Fayez Bahmad

Electrocochleography for Ménière's disease: is it reliable?,

INTRODUCTION Endolymphatic hydrops, the histopathological substrate of Ménières disease, is an almost universal finding in postmortem studies of patients with this disease. The cause of hydrops is still unknown, as is the mechanism by which it causes progressive dysfunction of the sensory organs of inner ear. The fluctuating course of the disease complicates the interpretation of certain tests, such as electrocochleography; thus, for some authors its diagnostic value is questionable. OBJECTIVE The aim of this study was to analyze the clinical applicability of electrocochleography in the diagnosis of hydrops. It is a valuable tool, but still generates conflicting opinions among otolaryngologists. METHODS Systematic review of the literature on electrocochleography in patients diagnosed with endolymphatic hydrops. RESULTS A total of 34 articles regarding the use of electrocochleography in patients with hydrops, from the year 2000 onwards, were selected. Of these, 15 were excluded from the review as they were not observational studies. Only one cross-sectional study addressing the clinical use of electrocochleography by otolaryngologists was included. CONCLUSION Electrocochleography is a valuable tool in the diagnosis of hydrops, as it is a non-invasive, easy to handle procedure, which offers new techniques to increase the sensitivity of the test, and thereby assists otolaryngologists in the management of Ménières disease.

Evaluation of vestibular evoked myogenic potentials (VEMP) and electrocochleography for the diagnosis of Ménière's disease

INTRODUCTION Ménières disease (MD) is an inner ear disorder characterized by episodic vertigo, tinnitus, ear fullness, and fluctuating hearing. Its diagnosis can be especially difficult in cases where vestibular symptoms are present in isolation (vestibular MD). The definitive diagnosis is made histologically and can only be performed post-mortem, after analysis of the temporal bone. Endolymphatic hydrops is a histopathological finding of the disease and occurs more often in the cochlea and saccule, followed by the utricle and semicircular canals. Vestibular evoked myogenic potentials (VEMP) emerged as the method of assessment of vestibular function in 1994. Until then, there was no unique way of assessing saccular function and the inferior vestibular nerve. Given that the saccule is responsible for most cases of severe hydrops, VEMP appears as a new tool to assist in the diagnosis of MD. OBJECTIVE To evaluate the sensitivity and specificity of VEMP and electrocochleography (EcochG) in the diagnosis of definite MD compared with clinical diagnosis. METHODS The study includes 12 patients (24 ears) diagnosed with definite MD defined according to the clinical criteria proposed by the American Academy of Otolaryngology - Head and Neck Surgery (AAO-HNS) in 1995, as well as 12 healthy volunteers allocated to the control group (24 ears). A clinical diagnosis by the AAO-HNS criteria was considered as the gold standard. All patients underwent an otoneurological examination, including pure tone and speech audiometry, VEMP, and extratympanic EcochG. The sensitivity and specificity to detect the presence or absence of disease were calculated, as well as their 95% confidence intervals. The reliability of VEMP and EcochG in both ears was assessed using the kappa index. RESULTS In both tests and in both ears, the ability to diagnose healthy cases was high, with specificity ranging from 84.6% to 100%. Moreover, the ability of the tests to diagnose the disease varied from low to moderate sensitivity, with values ranging from 37.5% to 63.6%. The agreement of both tests in the right ear, measured by the kappa coefficient, was equal to 0.54 (95% CI: 0.20-0.89), indicating a moderate agreement. In the left ear, that agreement was equal to 0.07 (95% CI: -0.33 to 0.46), indicating a weak correlation between the tests. The sensitivity of the VEMP for the right ear was 63.6% and for the left ear, 62.5%. The sensitivity of EcochG for the right ear was 63.6% and 37.5% for the left ear. CONCLUSION The specificity of both tests was high, and the sensitivity of VEMP was higher than that of EcochG.

Familial Alström syndrome: a rare cause of bilateral progressive hearing loss,

INTRODUCTION Alström Syndrome is a rare disease caused by mutations in ALMS1 gene. It is characterized by a progressive degeneration of sensory functions, resulting in visual and audiological impairment, as well as metabolic disturbances such as childhood obesity, hyperinsulinemia, and diabetes mellitus type 2. OBJECTIVE To report and discuss the genetic and audiological findings in two siblings with Alström syndrome. METHODS This was a prospective, analytical and descriptive study, using questionnaires, serial audiograms, otoacoustic emissions, and auditory brainstem response analysis, as well as molecular genetic analysis. RESULTS Both patients presented childhood-onset bilateral sensorineural hearing loss, which progressed to moderate impairment in the first case and severe hearing loss in the second. Otoacoustic emissions were absent, and auditory brainstem responses were bilaterally normal in both cases. CONCLUSION In the present patients, Alström Syndrome began with a neurosensory hearing loss in early childhood that progressed to a profound loss in ten to twenty years. The auditory lesions were cochlear in origen according to the otoacoustic emissions and auditory brainstem responses.

Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation

INTRODUCTION Auditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals. OBJECTIVE To verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy. METHODS A prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation. RESULTS Two participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications. CONCLUSION The use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills.

Facial Palsy-Specific Quality of Life in 920 Patients: Correlation With Clinician-Graded Severity and Predicting Factors: Quality of Life in Facial Palsy

To investigate the correlation between facial palsy severity and quality of life in a broad cohort of facial palsy patients and to elucidate factors that influence this relationship.

Hearing Rehabilitation through Bone-Conducted Sound Stimulation: Preliminary Results

Introduction  The bone-anchored hearing aid (BAHA) is a bone conduction system that transmits the sound directly to the inner ear by surpassing the skin impedance and the subcutaneous tissue. It is indicated for patients with mixed, conductive and unilateral sensorineural hearing loss who did not benefit from conventional hearing aids (HAs). Although the benefits from BAHA are well demonstrated internationally, this field still lacks studies in Brazil. Objective  To assess the auditory rehabilitation process in BAHA users through audiological, speech perception and tinnitus aspects. Methods  Individuals with hearing loss were assessed before and after the implantation. The participants were subjected to pure tone audiometry in free field, functional gain audiometry, speech perception tests, tinnitus handicap inventory (THI) in open format, and to the visual analog scale (VAS). Results  It was found that the participants benefited from the use of BAHA. The difference in the performance of the participants before and after the BAHA surgery was significant in terms of hearing acuity. There was no statistically significant difference in the speech perception tests. The tinnitus assessment showed that 80% of the participants scored slight tinnitus severity in THI after using a BAHA. Eighty percent of the participants classified their tinnitus as absent to mild in the VAS after the surgery. Conclusion  Based on the results of the current study, we can conclude that the participants improved both the auditory perception and the tinnitus handicap.

Polysomnographic Findings in Patients With Chronic Tinnitus

Objectives: Tinnitus is an auditory sensation in the absence of any external stimulus. It has a negative impact on quality of life and interferes with concentration, sleep, social activities, and even emotional stability. The aim of this study was to compare sleep architecture in patients with and without chronic subjective tinnitus. Methods: This was an observational, noninterventional, and prospective study. The sample consisted of 50 individuals of both sexes aged 20 to 60 years. Twenty-five patients with tinnitus constituted the study group, and for comparison, a control group consisting of 25 patients without reported tinnitus was formed. The patients underwent polysomnography and were administered the Epworth Sleepiness Scale, Tinnitus Handicap Inventory, and visual analog scales. Results: The group with tinnitus had higher mean values in sleep stages 1 and 2, and lower mean values in stage 3 and in rapid eye movement (REM) sleep, compared with the control group, and this difference was significant only for REM sleep (P = .031). This demonstrates that patients with tinnitus remained longer in shallow sleep and spent less time in deep sleep (stage 3) and REM sleep. Conclusions: This study shows that patients with tinnitus have significant alterations in REM sleep latency as well as the REM sleep phase.