Preceding Benign Paroxysmal Positional Vertigo as a Trigger for Persistent Postural-Perceptual Dizziness: Which Clinical Predictors?

OBJECTIVE: Persistent postural-perceptual dizziness (PPPD) is a syndrome described as secondary, when it is the consequence of an organic disorder (s-PPPD), or primary, when no somatic triggers can be identified. We evaluated a group of patients diagnosed as s-PPPD, with Benign Positional Paroxysmal Vertigo (BPPV) as the main somatic trigger, with the aim of identifying the predictive clinical elements of evolution towards PPPD. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: We evaluated 126 patients diagnosed with PPPD; 54 patients were classified as p-PPPD (43%) and 72 as s-PPPD (57%). Of these, 51 patients had BPPV as a somatic trigger of PPPD, and in this group, we evaluated the prevalence of some clinical features (age, sex, latency between the onset of BPPV and the final diagnosis, recurrence of BPPV and the presence of migraine headache) for comparison with a group of patients who suffered from BPPV without an evolution towards PPPD (control group). RESULTS: In the group with PPPD secondary to BPPV, we found a significantly higher mean age and a longer latency between the onset of BPPV and the final diagnosis compared to the control group. No difference between the two groups was found regarding sex, recurrence rate and the presence of migraine headache. CONCLUSIONS: The parameters most involved as potential precipitants of PPPD after BPPV were the age of the patients and a long latency between the onset of BPPV and the final diagnosis; the mean age of the subjects who developed PPPD following BPPV was significantly higher. These findings lead us to emphasize the importance of the early identification and treatment of BPPV, especially in older patients.

Titanium Biohybrid Middle Ear Prostheses: A Preliminary In Vitro Study.

Ossiculoplasty is a surgical operation performed to restore auditory transmission through the reconstruction of the ossicular chain using prosthetics. Tissue bioengineering has assumed a pivotal role in implementing alternatives to conventional ossicular middle ear replacement prostheses, to overcome extrusion while preserving acoustic properties. This in vitro study aims to explore, for the first time in current literature, the feasibility of a biohybrid middle ear prosthesis, composed of titanium surrounded by a bone extracellular matrix as bio-coating. We have hereby studied the adhesion and proliferation of human adipose-derived mesenchymal stem cells (hASC) on titanium scaffolds in vitro. Moreover, we identified the osteogenic differentiation of hASC using an immunofluorescence assay to analyze osteoblasts' gene expression profiles (Alp, Runx2, Col1a1, Osx, and Bglap), and we counted the presence of collagen as a marker of hASC's ability to secrete an extracellular matrix. We utilized scanning electron microscopy to evaluate the presence of an extracellular matrix on the scaffolds. Our preliminary data demonstrated the titanium's ability to support human adipose-derived mesenchymal stem cell colonization, proliferation, and osteoblastic differentiation, in order to obtain a biohybrid device. Our experience seems encouraging; thus, we advocate for further in vivo research to corroborate our results regarding bone transplantation.

Cochlear implantation after head and neck radiotherapy: A multicentric study and systematic review.

OBJECTIVE: The objective of this study is to assess whether cochlear implantation is feasible in patients treated with radiotherapy of the temporal bone (for diseased other than vestibular schwannoma), in terms of surgical management and auditory outcome. METHODS: A systematic review of the literature was performed, screening PubMed, Embase, and Scopus databases, according to PRISMA criteria, retrieving 12 articles. Moreover, 9 cases coming from a multicentric study involving Padova and Pisa University Hospitals, were added, for a total of 62 cases of irradiated patients receiving cochlear implantation. RESULTS: In our multicentric study we included a total of 9 patients, 6 males, and 3 females (mean age at first cochlear implantation was 53.89 years ± 21.07), while from the literature we considered 53 cases (mean age at first cochlear implantation 48.78 years ± 12.41). We considered for both groups a set of preoperative imaging abnormalities, intraoperative complications and possible postoperative complications. In our cohort, the preoperative pure tone average (PTA) in the implanted ear was 105.6 dB, and the postoperative one was 34.6 dB. In the literature the preoperative PTA was 111.5 dB (when reported), and the postoperative one 52.8 dB. CONCLUSION: Both our experience and the literature suggest that cochlear implantation in an irradiated ear is feasible with a good auditory outcome. Surgical difficulties and fitting challenges can be easily managed by professionals aware of these findings, making these patients good candidates to this rehabilitative option.

The Role of Bone-Anchored Hearing Devices and Remote Microphones in Children with Congenital Unilateral Hearing Loss.

Congenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA). This was a retrospective observational study on a group of children with UHL. The Simplified Italian Matrix Sentence Test was used for the assessment of speech reception threshold (SRT) in different conditions: speech and noise from the front (S0N0), speech at 45° from the side of the better ear and noise at 45° from the opposite side (SbNw), and vice versa (SwNb). Each test was conducted unaided, with a bone-anchored hearing device (BAHD), and with a remote microphone (RM) system. The use of a BAHD and RM led to an improvement in SRT in S0N0 and SwNb conditions. The SSD subgroup demonstrated significant benefits with both devices in SwNb, and the UAA subgroup from the use of BAHD in S0N0. In conclusion, the study underscores the potential benefits of both devices in enhancing speech perception for UHL children, providing insights into effective intervention strategies for these challenging cases.

Extrusion and Dislocation in Titanium Middle Ear Prostheses: A Literature Review.

Titanium middle ear (ME) prostheses are widely used in surgical practice due to their acoustic properties. However, they present a significant drawback shared by all synthetic materials currently in use for ME reconstruction: they can be rejected by the organism of the host. In this study, we aim to review the current literature on titanium partial ossicular replacement prostheses (PORPs) and total ossicular replacement prostheses (TORPs) extrusion and dislocation. Eighty articles were analysed after a full article review based on the inclusion and exclusion criteria. The most common indication for reconstruction was chronic otitis media with cholesteatoma. The average extrusion or dislocation rate was 5.2%, ranging from 0 to 35%. The average improvements in the air-bone gap were 12.1 dB (1.6 dB to 25.1 dB) and 13.8 (-0.5 dB to 22.7 dB) for the PORP and TORP groups, respectively. The data reported on this topic are highly variable, demonstrating that functional outcomes are difficult to predict in clinical practice. We believe that the current limitations could be overcome with technological developments, including bioengineering research focused on promoting prosthesis adaptation to the ME environment.

Current trends on subtotal petrosectomy with cochlear implantation in recalcitrant chronic middle ear disorders.

Objective: To establish the safety and effectiveness of subtotal petrosectomy with cochlear implantation in patients affected by chronic middle ear disorders to refractory to previous surgical treatments. Methods: A multicentre, retrospective study was conducted on patients affected by recalcitrant chronic middle ear disorders who underwent cochlear implantation in combination with subtotal petrosectomy. Patients' details were collected from databases of 11 Italian tertiary referral centres. Additionally, a review of the most updated literature was carried out. Results: 55 patients were included with a mean follow-up time of 44 months. Cholesteatoma was the most common middle ear recurrent pathology and 50.9% of patients had an open cavity. 80% of patients underwent a single stage surgery. One case of explantation for device failure was reported among the 7 patients with post-operative complications. Conclusions: Subtotal petrosectomy with cochlear implantation is a benchmark for management of patients with recalcitrant chronic middle ear disorders. A single stage procedure is the most recommended strategy. Optimal follow-up is still debated. Further studies are required to investigate the role of this surgery in paediatric patients.

Effects of a short period of postural training on postural stability and vestibulospinal reflexes.

The effects of postural training on postural stability and vestibulospinal reflexes (VSRs) were investigated in normal subjects. A period (23 minutes) of repeated episodes (n = 10, 50 seconds) of unipedal stance elicited a progressive reduction of the area covered by centre of pressure (CoP) displacement, of average CoP displacement along the X and Y axes and of CoP velocity observed in this challenging postural task. All these changes were correlated to each other with the only exception of those in X and Y CoP displacement. Moreover, they were larger in the subjects showing higher initial instability in unipedal stance, suggesting that they were triggered by the modulation of sensory afferents signalling body sway. No changes in bipedal stance occurred soon and 1 hour after this period of postural training, while a reduction of CoP displacement was apparent after 24 hours, possibly due to a beneficial effect of overnight sleep on postural learning. The same period of postural training also reduced the CoP displacement elicited by electrical vestibular stimulation (EVS) along the X axis up to 24 hours following the training end. No significant changes in postural parameters of bipedal stance and VSRs could be observed in control experiments where subjects were tested at identical time points without performing the postural training. Therefore, postural training led to a stricter control of CoP displacement, possibly acting through the cerebellum by enhancing feedforward mechanisms of postural stability and by depressing the VSR, the most important reflex mechanism involved in balance maintenance under challenging conditions.

Surgical Treatment of an Endolymphatic Sac Tumor.

Endolymphatic sac tumors (ELST) are low-grade papillary adenocarcinoma originating from the endolymphatic sac. Usually slow-growing, with local aggressiveness and a low risk of distant metastases, ELST can be sporadic but also frequently associated with von Hippel Lindau disease. The current treatment of ELST is primarily surgical resection. A 55-year-old woman accessed our otologic tertiary level referral center for a sudden worsening of hearing loss in her left ear and vertigo. A magnetic resonance (MRI) and computer tomography scan study subsequently showed a mass in the petrous bone; hence, the presence of an ELST was hypothesized. After embolization of the mass, the patient underwent surgical removal of the lesion. The resection of the mass was done through a translabirinthine approach, with an uneventful procedure. No residual disease remained after surgery. After 24 months of radiologic follow up with MRI, there are no signs of recurrence disease. This paper reports the management of this sporadic ELST, as well as the follow up results, providing clinicians this protocol for the handling of such a challenging otologic skull base surgery and rare disease.

Temperature-Sensitive Auditory Neuropathy: Report of a Novel Variant of OTOF Gene and Review of Current Literature.

Background and objectives: Otoferlin is a multi-C2 domain protein implicated in neurotransmitter-containing vesicle release and replenishment of the cochlear inner hair cell (IHC) synapses. Mutations in the OTOF gene have been associated with two different clinical phenotypes: a prelingual severe-to-profound sensorineural hearing loss (ANSD-DFNB9); and the peculiar temperature-sensitive auditory neuropathy (TS-ANSD), characterized by a baseline mild-to-moderate hearing threshold that worsens to severe-to-profound when the body temperature rises that returns to a baseline a few hours after the temperature has fallen again. The latter clinical phenotype has been described only with a few OTOF variants with an autosomal recessive biallelic pattern of inheritance. Case report: A 7-year-old boy presented a picture compatible with TS-ANSD exacerbated by febrile states or physical exercise with mild-to-moderate hearing loss at low and medium frequencies and a decrease in speech discrimination that worsened with an unfavorable speech-to-noise ratio. Otoacoustic emissions (OAEs) were present whereas auditory brainstem responses (ABRs) evoked by a click or tone-burst were generally absent. No inner ear malformations were described from the CT scan or MRI. Next-generation sequencing (NGS) of the known deafness genes and multi-phasic bioinformatic analyses of the data detected in OTOF a c.2521G>A missense variant and the deletion of 7.4 Kb, which was confirmed by array-comparative genomic hybridization (array-CGH). The proband's parents, who were asymptomatic, were tested by Sanger sequencing and the father presented the c.2521G>A missense variant. Conclusions: The picture presented by the patient was compatible with OTOF-induced TS-ANSD. OTOF has been generally associated with an autosomal recessive biallelic pattern of inheritance; in this clinical report, two pathogenic variants never previously associated with TS-ANSD were described.

Battery Lifespan of an Implantable Middle Ear Device.

BACKGROUND: The Carina system (Cochlear Ltd., Sydney, Australia) is a totally implantable device providing acoustic amplification in adult patients with moderate-to-severe sensorineural or mixed hearing loss. One of the main concerns about such a totally implantable device has been represented by the subcutaneous battery lifespan. The aim of this article is to report the analysis of battery performances in a series of Carina-implanted patients after a long follow up. METHODS: In this retrospective study, the technical data of a series of patients implanted with the Carina middle ear implant in our clinic have been analysed, extracting the data from the log of telemetric measures. RESULTS: The mean lifespan cutback was 0.43 h/years (from 0 to 0.71 h/year), with a strong negative significant correlation between the follow-up period and the percentage of battery residual lifespan. CONCLUSION: The lifespan of the Carina's battery seems consistent with the manufacturer statement of a pluri-decennial lifespan, avoiding the need of an early surgical substitution and providing a full day of use of the system even after up to 12 years from the implantation.

Bone-Anchored Hearing Aids Fitted According to NAL and DSL Procedures in Adults with Mixed Hearing Loss.

BACKGROUND: Bone-anchored hearing aids represent a valid alternative for patients with conductive/mixed hearing loss who cannot use hear- ing aids. To date, these devices have given good audiological results, thanks to various fitting prescription programs (i.e., National Acoustic Laboratories and Desired Sensation Level). The aim of this study is to compare 2 types of fitting algorithms (National Acoustic Laboratories and Desired Sensation Level) implemented for bone-anchored hearing devices. METHODS: We retrospectively enrolled 10 patients followed at our operative unit, suffering from bilateral symmetrical mixed hearing loss and who underwent bone-anchored hearing aid implantation. All patients experienced each prescriptive procedure, National Acoustic Laboratories and Desired Sensation Level, for 7 months (on average), and they were subjected to audiological tests and questionnaires to evaluate the best program. RESULTS: National Acoustic Laboratories and Desired Sensation Level prescriptions yielded similar results. Desired Sensation Level allowed less amplification of the low frequencies than the National Acoustic Laboratories prescription, and these differences were the only statistically sig- nificant. Desired Sensation Level allowed better disyllabic word and sentence recognition scores only in quiet and not in noisy conditions. The subjective questionnaires showed similar results. At the end of the trial sessions, more patients (60%) definitively chose the Desired Sensation Level program for their device. These patients were those with a worse hearing threshold. CONCLUSION: The 2 prescriptive programs allowed similar results although patients with a worse threshold seem to prefer the DSL program. This is the first evaluation of the 2 prescriptive programs, National Acoustic Laboratories versus Desired Sensation Level, for bone conduction devices available in the literature. Further studies are needed to confirm this initial finding.

Functional outcomes and complications in refractory dysthyroid optic neuropathy management: Experience with 3 different surgical protocols.

INTRODUCTION: Dysthyroid optic neuropathy (DON) is the most severe complication of Graves' orbitopathy (GO) and its management may require decompression surgery. Clear recommendations do not exist about which surgery should be performed and how extended the decompression should be. In this paper we present our experience regarding the management of DON via 3 different surgical protocols: a modified extended orbital apex decompression, a 2 walls decompression (inferior and lateral) and a 3 walls decompression (inferior, lateral and medial) and evaluate the functional outcomes. METHODS: Retrospective evaluation of subjects affected by DON not responding to medical therapy has been performed. All patients were submitted to pre- and post-operative ophthalmologic evaluations and orbital and sinuses CT scan in order to evaluate functional and surgical outcomes. RESULTS: 27 patients were enrolled in the study. Surgical procedures were performed on 42 orbits. A statistically significant post-operative improvement was recorded in visual acuity, proptosis, color vision and fundus oculi evaluation for all groups. No patient developed major or minor complications after surgery. CONCLUSIONS: Extended endonasal approach and 3 walls decompression have been proved effective in the management of DON. The choice between them is done according to degree of proptosis, general status and eye-surface damages.

A Rare Case of Perrault Syndrome with Auditory Neuropathy Spectrum Disorder: Cochlear Implantation Treatment and Literature Review.

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.

The Role of the Functional Head Impulse Test with and without Optokinetic Stimuli in Vestibular Migraine and Acute Unilateral Vestibulopathy: Discovering a Dynamic Visual Dependence.

(1) Background: Visually induced vertigo (i.e., vertigo provoked by moving visual scenes) can be considered a noticeable feature of vestibular migraines (VM) and can be present in patients suffering from acute unilateral vestibulopathy (AUV). Hypersensitivity to moving or conflicting visual stimulation is named visual dependence. (2) Methods: Visuo-vestibular interactions were analyzed via the functional Head Impulse Test (fHIT) with and without optokinetic stimulation (o-fHIT) in 25 patients with VM, in 20 subjects affected by AUV, and in 20 healthy subjects. We calculated the percentage of correct answers (%CA) without and with the addition of the optokinetic background (OB). (3) In VM groups, the %CA on the fHIT was 92.07% without OB and 73.66% with OB. A significant difference was found between %CA on the deficit side and that on the normal side in AUV, both without OB and with OB. (4) Conclusions: The fHIT results in terms of %CA with and without OB could be useful to identify the presence of a dynamic visual dependence, especially in patients suffering from VM. The difference in %CA with and without OB could provide instrumental support to help correctly identify subjects suffering from VM. We propose the use of the fHIT in clinical practice whenever there is a need to highlight a condition of dynamic visual dependence.

Extended-hearing targeted screening for congenital cytomegalovirus infection.

BACKGROUND: The importance of neonatal screening for congenital cytomegalovirus infection (cCMV) is widely shared in the world scientific literature. However, currently, no programs for universal neonatal screening for cCMV have been reported in the literature, and only a few experiences of hearing targeted cCMV screening have been published. In the Tuscany Region, Italy, screening for cCMV has been mandatory since 2008 for each newborn that result "refer" at the Transient Evoked Otoacoustics Emissions (TEOAE) test, and in our university hospital since 2012, it has been extended to some categories at risk of cCMV. METHODS: We present the results of the protocol for cCMV screening adopted at our Institution since 2012. RESULTS: From 2012 to 2017, 1615 newborns underwent cCMV screening. Twenty-five cases were positive for cCMV, (1.54% of all the newborn screened for cCMV and 0.19% of infants submitted to newborn hearing screening). Nineteen of these children (76%) had normal hearing, while 6 (24%) had a hearing deficit of variable degree. 2/25 (8%) cases presented a progression of the hearing deficit in the first months of life and no children had a late onset or fluctuating hearing loss. CONCLUSIONS: Our findings show how cCMV screening in newborns that result refer at TEOAE allows the detection of many cases; but many are still missed. It would be, therefore, important to adopt a universal newborn cCMV screening program or a program extended to newborns at higher risk.

Cochlear implant in prelingually hearing-impaired adults: prognostic factors and results.

OBJECTIVES: The aim of present study is to evaluate the impact of prognostic factors on the outcome in a group of prelingually hearing-impaired patients submitted to cochlear implantation (CI) at an adult age. METHODS: This is a retrospective study on a cohort of prelingually severe-to-profound hearing-impaired patients cochlear implanted in adulthood and followed by a single audiology centre. We correlated post-CI results in term of speech perception with patients' speech perception with hearing aids before implantation, history of progression of hearing loss (HL), and levels of education and cognition. The study group was composed of 49 patients. RESULTS: Post-CI open-set recognition score in silence and noise was significantly correlated with pre-CI open-set recognition score in silence and with background noise. Patients with a history of progression of HL gained significantly better results. Furthermore, we found higher improvements in patients with a higher level of education. CONCLUSIONS: Prelingually deafened patients implanted in adulthood achieved satisfactory results. Significantly better results were achieved by patients with better pre-operative speech perception scores, progressive HL and higher level of education.

Prognosis after acute unilateral vestibulopathy: Usefulness of the suppression head impulse paradigm (SHIMP).

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.

Lichen planus of the external auditory canal: Treatment options and review of literature.

Medical topic therapies can relieve symptoms associated with lichen planus of external auditory canal. Further, bone-anchored hearing devices represent an optimal solution for hearing restoration in otic lichen planus.

Cochlear Implant in the Elderly: Results in Terms of Speech Perception and Quality of Life.

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.

Newborn hearing screening protocol in tuscany region.

BACKGROUND: Newborn 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 PART: In 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. CONCLUSIONS: In 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.