Is intrauterine exposure to COVID-19 infection a risk factor for infant hearing loss?

PURPOSE: To determine whether exposure to intrauterine COVID-19 infection causes congenital or late-onset hearing loss in infants. MATERIAL AND METHOD: The hearing screening results of infants born in a tertiary hospital between March 2020 and April 2022 with and without a history of intrauterine exposure to COVID-19 infection (36 infants each) were retrospectively analyzed within one month after birth in all infants and additionally at six months after intrauterine COVID-19 infection exposure in the study group. The automated auditory brainstem response (AABR) test was used for the hearing evaluation. RESULTS: The polymerase chain reaction test was negative in study group exposed to intrauterine COVID-19 infection. The number of infants admitted to the intensive care unit (ICU), and the length of ICU stay were significantly higher in this group (p < 0.01). Six infants (16.6 %) in the study group failed the first AABR test bilaterally, but five of these infants passed the second AABR test. A bilateral severe sensorineural hearing loss was detected in one infant (2.77 %). All the infants in the study group underwent the AABR test again at six months, and all infants, except this infant, passed the test. In the control group, five infants (13.88 %) failed the first AABR test bilaterally, but they all passed the second test. CONCLUSIONS: Exposure to COVID-19 infection in the intrauterine period does not cause congenital or late-onset hearing loss (within six months) in infants; therefore, gestational COVID-19 infection is not a risk factor for infant hearing loss.

Association of Conductive Hearing Loss with the Structural Changes in the Organ of Corti.

OBJECTIVE: The aim of the study was to evaluate the association of conductive hearing loss (CHL) with the structural changes in the organ of Corti. METHODS: Twenty ears of 10 healthy adult Wistar albino rats were included in the study. The right ears (n = 10) of the animals served as controls (group 1), and no surgical intervention was performed in these ears. A tympanic membrane perforation without annulus removal was performed under operative microscope on the left ears (n = 5) in 5 of 10 animals (group 2). A tympanic membrane perforation with annulus removal was performed under operative microscope on the left ears (n = 5) of the remaining 5 animals (group 3). Auditory brainstem response testing was performed in the animals before the interventions. After 3 months, the animals were sacrificed, their temporal bones were removed, and inner ears were investigated using scanning electron microscopy (SEM). The organ of Corti was evaluated from the cochlear base to apex in the modiolar axis, and the parameters were scored semiquantitatively. RESULTS: In group 1, the pre- and post-intervention hearing thresholds were similar (p > 0.05). In group 2, a hearing decrease of at least 5 dB was encountered in all test frequencies (p > 0.05). In group 3, at the frequency range of 2-32 kHz, there was a significant hearing loss after 3 months (p < 0.01). After 3 months, the hearing thresholds in group 2 and 3 were higher than group 1 (p < 0.01). The hearing threshold in group 3 was higher than group 2 (p < 0.01). On SEM evaluation, the general cell morphology and stereocilia of the outer hair cells were preserved in all segments of the cochlea in group 1 with a mean SEM score of 0.2. There was segmental degeneration in the general cell morphology and outer hair cells in group 2 with a mean SEM score of 2.2. There was widespread degeneration in the general cell morphology and outer hair cells in group 3 with a mean SEM score of 3.2. The SEM scores of group 2 and 3 were significantly higher than group 1 (p < 0.05). The SEM scores of group 3 were significantly higher than group 2 (p < 0.05). CONCLUSION: CHL may be associated with an inner ear damage. The severity of damage appears to be associated with severity and duration of CHL. Early correction of CHL is advocated in order to reverse or prevent progression of the inner ear damage, which will enhance the success rates of hearing restoration surgeries. Subjective differences and compliance of the hearing aid users may be due to the impact of CHL on inner ear structures.

Genotypic-phenotypic features and enzyme replacement therapy outcome in patients with mucopolysaccharidosis VI from Turkey.

Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disorder (LSD) characterized by a chronic, progressive course with multiorgan involvement. In our study, clinical, biochemical, molecular findings, and response to enzyme replacement therapy (ERT) for at least 6 months were evaluated in 20 patients with MPS VI. Treatment effects on clinical findings such as liver and spleen sizes, cardiac and respiratory parameters, visual and auditory changes, joints' range of motions, endurance tests and changes in urinary glycosaminoglycan excretions, before and after ERT were analyzed. ERT caused increased physical endurance and decreased urinary dermatan sulfate/chondroitin sulfate ratios. Changes in growth parameters, cardiac, respiratory, visual, auditory findings, and joint mobility were not significant. All patients and parents reported out an increased quality of life, which were not correlated with clinical results. The most prevalent mutation was p.L321P, accounting for 58.8% of the mutant alleles and two novel mutations (p.G79E and p.E390 K) were found. ERT was a safe but expensive treatment for MPS VI, with mild benefits in severely affected patients. Early treatment with ERT is mandatory before many organs and systems are involved.