Can unilateral, progressive or sudden hearing loss be immune-mediated in origin?

OBJECTIVE: The aim of the present study was to demonstrate that the positivity of nonspecific immunological tests could be found not only in bilateral hearing loss but also in unilateral cases, either sudden or progressive. METHOD: An observational case series study included subjects suffering from unilateral or bilateral, sudden or progressive, symmetric or asymmetric sensorineural hearing loss (SNHL). All the patients underwent pure tone audiometry and the following battery of blood exams: anti-nuclear antibody (ANA), extractable nuclear antigen (ENA) antibody screening, anti-thyroperoxidase (anti-TPO), anti-thyroglobulin and anti-smooth muscle antibody (ASMA). RESULTS: The positivity to nonspecific immunological test was found in nearly 70% of the study groups. ASMA and ANA were found to be present in both bilateral and unilateral cases, without statistical difference. Considering the correlation between positivity/negativity and systemic autoimmune pathologies, in the bilateral forms of hearing loss, a high incidence of thyroid pathologies has been identified, with a higher percentage of systemic autoimmune diseases in respect to the normal population. CONCLUSIONS: The nonspecific autoimmune tests are worth to be performed also when SNHL is not bilateral and progressive, since an immunological mechanism could also underlie unilateral and sudden SNHL cases

Bone conductive implants in single sided deafness

Conclusion: The Bone Conductive Implants (BCI) showed to partly restore some of the functions lost when the binaural hearing is missing, such as in the single-sided deafness (SSD) subjects. The adoption of the single BCI needs to be advised by the clinician on the ground of a thorough counselling with the SSD subject. Objectives: To perform an overview of the present possibilities of BCI in SSD and to evaluate the reliability of the audiological evaluation for assessing the speech recognition in noise and the sound localization cues, as major problems related to the loss of binaural hearing. Method: Nine SSD subjects who underwent BCI implantation underwent a pre-operative audiological evaluation, consisting in the soundfield speech audiometry, as word recognition score (WRS) and sound localization, in quiet and in noise. Moreover, they were also tested for the accuracy of directional word recognition in noise and with the subjective evaluation with APHAB questionnaire. Results: The mean maximum percentage of word discrimination was 65.5% in the unaided condition and 78.9% in the BCI condition. The sound localization in noise with the BCI was better than the unaided condition, especially when stimulus and noise were on the same side of the implanted ear. The accuracy of directional word recognition showed to improve with BCI in respect to the unaided condition, in the BCI side, with either the stimulus on the implanted ear and the noise in the contralateral ear, or when both stimulus and noise were deliver to implanted ear

Idiopathic benign paroxysmal vertigo in children, a migraine precursor.

OBJECTIVES: We describe the case of a young girl in whom transient deafness occurred when her core body temperature rose. METHODS: The patient was referred for a series of audiological and neurologic evaluations performed over time in both afebrile and febrile states, as well as after a stress test (with a treadmill) in which the body temperature rise simulated the febrile state. RESULTS: The patient was found to have a temporary bilateral hearing loss, but had normal distortion product otoacoustic emissions. Moreover, auditory brain stem responses revealed the absence of neural synchrony when her core body temperature increased. CONCLUSIONS: These results are consistent with a temperature-dependent auditory neuropathy, a rare condition in which patients show normal outer hair cell function and abnormal neural function of the eighth cranial nerve. The symptom is reminiscent of Uhthoff's phenomenon, which is described as transient visual loss and is usually observed in multiple sclerosis. This case of temperature-dependent auditory neuropathy is noteworthy because it sheds light on a disorder of which there have been few reports in the literature. We discuss its similarity to Uhthoff's phenomenon

Patient satisfaction after auditory implant surgery. ten-year experience from a single implanting unit center

Conclusions: The satisfaction rate of the subjects with an auditory implant appears strictly related to the resulting auditory improvement, and the surgical variables would play a prevailing role in respect to the esthetic factors. Objectives: To assess the rate of satisfaction in subjects who underwent the surgical application of an auditory device at a single Implanting Center Unit. Method: A series of validated questionnaires has been administered to subjects who underwent the surgical application of different auditory devices. The Glasgow Benefit Inventory (GBI), the Visual Analog Scale (VAS), and the Abbreviated Profile of Hearing Aid Benefit (APHAB) have been used to compare the implanted situation with the hearing-aided one; a percutaneous bone conductive implant (pBCI) with an active middle ear implant (AMEI) on the round window in mixed hearing loss; and an invisible, fully-implantable device with a frankly and bulky semi-implantable device. Results: The mean GBI scores were higher in Vibrant Soundbridge (VSB)VR and BonebridgeVR subjects, without significant differences among the various devices. The mean VAS score increased for all the devices in comparison with the conventional hearing aid. The mean APHAB score was similarly better in the implanted condition as total and partial scores

Delayed Effect of Active Pressure Treatment on Endolymphatic Hydrops

OBJECTIVE: To identify eventual correlations between the effect of low-pressure treatment and endolymphatic hydrops in Ménière patients. MATERIAL AND METHODS: The study group consisted of subjects affected by definite Ménière disease (2015) and a severe degree of disability, who received a ventilation tube with or without a low-pressure treatment before undergoing a surgical procedure (vestibular neurectomy). After the placement of the ventilation tube, the subjects were either left alone with the tube or received 1 month of self-administered low-pressure therapy with a portable device. In all subjects, an electrocochleography (ECochG) was performed and specific questionnaires - Dizziness Handicap Inventory (DHI) and Functional Scale Level (FSL) - were completed before starting either arm of treatment, at the end of treatment, and then 3 and 6 months later. RESULTS: All selected subjects presented with an ECochG pattern that was indicative of endolymphatic hydrops before starting either treatment. At the end of pressure treatment, 80% showed symptomatic improvement while maintaining the hydropic ECochG pattern. At the 3-month control stage, the hydropic pattern resulted normalized (<0.5) in all the improved subjects. CONCLUSIONS: Although 1 month of low-pressure treatment provided a positive symptomatological outcome, normalization of the hydropic ECochG parameters occurred only at a later time. Therefore, it is possible to assume that endolymphatic hydrops could be concurrent with a non-symptomatic stage of Ménière disease, and that the anti-hydropic effect of the low-pressure treatment, if any, would present with a certain delay after its completion

Hearing loss in autoimmune disorders

Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity accounting for less than 1% of all cases of hearing loss; however, this could be an underestimation based on the absence of specific diagnostic tests and the complexity of differential diagnosis. The disease could be localized only in the ear or hearing loss could be a symptom of a systemic autoimmune disease

Is the human round window really round? An anatomic study with surgical implications.

Human round window (RW) presents anatomic variations that may influence surgical approach. The true shape of the human RW has been divisive since its first description in 1772 by Antonio Scarpa. Introduction of novel surgical strategies in recent years have raised its significance. Here, the human RW size and shape variations were documented in microdissected human temporal bones. An archival collection of human microdissected temporal bones was analyzed. RW rim could be delineated and photographed from the labyrinthine aspect and its topography assessed. Human RW is seldom round but ovoid or orthogonal, skewed, and nonplanar (saddlelike). Membrane is fan shaped or conical with an anteroinferior and a posterosuperior part. The mean longest diameter was 1.90 mm, and the smallest one is 1.54 mm. The mean diameter from the crista fenestra was 1.31 mm. The mean area of the RW was 2.08 mm, which varied between 0.99 and 3.20 mm. The crista fenestrae of the anterior component form a "doorstep" that may limit the entry to the scala tympani from the RW niche. The alternate anatomic features of the human RW may influence its surgical access and designs of implants aimed at targeting this region

Esteem® middle ear device versus conventional hearing aids for rehabilitation of bilateral sensorineural hearing loss

This retrospective study aimed to evaluate the efficacy of the Esteem ® middle ear implant in sensorineural hearing loss (SNHL) of different degree as well as to compare it with that obtained with conventional hearing aids. Fifteen out of 30 adults patients who received an Esteem ® middle ear device for rehabilitation of sensorineural hearing loss met the primary eligibility criterion of prior, continuous use of conventional hearing aids. Study population included moderate-to-severe SNHL (8 patients) and severe-to-profound SNHL (7 patients). Audiometric measurements included free-field pure-tone and speech audiometry in Esteem ®-aided, HA-aided, and baseline threshold. For speech audiometry, speech reception threshold (SRT) and word recognition score (WRS) were assessed. Subjective benefit was evaluated by Client Oriented Scale of Improvement (COSI) questionnaire. In all the subjects, SRT and WRS showed improvement both with conventional HA and Esteem® in respect to the unaided situation. Although not statistically significant, a slight prevalence of the Esteem® performances was recorded both audiometrically and as subjective satisfaction score. The Esteem® middle ear device demonstrated appreciable benefit for rehabilitation of SNHL of different degree, comparable to what can be achieved by conventional hearing aids. In addition, this rehabilitative process may enable also individuals presenting with severe-to-profound SNHL to achieve remarkable functional outcomes. © 2012 Springer-Verlag Berlin Heidelberg

On the Anatomy of the ‘Hook’ Region of the Human Cochlea and How It Relates to Cochlear Implantation

Background: The optimal insertion route for an electrode array in hearing preservation cochlear implantation (CI) sur- gery is still tentative. Both cochleostomy (CO) and round window (RW) techniques are used today. In the present study we analyzed size variations and topographic anatomy of the ‘hook’ region of the human cochlea to better compre- hend the Testo effects of various electric array insertion modes. Material and Methods: Size variations of the cochle- ar ‘hook’ region were assessed in 23 human, microdissected temporal bones by measuring the distances between the oval and round windows, also outlining the spiral ligament/ spiral lamina. Influence of size variations on spiral ligament position and fundamentals for different surgical approaches were evaluated in a subset of ‘small’ and ‘large’ cochleae per- forming different types of CO. In addition, the relationship between the microdissected accessory canal housing the inferior cochlear vein and the RW was analyzed. Results: The lateral vestibular wall and the cochlear ‘hook’ displayed large anatomic variations that greatly influenced the size of the potential surgical area. Results showed that only very inferi- orly located CO entered the scala tympani without causing trauma to the spiral ligament and spiral lamina. An inferior approach may challenge the inferior cochlear vein. Conclu- sion: Preoperative assessment of the distance between the round and oval windows may direct the surgeon before CI hearing-preservation surgery. CO techniques, especially in ‘small’ ears, may lead to frequent damage to the inner ear structures. In those cases with substantial residual hearing, CI surgery may be better performed through a RW approach