Unusual presentation of temporal bone involvement in Churg–Strauss syndrome

from the Journal of Laryngology and Otology

Objective: To present a case of a 60-year-old male with a history of sudden onset sensorineural hearing loss due to Churg–Strauss syndrome.

Case report: The patient had a 20-year history of asthma and recurrent right otitis media and a nasal polypectomy four years prior to presenting with ear symptoms. Ear, nose and throat involvement is common in Churg–Strauss syndrome, usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps.

Conclusions: Otolaryngologists play an important role in making an early diagnosis of this disease. To our knowledge this is the first case of Churg–Strauss syndrome primarily presenting with otological pathology: left sensorineural hearing loss and right otitis media.

Temporal bone study of development of the organ of Corti: correlation between auditory function and anatomical structure

from the Journal of Laryngology and Otology

Objective: To study the development of the organ of Corti in the human cochlea, and to correlate our findings with the onset of auditory function.

Material and methods: Step sections of 81 human fetal temporal bones were studied, from eight weeks of gestation to full term.

Results: By the end of the 10th week, the tectorial membrane primordium could be traced even in the most apical turns. Individual hair cells became identifiable at the basal turn at 14 weeks. At the same time, a small but well formed oval space was observed between the inner and outer hair cells in the basal turn. This does not correspond to the tunnel of Corti, as is erroneously quoted in the literature, as the individual pillar cells develop at later stages. Between 14 and 15 weeks, Hensen’s cells were recognised for the first time. Individual pillar cells were identifiable at 17 weeks and the tunnel of Corti opened at 20 weeks. By 25 weeks, the cochlea had reached its adult size, but continued to develop until full term.

Discussion and conclusions: A temporal coincidence of different developmental events is responsible for early fetal audition at 20 weeks, including growth of pillar cells, opening of the tunnel of Corti and regression of Kollicker’s organ, with the subsequent formation of the inner spiral sulcus and then separation of the tectorial membrane. The fine structures of the organ of Corti continue to develop well after the 25th week, and this may well alter the mechanical properties of the vibrating parts of the cochlea, which may in turn account for the frequency shift observed in preterm infants. These changes will have to be taken into account in the development of prenatal hearing screening tests.

Sacculo-collic response in otosclerosis and following successful stapes surgery

from the Journal of Laryngology and Otology

Introduction: The sacculo-collic reflex is believed to be a short latency, otolith-mediated myogenic response to sound. With the application of air-conducted sound, one would expect an absent response in stapes fixation, as a fixed stapes footplate will not transmit a pressure wave to the saccule.

Methods: Fifty patients (70 stapes surgery ears, 26 otosclerotic ears and four normal ears) and 40 controls underwent repeated sacculo-collic tests.

Results: The results support the proposed mechanism for the sacculo-collic response. The study also suggests that, whilst stapedotomy piston prostheses are effective in the reversal of conductive hearing loss, they produce an insufficient pressure wave to elicit a myogenic response to sound.

Conclusion: The sacculo-collic test could be a useful tool for screening otolith function and inferior vestibular nerve integrity, but further work is needed to determine the effect of stapes surgery on saccular function.

Effects of antituberculosis treatment on self assessment, perceptual analysis and acoustic analysis of voice quality in laryngeal tuberculosis patients

from the Journal of Laryngology and Otology

Objectives: To evaluate the effects of antituberculosis treatment on the voice quality of laryngeal tuberculosis patients, measured by patient self-assessment, perceptual analysis and acoustic analysis.

Materials and methods: A total of 14 laryngeal tuberculosis patients were enrolled. Laryngeal tuberculosis was established either by biopsy and histopathological examination or by rapid regression of the laryngeal lesions after antituberculosis medication. Before and after treatment, all patients were evaluated perceptually (on a scale of zero to three), and 12 assessed their own voices using the voice handicap index-10 scale. Acoustic analysis was performed to allow objective evaluation.

Results: Patients’ ages ranged from 21 to 72 years (mean, 41). The male to female ratio was 12:2. Eight patients (57 per cent) had tuberculous involvement of the epiglottis, four (28 per cent) had involvement of the aryepiglottic fold and eight (57 per cent) had involvement of the false vocal folds. The glottis was the less commonly involved part of the larynx, including true vocal folds (28 per cent, n = 4) and posterior commissure (14 per cent, n = 2). Perceptual evaluation, on a scale of zero to three, gave the patients a median score of six; after commencement of treatment, the median score decreased to two. The mean voice handicap index-10 score decreased from 24 to 12 after treatment. An obvious improvement in acoustic analytical parameters was also found following treatment.

Conclusions: Antituberculosis treatment clearly improved the voice outcomes of laryngeal tuberculosis patients, according to self-assessment, perceptual analysis and acoustic analysis.

Another reason for intra-operative imaging during cochlear implantation

from the Journal of Laryngology and Otology

Objective: We report a case of a rare cochlear implant complication: the introduction of the electrode array into the superior semicircular canal, with intra-operative measurements of neural response reactions suggesting reasonable functioning of the implant.

Case report: A two-year old patient affected by congenital, profound, sensorineural deafness underwent bilateral cochlear implantation at the ENT clinic of the ‘La Sapienza’ University of Rome. Two Clarion 90k devices were implanted, and electrophysiological and radiological checks were performed. After the introduction of the array in the right side, neural response imaging was performed, and a neural potential was found only on two apical electrodes, at a stimulation intensity of 431 clinical units. The situation differed on the left side, where neural response imaging was present at a stimulation intensity of 300 clinical units on the two electrodes tested (one apical electrode (number three), and one middle electrode (number nine)). Intra-operative radiological assessment with a transorbital plain films was performed as usual in order to assess the position of the electrodes inside the cochlea. This radiography showed the electrode array to be in the superior semicircular canal in the right ear.

Conclusion: Intra-operative monitoring tests during cochlear implant surgery play different roles; measurement of impedances and neural response imaging can evaluate the integrity of implant electrodes and the status of the electrode–cochlea interface, but it must not be the sole way in which correct positioning of the array is confirmed. In our opinion, intra-operative radiological assessment is mandatory during cochlear implant surgery.

Working Memory Has Limited ‘Slots’

from Medical News Today.com

A new study by researchers at UC Davis shows how our very short-term “working memory,” which allows the brain to stitch together sensory information, operates. The system retains a limited number of high-resolution images for a few seconds, rather than a wider range of fuzzier impressions.

Palmtop Computing Helps Autistics Communicate

from MedGadget.com

Canada’s CTV News is reporting on how autistic children at the Bloorview Kids Rehab, a teaching hospital affiliated with the University of Toronto, are using special software on Pocket PC-like devices to more comfortably communicate with people around them.

The neural circuit for tone-specific plasticity in the auditory system elicited by conditioning

from Learning & Memory

No abstract available

Early and later vocalization skills in children with and without cleft palate

from the International Journal of Pediatric Otorhinolaryngology

Objective
The purpose of this study was to describe the early vocalization skills in children with cleft lip and palate (CLP) at 6 and 12 months of age and compare these early vocalization measures to later speech and vocabulary development at 30 months of age.

Methods
The participants in the study included 13 children without cleft lip or palate (NCLP) who were typically developing and 13 children with CLP matched for age, gender and socioeconomic status. Standardized measures of cognition, language, hearing, and prelinguistic vocalization measures were administered at 6 and 12 months and speech production, and vocabulary measures were collected at 30 months of age.

Results
Group differences were observed in both receptive and expressive language development at 12 and 30 months of age. Group differences were observed in the frequency of babbling and Mean Babbling Level at 12 months and speech sound accuracy and vocabulary production at 30 months of age. Significant correlation coefficients were observed between babbling frequency at 6 months and consonant inventory size, vocabulary at 30 months for the children with clefts and PCC-R for noncleft children.

Conclusions
This study documented that young children with clefts have persistent vocalization and vocabulary deficits well beyond palate closure. Measures of babbling frequency, Mean Babbling Level and consonant inventories provide clinically effective means of identifying these early deficits. Additionally, these measures may provide a tool for monitoring the effects of early intervention programs that promote facilitation of sound and vocabulary development.

Speech and language development in toddlers with and without cleft palate

from the International Journal of Pediatric Otorhinolaryngology

Objective
The effect of early palate closure on speech and language development in children with cleft palate.

Design
Comparative study.

Setting
University Medical Center Groningen, Cleft Palate Team (The Netherlands).

Materials and methods
Forty-three toddlers with cleft palate and thirty-two toddlers without cleft palate were analyzed with standardized tests for language comprehension and language production. Moreover articulation and hyper nasality were examined by trained speech therapists.

Results
For language comprehension, language production and articulation there were no significant differences between the children with and without cleft lip and/or palate. This is despite the high percentage of conductive hearing loss (55%) in children with clefts. Significant difference was found for hyper nasality (mean: 35% vs. 0%, p = 0.001). In both groups articulation problems raise to a higher percentage than language production problems (63–20%; 24–4%).

Conclusions
Early surgical treatment is effective for a part of the communicative development, i.e. language development and articulation. Besides conductive hearing loss hyper nasality remains a serious problem in 30–50% of the children with cleft palate. Therefore, speech therapy and pharyngoplasty also are part of the treatment procedure. Because of the high amount articulation problems in all children, standards for articulation development are perhaps too strict. Future research should be carried out after normal variations in articulation development.