Objectives: The aim of this study was to evaluate the results after cochlear implant (CI) in elderly patients and to compare them to those obtained in younger adult patients. A possible correlation between the results in older CI recipients and some variables of the patients was also investigated. Methods: The sample consisted of 107 patients over 40 years of age at surgery divided into 3 subgroups according to age at implantation. Results in terms of speech perception in silence and with background noise were compared between the 3 groups. The patients also completed the Glasgow Benefits Inventory (GBI) questionnaire. Results: Older patients achieved significant benefits after CI with regard to speech perception in silence and with background noise. We also recorded benefits for the GBI. Discussion: In line with the literature, our data confirm that CI is an effective procedure also in elderly patients, conferring benefits in speech perception and quality of life.

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.

OBJECTIVES: This cross-sectional study aims to describe the features of the suppression head impulse paradigm (SHIMP) in acute unilateral vestibulopathy (AUV) and to define its role in predicting the recovery of patients. METHODS: Thirty patients diagnosed with AUV were retrospectively analyzed. The dizziness handicap inventory score and video head impulse test parameters performed 4–8 weeks from the AUV onset constituted the main outcome measures. Patients with a worse recovery (Group 1) and patients who recovered spontaneously (Group 2) were compared. RESULTS: The SHIMP vestibulo-ocular reflex (VOR) gain was statistically significantly lower than the conventional head impulse paradigm (HIMP) VOR gain (P <  0.001). The SHIMP VOR gain was negatively correlated with the DHI (P <  0.001) and was positively correlated with the HIMP VOR gain (P <  0.001) and the SHIMP overt saccades (%) (P <  0.001). Patients with a worse recovery exhibited the following: higher DHI (P <  0.001), lower SHIMP and HIMP VOR gain (P <  0.001 and P = 0.007, respectively), and lower SHIMP and greater HIMP overt saccade prevalence values (P = 0.007 and P = 0.032, respectively). CONCLUSIONS: The SHIMP and HIMP help in improving our approach to AUV. SHIMP appears to better identify the extent of the vestibular damage in patient suffering from AUV than HIMP and could provide interesting information about the course of the disease. Particularly, the analysis of SHIMP VOR gain and overt saccade prevalence would provide useful information about the recovery of patients.

BackgroundNewborn hearing screening has to be considered the first step of a program for the identification, diagnosis, treatment and habilitation/rehabilitation of children with hearing impairment.Main PartIn Tuscany Region of Italy, the universal newborn hearing screening is mandatory since november 2007. The first guidelines for the execution of the screening have been released in June 2008; then many other Italian regions partially or totally adopted these guidelines. On the basis of the experience from 2008 and according to the recent evidences in the scientific literature, a new screening protocol was released in Tuscany region. The new protocol is an evolution of the previous one. Some issues reported in the previous protocol and in the Joint Committee on Infant Hearing statement published in 2007 were revised, such as the risk factors for auditory neuropathy and for late onset, progressive or acquired hearing loss. The new updated guidelines were submitted to the Sanitary Regional Council and then they have been approved in August 2016. The updated screening protocol is mainly aimed to identify newborns with a congenital moderate-to-profound hearing loss, but it also provides indications for the audiological follow-up of children with risk’s factor for progressive or late onset hearing loss; further it provides indications for the audiological surveillance of children at risk for acquired hearing impairment. Then, in the new guidelines the role of the family paediatrician in the newborn hearing screening and audiological follow-up and surveillance is underscored. Finally the new guidelines provide indications for the treatment with hearing aids and cochlear implant, in accordance with the recent Italian Health Technology Assessment (HTA) guidelines.ConclusionsIn the paper we report the modality of execution of the universal newborn hearing screening in the Tuscany Region, according to the recently updated protocol. The main features of the protocol and the critical issues are discussed.Electronic supplementary materialThe online version of this article (10.1186/s13052-017-0397-1) contains supplementary material, which is available to authorized users.

Perrault syndrome (PRLTS) is a rare autosomal recessive disorder characterised by ovarian failure in females and sensorineural hearing loss (SNHL) in both genders. In the present paper we describe a child affected by PRLTS3, due to CLPP homozygous mutations, presenting auditory neuropathy spectrum disorder (ANSD) with bilateral progressive SNHL. This is the first case reported in the literature of an ANSD in PRLTS3. CLPP is a nuclear encoded mitochondrial protease directed at the mitochondrial matrix. It is encoded on chromosome 19. This protease participates in mitochondrial protein quality control by degrading misfolded or damaged proteins, thus maintaining the normal metabolic function of the cell. In PRLTS3, the peptidase activity of CLPP is suppressed. Neurological impairments involved in PRLTS3 suggest that the pathogenic mutations in CLPP might trigger a mitochondrial dysfunction. A comprehensive description of the clinical and audiological presentation, as well as the issues related to cochlear implant (CI) procedure and the results, are addressed and discussed. A brief review of the literature on this topic is also provided.