils (114 males and 59 females, aged 6–10xa0years; mean age 8.80xa0±xa01.53) and 116 middle school students (60 males and 56 females, aged 11–14xa0years; mean age 12.6xa0±xa00.9) 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 13–14xa0years. 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.

Cochlear active mechanisms in young normal-hearing subjects affected by Williams syndrome: Time-frequency analysis of otoacoustic emissions

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.

The influence of music on static posturography

It is well known that high intensity sounds modify balance by activating the saccule, which is sensitive to both vestibular and acoustic stimuli. Few studies have examined the effects of music on the postural responses in healthy subjects. The aim of this study was to evaluate the influence of different types of music (Mozart, Köhler, Köhler with a carrier of 12 KHz and subjects favourite music) on twelve healthy subjects standing on a stabilometric platform. With each type of music, all subjects underwent static posturography with eyes opened and eyes closed, and with and without foam pads. We evaluated the length and the surface of body sway and the correlation between them, and we analyzed the visual, vestibular and somatosensory sub-components. Listening to different types of music did not significantly change the stabilometric variables, with the exception of listening to Mozarts Jupiter, which caused a significant reduction in the visual component with a consequent increase in both the vestibular and somatosensory inputs. Further studies are needed to determine the effect of Mozarts music in modifying the sensory strategy in the rehabilitation of patients with vestibular impairments.

Audiological findings in Williams syndrome: A study of 69 patients

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 15u2009dB 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 15u2009dB 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.

Prevalence and Voice Characteristics of Laryngeal Pathology in an Italian Voice Therapy-seeking Population.

OBJECTIVESnThe aim of this study was to determine the prevalence and clinical characteristics of voice disorders in a large group of patients seeking voice therapy.nnnSTUDY DESIGNnThis is a prospective prevalence study.nnnMETHODSnA total of 821 patients were enrolled. Each patient was evaluated following a multidimensional protocol including videolaryngostroboscopy, perception, acoustics, aerodynamics, and self-rating by the patient. Data regarding age, gender, tobacco use, gastroesophageal reflux disease (GERD), and professional voice use were collected and analyzed.nnnRESULTSnBased on videolaryngoscopic findings, the sample group was divided into patients with functional dysphonia (nu2009=u2009155), patients with organic dysphonia (nu2009=u2009359), and patients with dysphonia due to movement disorders (nu2009=u2009307). The most frequently detected pathologies were vocal fold paralysis, muscle tension dysphonia, and vocal fold edema. Children (nu2009=u200941) and adolescents (nu2009=u200943) represented a minority of the sample group. Dysphonia was significantly more common in women. Organic dysphonia was more common in children and adolescents. GERD was suspected in 382 patients and confirmed in 83 of them; 164 patients were smokers. Professional voice users composed the large majority of the working population and were more frequently affected by organic dysphonia. Patients with dysphonia due to movement disorders presented a worse voice quality and voice-related quality of life.nnnCONCLUSIONSnIn patients seeking voice therapy, there are more females than males, children and adolescents represent a minority of the sample, professional voice users more commonly present organic dysphonia, and patients with dysphonia due to movement disorders show significantly worse voice quality.

Electromyographic activity of sternocleidomastoid and masticatory muscles in patients with vestibular lesions.

This study evaluated the electromyographic characteristics of masticatory and neck muscles in subjects with vestibular lesions. Surface electromyography of the masseter, temporalis and sternocleidomastoid muscles was performed in 19 patients with Ménières disease, 12 patients with an acute peripheral vestibular lesion, and 19 control subjects matched for sex and age. During maximum voluntary clenching, patients with peripheral vestibular lesions had the highest co-contraction of the sternocleidomastoid muscle (analysis of covariance, p=0.02), the control subjects had the smallest values, and the patients with Ménières disease had intermediate values. The control subjects had larger standardized muscle activities than the other patient groups (p=0.001). In conclusion, during maximum voluntary tooth clenching, patients with vestibular alterations have both more active neck muscles, and less active masticatory muscles than normal controls. Results underline the importance of a more inclusive craniocervical assessment of patients with vestibular lesions.

Audiological follow-up of 24 patients affected by Williams syndrome

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.

Audiological and vestibular findings in the Kabuki syndrome

Since the first description of Kabuki syndrome (KS) in 1981, over 350 cases from a variety of countries have been reported. Even though otolaryngological manifestations are common in KS, only a limited number of the reports provide audiological and vestibular data. The aim of the present study was to investigate the vestibular function and describe the audiological findings in KS. The present study reports no audiological and vestibular features in a group of 10 KS patients (7 males, 3 females), with chronological age ranging from 10 to 25 years (mean ageu2009=u200914.5): a complete otoneurological and audiological work‐up was performed for each patient and included where possible, the measurement of vestibular evoked potentials, caloric tests and static posturography. Hearing loss was found in 65% showing a mix or a conductive impairment; moreover the vestibular function was normal in 95% of the examined ears. In conclusion, audiological and vestibular examination should be considered when evaluating KS subjects.

Bilateral sudden sensorineural hearing loss and chronic venous cerebrospinal insufficiency: a case report.

Objectives: We report a case of bilateral sudden sensorineural hearing loss (SSHL) in a patient suffering from chronic venous cerebrospinal insufficiency (CCSVI). Methods: Audiometric testing confirmed bilateral sensorineural hearing loss with hypoexcitability to caloric stimulation on the left side and echo-colour Doppler examination showed abnormal cerebral venous deficiency. Results: The patients condition improved after 15 days following medical treatment. Conclusions: CCSVI may explain the anatomical background which provides a predisposing factor for SSHL although further studies are needed to verify whether this observation is casual or coincidental.

Audiovestibular disorders as autoimmune reaction in patients with melanoma

Melanoma is an aggressive form of cancer derived from neuroectodermal melanocytes. Melanocytes are present in the skin and hair follicles, as well as in the eye (iris and choroids), the leptomeninges, the anal canal and the inner ear. In the inner ear melanocytes are found both in the intermediate layer of the stria vascularis of the cochlea and in the dark cells of the vestibular organs. They are believed to play an important role in the production of endolymphatic potentials and in the maintenance of normal volumes of the inner ear fluids. Recently, audiovestibular dysfunctions have been demonstrated in patients treated with immunotherapy for metastatic melanoma and have been related to an autoimmune attack on the normal melanocytes of the inner ear. Melanoma is an immunogenic tumor type frequently associated with spontaneous autoimmune manifestations which seem to be associated with better prognosis. The melanoma-associated antigens are also expressed in normal melanocytes in the skin, eye and ear. We hypothesize that inner ear melanocytes could be a target of an autoimmune process in patients affected by melanoma. The immune system could produce antibodies that cross-react with both the melanoma cells and the labyrinth melanocytes causing an altered homeostasis of endolymphatic liquids and provoking some labyrinthic disorders such as vertigo, hearing loss, aural fullness and tinnitus resembling or influencing Ménières disease. In this perspective, audiovestibular disorders could be interpreted as an attempt by the individual immune system to develop anti-tumor response. In patients affected by melanoma an autoimmune genesis has already been advocated for ocular symptoms in melanoma-associated retinopathy, where the cross-reaction happens against retinal cells. A possible role of inner ear melanocytes should be considered as a potential cause of audiovestibular disorders. Further research is needed to demonstrate a connection between melanoma and labyrinth dysfunctions such as in melanoma-associated retinopathy.