Objectives: 1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis.
Study Design: Retrospective audiometric database and chart review.
Setting: Tertiary referral center.
Patients: Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram.
Intervention: Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition.
Main Outcome Measure: BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population).
Results: Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002).
Conclusion: The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for tested frequencies among this subgroup.
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http://dx.doi.org/10.1097/MAO.0000000000001234 | DOI Listing |
FASEB J
January 2025
Laboratory of Molecular Pharmacology, Biosignal Research Center, Kobe University, Kobe, Japan.
DFNA1 (deafness, nonsyndromic autosomal dominant 1), initially identified as nonsyndromic sensorineural hearing loss, has been associated with an additional symptom: macrothrombocytopenia. However, the timing of the onset of hearing loss (HL) and thrombocytopenia has not been investigated, leaving it unclear which occurs earlier. Here, we generated a knock-in (KI) DFNA1 mouse model, diaphanous-related formin 1 (DIA1), in which Aequorea coerulescens green fluorescent protein (AcGFP)-tagged human DIA1(p.
View Article and Find Full Text PDFMov Disord
January 2025
Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Objective: Pathogenic variants in B-cell receptor-associated protein (BCAP31) are associated with X-linked, deafness, dystonia and cerebral hypomyelination (DDCH) syndrome. DDCH is congenital and non-progressive, featuring severe intellectual disability (ID), variable dysmorphism, and sometimes associated with shortened survival. BCAP31 encodes one of the most abundant chaperones, with several functions including acting as a negative regulator of endoplasmic reticulum (ER) calcium ion (Ca) concentration.
View Article and Find Full Text PDFTher Adv Ophthalmol
January 2025
Department of Ophthalmology, Zealand University Hospital, Roskilde, Denmark.
Background: Age-related macular degeneration (AMD) is a prevalent cause of irreversible vision loss among the elderly. The prevalence and detailed characteristics of Charles Bonnet syndrome (CBS) remain largely unexplored in patients with geographic atrophy (GA) secondary to AMD.
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Cureus
December 2024
Ear Nose and Throat, Bahrain Defense Force Hospital, Royal Medical Services, Manama, BHR.
Objective: The aim of this study was to assess hearing level of preschoolers with delayed speech in order to detect any underlying hearing loss Methods: In this research we targeted preschool children with speech delay, who have not been previously diagnosed with any medical or psychological illnesses. A total of 54 preschool speech-delayed children were audiologically assessed in our clinic in the past year. The age at time of referral ranged from two to 7.
View Article and Find Full Text PDFNatl J Maxillofac Surg
November 2024
Department of ENT, Jubilee Mission Medical College and Research Institute, Thrissur, Kerala, India.
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