Sequential bilateral cochlear implant: long-term speech perception results in children first implanted at an early age.

PURPOSE: The study aims to assess the benefit of sequential bilateral cochlear implantation in children with congenital bilateral profound hearing loss, submitted to the first implant at an early age. METHODS: We enrolled all the bilateral sequential cochlear implanted children who received the first implant within 48 months and the second within 12 years of age at our Institution. The children were submitted to disyllabic word recognition tests and Speech Reception Threshold (SRT) assessment using the OLSA matrix sentence test with the first implanted device (CI1), with the second implanted device (CI2), and with both devices (CIbil). Furthermore, we measured the datalogging of both devices. Then we calculated the binaural SRT gain (b-SRTgain) and checked the correlations between speech perception results and the b-SRTgain with the child's age at CI1 and CI2, DELTA and the datalogging reports. RESULTS: With the bilateral electric stimulation, we found a significant improvement in disyllabic word recognition scores and in SRT. Moreover, the datalogging showed no significant differences in the time of use of CI1 and CI2. We found significant negative correlations between speech perception abilities with CI2 and age at CI2 and DELTA, and between the SRT with CI1 and the b-SRTgain. CONCLUSIONS: From this study we can conclude that in a sequential CI procedure, even if a short inter-implant delay and lower ages at the second surgery can lead to better speech perception with CI2, children can benefit from bilateral stimulation independently of age at the second surgery and the DELTA.

Cochlear implantation in patients with asymmetric hearing loss: reporting and discussing the benefits in speech perception, speech reception threshold, squelch abilities, and patients' reported outcomes.

OBJECTIVES: This study presents the results obtained in a group of patients with asymmetric hearing loss undergoing cochlear implantation at our institution. Prognostic factors are discussed in relation to different rehabilitative approaches for asymmetric hearing loss remediation. The current literature is also discussed. METHODS: Nineteen adult patients with post-verbal asymmetric hearing loss were enrolled. The results were assessed by means of a speech perception test, completed in silence and with background noise, and a speech reception threshold test (Oldenburg Sentence Test). The subjectively perceived benefits were assessed using the Speech, Spatial and Qualities of Hearing Scale. RESULTS: Statistically significant improvements were achieved by all patients in terms of speech perception and speech reception threshold, and in subjective benefits. CONCLUSION: The results confirm the literature findings which suggest that patients with asymmetric hearing loss generally gain substantial benefit from cochlear implantation because of the binaural input, with significant improvement in speech perception abilities in noise, speech reception threshold, and squelch abilities.

Enlarged vestibular aqueduct and Mondini Malformation: audiological, clinical, radiologic and genetic features.

PURPOSE: When referring to enlarged vestibular aqueduct (EVA) we should differentiate between nonsyndromic enlarged vestibular aqueduct (NSEVA) and Pendred Syndrome (PDS), a disease continuum associated with pathogenic sequence variants of Pendrin's Gene (SLC26A4) in about half of the cases. The study was aimed to analyse the clinical and audiological features of a monocentric cohort of Caucasian patients with NSEVA/PDS, their genetic assessment and morphological inner ear features. METHODS: We retrospectively reviewed the audiologic, genetic and anamnestic data of 66 patients with NSEVA/PDS followed by our audiology service. RESULTS: SLC26A4 mutations was significantly correlated with the presence of PDS rather than NSEVA (p < 0.019), with the expression of inner ear malformations (p < 0.001) and with different severity of hearing loss (p = 0.001). Furthermore, patients with PDS showed significantly worse pure tone audiometry (PTA) than patients with NSEVA (p = 0.001). Anatomically normal ears presented significantly better PTA than ears associated with Mondini Malformation or isolated EVA (p < 0.001), but no statistically significative differences have been observed in PTA between patients with Mondini Malformation and isolated EVA. CONCLUSION: NSEVA/PDS must be investigated in all the congenital hearing loss, but also in progressive, late onset, stepwise forms. Even mixed or fluctuating hearing loss may constitute a sign of a NSEVA/PDS pathology. Our findings can confirm the important role of SLC26A4 mutations in determining the phenotype of isolated EVA/PDS, both for the type/degree of the malformation, the hearing impairment and the association with thyroid dysfunction.

Comparison of a purely endoscopic three-layer technique versus pericranial flap for reconstruction of anterior skull base defects after sino-nasal tumor resection: assessment of postoperative frontal lobe sagging and frontal lobe falling.

BACKGROUND: The evolution of endoscopic skull base approaches has enabled surgeons to manage selected skull base tumors through a transnasal endoscope-assisted approach. On the other side, more extensive lesions may require a combined cranioendoscopic approach. In this paper, we analysed and compared the incidence of frontal lobe sagging after endoscopic multilayer (EM) reconstruction versus pericranial flap (PF) reconstruction. METHODOLOGY: Subjects were selected retrospectively according to specific inclusion and exclusion criteria. The degree of frontal lobe sagging after surgery was calculated based on the most inferior position of the frontal lobe relative to the nasion-sellar line defined on preoperative and postoperative imaging. A positive value signified upward displacement, and a negative value represented frontal lobe sagging. RESULTS: Twenty subjects were enrolled in our study. In the EM technique group the average frontal lobe displacement was -2,34 ± 1,55 mm. The average postoperative frontal lobe sagging was -0,45 ± 8,92 mm in subjects reconstructed with the PF. The skull base defect size correlated with the degree of frontal lobe sagging in subjects reconstructed with the PF, but not in the other group and when merging the two groups. CONCLUSIONS: In conclusion, the EM technique and the PF reconstruction showed a good reliability for the closure of anterior skull base defects. Moreover the PF seemed to prevent frontal lobe sagging but, for larger skull base defects, it could be useful to be combined with other autologous or heterologous materials to avoid the frontal lobe falling.

Transtympanic Hearing Aid: exploratory study on a new device.

SUMMARY: In this paper, we present the preliminary results achieved with a transtympanic hearing aid (THA). This is a modified digital, open-fit external hearing aid (HA) designed for acute study only, which allows coupling with a pre-implanted ventilation tube. The THA conveys amplified sound directly onto the round window, bypassing the ossicular chain, in contrast with traditional HAs that convey sound onto the second or third portion of the external auditory canal. The THA has been developed as an alternative to standard HAs and active middle ear implants for patients who are unsatisfied with traditional HA outcomes and want to avoid middle-ear implantation. The results achieved using the THA were compared to those obtained with an equivalent device, the Latitude 8 Moxi 13 (Moxi), uncoupled from the ventilation tube, and placed onto the outer ear. For this purpose, 12 patients with conductive (1/12), sensorineural (3/12), or mixed (8/12) hearing loss from moderate to severe, with a pre-implanted ventilation tube, underwent audiological evaluation with both the THA and the Latitude 8 Moxi 13 (Moxi). Our initial results showed that the THA provided significant improvement in the warble tone results in comparison to the Moxi. Moreover, patients with a PTA between 41 and 90 also achieved better results in terms of speech recognition using the THA in comparison to the Moxi. In conclusion, these outcomes provide the first evidence of the potential benefits of the THA over standard open-fit HAs. Nevertheless, these preliminary outcomes require further confirmation.

Ultra-slow mechanical stimulation of olfactory epithelium modulates consciousness by slowing cerebral rhythms in humans.

The coupling between respiration and neural activity within olfactory areas and hippocampus has recently been unambiguously demonstrated, its neurophysiological basis sustained by the well-assessed mechanical sensitivity of the olfactory epithelium. We herein hypothesize that this coupling reverberates to the whole brain, possibly modulating the subject's behavior and state of consciousness. The olfactory epithelium of 12 healthy subjects was stimulated with periodical odorless air-delivery (frequency 0.05 Hz, 8 s on, 12 off). Cortical electrical activity (High Density-EEG) and perceived state of consciousness have been studied. The stimulation induced i) an enhancement of delta-theta EEG activity over the whole cortex mainly involving the Limbic System and Default Mode Network structures, ii) a reversal of the overall information flow directionality from wake-like postero-anterior to NREM sleep-like antero-posterior, iii) the perception of having experienced an Altered State of Consciousness. These findings could shed further light via a neurophenomenological approach on the links between respiration, cerebral activity and subjective experience, suggesting a plausible neurophysiological basis for interpreting altered states of consciousness induced by respiration-based meditative practices.

Cochlear implant in prelingually deafened oralist adults: speech perception outcomes, subjective benefits and quality of life improvement.

The aim of this study is to report our results in a group of prelingually deafened adults, who followed an oralist rehabilitation programme, and submitted to cochlear implant at our institution. We evaluated 30 prelingually deafened adult patients, 18 males and 12 females, median age 35 years, of a group of 36 prelingually deafened adult patients consecutively submitted to unilateral cochlear implantation at the ENT Unit of the University of Pisa. After implantation, patients achieved significant benefits in terms of speech perception skills, including the ability to have telephone conversations in some cases, quality of life and their own perception of disability. According to literature data, the results herein reported are quite variable but generally satisfactory. Procedures other than traditional speech perception measures should be used to evaluate the benefits of cochlear implant in such patients, to compressively evaluate the global benefits, not only in terms of speech perception, but also in terms of quality of life and daily life.

Stabilometric findings in patients affected by organic dysphonia before and after phonomicrosurgery.

The purpose of this study is to understand if there is any alteration in the posture of patients affected by organic dysphonia and describe possible postural modifications after phonomicrosurgery on the vocal folds. Forty subjects (22 males, 18 females; mean age 32.6 ± 7.5 years) suffering from organic dysphonia (15 cases of polyps, 11 submucosal retention cysts, 10 bilateral fibrous vocal fold nodules and 4 bilateral Reinke's oedema) were examined by open-eye and closed-eye posturography while breathing spontaneously before surgery, 24 hours after surgery and after 6 months. The variables taken into account were: the coordinates of the centre of pressure on both frontal and sagittal planes, length and surface of the track, mean velocity of the oscillations and relative standard deviations, spectral analysis of oscillation frequency, statokinesigram and stabilogram values. No characteristic pathological pattern was seen in basal stabilometry in any of the subgroups (polyps, cysts, Reinke's oedema). Only the subgroup of patients with fibrous vocal fold nodules (8/10; 80%) showed a slight forward shift from the centre of gravity when analysed in both open-eye and closed-eye posturography. A comparison performed within the same subgroup using open-eye and closed-eye posturography before and after surgery revealed no significant difference in any of the parameters being studied. The use of static stabilometry in this study demonstrates the absence of characteristic postural alterations in patients affected by organic dysphonia and also excludes that simple removal of the vocal fold lesion can change posture.

A review of new insights on the association between hearing loss and cognitive decline in ageing.

Age-related hearing loss (ARHL) has a multifactorial pathogenesis and it is an inevitable hearing impairment associated with reduction of communicative skills related to ageing. Increasing evidence has linked ARHL to more rapid progression of cognitive decline and incidental dementia. Many aspects of daily living of elderly people have been associated to hearing abilities, showing that hearing loss (HL) affects the quality of life, social relationships, motor skills, psychological aspects and function and morphology in specific brain areas. Epidemiological and clinical studies confirm the assumption of a relationship between these conditions. However, the mechanisms are still unclear and are reviewed herein. Long-term hearing deprivation of auditory inputs can impact cognitive performance by decreasing the quality of communication leading to social isolation and depression and facilitate dementia. On the contrary, the limited cognitive skills may reduce the cognitive resources available for auditory perception, increasing the effects of HL. In addition, hearing loss and cognitive decline may reflect a 'common cause' on the auditory pathway and brain. In fact, some pathogenetic factors are recongised in common microvascular disease factors such as diabetes, atherosclerosis and hypertension. Interdisciplinary efforts to investigate and address HL in the context of brain and cognitive ageing are needed. Surprisingly, few studies have been adressed on the effectiveness of hearing aids in changing the natural history of cognitive decline. Effective interventions with hearing aids or cochlear implant may improve social and emotional function, communication, cognitive function and positively impact quality of life. The aim of this review is to overview new insights on this challenging topic and provide new ideas for future research.

Idiopathic sensorineural hearing loss in the only hearing ear.

A retrospective chart review was used for 31 patients with sudden, progressive or fluctuating sensorineural hearing loss (SHL) in the only hearing ear who had been consecutively evaluated at the ENT, Audiology and Phoniatrics Unit of the University of Pisa. The group of patients was evaluated with a complete history review, clinical evaluation, imaging exam (MRI, CT), audiologic tests (tone and speech audiometry, tympanometry, study of stapedial reflexes, ABR and otoacoustic emission) evaluation. In order to exclude genetic causes, patients were screened for CX 26 and CX30 mutations and for mitochondrial DNA mutation A1555G. Patients with sudden or rapidly progressive SHL in the only hearing ear were treated with osmotic diuretics and corticosteroids. In patients who did not respond to intravenous therapy we performed intratympanic injections of corticosteroid. Hearing aids were fitted when indicated and patients who developed severe to profound SHL were scheduled for cochlear implant surgery. The aim of this study is to report and discuss the epidemiology, aetiopathogenesis, therapy and clinical characteristic of patients affected by SHL in the only hearing hear and to discuss the issues related to the cochlear implant procedure in some of these patients, with regard to indications, choice of the ear to implant and results.

Aetiologic diagnosis of hearing loss in children identified through newborn hearing screening testing.

With the implementation of universal newborn hearing screening (UNHS) programmes and early diagnosis and treatment of hearing problems, the need has clearly emerged to implement and carry out a systematic and coordinated protocol for the aetiological diagnosis of permanent hearing impairment (PHI). Within the framework 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", it has been decided to consider the problems relative to aetiological diagnosis of child PHI within UNHS programmes. The specific objective was to apply a shared diagnostic protocol that can identify the cause in at least 70% of cases of PHI. For this part of the project, four main recommendations were identified that can be useful for an efficient aetiological diagnosis in children affected by PHI and that can offer valid suggestions to optimise resources and produce positive changes for third-level audiologic centres.

Early care in children with permanent hearing impairment.

The implementation of regional protocols for newborn hearing screening and early audiologic diagnosis represent the first step of the entire diagnostic, rehabilitative and prosthetic programme for children with permanent hearing impairment. The maximum benefit of early diagnosis can indeed be obtained only by prompt rehabilitation aimed at fostering the child's communicative, linguistic and cognitive development. Within the framework of the CMM 2013 project of the Ministry of Health entitled "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", the problems concerning the promotion of the global development of children with PHI through an early rehabilitation project based on shared knowledge and scientific evidence. In this project, our specific aim was to define the features and modes of access to a precise and specialised rehabilitation project for the small hearing-impaired child within three months from audiologic diagnosis. Three main recommendations relative to assessment and rehabilitation aspects of early care emerged from the study.

Long-term follow up of sudden sensorineural hearing loss patients treated with intratympanic steroids: audiological and quality of life evaluation.

OBJECTIVE: To evaluate the long-term stability of intratympanic steroids and investigate the 'real' impact of sudden sensorineural hearing loss on patients. METHOD: A total of 14 patients treated with intratympanic steroids were evaluated by audiometric and vestibular examinations. The modified Glasgow Benefit Inventory was used to evaluate quality of life changes after intratympanic steroid treatment. RESULTS: There was no significant difference between pure tone average post-intratympanic steroids and at follow up. The general Glasgow Benefit Inventory score was not significantly associated with the presence of tinnitus or dizziness, or with patient age. The change in pure tone average after intratympanic steroid treatment did not correlate with social or physical scores, but correlated strongly with the general Glasgow Benefit Inventory score (p = 0.0023). Intratympanic steroid administration led to a stable improvement in hearing. Quality of life assessment showed that patients can feel satisfaction regardless of the hearing outcome. Patients who regained a social hearing level expressed greater satisfaction than patients without serviceable hearing. Overall, quality of life improvement was not related to hearing improvement. CONCLUSION: Sudden sensorineural hearing loss is devastating. Considering the audiological effects alone ignores the 'human' perspective. Audiological success can correlate with poor quality of life outcome.

Mitochondrial DNA (mtDNA) haplotypes and dysfunctions in presbyacusis.

The aim of this study was to investigate the presence of mitochondrial DNA (mtDNA) alterations and metabolic dysfunctions in patients with presbyacusis, and to discover correlations between presbyacusis and the degree of hearing loss and mitochondrial damage. Seventy patients with presbyacusis were examined, including 40 Egyptian patients and 30 Italian patients. Forty eight normal subjects were included as control group, including 24 Egyptians and 24 Italians. There was no common point mutation, and A1555G, A3243G, A7445G not were detected in any patients or controls. Haplogroup U was significantly common in patients in comparison to controls. Mutation of antioxidant genes (GSTT1, GSTM1) were significantly present in only Italian patients compared to Italian controls.

Neonatal screening for congenital cytomegalovirus infection in preterm and small for gestational age infants.

Congenital cytomegalovirus (CMV) infection affects many organs: reticuloendothelial and central nervous system are particularly involved. Congenital CMV infection is the leading cause of non-genetic sensorineural hearing loss. Hearing impairment can be present at birth or it can occur months or even years after birth. It is as well an important risk factor for antenatal stillbirth, preterm birth and small for gestational age (SGA) condition. For these reasons we should early identify congenital CMV infection investigating at least at risk newborns such as preterm or SGA babies given that a simple and standardized method for a large scale screening program is lacking. In our study, we found an association between congenital CMV infection and preterm births (3.03%) and with SGA condition (3.7%). Consequently, routine CMV urine detection should be performed at least in all babies born before 37 weeks of gestational age and in term SGA newborns.

A rare case of nasopharyngeal pleomorphic adenoma.

Salivary gland tumors are rare. The majority of these tumors are benign and about 70% are pleomorphic adenomas (PA). Nasopharynx is an unusual site for the PA tumor. Only six cases are presented in the literature from 1990 to 2011. The diagnosis of this disease is linked to the sum of imaging tests, clinical and histological study of the mass of the above. The radiologic features of noninvasion of surrounding structures give the first clue to the benign lesion. From the review of the literature and our experience is identified as the gold standard in surgical treatment for this condition. Thanks to the endoscopic surgery, we can perform a complete resection of the lesion without damaging adjacent structures. We presented a case of pleomorphic adenoma of nasopharynx with literature review.

[Cochlear implant in children: rational, indications and cost/efficacy]. / L'impianto cocleare nel bambino: razionale, indicazioni, costo/efficacia.

A cochlear implant (CI) is a partially implanted electronic device that can help to provide a sense of sound and support speech to severely to profoundly hearing impaired patients. It is constituted by an external portion, that usually sits behind the ear and an internal portion surgically placed under the skin. The external components include a microphone connected to a speech processor that selects and arranges sounds pucked up by the microphone. This is connected to a transmitter coil, worn on the side of the head, which transmits data to an internal receiver coil placed under the skin. The received data are delivered to an array of electrodes that are surgically implanted within the cochlea. The primary neural targets of the electrodes are the spiral ganglion cells which innervate fibers of the auditory nerve. When the electrodes are activated by the signal, they send a current along the auditory nerve and auditory pathways to the auditory cortex. Children and adults who are profoundly or severely hearing impaired can be fitted with cochlear implants. According to the Food and Drug Administration, approximately 188,000 people worldwide have received implants. In Italy it is extimated that there are about 6-7000 implanted patients, with an average of 700 CI surgeries per year. Cochlear implantation, followed by intensive postimplantation speech therapy, can help young children to acquire speech, language, and social skills. Early implantation provides exposure to sounds that can be helpful during the critical period when children learn speech and language skills. In 2000, the Food and Drug Administration lowered the age of eligibility to 12 months for one type of CI. With regard to the results after cochlear implantation in relation to early implantation, better linguistic results are reported in children implanted before 12 months of life, even if no sufficient data exist regarding the relation between this advantage and the duration of implant use and how long this advantage persists in the subsequent years. With regard to cochlear implantation in children older than 12 months the studies show better hearing and linguistic results in children implanted at earlier ages. A sensitive period under 24-36 months has been identified over which cochlear implantation is reported to be less effective in terms of improvement in speech and hearing results. With regard to clinical effectiveness of bilateral cochlear implantation, greater benefits from bilateral implants compared to monolateral ones when assessing hearing in quiet and in noise and in sound localization abilities are reported to be present in both case of simultaneous or sequential bilateral implantation. However, with regard to the delay between the surgeries in sequential bilateral implantation, although benefit is reported to be present even after very long delays, on average long delays between surgeries seems to negatively affect the outcome with the second implant. With regard to benefits after cochlear implantation in children with multiple disabilities, benefits in terms of speech perception and communication as well as in quality of the daily life are reported even if benefits are slower and lower in comparison to those generally attained by implanted children without additional disabilities. Regarding the costs/efficacy ratio, the CI is expensive, in particular because of the cost of the high technological device, long life support, but even if healthcare costs are high, the savings in terms of indirect costs and quality of life are important. The CI, in fact, has a positive impact in terms of quality of life.

The use and role of the Ambulatory Phonation Monitor (APM) in voice assessment.

Vocal load plays a significant role in the aetiology of voice disorders and influences the response to treatment. For this reason, many researchers have focused their attention on how a voice is used, especially when vocal load is increased, during working hours for instance. The majority of studies in this regard have been performed by recording vocal parameters for brief periods with the aid of microphones. The first devices produced recorded only a few parameters and for relatively short periods of time, and since microphones were used there was a problem with both privacy and background noise such as the inclusion of voices from nearby people. Recently, microprocessors that can monitor a voice for an entire day have been developed; these use miniaturised accelerometers as vocal sensors. The latest commerciallyavailable version is the Ambulatory Phonation Monitor (APM) (KayPENTAX, Lincoln Park, NJ, USA) which can record several vocal parameters for over 18 hours and supply a series of graphic representations of the variations in these parameters during the recording period. In particular, the APM permits recording vocal load by measuring the cycle dose and distance dose, and evaluates vocal intensity (dB sound pressure level [SPL]), fundamental frequency and total phonation time. This report describes the APM, the use of an accelerometer as a vocal sensor, the importance of its calibration and the parameters it records. In particular, details are given on phonation time, variations in frequency, vocal intensity, phonation density and vocal dose. The role of the APM in voice studies is also described, in addition to its potential clinical applications as demonstrated by the few reports available in the literature. We also discuss our experience with the device in groups of euphonic and dysphonic elementary school teachers.

Cochlear implant in incomplete partition type I.

In this investigation, we report on 4 patients affected by incomplete partition type I submitted to cochlear implant at our institutions. Preoperative, surgical, mapping and follow-up issues as well as results in cases with this complex malformation are described. The cases reported in the present study confirm that cochlear implantation in patients with incomplete partition type I may be challenging for cochlear implant teams. The results are variable, but in many cases satisfactory, and are mainly related to the surgical placement of the electrode and residual neural nerve fibres. Moreover, in some cases the association of cochlear nerve abnormalities and other disabilities may significantly affect results.