Background And Aims: Inflammatory bowel disease (IBD) has many characteristics of autoimmune diseases. Sensorineural hearing loss has been reported in many autoimmune diseases. Little is known about hearing loss in patients with IBD.
Methods: A prospective blinded comparative study was conducted over a 3-year period. IBD patients and controls underwent a complete otorhinolaryngeal examination and eudiometry test.
Results: Altogether 105 participants (76 patients and 29 controls) took part in this study. Mean age was 36, 51 % were males, and 40 % of the patients were presently hospitalized due to IBD exacerbation. Audiometric examination revealed that any hearing loss (mild to severe) was found in 29 (38 %) of the IBD population, compared to 4 (14 %) of the control group (p = 0.02). Extraintestinal manifestation (EIM) was present in 33/76 (43 %) of IBD patients. Any hearing loss and moderate to severe hearing loss were found in 17/33 (52 %) and 7/33 (21 %) in the EIM-positive group compared to 12/43 (28 %) and 4/43 (9 %) in the EIM-negative group (p = 0.036 and p = 0.14, respectively). Out of patients over the age of 40 with other EIMs, all 11/11 (100 %) of patients had any hearing loss compared to 8/12 (66 %) of patients over the age of 40 without other EIMs, p = 0.035.
Conclusions: Hearing loss may be another EIM of IBD. It is found in 38 % of IBD patients and in up to 52 % of patients with other EIMs and increases over the age of 40. Early hearing evaluation should be recommended to these high-risk IBD patients.
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http://dx.doi.org/10.1007/s10620-016-4074-9 | DOI Listing |
Hum Genet
January 2025
Division of Hearing and Balance Research, National Institute of Sensory Organs, NHO Tokyo Medical Center, 2-5-1 Higashigaoka, Meguro-Ku, Tokyo, 152-8902, Japan.
There are hundreds of rare syndromic diseases involving hearing loss, many of which are not targeted for clinical genetic testing. We systematically explored the genetic causes of undiagnosed syndromic hearing loss using a combination of whole exome sequencing (WES) and a phenotype similarity search system called PubCaseFinder. Fifty-five families with syndromic hearing loss of unknown cause were analyzed using WES after prescreening of several deafness genes depending on patient clinical features.
View Article and Find Full Text PDFArch Dermatol Res
January 2025
Tianjin Medical University, Tianjin, 300102, China.
Objective: This study aims to investigate the genetic link between psoriasis and sudden sensorineural hearing loss (SSNHL).
Methods: From a genetic standpoint, this study further highlighted the connection between psoriasis and SSNHL. Single nucleotide polymorphisms (SNPs) connected to SSNHL could be found using a genome-wide association study from the IEU OpenGWAS project website.
BMC Neurol
January 2025
Faculty of Medicine, Department of Neurology, Al-Quds University, Jerusalem, Palestine.
Background: Vanishing white matter disease (VWMD) is a rare autosomal recessive leukoencephalopathy. It is typified by a gradual loss of white matter in the brain and spinal cord, which results in impairments in vision and hearing, cerebellar ataxia, muscular weakness, stiffness, seizures, and dysarthria cogitative decline. Many reports involve minors.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Audiovestibular Medicine, St George's Hospital, London, UK.
A toddler presented to audiovestibular medicine with mild bilateral, sensorineural hearing loss identified via the Newborn Hearing Screening Programme. This report focuses on the early clinical assessment and aetiological investigation which prompted testing for metabolic disease and highlights the parents' perspective. Early investigation led to a relatively early diagnosis of mucopolysaccharidosis (MPS) type IIIA: Sanfilippo disease which enabled the family to access a novel treatment option which otherwise would not have been possible.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Haematology, Mid Yorkshire Hospitals NHS Trust, Wakefield, UK
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