Fitting range of the BAHA Intenso.

Audiometric characteristics of a recently introduced more powerful, behind-the-ear, BAHA sound processor, the BAHA Intenso, were evaluated in 23 patients with contra-indications for using conventional hearing aids. All patients had mixed hearing loss with boneconduction thresholds ranging between 30 and 50 dB HL. Boneconduction gain, defined as the difference in aided free-field detection thresholds and unaided boneconduction thresholds, was calculated at the octave frequencies between 500 Hz and 4 kHz. Median boneconduction gain of the Intenso ranges from 0 dB at 500 ;Hz to 12 ;dB at 2 kHz with substantial interindividual variability. The upper limit of the BAHA Intenso's fitting range was established by requiring aided speech reception thresholds with CVC-monosyllables of at most 60 dB SPL. The fitting range of the BAHA Intenso appeared to be limited to 42, 44, 58, and 48 dB HL for boneconduction thresholds at 0.5, 1, 2, and 4 ;kHz, respectively. Loudness growth functions at 0.5 and 3 kHz as obtained with 7-point categorical scaling showed an adequate aided dynamic range.

The voice handicap of student-teachers and risk factors perceived to have a negative influence on the voice.

A cross-sectional questionnaire survey was performed. The objectives of the study were to assess the psychosocial impact of current voice complaints as perceived by student-teachers with voice complaints in comparison with student-teachers without voice complaints, and to observe the pattern of risk factors in relation to their voice handicap. Subjects in the general population without a voice-demanding profession were selected as a reference group for limited comparison with the total group of student-teachers (future professional voice users). The respondents to the questionnaires were anonymous. Among the student-teachers, 17.2% reported current voice complaints in comparison with 9.7% of the reference group, and the odds ratio was 1.94, which showed the relative risk. Student-teachers had significantly greater total Voice Handicap Index (VHI) scores than the reference group (P = 0.034). The VHI subscale scores were not significantly different (P > 0.05). Student-teachers who reported current voice complaints had a significantly higher total VHI and subscale scores than student teachers without voice complaints (P < 0.001). Of the student-teachers without voice complaints, 17.0% had VHI scores greater than the 75th percentile. These persons may be neglecting their voice handicap and probably represent the false-negative cases in the estimation of voice complaints. Logistic regression analysis of each of the given risk factors with the VHI as the independent variable showed that the perceived negative influence of the given risk factors on their voices was significantly greater with increasing VHI scores across the VHI range. A significant correlation was observed between the number of perceived risk factors and increasing VHI scores across the VHI range. An increased awareness of risk factors in relation to their voice handicap would serve to motivate student-teachers to change factors that contributed to their voice problem. Attention to all risk factors, which the subjects perceive to be a risk, would aid in effective management of their voice handicap.

The effect of the "floating mass transducer" in the middle ear on hearing sensitivity.

OBJECTIVE: The Vibrant soundbridge is a semi-implantable hearing device; the transducer is implanted, coupled directly to the incus. The influence of the implant surgery and the presence of the transducer on hearing sensitivity was studied in six implanted subjects. STUDY DESIGN: Longitudinal case reports. SETTING: Tertiary referral center. Subjects. The subjects had bilateral sensorineural hearing loss with an average hearing loss of 40 to 70 dB HL. RESULTS: In five of the six subjects, no long-term effect of the surgery or the presence of the transducer on hearing thresholds was found. In the remaining subject, a deterioration in hearing thresholds was found of 20 dB, with a high and low frequency component. In the 2-kHz region, hearing sensitivity was not deteriorated. In addition, chronic negative middle ear pressure occurred after surgery. CONCLUSION: Hearing thresholds did not change significantly in five of the six patients after placement of the "floating mass transducer." It was speculated that the high frequency component of the hearing deterioration in the remaining patient was caused by cochlear damage caused by the surgery and that the low frequency component was caused by the chronic aeration problems indirectly related to the surgery.

Progressive sensorineural hearing loss and a widened vestibular aqueduct in Pendred syndrome.

Pendred syndrome is an autosomal recessive inherited disorder. Obligatory features are profound deafness in childhood and defective organic binding of iodine in the thyroid gland. Therefore, goiter is a common symptom. Hypoplasia of the cochlea is another feature. Recently, the gene for Pendred syndrome was identified. We describe a boy whose sensorineural hearing loss in both ears progressed rapidly from about 50 to 60 dB at the age of 3 years and 3 months to more than 100 dB at the age of 4 years and 4 months. This loss was preceded by a medical history of a progressive hearing loss. The progressive nature of the hearing loss motivated a search for the cause. Dysplasia of the cochlea and a widened vestibular aqueduct were found. The results of thyroid function tests were normal, but he had an elevated level of thyroglobulin. The diagnosis of Pendred syndrome was confirmed by the positive results of a potassium perchlorate test, indicating defective organic binding of iodine in the thyroid gland. It is possible that the widened vestibular aqueduct was responsible for the increase in the hearing impairment. Aside from the branchio-otorenal syndrome, Pendred syndrome is the only other known genetic disorder with a widened vestibular aqueduct. If a child has progressive sensorineural deafness and a widened vestibular aqueduct, it is important to consider a diagnosis of Pendred syndrome. A widened vestibular aqueduct may help to elucidate the pathophysiologic characteristics of hearing loss in these genetic types of deafness in childhood.

Usher syndrome. A temporal bone report.

The bilateral temporal bones of a deceased 84-year-old man who had been suffering from Usher syndrome were examined using light microscopy. Histopathologic examination disclosed degeneration of the organ of Corti that was most profound in the basal turn, degeneration of cochlear neurons in all of the turns, and severe loss of spiral ganglia in both cochleas. Endolymphatic hydrops of unknown cause and a functionally unimportant pit malformation in the macular utricle were observed in the right cochlea. We compared the aforementioned findings with temporal bone reports cited in the literature.

Noninvasive assessment of the intralabyrinthine pressure. A new technique applied to patients with X-linked progressive mixed deafness syndrome with perilymphatic gusher during stapes surgery.

In this report, we describe the findings of a noninvasive assessment of the intralabyrinthine pressure in two patients from a family with X-linked progressive, mixed deafness syndrome in whom a perilymphatic gusher occurs during stapes surgery. The so-called tympanic membrane displacement measurement technique could be successfully applied in these two patients (from a total of five patients who were studied) because they still showed a stapedial reflex at 1 kHz, which is mandatory for application of the tympanic membrane displacement measurement technique. The findings were compared with those of age-related control subjects and indicated a significantly elevated intralabyrinthine pressure in the two patients who were observed. The results suggest that the tympanic membrane displacement measurement technique may serve as a screening test in audiological diagnostic studies of perilymphatic hypertension.

Fibroinflammatory pseudotumor of the ear. A locally destructive benign lesion.

We describe three cases of a fibroinflammatory pseudotumor (tumefactive fibroinflammatory lesion) of the middle and inner ear. The patients presented with total deafness in the affected ear and no response to caloric stimulation. The computed tomographic pattern showed destruction of inner ear structures and a typical widening of parts of the labyrinth. Magnetic resonance imaging performed in all three patients showed an extension greater than expected based on computed tomographic images of both areas of destruction, as well as areas of radiologic normality. An enhancing mass was seen in the inner ear with a characteristic extension into both the internal auditory canal and the middle ear. A transotic approach or subtotal petrosectomy was used to remove the tumor in all three cases. Although histologically benign, these tumors are locally destructive and, as such, behave like a neoplastic lesion. They are composed of fibrovascular tissue admixed with chronic inflammatory cells. To our knowledge, this is the first report on pseudotumors of the middle ear, inner ear, and internal auditory canal. Inflammatory pseudotumor used to be a somewhat confusing term for a recognized entity of unknown origin. It is likely that infection is an important contributing factor in the development of these lesions. Although surgical removal seems to be the treatment of choice, no clear judgment of its prognosis can be made owing to the rarity of this tumor.

Speech recognition in patients after successful surgery for unilateral congenital ear anomalies.

It is generally assumed that auditory stimulation since birth is important for the proper development of the central auditory nervous system. Whether auditory deprivation occurs in man and, therefore, whether it may be considered as a contraindication to surgery in unilateral congenital middle ear anomalies and atresias is the subject of the present study. Speech recognition during both monaural and binaural presentation was studied in patients who had successful surgery for a unilateral congenital ear anomaly. In binaural speech recognition tests, the average results of the patient group (N = 13) and a group of subjects with normal hearing proved to be comparable. The average speech recognition score using monaurally presented band-pass filtered speech was 84% +/- 8% and 77% +/- 10% for the unoperated (normal) and operated ears, respectively (statistically significant). Speech-to-noise ratios of -5.6 +/- 0.7 dB and -3.9 +/- 1.6 db were found in the normal and operated ears, respectively (statistically significant). It is concluded that, in general, the speech recognition scores of the operated ears were satisfactory, but poorer than those of the normal ears.

Myringochorda-vestibulopexy: a new method for total replacement of the ossicular chain.

A new method is described for total replacement of the ossicular chain if the ossicles are absent and malleovestibulopexy does not form a solution. One end of a 0.4-mm thick Teflon-platinum piston was fixed to the chorda tympani, and the other end was inserted into an opening made during stapedotomy. Contact between the piston and tympanic membrane was achieved by placing autologous cartilage between the posterior upper quadrant of the tympanic membrane and the chorda. The results of a second patient at 2-year follow-up are presented.

Persistent stapedial artery: does it prevent successful surgery?

Because of the important function of the embryologic stapedial artery, it is taken for granted by many surgeons that the finding of such a persistent artery in postnatal humans during middle ear surgery should urge maximal caution in order not to damage the artery. Often, discontinuation of the surgery is recommended. Yet this attitude is based on theoretic considerations rather than on any clinical evidence of complications following injury to this vessel. The present paper describes the embryology in relation to this specific aspect and reviews the literature on the persistent stapedial artery, emphasizing the papers dealing with injury to this vessel. In addition, we report 4 cases of persistent stapedial artery from the files of almost 20,000 patients in whom tympanotomy was performed. From all these data we conclude that injury to this artery or even complete section probably does not cause major, if any, postoperative sequelae, and that consequently, middle ear surgery is not necessarily hindered by the presence of this vessel.

Classification of congenital middle ear anomalies. Report on 144 ears.

The surgical findings in 144 successive ears operated on for congenital conductive hearing loss were analyzed, and the results were evaluated in terms of hearing gain. All the patients underwent middle ear surgery at the University Hospital Nijmegen between 1964 and 1990. A classification system was developed to analyze the findings. Class 1 comprises ears with congenital isolated stapes ankylosis. Class 2 comprises ears with congenital stapes ankylosis in combination with a congenital anomaly of the ossicular chain. Class 3 comprises ears with congenital anomalies of the ossicular chain and at least a mobile stapes footplate. Class 4 comprises ears with aplasia or severe dysplasia of the oval window or round window.

Acquired atresia of the external auditory canal. Surgical treatment and results.

Atresia of the external ear canal following recurrent external otitis or surgery for chronic otitis media was treated in 17 ears with a canalplasty procedure using a retroauricular incision. The stenotic meatus was widened by removing fibrous tissue, and the tympanic membrane was deepithelialized. The posterior, superior, and inferior bony canal was widened until the first mastoid cells were encountered. The anterior canal was widened in cases where there was an anterior bony overhang. The bony canal was lined with split-thickness skin grafts. Meatoplasty was performed, and split-thickness skin grafts were grafted onto the margins of the meatoplasty to cover the lateral part of the ear canal. Preoperative and postoperative surgical results, including hearing levels, are presented and compared with those from the few other series from the literature.

Cavernous hemangioma of the internal acoustic canal.

A case report of a 39-year-old man suffering from left-sided, progressive hearing loss is presented. As well, the patient noted ipsilateral recurrent facial paralysis. MRI succeeded in confirming the presence of a tumor in the internal acoustic canal 18 months after the first MRI.

Isolated congenital stapes ankylosis: surgical results in 32 ears and a review of the literature.

Isolated congenital stapes ankylosis is described in 32 operated ears from 28 patients. In 27 ears, a regular stapedectomy was performed. In the remaining 5 ears, 2 had stapes gushers, 2 had bony stapedial tendons, and 1 had an aberrant facial nerve crossing the oval window. The average individual hearing gain was 20 dB. The mean hearing gain for the 24 successful stapedectomies was 29 dB. In 24 of 32 ears (75%), an essential hearing gain of at least 15 dB Fletcher's index hearing threshold could be achieved. A Fletcher's index not exceeding 30 dB could be achieved in 19 of 32 ears (60%), in spite of several cases with a sensorineural component in the hearing loss. A review of the literature and overview of longer series with isolated congenital stapes ankylosis is presented.

An autosomal dominant inherited syndrome with congenital stapes ankylosis.

A newly recognized autosomal dominant inherited syndrome associated with congenital conductive deafness, hyperopia, broad thumbs, broad first toes, short distal phalanges, and syndactyly is reported. The conductive loss was the result of congenital stapes ankylosis and, in two cases, was associated with ankylosis of the short process of the incus in the fossa incudis. Stapedectomy improved hearing in these patients. Fused cervical vertebrate are also an associated feature.

The gene for X-linked progressive mixed deafness with perilymphatic gusher during stapes surgery (DFN3) is linked to PGK.

A linkage analysis has been performed in a large Dutch kindred with progressive mixed deafness with perilymphatic gusher during stapes surgery (DFN3) using a panel of X-chromosomal RFLPs. Tight linkage (zmax = 3.07 at 0 = theta = 0.00) was demonstrated with the locus for phosphoglycerate kinase (PGK), which is located at Xq13. Tight linkage was excluded for DXS9 (probe RC8) and DXS41 (probe 99.6) on Xp and for blood clotting factor 9 (FIX) on distal Xq. Deafness is one of the predominant clinical features in males with deletions of the Xq21 band. Our results suggest that this association may be due to involvement of the DFN3 gene.