Italian fast speech reception threshold test, a new method to investigate adults auditory impairment in noise.

INTRODUCTION: Purpose of our study is to compare two competing methods of performing bisyllabic words speech audiometry for the detection of the 50% speech reception threshold in noise (SRT50). METHODS: Classic method is performed submitting multiple words lists at fixed signal to noise ratio. A newer Fast method - Italian Fast Speech Reception Threshold 50 (IFastSRT50) - is performed by means of a program software with a single list of bisyllabic words and noise intensity shifting. RESULTS: Means comparison between SRT50 Classic and IFastSRT50 shows a slight significant correlation (r=0.263; p=0.044) and a wide significant difference: SRT50 Classic=-2.763dB (SD=4.1) and IFastSRT50=-7.803dB (SD=2.1) (P < 0.0001). There is high difference between test execution time means (SRT50 Classic=11min, IFastSRT50 =2min; P < 0.0001). Correlation between test results and execution times was higher in for SRT50 Classic than IFastSRT50. CONCLUSION: IFastSRT50 test is a reliable method to quickly investigate signal to noise ratio needed to obtain 50% of recognition scores with bisyllabic words, it allows less execution time than SRT50 Classic method and can avoid patients fatigue and other limitations of different speech discrimination tests in noise as sentences based ones.

3D Printed Piezoelectric BaTiO3/Polyhydroxybutyrate Nanocomposite Scaffolds for Bone Tissue Engineering.

Bone defects are a significant health problem worldwide. Novel treatment approaches in the tissue engineering field rely on the use of biomaterial scaffolds to stimulate and guide the regeneration of damaged tissue that cannot repair or regrow spontaneously. This work aimed at developing and characterizing new piezoelectric scaffolds to provide electric bio-signals naturally present in bone and vascular tissues. Mixing and extrusion were used to obtain nanocomposites made of polyhydroxybutyrate (PHB) as a matrix and barium titanate (BaTiO3) nanoparticles as a filler, at BaTiO3/PHB compositions of 5/95, 10/90, 15/85 and 20/80 (w/w%). The morphological, thermal, mechanical and piezoelectric properties of the nanocomposites were studied. Scanning electron microscopy analysis showed good nanoparticle dispersion within the polymer matrix. Considerable increases in the Young's modulus, compressive strength and the piezoelectric coefficient d31 were observed with increasing BaTiO3 content, with d31 = 37 pm/V in 20/80 (w/w%) BaTiO3/PHB. 3D printing was used to produce porous cubic-shaped scaffolds using a 90° lay-down pattern, with pore size ranging in 0.60-0.77 mm and good mechanical stability. Biodegradation tests conducted for 8 weeks in saline solution at 37 °C showed low mass loss (∼4%) for 3D printed scaffolds. The results obtained in terms of piezoelectric, mechanical and chemical properties of the nanocomposite provide a new promising strategy for vascularized bone tissue engineering.

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.

The Audiological Follow-Up of Children with Symptomatic Congenital Cytomegalovirus Infection: An Experience in Two Italian Centers.

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is the leading cause of non-hereditary sensorineural hearing loss in children. While about 10% of children reportedly display symptoms at birth, 85-90% of cCMV infection cases are asymptomatic. However, 10-15% of these asymptomatic infants may later develop hearing, visual, or neurodevelopmental impairments. This study aimed to evaluate the impact of cCMV infection on newborns' hearing function with a particular emphasis on progressive and late-onset cases. METHODS: This study is a retrospective chart analysis with longitudinal character and was conducted in two Italian centers: Center 1 (from 1 November 2007 to 31 December 2021) and Center 2 (from 1 January 2012 to 31 December 2021). Data collected included newborn hearing screening results, characterization of hearing loss (unilateral/bilateral, degree of impairment), and audiological follow-up. RESULTS: The cohort consisted of 103 children (42% males, 58% females). In total, 28 children presented with hearing impairment; 71.4% (20 out of 28) of the cases of hearing loss were severe/profound, with 35.7% of the cases due to unilateral hearing loss. Out of twenty-eight, six experienced progression of hearing loss and four had late-onset hearing loss. CONCLUSIONS: In the absence of universal cCMV screening, hearing screening at birth for cCMV remains a critical factor for early diagnosis. A significant percentage of children affected by cCMV with normal audiological evaluations at birth is easily lost to follow-up. Close collaboration between neonatologists, pediatricians, and audiological services is fundamental to ensure timely diagnosis and treatment of cCMV-related hearing loss.

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.

Piezoelectric Signals in Vascularized Bone Regeneration.

The demand for bone substitutes is increasing in Western countries. Bone graft substitutes aim to provide reconstructive surgeons with off-the-shelf alternatives to the natural bone taken from humans or animal species. Under the tissue engineering paradigm, biomaterial scaffolds can be designed by incorporating bone stem cells to decrease the disadvantages of traditional tissue grafts. However, the effective clinical application of tissue-engineered bone is limited by insufficient neovascularization. As bone is a highly vascularized tissue, new strategies to promote both osteogenesis and vasculogenesis within the scaffolds need to be considered for a successful regeneration. It has been demonstrated that bone and blood vases are piezoelectric, namely, electric signals are locally produced upon mechanical stimulation of these tissues. The specific effects of electric charge generation on different cells are not fully understood, but a substantial amount of evidence has suggested their functional and physiological roles. This review summarizes the special contribution of piezoelectricity as a stimulatory signal for bone and vascular tissue regeneration, including osteogenesis, angiogenesis, vascular repair, and tissue engineering, by considering different stem cell sources entailed with osteogenic and angiogenic potential, aimed at collecting the key findings that may enable the development of successful vascularized bone replacements useful in orthopedic and otologic surgery.

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.

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.

Binaural hearing restoration with a bilateral fully implantable middle ear implant.

AIM: The fully implantable middle ear implant (C-FI-MEI) is designed for patients with moderate-to-severe sensorineural hearing loss or those with mixed hearing loss. To analyze the audiological post-operative results of subjects bilaterally implanted with C-FI-MEI. MATERIALS AND METHODS: Retrospective study: 14 patients with bilateral, moderate-to-severe, sensorineural or mixed hearing loss were treated. This clinical sample included 14 cases bilaterally implanted (13 sequentially, 1 simultaneously). The evaluation at each follow-up after surgery included otologic examination, a structured interview, and different audiological tests composed of pure tone audiometry, speech in quiet and in noise test, and localization task. The mean follow-up was 67.2 ± 33 months. RESULTS: There were no significant differences between pre and post-operative pure tone averages. The patients showed no significant differences between pre-operatively aided and C-FI-MEI implant-aided conditions in terms of word recognition score. Speech perception in noise under different loudspeaker arrangements and localization tests demonstrated a binaural advantage in bilaterally implanted patients. The mean daily use time was 17.4 and 16.7 h, respectively, for right and left side. CONCLUSION: The results for the 14 patients, bilaterally implanted with C-FI-MEI, suggest that bilateral implantation of fully implantable middle ear hearing devices is an effective procedure. LEVEL OF EVIDENCE: 4.

Bone Anchored Hearing Aids for the Treatment of Asymmetric Hearing Loss.

OBJECTIVES: Asymmetric hearing loss is generally defined as a moderate-to-profound hearing loss in the poorer ear and a mild-to-moderate hearing loss in the better ear. Application of a bone conduction hearing aid is one of the possible treatments for the poorer ear in asymmetric hearing loss. However, the device essentially stimulates the contralateral better ear, precluding true binaural hearing. The aim of this paper is to evaluate the application and utility of bone-anchored hearing aids in the treatment of asymmetric hearing loss. MATERIALS AND METHODS: We retrospectively evaluated 215 implanted subjects in our clinic and extracted a series of 27 patients affected by asymmetric hearing loss and treated with bone-anchored hearing aids. All 27 subjects had a mixed hearing loss after middle ear surgery. The preoperative and postoperative audiological data of these patients were collected and analyzed. RESULTS: The audiological tests showed an improvement in the performance of hearing perception of sound and speech in quiet and noise. Moreover, the subjects have positively answered the questionnaires administered to evaluate subjective benefits. All subscales of the abbreviated profile of hearing aid benefit and speech, spatial, and qualities of hearing scale showed a significant improvement with the device. CONCLUSION: Bone-anchored hearing aids are a suitable treatment for asymmetric hearing loss. When other devices cannot be utilized or are not indicated, the bone conduction devices may allow good audiological results.

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.

Middle Ear Neuroendocrine Adenoma: A Case Report and Literature Review.

Neuroendocrine adenomas of the middle ear are rare tumors that represent less than 2% of primary tumors of the ear. In this paper, we describe a case of a 40-year-old woman who developed neuroendocrine adenoma of the middle ear. The specific management strategy for this rare tumor is unclear; information in the available literature on the management of this tumor is varied. However, an extensive demolition seems to be the gold standard treatment for this tumor to avoid recurrence and regional metastases in the lymph node or distant metastases. For the present case, we performed an incisional biopsy to confirm the diagnosis, and thereafter, we performed a canal-wall-down tympanoplasty. For cases like the present one, careful long-term clinical and instrumental follow-up is required to monitor progress and facilitate patient recovery.

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.

Extensive Skull Base Osteomyelitis Secondary to Malignant Otitis Externa.

Skull base osteomyelitis is a severe complication of malignant otitis externa that affects the marrow of the temporal, sphenoid, and occipital bones. Skull base osteomyelitis is usually diagnosed based on clinical, microbiological, and radiological findings. Here, we present the imaging findings of a 76-year-old man who initially presented with right-sided malignant otitis externa, with the involvement of the otomastoid structures and ipsilateral temporal bone. Over the following 3 years, despite specific extended antibiotic therapy, the skull base osteomyelitis entirely involved the skull base, up to the contralateral petrous portion of the temporal bone, and it affected the cervical vertebral processes. This report describes an exceptional extent of unilateral malignant otitis externa with a severe involvement of the skull base on the contralateral side and the cervical spine.