A potential serological biomarker for inner ear pathologies: OTOLIN-1.

Objective: The goal of the study was to determine whether the level of OTOLIN-1, a protein whose expression is highly restricted to the inner ear,is increased in the body fluids of patients with inner ear disorders in comparison to healthy subjects. Methods: In the preliminary part of the study, OTOLIN-1 levels were measured in the serum, urine, and saliva of patients with an acute onset of Ménière´s disease and in healthy individuals. Subsequently, only serum OTOLIN-1 levels were taken into account and were compared between patients with acute onset of Ménière´s disease, sudden hearing loss, vestibular neuritis and healthy subjects. Results: The most reliable diagnostic parameter was OTOLIN-1 levels in serum. Serum samples of patients with Ménière's disease and sudden hearing loss showed significantly higher OTOLIN-1 levels than those from healthy individuals. In addition, there was no significant difference between the serum concentration of OTOLIN-1 in patients with vestibular neuritis and the control group. Conclusions: Serum levels of OTOLIN-1 can potentially be used as a biomarker for acute onset of inner ear disorders due to its significant increase in patients with acute Meniere´s disease and sudden hearing loss in comparison to healthy individuals.

Advanced Imaging of the Vestibular Endolymphatic Space in Ménière's Disease.

The diagnosis of "definite" Méniére's disease (MD) relies upon its clinical manifestations. MD has been related with Endolymphatic Hydrops (EH), an enlargement of the endolymphatic spaces (ES) (cochlear duct, posterior labyrinth, or both). Recent advances in Magnetic Resonance (MR) imaging justify its increasing role in the diagnostic workup: EH can be consistently recognized in living human subjects by means of 3-dimensional Fluid-Attenuated Inversion-Recovery sequences (3D-FLAIR) acquired 4 h post-injection of intra-venous (i.v.) Gadolinium-based contrast medium, or 24 h after an intratympanic (i.t.) injection. Different criteria to assess EH include: the comparison of the area of the vestibular ES with the whole vestibule on an axial section; the saccule-to-utricle ratio ("SURI"); and the bulging of the vestibular organs toward the inferior 1/3 of the vestibule, in contact with the stapedial platina ("VESCO"). An absolute link between MD and EH has been questioned, since not all patients with hydrops manifest MD symptoms. In this literature review, we report the technical refinements of the imaging methods proposed with either i.t. or i.v. delivery routes, and we browse the outcomes of MR imaging of the ES in both MD and non-MD patients. Finally, we summarize the following imaging findings observed by different researchers: blood-labyrinthine-barrier (BLB) breakdown, the extent and grading of EH, its correlation with clinical symptoms, otoneurological tests, and stage and progression of the disease.

Comparison of superelastic nitinol stapes prostheses and platin teflon stapes prostheses.

INTRODUCTION: Stapes plasty is an established procedure to improve hearing in case of otosclerosis. By reinforcing the prostheses, an optimization of the prosthesis could be achieved. The aim of the study was to evaluate the audiological results of the Nitinol Superelastic stapes prosthesis compared with a Platin-Teflon prosthesis. MATERIAL AND METHODS: In a retrospecitve setting, the audiological results of nine patients who have been implanted with a Nitinol Alloy Superelastic stapes prothesis were compared with the results of ten patients, which have been implanted with a Platinum-Teflon piston prostesis. All operations were performed by the same experienced ear surgeon. RESULTS: The Nitinol-group presented an average of postoperative air-bone-gap over all frequencies (0.5-6 kHz) of all patients of 15.1 dB. In the Platin-Teflon group, the average of postoperative air-bone-gap was 14.9 dB. Moreover, the post surgery delta-bone-conduction pre- to post surgery showed no differences (0.5-6 kHz). There were no surgical complications in both groups. CONCLUSION: Based on the patient collective, stapes plasty using a Nitinol Superelastic stapes prosthesis is a sufficient method for improving the hearing conduction. DESIGN: Retrospective case series. SETTING: University Department.

Proteome Analysis of Human Perilymph Using an Intraoperative Sampling Method.

The knowledge about the etiology and pathophysiology of sensorineural hearing loss (SNHL) is still very limited. This study aims at the improvement of understanding different types of SNHL by proteome analysis of human perilymph. Sampling of perilymph was established during inner ear surgeries (cochlear implantation, vestibular schwannoma surgeries), and safety of the sampling method was determined by checking hearing threshold with pure-tone audiometry postoperatively. An in-depth shot-gun proteomics approach was performed to identify cochlear proteins and the individual proteome in perilymph of patients. This method enables the identification and quantification of protein composition of perilymph. The proteome of 41 collected perilymph samples with volumes of 1-12 µL was analyzed by data-dependent acquisition, resulting in overall 878 detected protein groups. At least 203 protein groups were solely identified in perilymph, not in reference samples (serum, cerebrospinal fluid), displaying a specific protein pattern for perilymph. Samples were grouped by patient's age and surgery type, leading to the identification of some proteins specific to particular subgroups. Proteins with different abundances between different sample groups were subjected to classification by gene ontology annotations. The identified proteins might serve as biomarkers to develop tools for noninvasive inner ear diagnostics and to elucidate molecular profiles of SNHL.

Outer ear canal sound pressure and bone vibration measurement in SSD and CHL patients using a transcutaneous bone conduction instrument.

The intraoperative and postoperative objective functional assessment of transcutaneous bone conduction implants is still a challenge. Here we compared intraoperative Laser-Doppler-vibrometry (LDV, Polytec Inc.) to measure vibration of the bone close to the implant to Outer Ear Canal Sound Pressure Level (OEC-SPL) measurements. Twelve single sided deafness (SSD) patients with contralateral intact ossicular chains and eight bilateral conductive hearing loss (CHL) patients were included in the study. SSD patients had a minor average air-bone-gap (ABG) of 0.4 ± 0.4 dB (0.5, 1, 2, 4 kHz mean value (MV) ± standard deviation (SD)) on the contralateral side where a normal transmission between cochlea and the tympanic membrane can be assumed. CHL patients had an impaired middle ear transmission with a mean ABG of 46.0 ± 7.9 dB (MV±SD). Vibration and OEC-SPL responses could reliably be recorded with a minimal signal-to-noise ratio of at least 12 dB. Average OEC-SPL on the contralateral side and intraoperative vibration measurements were strongly correlated in SSD (r2 = 0.75) and CHL (r2 = 0.86) patients. The correlation in individual results between OEC-SPL and vibration measurements was weak, indicating some underlying inter-individual variability. The high correlation of average responses showed that OEC-SPL are closely linked to bone vibration, although both cannot be equivalently used for intraoperative testing due to the high variability in individual results. On the other hand, OEC-SPL provides an easy and affordable measurement tool to monitor stability and functionality postoperatively using individual reference measurements. We observed no significant differences (t-test, p < 0.05) by comparing results from contralateral OEC-SPL in twelve SSD and eight CHL patients at frequencies between 0.5 and 8 kHz. This implies that the part of the measured sound pressure in the ear canal originating from the cochlea and emitted by the tympanic is not dominant and OEC-SPL is mainly due to vibration of the external ear-canal walls as the only other pathway of BC sound to reach the ear canal. In addition, the transcranial attenuation (contralateral outer ear canal sound pressure divided by ipsilateral) was compared to previous studies measuring vibration by LDV and accelerometer. The trend in the average transcranial attenuation in patients was similar to previous studies measuring the OEC-SPL with less than 5 dB difference.

Mutational analysis for GJB2, GJB6, and GJB3 genes in Campania within a universal neonatal hearing screening programme.

OBJECTIVE: To determine the incidence of GJB2 and GJB3 mutations and of two deletions upstream of the GJB6 gene in infants of the Campania region of southern Italy. DESIGN: DNA samples from non-syndromic hearing-impaired infants enrolled in a neonatal screening programme for sensorineural hearing loss were analysed by PCR and by direct sequencing. The audiological features of infants with biallelic GJB2 mutations were also examined to identify genotype-phenotype correlations. STUDY SAMPLE: Molecular analyses were carried out in 129 affected and five unaffected infants. RESULTS: A genetic etiology of hearing loss was identified in 28% of infants, including several at environmental risk of hearing loss. Neither GJB6 nor GJB3 (a gene not previously investigated in the Campania population) mutations were found. CONCLUSIONS: This study confirms the importance of universal neonatal hearing screening. The identification of a genetic cause in infants at environmental risk indicates that such infants should be included when investigating etiology. We confirm that also in our geographical area, c.35delG homozygotes tend to have severe symmetrical hearing loss, whereas hearing impairment is milder in compound heterozygotes.

The development of language in babies and the role of the family.

Nowadays for deaf children there are many new opportunities that provide enhanced ways of hearing stimulation.These concerns may result as much more significant as the family is engaged in the stimulation program. An NHS program in Campania was launched in 2005. According to these concerns, the family plays a central role both in the diagnostic and the therapeutic intervention of the impaired children. As paradigm of the important role of the family in the rehab project is a comparison between two kids in charge by our team. The outcomes are better in children who received stimulation by the family.