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The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere’s disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet’s disease, Cogan’s syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides.

Sensorineural systems play a crucial role in the diagnosis, treatment and management of several neurological disorders. The function of the eye and ear represents a unique window for testing various conditions in cognitive decline or dementia. Touch and smell have also been found to be strongly involved in neurodegenerative conditions, and their decline has been significantly associated with the progression of the disease; hence, the idea that restoring sensory function in cognitively impaired adults might enable a significant improvement in their cognitive status, reducing the worldwide incidence and prevalence of dementia. Not all sensorineural ‘windows' can benefit equally from the same procedures; however, hearing and vision can certainly gain the most from dependable therapeutic and other diagnostic options. The ear, including the vestibular system, deserves an honored place among the sensory organs in this context due mainly to the sophisticated electrical devices available that have amply demonstrated their effectiveness in treating hearing loss. Restoring an individual's hearing can reduce the cognitive ‘load', i.e. the neural activity needed to understand/recognize the spoken word - an activity that becomes more demanding if the brain is obliged to recruit different neural populations to achieve the same performance, as happens in older adults with sensory impairments. The sensory interfaces may also facilitate the early diagnosis of conditions characterized by a lengthy preclinical phase, as well as enabling noninvasive, follow-up procedures to assess the outcome of rehabilitation measures and distinguish physiological brain aging from neurodegenerative disorders. The present study is a brief literature review on the issues and prospects relating to the unique relationship between hearing and cognitive decline, with a general introduction to the main topics before focusing on rehabilitation training with hearing aids and cochlear implants to combat cognitive decline.

Untreated hearing loss has an effect on cognition. It is hypothesized that the additional processing required to compensate for the sensory loss affects the cognitive resources available for other tasks and that this could be mitigated by a hearing device. The impact on cognition of cochlear implants (CIs) was tested in 100 subjects, ≥60 years old, with bilateral moderately-severe to profound post linguistic deafness using hearing aids. Data was compared pre and 12 and 18 months after cochlear implantation for the speech spatial qualities questionnaire, Mini Mental State Examination (MMSE), Trail making test B (TMTB) and digit symbol coding (DSC) from the Wechsler Adult Intelligence Scale version IV and finally the timed up and go test (TUG). Subjects were divided into young old (60-64), middle old (65-75) and old old (75+) groups. Cognitive test scores and times were standardized according to available normative data. Hearing significantly improved pre- to post-operatively across all age groups. There was no change post-implant in outcomes for TMTB, TUG or MMSE tests. Age-corrected values were within normal expectations for all age groups for the TUG and MMSE. However, DSC scores and TMTB times were worse than normal. There was a significant increase in DSC scores between baseline and 12-months for 60- to 64-year-olds ( [153] = 2.608, = 0.027), which remained at 18 months ( [153] = 2.663, = 0.023). The improved attention and processing speed in the youngest age group may be a consequence of reallocation of cognitive resources away from auditory processing due to greatly improved hearing. The oldest age group of participants had cognition scores closest to normal values, suggesting that only the most able older seniors tend to come forward for a CI. Severe to profoundly deaf individuals with hearing aids or cochlear implants were still poorer than age-equivalent normally hearing individuals with respect to cognitive flexibility, attention, working memory, processing speed and visuoperceptual functions. Due to a lack of data for the TUG, TMTB and DSC in the literature for hearing impaired individuals, the results reported here provide an important set of reference data for use in future research.

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

Epidemiological studies show increasing prevalence rates of cognitive decline and hearing loss with age, particularly after the age of 65 years. These conditions are reported to be associated, although conclusive evidence of causality and implications is lacking. Nevertheless, audiological and cognitive assessment among elderly people is a key target for comprehensive and multidisciplinary evaluation of the subject's frailty status. To evaluate the use of tools for identifying older adults at risk of hearing loss and cognitive decline and to compare skills and abilities in terms of hearing and cognitive performances between older adults and young subjects, we performed a prospective cross-sectional study using supraliminal auditory tests. The relationship between cognitive assessment results and audiometric results was investigated, and reference ranges for different ages or stages of disease were determined. Patients older than 65 years with different degrees of hearing function were enrolled. Each subject underwent an extensive audiological assessment, including tonal and speech audiometry, Italian Matrix Sentence Test, and speech audiometry with logatomes in quiet. Cognitive function was screened and then verified by experienced clinicians using the Montreal Cognitive Assessment Score, the Geriatric Depression Scale, and further investigations in some. One hundred twenty-three subjects were finally enrolled during 2016-2019: 103 were >65 years of age and 20 were younger participants (as controls). Cognitive functions showed a correlation with the audiological results in post-lingual hearing-impaired patients, in particular in those affected by slight to moderate hearing loss and aged more than 70 years. Audiological testing can thus be useful in clinical assessment and identification of patients at risk of cognitive impairment. The study was limited by its sample size (CI 95%; CL 10%), strict dependence on language, and hearing threshold. Further investigations should be conducted to confirm the reported results and to verify similar screening models.

Objectives Several studies have demonstrated a high prevalence of voice disorders in teachers, together with the personal, professional and economical consequences of the problem. Good primary prevention should be based on 3 aspects: 1) amelioration of classroom acoustics, 2) voice care programs for future professional voice users, including teachers and 3) classroom or portable amplification systems. The aim of the study was to assess the benefit obtained from the use of portable amplification systems by female primary school teachers in their occupational setting. Materials and Methods Forty female primary school teachers attended a course about professional voice care, which comprised two theoretical lectures, each 60 min long. Thereafter, they were randomized into 2 groups: the teachers of the first group were asked to use a portable vocal amplifier for 3 months, till the end of school-year. The other 20 teachers were part of the control group, matched for age and years of employment. All subjects had a grade 1 of dysphonia with no significant organic lesion of the vocal folds. Results Most teachers of the experimental group used the amplifier consistently for the whole duration of the experiment and found it very useful in reducing the symptoms of vocal fatigue. In fact, after 3 months, Voice Handicap Index (VHI) scores in “course + amplifier” group demonstrated a significant amelioration (p = 0.003). The perceptual grade of dysphonia also improved significantly (p = 0.0005). The same parameters changed favourably also in the “course only” group, but the results were not statistically significant (p = 0.4 for VHI and p = 0.03 for perceptual grade). Conclusions In teachers, and particularly in those with a constitutional weak voice and/or those who are prone to vocal fold pathology, vocal amplifiers may be an effective and low-cost intervention to decrease potentially damaging vocal loads and may represent a necessary form of prevention.

Background At the present time, 50 to 60% of the population above 70 years of age suffers from a hearing impairment and from 0.6 to 1.1% has a severe to profound loss, which cannot benefit from an hearing aid. Moreover, it is expected that this prevalence will grow by more than two-fold in the next 40 years. There is strong evidence that hearing loss in older adults is associated with both cognitive load and social isolation, which in turn, are associated with cognitive and physical functioning. Cochlear implant (CI) dramatically improves sound audibility and speech understanding. The aim of this paper was to analyze outcome and complications of CI treatment in elderly patients. Methods A retrospective study on 17 patients, aged at implantation between 65 and 79 years (mean = 70.47 ± 3.94), unilaterally implanted for severe to profound bilateral hearing loss. The following data were statistically evaluated: pre-implant pure-tone threshold and tests of speech recognition, both with hearing aid that without; post-implant threshold and speech perception with CI off and on. Moreover, statistical correlations of PTA improvement between two age groups (65 to 70 and over 70 years) were carried out. Results Mean PTA improved from 111.25 (± 17.51) (pre-implant) to 43.81 (± 9.27) (post-implant); and the mean SRT improved from 90 dB to 65 dB. Moreover there was no statistical difference in PTA improvement between the two age groups (65 to 70 and over 70 years). No severe per- or post-operative surgical complications were noted. Discussion In the elderly, CI is a safe procedure that significantly improves hearing threshold (p < 0.00001) and speech perception (p < 0.01). Support of family and professionals, as well as duration of deafness and pre-implant scores greatly influence the results of rehabilitation and its perceived benefit. CI should not be denied in older individuals who are otherwise in good health.

The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech–language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 ± 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.

Purpose To describe our institutional experience in cochlear implantation after vestibular schwannoma (VS) resection, and compare the audiological outcomes between sporadic and neurofibromatosis type 2 (NF2) VS sub-cohorts of patients, and in relation to preoperative contralateral hearing. Methods Seventeen patients (8 sporadic and 9 NF2-associated VSs) who had undergone VS resection and cochlear implant (CI) were analyzed retrospectively. Audiological outcomes at 24 months were correlated with preoperative clinical variables. The results according to VS type (sporadic vs. NF2-associated) and contralateral hearing (impaired vs. normal) were compared. Results Fourteen CIs were actively used by the patients (77.8%). Twenty-four months after CI activation, the median postoperative PTA (pure tone average) was 45.6 dB nHL and a measurable WRS (Word Recognition Score) was achieved by 44.4% of patients (median WRS = 40%). The median postoperative PTA in the implanted ear resulted better in the group with an impaired contralateral hearing (36.3 dB nHL vs. 78.8 dB nHL, p  = 0.019). Good preoperative contralateral hearing status (A–B classes of AAO-HNS) was a negative prognostic factor for CI performance on open-set discrimination (OR = 28.0, 95% CI 2.07–379.25, p  = 0.012). Conclusions CI is a viable rehabilitative option for patients with sporadic or NF2-associated VS. A good contralateral hearing adversely affects CI outcome and should be taken into consideration for patients’ selection and rehabilitation programs.

During COVID-19 pandemic, protective measures such as social distancing and face masks posed a challenge in daily communication, in this context the elderly are one of the most at risk categories as widely exposed to hearing loss. This article focuses on how the COVID-19 pandemic affected verbal communication, especially on those people that even in normal conditions present an increased difficulty in speech perception. Special attention has been paid to hearing aids and cochlear implant users, these devices indeed can be affected by a speech intelligibility reduction and could be uncomfortable if used together with face masks. Possible alternatives and solutions will be proposed to reduce the negative impacts of face coverings on communication, to enhance speech intelligibility and to manage wearability of hearing rehabilitation devices.