Successful treatment of sleep paralysis with the Sleep Position Trainer: a case report.

Sleep paralysis is a period of paralysis at either sleep onset or upon awakening and is often accompanied by terrifying hallucinations. We report a case of a 32-year-old healthy men with a history of mild positional obstructive sleep apnea and sleep paralysis. The positional sleep apnea was successfully treated with the Sleep Position Trainer. Remarkably, he did no longer experience episodes of sleep paralysis since using the Sleep Position Trainer. This case highlights a possible elegant noninvasive long-term solution for the treatment of sleep paralysis. CITATION: Cui N, van Looij MA, Kasius KM. Successful treatment of sleep paralysis with the Sleep Position Trainer: a case report. J Clin Sleep Med. 2022;18(9):2317-2319.

No COVID-19 in Patients With Sudden Sensorineural Hearing Loss (SSNHL).

BACKGROUND: Various case reports have described sudden sensorineural hearing loss (SSNHL) in patients with the 2019 novel coronavirus disease (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Our aim was to determine the incidence of COVID-19 in patients with SSNHL. METHODS: All consecutive patients with audiometric confirmed SSNHL between November 2020 and March 2021 in a Dutch large inner city teaching hospital were included. All patients were tested for COVID-19 by polymerase-chain-reaction (PCR) and awaited the results in quarantine. RESULTS: Out of 25 patients, zero (0%) tested positive for COVID-19. Two patients had previously tested positive for COVID-19: at three and eight months prior to the onset of hearing loss. CONCLUSIONS: This is the largest series to date investigating COVID-19 in SSNHL patients. In this series there is no apparent relationship between SSNHL and COVID-19.

Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients.

Tracheal agenesis is a rare congenital malformation, which is usually fatal in the newborn period. Its incidence is approximately 1 in 50,000 births. Presentation is with respiratory insufficiency and no audible cry. Other anomalies are found in most cases. Six cases of tracheal agenesis were seen in our hospital since 1988. Their medical records were reviewed. Three of our cases classify as Floyd's type III, two as Floyd's type II and one as Floyd's type I. Associated anomalies were found in five cases. The classification of tracheal agenesis, associated anomalies and potential therapeutic options are discussed.

Impact of conventional anesthesia on auditory brainstem responses in mice.

Anesthesia is known to affect the auditory brainstem response (ABR) in animals often used in hearing research. This study describes the differences in ABRs between awake and anesthetized FVB/N mice. Intracranial electrodes connected to a head fixation pedestal were used for click-evoked ABR recordings. This pedestal served to immobilize mice, either awake or under anesthesia, in a 'free' sound field. The presence of myogenic noise in the awake condition obviously increases recording time. However it is demonstrated that recording times can be significantly reduced by increasing the stimulus repetition rate from 23 up to 80 impulses per second. This causes only a small but significant increase in absolute peak latencies in the awake condition, but has no significant effect on the overall ABR-waveform, nor on the ABR-threshold, nor on the ABR interpeak latencies, nor on the absolute peak latencies in the anesthetized condition. Anesthesia with ketamine/xylazine caused a significant prolongation of ABR-peak latencies and interpeak latencies as well as a significant upward shift (8.0+/-1.8 dB) of ABR-thresholds as compared to the awake condition. Under anesthesia the measurement accuracy of peak latencies, interpeak latencies and thresholds decreases. In conclusion, the awake condition is preferable for more accurate measurements of ABR characteristics, in spite of the myogenic noise concomitant with this condition.

Characteristics of hearing loss in HDR (hypoparathyroidism, sensorineural deafness, renal dysplasia) syndrome.

Haploinsufficiency of the zinc finger transcription factor GATA3 causes the triad of hypoparathyroidism, deafness and renal dysplasia, known by its acronym HDR syndrome. The purpose of the current study was to describe in detail the auditory phenotype in human HDR patients and compare these to audiometrical and histological data previously described in a mouse model of this disease. Pure tone audiometry, speech audiometry, speech in noise, auditory brainstem responses and transiently evoked otoacoustic emissions were measured in 2 patients affected by HDR syndrome. Both patients were affected by a moderate-to-severe sensorineural hearing loss. Speech reception thresholds were shifted and speech recognition in noise was disturbed. No otoacoustic emissions could be generated in either patient. Auditory brainstem response interpeak intervals were normal. The human and murine audiological phenotypes seem to correspond well. Hearing loss in HDR syndrome is moderate to severe, seems to be slightly worse at the higher end of the frequency spectrum and may be progressive with age. The absence of otoacoustic emissions and the loss of frequency selectivity suggest an important role for outer hair cells in causing the hearing loss.

Hearing loss following Gata3 haploinsufficiency is caused by cochlear disorder.

Patients with HDR syndrome suffer from hypoparathyroidism, deafness, and renal dysplasia due to a heterozygous deletion of the transcription factor GATA3. Since GATA3 is prominently expressed in both the inner ear and different parts of the auditory nervous system, it is not clear whether the deafness in HDR patients is caused by peripheral and/or central deficits. Therefore, we have created and examined heterozygous Gata3 knockout mice. Auditory brainstem response (ABR) thresholds of alert heterozygous Gata3 mice, analyzed from 1 to 19 months of age, showed a hearing loss of 30 dB compared to wild-type littermates. Neither physiological nor morphological abnormalities were found in the brainstem, cerebral cortex, the outer or the middle ear. In contrast, cochleae of heterozygous Gata3 mice showed significant progressive morphological degeneration starting with the outer hair cells (OHCs) at the apex and ultimately affecting all hair cells and supporting cells in the entire cochlea. Together, these findings indicate that hearing loss following Gata3 haploinsufficiency is peripheral in origin and that this defect is detectable from early postnatal development and maintains through adulthood.