Author's reply to the Letter to the Editor "Therapeutic strategies in the treatment of Menière's disease: the Italian experience".

There is still controversy on the role of hyperinsulinemia and endogenous antisecretory factor (EAF) in Meniere's disease. In the literature, there is no mention on glucose uptake or specially processed cereals (SPC). Nevertheless, the role of EAF and its inhibition by SPC should be taken into account in future research. In case of patients not responding to medical treatment and dietary changes, intratympanic (IT) treatment has been suggested. In Italy, generalists consider IT steroids as the first-line treatment, while neurotologists suggest IT gentamicin. We agree with the authors that low-dose IT gentamicin has a high chance to treat vertigo with minimal risks for hearing; however, a recent randomized, double-blind, comparative trial has not proven the superiority of gentamicin to IT steroids suggesting the choice "should be made based on clinical knowledge and patient circumstances". IT steroids have the advantage to preserve both cochlear and vestibular function, while IT gentamicin has an ablative effect especially on the vestibular function.

Ocular and cervical vestibular-evoked myogenic potentials in idiopathic sudden sensorineural hearing loss (ISSHL) without vertigo: VEMPs in ISSHL.

PURPOSE: Idiopathic sudden sensorineural hearing loss (ISSHL) is a hearing impairment greater than 30 dB at three consecutive frequencies developing in less than 3 days. The aim of this study was to evaluate VEMPs and caloric testing in patients affected by ISSHL without vertigo. METHODS: We retrospectively evaluated 35 subjects affected by ISSHL. The audio-vestibular investigation consisted of pure-tone and speech audiometry, impedance, bithermal caloric testing, oVEMPs and cVEMPs. RESULTS: VEMPS were abnormal in 21 patients (60%): cVEMPs in 12 (34.2%) and oVEMPs in 19 (54.2%). 10 patients (28.5%) presented with both c-and oVEMPs altered. Subjects without vestibular involvement showed a significant lower PTA in the affected ear both at admission and at discharge. Recovery rate was significantly higher in group without vestibular involvement. CONCLUSION: The evaluation of the vestibular system can be useful for predicting the auditory recovery in patients without vertigo also.

Therapeutic strategies in the treatment of Menière's disease: the Italian experience.

PURPOSE: Ménière's disease (MD) is an inner ear disorder of unknown etiology, whose pathological substrate is the endolymphatic hydrops. Different treatments have been proposed; however, evidence of their effectiveness is lacking. The aim of this study was to evaluate by a questionnaire which medical and surgical treatments are used in Italy for the treatment of MD and to compare them with those proposed in other countries. METHODS: An electronic questionnaire of 40 questions was formulated and sent to Italian otolaryngologist (ENT) divided into two groups: Group 1 ("generalists" 60.8%) and Group 2 ("neurotologist- NO" 39.2%). RESULTS: One hundred and twenty five ENT replied. Treatment of the acute phase, apart from symptomatics, was based on diuretics that are prescribed by 83.5% of respondents, steroids, prescribed by 66.7%, and vasodilators, prescribed by 22%. In the intercritical phase, 87.2% of respondents recommended low-salt diet, 78.4% of respondents prescribed betahistine, and 52.8% diuretics. Statistical analysis did not show correlation neither with the declared specialization nor with the number of patients treated. In case of failure of medical treatment, IT gentamicin was suggested by 48.8% of the respondents and IT steroids by 40.8%. Statistical analysis showed that generalists prefer IT steroids and NO IT gentamicin (p 0.019). In case of failure of both medical treatment and IT treatment, vestibular neurectomy was indicated by 58.4% of the respondents, 6.4% indicated endolymphatic sac surgery, and 2.4% surgical labyrinthectomy. CONCLUSION: In Italy, the treatment of MD stand on a gradual approach that starts from the dietary-behavioral changes and a pharmacological therapy based on betahistine. In refractory cases, IT treatment initially with steroids and, therefore, with gentamicin allows the control in vertigo in the majority of cases. In case of failure of IT treatment, VNS is the surgery of choice.

Comorbidities and recurrence of benign paroxysmal positional vertigo: personal experience.

OBJECTIVE: The aim of this study is to evaluate the correlation between clinical features of benign paroxysmal positional vertigo (BPPV) and age, sex, trauma, presence of one or more comorbidities such as cardiovascular, neurological, endocrinological, metabolic, psychiatric diseases. DESIGN: Retrospective review of medical records (chart review). STUDY SAMPLE: A total of 475 patients aged from 14 to 87 years, affected by BPPV. RESULTS: Recurrence of BPPV occurred in 139/475 patients (29.2%). The recurrence rate was significantly higher in female and older patients. Comorbidities were present in 72.6% of subjects with recurrent BPPV vs. 48.9% of patients with no recurrence (p < 0.01). Forty-two patients (8.8%) reported a cranial trauma as a triggering event. Post-traumatic patients showed a significantly higher persistence rate (45.2%) compared to patients affected by non-traumatic BPPV (20.5%). Recurrence rates are overlapping between the two groups. CONCLUSION: Our results confirm the association between recurrence of BPPV and age, female sex, and presence of comorbidities. The correlation is stronger in patients affected by multiple associated diseases; the most frequently involved pathologies are psychiatric disorders, followed by neurological and vascular diseases. Collecting a complete medical history is important for prognostic stratification and detection of potential underlying pathological conditions.

Moderate OSAS and turbinate decongestion: surgical efficacy in improving the quality of life and compliance of CPAP using Epworth score and SNOT-20 score.

SUMMARY: Drug-induced sleep endoscopy (DISE) is an important procedure in diagnostic pathway of patients affected by moderate OSAS. However, the Italian National Health System does not provide any compatible Diagnosis-related-group (DRG) code codification for DISE, which makes it impossible to obtain regional reimbursement. In order to overcome this problem, DISE is usually associated with other codified surgical procedures. The aim of our study is to assess the association of turbinate decongestion (TD) and DISE in order to combine in a single operating session diagnostic and therapeutic procedures. The objective of our work is to assess the role of nasal surgery on symptoms of moderate OSA. Recent studies have confirmed that isolated nasal surgery improves quality of life (QOL), but not the apnoea hypopnoea index (AHI) during polygraph registration. We enrolled 30 patients, aged between 29 and 64 years (mean 50.53 ± 9.20), 26 males and 4 females, with a mean BMI of 26.07 ± 2.81 kg/m2, who were affected by moderate OSAS. All patients underwent otolaryngologycal pre-operative evaluation, home respiratory polygraph and subjective evaluation through Sino-Nasal-Outcome Test (SNOT-20) and Epworth Sleepiness Scale (ESS). During the same surgery session, they underwent DISE and TD. Patients were re-evaluated six months later using the same questionnaires. We observed a significant improvement (p #x003C; 0.05) in both the mean ESS index (6.03 ± 2.75 vs 4.16 ± 4.63) and total SNOT score (22.53 ± 12.16 vs 13.23 ± 10.82). Significant differences (p #x003C; 0.05) were also identified for partial SNOT questions 1-11 (9.1 ± 5.11 vs 6.13 ± 4.12) and 11-20 (13.36 ± 10.20 vs 7.13 ± 9.644). The results of the present study confirm that TD alone can improve sleepiness, QOL and nasal symptoms. Thus, in absence of a National Health System recognition for DISE, the association of this procedure with TD can be useful for diagnostic and therapeutic management of OSAS, improving CPAP compliance and adherence, reducing sleepiness, ameliorating nasal symptoms and therefore QOL.

Correlation between musical aptitude and learning foreign languages: an epidemiological study in secondary school Italian students.

SUMMARY: The aim of this study was to assess if a correlation exists between language learning skills and musical aptitude through the analysis of scholarly outcomes concerning the study of foreign languages and music. We enrolled 502 students from a secondary Italian school (10-14 years old), attending both traditional courses (2 hours/week of music classes scheduled) and special courses (six hours). For statistical analysis, we considered grades in English, French and Music. Our results showed a significant correlation between grades in the two foreign languages and in music, both in the traditional courses and in special courses, and better results in French than for special courses. These results are discussed and interpreted through the literature about neuroanatomical and physiological mechanisms of foreign language learning and music perception.

Tensor veli palatini electromyography for monitoring Eustachian tube rehabilitation in otitis media.

BACKGROUND: The pathogenesis of otitis media is related to Eustachian tube dysfunction. The tensor veli palatini muscle actively opens the Eustachian tube and promotes middle-ear ventilation. This study describes a technique for paratubal electromyography that uses a surface, non-invasive electrode able to record tensor veli palatini muscle activity during swallowing. METHODS: Twenty otitis media patients and 10 healthy patients underwent tensor veli palatini electromyography. Activity of this muscle before and after Eustachian tube rehabilitation was also assessed. RESULTS: In 78.5 per cent of patients, the electromyography duration phase and/or amplitude were reduced in the affected side. The muscle action potential was impaired in all patients who underwent Eustachian tube rehabilitation. CONCLUSION: This study confirmed that Eustachian tube muscle dysfunction has a role in otitis media pathogenesis and showed that muscle activity increases after Eustachian tube rehabilitation therapy.

Vascular endothelial growth factor (VEGF) expression in noise-induced hearing loss.

Noise-induced hearing loss has been associated with alterations in cochlear blood flow. Our study analyzed the expression of Vascular Endothelial Growth Factor (VEGF) and its functional receptors, Flt-1 and Flk-1, in the cochlear structures of noise-exposed and unexposed guinea pigs. VEGF is a prototypical angiogenic agent, with multiple functions on vascular biology, ranging from vascular permeability to endothelial cell migration, proliferation, differentiation, and survival. Acoustic trauma was induced by a continuous pure tone of 6 kHz, at 120 dB SPL for 30 min. Auditory function was evaluated by electrocochleographic recordings at 2-20 kHz for 7 days. Noise-induced cochlear morphological changes were studied by immunohistochemistry and scanning electron microscopy. The expression of VEGF and its receptors was examined by immunohistochemistry and western blotting analysis. The hearing threshold shift reached a level of 60 dB SPL on day 1 after trauma and underwent a partial recovery over time, reaching a value of about 20 dB SPL on day 7. Outer hair cell loss was more prominent in the area located 14-16 mm from the apex. Increased cochlear VEGF expression was observed in noise-exposed animals, in particular at the level of stria vascularis, spiral ligament, and spiral ganglion cells. No changes were observed in the expression of VEGF-receptors. Our data suggest a role for VEGF in the regulation of the vascular network in the inner ear after acoustic trauma and during auditory recovery, with potentially important clinical and therapeutic implications.

Auditory cortical activation in severe-to-profound hearing-impaired patients monitored by SPET.

Single photon emission tomography was used to map blood flow increase in temporal and parietal cortex after auditory stimulation in 25 subjects: 10 normal-hearing, 10 severe-profound hearing-impaired and 5 totally deaf. After a 500 Hz pure tone stimulation, a marked perfusion increase was observed, particularly at the level of the contralateral auditory temporal cortex. Blood flow increase in temporal and parietal cortical areas of normal subjects was significantly higher than that observed in severe-to-profound hearing-impaired patients. In all cases, following 500 Hz pure tone acoustic stimulation, the most lateral sagittal slice tomograms (48.75 and 56.25 mm) showed the highest blood flow increase. Statistically significant differences were also observed between normal subjects and hearing-impaired patients in the 48.75 mm sagittal tomogram. In 2 hearing-impaired patients, the single photon emission tomography pattern showed activation of the intermediate sagittal tomogram, suggesting a possible new tonotopic cortical arrangement. No significant activation was present in totally deaf patients. In conclusion, Single Photon Emission Tomography appears to be a useful tool in the evaluation of auditory cortical activation and cortical plasticity, in severe-to-profound hearing-impaired patients. Moreover, it could be a useful test for the study of auditory central pathways.

Achieving effective hearing aid fitting within one month after identification of childhood permanent hearing impairment.

Diagnosis of child permanent hearing impairment (PHI) can be made with extreme timeliness compared to the past thanks to improvements in PHI identification through newborn hearing screening programmes. It now becomes essential to provide an effective amplification as quickly as possible in order to restore auditory function and favour speech and language development. The early fitting of hearing aids and possible later cochlear implantation indeed prompts the development of central auditory pathways, connections with secondary sensory brain areas, as well as with motor and articulatory cortex. The aim of this paper is to report the results of a strategic analysis that involves identification of strengths, weaknesses, opportunities and threats regarding the process of achieving early amplification in all cases of significant childhood PHI. The analysis is focused on the Italian situation and is part of the Italian Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children".

Scanning electron microscopy of cochlea in new-born rats exposed to hyperbaric oxygen: preliminary report.

The aetiology of hearing loss in new-borns in neonatal intensive care is still debated. While the physiopathology of brain, lung and retina damage related to oxygen supplementation has been widely described, no studies have been carried out to define the relationship between hearing loss and supplementation of oxygen in new-borns. In the present investigation, the cochlear morphology of new-born rats was evaluated by means of scanning electron microscopy in order to assess morphological changes after supplemental oxygen administration. After treatment, electron microscopy revealed many changes in the morphology of outer hair cells, if compared to normal rats of the same age. The results suggest that cochlear changes are similar to those previously observed in other regions and may be related to a vascular mechanism of hypoxia-ischaemia and neovascularization and/or an oxidative stress.

Tensor veli palatini electromyography with surface electrode applied transnasally.

A new technique is proposed for paratubal electromyography, using a surface, non-invasive, electrode applied transnasally under nasopharyngoscope guidance. This electrode records activity of the tensor veli palatini muscle during swallowing. This technique is of interest for two reasons: endoscopic guidance offers the possibility to check correct positioning of the electrode recording at tensor veli palatini muscle level. Introduction of the non-invasive surface electrode is simple and not painful.

Can chronic nasal obstruction cause dysfunction of the paratubal muscles and otitis media? An experimental study in developing Wistar rats.

OBJECTIVE: To quantitatively analyze modifications of the paratubal muscles in developing Wistar rats following nasal obstruction. MATERIAL AND METHODS: Twenty-four Wistar rats were used. Twelve were examined at 6, 8 and 12 weeks after birth and were considered normal controls. The nostrils of the remaining 12 rats were bilaterally obstructed by means of a synthetic resin 28 days after birth. The animals were sacrificed at either 2, 4 or 8 weeks after nostril occlusion. Serial sections were made in the dorsoventral plane and stained with hematoxylin-eosin. Four 5 x 5 microm2 areas, selected within the paratubal muscles, were histologically analyzed and the number of muscular fibers was counted manually. RESULTS: The number of tensor veli palatini muscle fibers progressively decreased in the obstructed rats compared with age-matched normal controls and in those that had been obstructed for 4 and 8 weeks these reductions were statistically significant. CONCLUSION: The correct development of the paratubal muscles seems to be linked to physiological nasal breathing and is negatively affected by oral breathing.

Vestibulo-ocular reflex modification after virtual environment exposure.

Immersion in an illusory world is possible by means of virtual reality (VR), where environmental perception is modified by artificial sensorial stimulation. The application of VR for the assessment and rehabilitation of pathologies affecting the vestibular system, in terms of both diagnosis and care, could represent an interesting new line of research. Our perception of reality is in fact based on static and dynamic spatial information perceived by our senses. During head movements in a virtual environment the images on the display and the labyrinthine information relative to the head angular accelerations differ and therefore a visuo-vestibular conflict is present. It is known that mismatches between visual and labyrinthine information may modify the vestibulo-oculomotor reflex (VOR) gain. We studied the post-immersion modifications in 20 healthy subjects (mean age 25 years) exposed to a virtual environment for 20 min by wearing a head-mounted display. VOR gain and phase were measured by means of harmonic sinusoidal stimulation in the dark before, at the end of and 30 min after VR exposure. A VOR gain reduction was observed in all subjects at the end of VR exposure which disappeared after 30 min. Our data show that exposure to a virtual environment can induce a temporary modification of the VOR gain. This finding can be employed to enable an artificial, instrumental modification of the VOR gain and therefore opens up new perspectives in the assessment and rehabilitation of vestibular diseases.

Postural control and glycerol test in Ménière's disease.

Ménière's disease is a clinical disorder, characterized by fluctuating hearing loss, recurrent spontaneous episodic vertigo, tinnitus and aural fullness, which may be defined as the idiopathic syndrome of endolymphatic hydrops. The most important test for diagnosis of Ménière's disease is the glycerol test. This is a simple and rapid method and several authors have confirmed its efficiency for identifying endolymphatic hydrops. This test provides information on the cochlear response to the osmotic changes produced by glycerol in the inner ear, whereas modifications in the vestibular labyrinth are usually not evaluated. The aim of this study was to evaluate the effects of glycerol on postural control during attacks of Ménière's disease, and to correlate this data with data on cochlear function. After the glycerol test, an improvement in postural control was recorded in 70% of patients, with all patients reporting a recovery of vertigo. The impairment of postural control during endolymphatic hydrops could be related to a pressure increase in the labyrinth, which interferes with the normal dynamics of the endolymph, and a rapid functional recovery could occur during an osmotic depletion. Dynamic posturography improves the sensitivity of the glycerol test and may therefore be useful in the diagnosis and staging of Ménière's disease.

SPET assessment of auditory cortex stimulation and tonotopic spatial distribution in auditory brainstem implant.

Activation of the auditory cortex by multifrequency acoustic stimuli has been evaluated using Single Photon Emission Tomography in a case of auditory brainstem implant after activation of 6 and 11 electrodes. Before implantation, no activation of the auditory cortex has been observed after acoustic stimulation. Following auditory brainstem implant, the stimulation of 11 electrodes showed an activation value, in terms of blood flow increase, of the contralateral temporal cortex similar to that obtained with 6 electrodes (47.70 vs. 43.76%), but a significantly stronger activation was present in the contralateral parietal region (29.59 vs. 14.73%), in the homolateral temporal area (22.02 vs. 10.46%) and, especially, in the homolateral parietal zone (16.6 vs. 4.33%). The strongest activation in the contralateral temporal cortex was detected in the sagittal tomogram at 26.25 mm from the midline, that is in the areas where high frequencies are projected, both with 6 and 11 active electrodes. The medio-lateral auditory cortex, where the middle and lower frequencies are projected, showed an overall lower activation which was however significantly lower with 6-electrode stimulation. Stimulation of the surface of cochlear nuclei determines mainly an activation of the high frequency domain, independently of the electrodes number. This finding may explain the better results of cochlear implants in comparison with auditory brainstem implant and could justify the use of needle electrodes in auditory brainstem implant. In conclusion, Single Photon Emission Tomography can be considered useful in evaluating auditory brainstem implant placement and function. It is also able to define the effectiveness of acoustic stimulation, the degree and tonotopic spatial distribution of auditory cortex activation.

Transmastoid approach to repair meningoencephalic herniation in the middle ear.

Meningoencephalic herniation (MEH) in the middle ear and mastoid is a rare pathological entity with possible life-threatening complications. We treated 24 patients with a trans-mastoid approach, and the bony defect was closed by heterologous materials positioned in a multilayer fashion. The cause of the bony defect were chronic otitis media with cholesteatoma, iatrogenic, spontaneous and post-traumatic. The major presenting symptoms were meningitis, headache, conductive hearing loss, cerebrospinal fluid (CSF leak), neurologic deficit and pneumoencephalus, and stenosis of a canal wall down cavity. During follow-up, no patient developed complications due to surgery or related to the pathology, and imaging showed a stable occlusion of the bony defect. Different surgical treatments have been proposed to repair MEH, and the choice is based on the localization and size of the bony defect, preoperative auditory function and the presence of a coexisting pathology. We propose the use of collagenous membranes and bone substitutes for reconstruction of the floor of the middle fossa.

Alternative treatment for otitis media with effusion: eustachian tube rehabilitation.

In this study, we evaluated the effectiveness of eustachian tube rehabilitation (ETR) as treatment for otitis media with effusion (OME). Thirty-five children with persistent OME were enrolled. Patients were divided into three groups: group I (isolated OME); group II (OME and atypical swallowing); group II (OME, habitual mouth breathing and atypical swallowing). All children underwent ETR. Otomicroscopy and tympanograms were performed before treatment, and at one and three months following ETR. Considering the overall patient population after ETR (one and three months later), the prevalence of type A tympanogram increased significantly compared to before therapy (p < 0.005), while the prevalence of type B tympanogram decreased significantly (p < 0.005). We found significant differences between pre- and both post-therapy control in groups I and II. However, children in group II experienced significant improvement of middle ear conditions only three months after the end of therapy (p < 0.005). On the basis of the physiopathologic knowledge of OME and the underlying principles of ETR, we conclude that ETR can be considered a useful therapy in management of OME.

Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology.

Hearing loss is one of the most common disabilities and has lifelong consequences for affected children and their families. Both conductive and sensorineural hearing loss (SNHL) may be caused by a wide variety of congenital and acquired factors. Its early detection, together with appropriate intervention, is critical to speech, language and cognitive development in hearing-impaired children. In the last two decades, the application of universal neonatal hearing screening has improved identification of hearing loss early in life and facilitates early intervention. Developments in molecular medicine, genetics and neuroscience have improved the aetiological classification of hearing loss. Once deafness is established, a systematic approach to determining the cause is best undertaken within a dedicated multidisciplinary setting. This review addresses the innovative evidences on aetiology and management of deafness in children, including universal neonatal screening, advances in genetic diagnosis and the contribution of neuroimaging. Finally, therapy remains a major challenge in management of paediatric SNHL. Current approaches are represented by hearing aids and cochlear implants. However, recent advances in basic medicine which are identifying the mechanisms of cochlear damage and defective genes causing deafness, may represent the basis for novel therapeutic targets including implantable devices, auditory brainstem implants and cell therapy.