42 research outputs found
Hearing loss in autoimmune disorders
No full textAutoimmune 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.
No full textHuman 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
On the Anatomy of the ‘Hook’ Region of the Human Cochlea and How It Relates to Cochlear Implantation
No full textBackground: 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
Idiopathic benign paroxysmal vertigo in children, a migraine precursor.
No full textOBJECTIVES: 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
Bone conductive implants in single sided deafness
No full textConclusion: 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
A new semi-implantable middle ear implant for sensorineural hearing loss. three-years follow-up in a pilot patient’s group
No full textObjective: The aim of this article is to report on the long-term follow-up of a new semi-implantable middle ear device utilized for restoration of moderate-to-severe sensorineural hearing loss in a first series of subjects. Methods: Three subjects, affected by sensorineural hearing loss, have undergone implantation of Maxum® middle ear implant, via a transcanal approach. They all underwent an auditory assessment, paying particular attention on the pre- versus post-operative hearing levels under the unaided, best-fitted hearing aided and implant-aided conditions. The audiometric evaluation has been repeated 3 years after implantation and implemented by questionnaires aiming at the evaluation of the quality of life. Results: The post-operative hearing threshold and discrimination in quiet appear to be similar or better than those provided by conventional hearing aids, with a concomitant improvement of the subjects’ quality of life. The application of the present device showed to be easy and reproducible, with no severe adverse effects recorded at the 3-years follow-up. Mild issues due to the external component were also observed, such as difficulty of keeping it continuously in place due to excess canal sweating in one subject, and a temporary loss of stability due to occurring irregularities of the external coating in another subject. Conclusions: Long-term, preliminary data reveal that the Maxum® device may provide equal or better functional gain and word recognition scores in quiet in patients with moderate-to-severe sensorineural hearing loss, in comparison to optimally fitted hearing aids, with a satisfactory improvement of their quality of life
Esteem® middle ear device versus conventional hearing aids for rehabilitation of bilateral sensorineural hearing loss
No full textThis 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
Is the Bone-Conduction Headband Test Useful for Predicting the Functional Outcome of a Round Window Active Middle Ear Implant?
No full textObjective: To estimate the reliability of the Bone Conduction-HeadBand (BC-HB) test for predicting the postoperative functional outcome of a round-window (RW) vibroplasty. Study Design: Within-subject comparison of the functional results of the BC-HB test, which is routinely used for the preoperative evaluation of a bone-conduction transducer, with an active middle ear implant (AMEI) placed onto the round window. Setting: Tertiary referral university hospital center. Patients: Seven patients with similar anatomic (absent stapes superstructure) and functional (moderate, mixed hearing loss) sequelae from open tympanoplasty technique. Intervention: All subjects underwent preoperative audiologic assessment with the BC-HB. Subsequently, all subjects underwent surgical placement of an AMEI onto the round window. Main Outcome Measure: Pure tone and speech audiometry in quiet and noise were assessed. Additionally, evaluation of specific satisfactory targets was performed using the Client Oriented Scale of Improvement. Results: Pure tone and speech audiometry in quiet established that both devices had very similar performance and provided remarkable improvement compared with the unaided condition. However, high-frequency gain and speech audiometry in noise demonstrated better performance with RW-AMEI. Conclusion: In patients presenting with mixed hearing loss as a sequela from middle ear surgery, the preoperative BC-HB test may be helpful in predicting the final functional outcome and patient satisfaction with RW-AMEI
Idiopathic Benign Paroxysmal Positional Vertigo (BPPV) . Recurrent versus alternating relapses
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